JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. - ,NO.- ,2014 ª 2014 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION ISSN 0735-1097/$36.00 PUBLISHED BY ELSEVIER INC. http://dx.doi.org/10.1016/j.jacc.2014.08.003

1 APPROPRIATE USE CRITERIA 55 2 56 3 57 4 ACC/AAP/AHA/ASE/HRS/ 58 5 59 6 SCAI/SCCT/SCMR/SOPE 60 7 61 8 2014 Appropriate Use Criteria for 62 9 63 10 Initial Transthoracic Echocardiography 64 11 65 12 in Outpatient Pediatric Cardiology 66 13 67 14 A Report of the American College of Cardiology Appropriate Use Criteria Task Force, 68 15 American Academy of Pediatrics, American Heart Association, American Society of 69 16 Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and 70 17 Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular 71 18 Magnetic Resonance, and Society of Pediatric Echocardiography 72 19 73 20 74 21 75 22 76 Q1 Writing Group for Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, Wyman W. Lai, MD, MPH, FACC, FASE 23 77 Echocardiography Chair Leo Lopez, MD, FACC, FAAP, FASE 24 78 in Outpatient Ritu Sachdeva, MD, FACC, FAAP, FASE 25 79 Pediatric Pamela S. Douglas, MD, MACC, FAHA, FASE 26 80 Cardiology Benjamin W. Eidem, MD, FACC, FASE 27 81 28 82 29 83 30 Rating Panel Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, Richard Lockwood, MD** 84 yy 31 Chair* G. Paul Matherne, MD, MBA, FACC, FAHA 85 zz 32 Pamela S. Douglas, MD, MACC, FAHA, FASE, Moderator* David Nykanen, MD, FACC 86 yy 33 Catherine L. Webb, MD, FACC, FAHA, FASE 87 y 34 Louis I. Bezold, MD, FACC, FAAP, FASE Robert Wiskind, MD, FAAP* 88 35 William B. Blanchard, MD, FACC, FAHA, FAAP* 89 36 Jeffrey R. Boris, MD, FACC* 90 z *American College of Cardiology representative. yAmerican Academy of 37 Bryan Cannon, MD Pediatrics representative. zHeart Rhythm Society representative. 91 Gregory J. Ensing, MD, FACC, FASEx 38 xAmerican Society of Echocardiography representative. jjSociety of 92 jj 39 Craig E. Fleishman, MD, FACC, FASE Pediatric Echocardiography representative. {Society for Cardiovascular 93 { Magnetic Resonance representative. #Society of Cardiovascular Computed 40 Mark A. Fogel, MD, FACC, FAHA, FAAP 94 yy B. Kelly Han, MD, FACC# Tomography representative. **Health Plan representative. American 41 Heart Association representative. zzSociety for Cardiovascular 95 42 Shabnam Jain, MD, MPH, FAAP* Angiography and Interventions representative. 96 jj 43 Mark B. Lewin, MD 97 44 98 45 99 This document was approved by the American College of Cardiology Board of Trustees in June 2014. 46 The American College of Cardiology requests that this document be cited as follows: 100 47 Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 appropriate use criteria 101 48 for initial transthoracic echocardiography in outpatient pediatric cardiology: a report of the American College of Cardiology Appropriate Use Criteria 102 Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for 49 Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and 103 50 Society of Pediatric Echocardiography. J Am Coll Cardiol 2014;XX:xxx-xx. 104 51 This document is copublished in the Journal of the American Society of Echocardiography. 105 52 Copies: This document is available on the World Wide Web site of the American College of Cardiology (www.acc.org). For copies of this document, 106 please contact Elsevier Inc. Reprint Department, fax (212) 633-3820, e-mail [email protected]. 53 Permissions: Modification, alteration, enhancement, and/or distribution of this document are not permitted without the express permission of the 107 54 American College of Cardiology. 108

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109 Appropriate Manesh R. Patel, MD, FACC, Chair Pamela S. Douglas, MD, MACC, FAHA, FASE 163 110 Use Criteria Christopher M. Kramer, MD, FACC, FAHA, Co-Chair Robert C. Hendel, MD, FACC, FAHA, FASNC 164 111 Task Force Bruce D. Lindsay, MD, FACC 165 112 Steven R. Bailey, MD, FACC, FAHA, FSCAI Leslee J. Shaw, PhD, FACC, FASNC, FAHA 166 113 Alan S. Brown, MD, FACC L. Samuel Wann, MD, MACC 167 114 John U. Doherty, MD, FACC, FAHA Joseph M. Allen, MA 168 115 169 116 170 117 TABLE OF CONTENTS 171 118 172 119 173 ABSTRACT ...... XX Figure 5. Palpitaions and Arrhythmias ...... xx 120 174 121 Figure 6. Murmur ...... xx 175 PREFACE ...... XX 122 176 9. DISCUSSION ...... XX 123 177 1. INTRODUCTION ...... XX 124 Assumptions and Definitions ...... xx 178

125 Indications and Ratings ...... xx 179 2. METHODS ...... XX 126 180 Comparison with the Adult Cardiology AUC ...... xx 127 Figure 1. AUC Development Process ...... xx 181 128 Limitations ...... xx 182 129 3. GENERAL ASSUMPTIONS ...... XX 183 Use of AUC to Improve Care ...... xx 130 184 Figure 2. Factors Influencing Outcomes 131 ...... 185 of an Imaging Study xx 10. CONCLUSIONS ...... XX 132 186 133 187 4. DEFINITIONS ...... XX APPENDIX A 134 188 Appropriate Use Criteria for Initial Transthoracic 135 5. ABBREVIATIONS ...... XX 189 Echocardiography in Outpatient Pediatric Cardiology: 136 190 Participants ...... xx 137 6. RESULTS ...... XX 191 138 192 APPENDIX B 139 7. TRANSTHORACIC ECHOCARDIOGRAPHY IN 193 140 OUTPATIENT PEDIATRIC CARDIOLOGY: Relationships With Industry (RWI) and 194 Other Entities ...... xx 141 APPROPRIATE USE CRITERIA (BY INDICATION) ..... XX 195 142 196 Table 1. and Arrhythmias ...... xx 143 ABSTRACT 197 144 Table 2. Syncope ...... xx 198 145 The American College of Cardiology (ACC) participated in 199 Table 3. ...... xx 146 a joint project with the American Society of Echocardi- 200 ...... 147 Table 4. Murmur xx ography, the Society of Pediatric Echocardiography, and 201 148 Table 5. Other Symptoms and Signs ...... xx several other subspecialty societies and organizations to 202 149 establish and evaluate Appropriate Use Criteria (AUC) for 203 Table 6. Prior Test Results ...... xx 150 the initial use of outpatient pediatric echocardiography. 204 151 Table 7. Systemic Disorders ...... xx Assumptions for the AUC were identified, including the 205 152 fi 206 Table 8. Family History of in fact that all indications assumed a rst-time transthoracic 153 Patients Without Signs or Symptoms and Without echocardiographic study in an outpatient setting for pa- 207 154 Confirmed Cardiac Diagnosis ...... xx tients without previously known heart disease. The defi- 208 155 nitions for frequently used terminology in outpatient 209 Table 9. Outpatient Neonates Without Post-Natal 156 Cardiology Evaluation ...... xx pediatric cardiology were established using published 210 157 guidelines and standards and expert opinion. These AUC 211 158 8. FLOW DIAGRAMS FOR COMMON serve as a guide to help clinicians in the care of children 212 fi 159 PATIENT SYMPTOMS ...... XX with possible heart disease, speci cally in terms of when 213 160 a transthoracic echocardiogram is warranted as an initial 214 Figure 3. Chest Pain ...... xx 161 diagnostic modality in the outpatient setting. They are 215 162 Figure 4. Syncope ...... xx alsoausefultoolforeducationandprovidethe 216

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217 infrastructure for future quality improvement initiatives increase in the utilization of such technologies. As these 271 218 as well as research in healthcare delivery, outcomes, and imaging technologies and clinical applications continue to 272 219 resource utilization. advance, the healthcare community needs to understand 273 220 To complete the AUC process, the writing group iden- how best to incorporate these options into daily clinical 274 221 tified 113 indications based on common clinical scenarios care and how to choose between new and long-standing, 275 222 and/or published clinical practice guidelines, and each established imaging technologies. In an effort to res- 276 223 indication was classified into 1 of 9 categories of common pond to this need and to ensure the effective use of 277 224 clinical presentations, including palpitations, syncope, advanced diagnostic imaging tools and procedures, the 278 225 chest pain, and murmur. A separate, independent rating AUC project was initiated. The AUC in this document have 279 226 panel evaluated each indication using a scoring scale of 1 been developed in order to promote effective patient 280 227 to 9, thereby designating each indication as “Appropriate” care, better clinical outcomes, and improved resource 281 228 (median score 7 to 9), “May Be Appropriate” (median utilization. This set of AUC should be useful not only for 282 229 score 4 to 6), or “Rarely Appropriate” (median score 1 to pediatric cardiologists, but also for general pediatricians 283 230 3). Fifty-three indications were identified as Appropriate, and family practitioners, who are frequently the first cli- 284 231 28 as May Be Appropriate, and 32 as Rarely Appropriate. nicians to consider the need for this modality. 285 232 Although AUC have been established for echocardiog- 286 PREFACE 233 raphy in adult patients (1–3), a similar document for pe- 287 234 diatric patients has not yet been published. This is partly 288 In an effort to respond to the need for the rational use of 235 because the scope of such a document would require an 289 services in the delivery of high quality care, the ACC has 236 impossibly extensive list, if criteria were developed for 290 undertaken a process to determine the appropriate use of 237 each congenital cardiac malformation and its variants 291 cardiovascular imaging and procedures for selected pa- 238 before and after intervention. Guidelines and standards 292 tient indications. 239 for performing a pediatric echocardiogram, as well as 293 AUC publications reflect an ongoing effort by the ACC 240 recommendations for quantification methods, have 294 to critically and systematically create, review, and cate- 241 already been published (4,5). However, the questions 295 gorize clinical situations where diagnostic tests and pro- 242 often raised by AUC of “when to do” and “how often to 296 cedures are utilized by physicians caring for patients with 243 do” a pediatric echocardiogram still remained. 297 known or suspected cardiovascular diseases. The process 244 To address these concerns, the American College of 298 is based on current understanding of the technical capa- 245 Cardiology initiated an AUC document on pediatric 299 bilities of the imaging modalities and procedures exam- 246 echocardiography in the outpatient setting, since outpa- 300 ined. Although not intended to be entirely comprehensive 247 tient care is an important component of clinical pediatric 301 due to the wide diversity of clinical disease, the in- 248 cardiology. Children with heart disease represent a widely 302 dications are meant to identify common scenarios 249 varied group of patients, frequently characterized by 303 encountered by the majority of contemporary practices. 250 complex anatomic malformations requiring lifelong 304 Given the breadth of information they convey, the in- 251 follow-up. While echocardiography is the primary diag- 305 dications do not directly correspond to the International 252 nostic modality for children with established congenital 306 Classification of Diseases (ICD) system. 253 and acquired heart disease, the scope of the current 307 The ACC believes that careful blending of a broad range 254 document has been limited to first-time outpatient 308 of clinical experiences and available evidence-based infor- 255 transthoracic echocardiographic studies in patients 309 mation will help guide a more efficient and equitable allo- 256 without previously known cardiac abnormalities. This 310 cation of health care resources in cardiovascular imaging. 257 narrower set of clinical presentations has been chosen 311 The ultimate objective of AUC is to improve patient care 258 because of the high volume of such testing within pedi- 312 and health outcomes in a cost-effective manner, but they 259 atric cardiology. In addition, this initiative has established 313 are not intended to ignore ambiguity and nuance intrinsic 260 the infrastructure to develop additional AUC for pediatric 314 to clinical decision-making. Local parameters, such as the 261 and congenital echocardiography in other settings. 315 availability or quality of equipment or personnel, may in- 262 316 fluence the selection of certain tests or procedures. AUC 263 2. METHODS 317 thus should not be considered substitutes for sound clinical 264 318 judgment and practice experience. 265 This document covers a wide array of potential signs 319 266 1. INTRODUCTION and symptoms associated with cardiovascular disease in 320 267 pediatric patients. A standardized approach was used to 321 268 Improvements in cardiovascular imaging technologies create different categories of indications with the goal of 322 269 and their application, particularly with increasing thera- capturing actual clinical scenarios, without making the list 323 270 peutic options for cardiovascular disease, have led to an of indications excessively long. Indications were created 324

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325 to represent most of the possible uses of echocardiography should be categorized as Appropriate care, May Be 379 326 in the outpatient pediatric setting rather than limiting the Appropriate care, or Rarely Appropriate care, and was 380 327 AUC to indications for which evidence was available. provided the following definition of appropriate use: 381 328 To identify and categorize the indications, a writing An appropriate imaging study is one in which the ex- 382 329 group of pediatric echocardiography experts was formed pected incremental information, combined with clinical 383 330 of representatives from a variety of organizations and judgment, exceeds the expected negative consequences1 384 331 societies. Wherever possible during the writing process, by a sufficiently wide margin for a specific indication 385 332 the group members would map the indications to rele- that the procedure is generally considered acceptable 386 333 vant clinical guidelines and key publications or refer- care and a reasonable approach for the indication. 387 334 ences (See Online Appendix). Once the indications were The rating panel scored each indication as follows: 388 335 formed, they were reviewed and critiqued by the parent Median Score 7 to 9: Appropriate test for specificindi- 389 336 AUC Task Force and numerous external reviewers repre- cation (test is generally acceptable and is areasonable 390 337 senting all pediatric cardiovascular specialties and pri- approach for the indication). 391 338 mary care. After the writing group incorporated this An appropriate option for management of patients in this 392 339 initial feedback, the indications were sent to an inde- population due to benefits generally outweighing risks; 393 340 pendent rating panel comprised of additional experts in effective option for individual care plans although not 394 341 the pediatrics and pediatric cardiology realm, before be- always necessary depending on physician judgment and 395 342 ing sent back to the writing group for additional vetting. patient specific preferences (i.e., procedure is generally 396 343 Each indication was then rated and classified as either acceptable and is generally reasonable for the indication). 397 344 “Appropriate care”, “MayBeAppropriatecare”,or Median Score 4 to 6: May Be Appropriate test for specific 398 345 “Rarely Appropriate care” based on these multiple rounds indication (test may be generally acceptable and may be a 399 346 of review and revision (see Figure 1). reasonable approach for the indication). May Be Appro- 400 347 A detailed description of the methods used for rating the priate also implies that more research and/or patient in- 401 348 selected clinical indications is found in a previous publi- formation is needed to classify the indication definitively. 402 349 cation, “ACCF Proposed Method for Evaluating the At times an appropriate option for management of pa- 403 350 Appropriateness of Cardiovascular Imaging,” (6) as well as tients in this population due to variable evidence or lack of 404 351 the updated version, “Appropriate Use of Cardiovascular agreement regarding the benefits risks ratio, potential 405 352 Technology: 2013 ACCF Appropriate Use Criteria benefit based on practice experience in the absence of evi- 406 353 Methodology Update: A Report of the American College dence, and/or variability in the population; effectiveness 407 354 of Cardiology Foundation Appropriate Use Criteria Task for individual care must be determined by a patient’s 408 355 Force” (7).Briefly, this process combines evidence-based physicianinconsultationwiththepatientbasedonaddi- 409 356 medicine and practice experience and engages a rating tional clinical variables and judgment along with patient 410 357 panel in a modified Delphi exercise. Other steps are preferences (i.e., procedure may be acceptable and may be 411 358 convening a formal writing group with diverse expertise reasonable for the indication). 412 359 in pediatric imaging and clinical care, circulating the Median Score 1 to 3: Rarely Appropriate test for specific 413 360 indications for external review prior to being sent to the indication (test is not generally acceptable and is not a 414 361 rating panel, ensuring an appropriate balance of reasonable approach for the indication). 415 362 expertise and practice areas among the rating panelists, Rarely an appropriate option for management of 416 363 developing a standardized rating package that includes patients in this population due to the lack of a clear benefit/ 417 364 relevant evidence, and establishing formal roles for risk advantage; rarely an effective option for individual 418 365 facilitating panel interaction at the face-to-face meeting. care plans; exceptions should have documentation of the 419 366 The rating panel first evaluated the indications inde- clinical reasons for proceeding with this care option 420 367 pendently. Then, the panel was convened for a face-to-face (i.e., procedure is not generally acceptable and is not 421 368 meeting for discussion of each indication. At this meeting, generally reasonable for the indication). 422 369 panel members were given their scores and a blinded The division of the numerical scores into 3 levels of 423 370 summary of their peers’ scores. After the meeting, panel appropriateness is somewhat arbitrary and the numeric 424 371 members were then asked to independently provide their designations should be viewed as existing on a contin- 425 372 final scores for each indication (See Online Appendix). uum. Further, there may be diversity in clinical opinion 426 373 Although panel members were not provided explicit for particular clinical scenarios, such that scores in the 427 374 cost information to help determine their appropriate use 428 375 ratings, they were asked to implicitly consider cost as an 429 376 additional factor in their evaluation of appropriate use. In 430 1Negative consequences include the risks of the procedure (i.e., radiation or 377 rating these criteria, the AUC Rating Panel was asked to contrast exposure) and the downstream impact of poor test performance such as 431 378 assess whether the use of the test for each indication delay in diagnosis (false negatives) or inappropriate diagnosis (false positives). 432

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433 487 434 488 Develop list of indicaƟons, Literature review and 435 assumpƟons, and definiƟons Guideline Mapping 489 436 490 Review Panel >30 members 437 provide feedback 491

438 on Development 492 Ɵ

439 WriƟng Group revises indicaƟons 493 440 Indica 494 441 495 RaƟng Panel rates the IndicaƟons 442 in two rounds 496

443 on 497 Ɵ 444 1st round – No InteracƟon 498

445 2nd Round – Panel InteracƟon 499 Determina

446 Appropriateness 500 447 Appropriate Use Score 501 448 (7-9) Appropriate 502

449 (4-6) May Be Appropriate 503

450 (1-3) Rarely Appropriate 504 ProspecƟve comparison ProspecƟve clinical 451 with clinical records decision aids 505 on

452 Ɵ 506 453 507 Valida 454 % Use that is Appropriate, Increase Appropriate Use 508 May Be Appropriate, or 455 Rarely Appropriate 509 456 510 457 511 FIGURE 1 AUC Development Process 458 512 459 513 460 intermediate level of appropriate use should be labeled 3. GENERAL ASSUMPTIONS 514 461 “May Be Appropriate,” as critical patient or research data 515 462 may be lacking or discordant. This designation should be 1. This document will address the initial use of outpa- 516 463 a prompt to the field to carry out definitive research tient transthoracic echocardiography (TTE) during 517 464 investigation whenever possible. It is anticipated that the pediatric (# 18 years of age) outpatient care. Although 518 465 AUC reports will continue to be revised as further data are TTE is also an essential tool in hospitalized patients, 519 466 generated and information from criteria implementation discussion of indications for this use is beyond the 520 467 is accumulated. scope of this document. 521 468 To prevent bias in the scoring process, the rating panel, 2. This AUC document will not address the use of TTE in 522 469 by design, included a minority of specialists in pediatric patients with previously known structural, functional, 523 470 echocardiography. Specialists, while offering important or primary electrical cardiac abnormalities. 524 471 clinical and technical insights, might have a natural ten- 3. A comprehensive TTE examination may include 2- 525 472 dency to rate the indications within their specialty as more dimensional, M-mode, and 3-dimensional imaging as 526 473 appropriate than non-specialists. In addition, care was well as spectral and color Doppler evaluation, all of 527 474 taken to provide objective, nonbiased information, in- which are important elements (9–11) to evaluate rele- 528 475 cluding guidelines and key references, to the rating panel. vant cardiac structures and hemodynamics. A 529 476 The level of agreement among panelists was analyzed comprehensive TTE report includes interpretation of 530 477 based on the RAND Corporation’sBIOMEDConcerted all aspects of the TTE. 531 478 Action on Appropriateness rule (8) for a panel of 14 to 4. The use of transesophageal or stress echocardiogra- 532 479 16 members. As such, agreement was defined as an phy will not be addressed in this document. 533 480 indication where 4 or fewer panelists’ ratings fell outside 5. This document assumes that any other more defini- 534 481 the 3-point region containing the median score. tive diagnostic test, including but not limited to 535 482 Disagreement was defined as occurring when at least 5 electrocardiogram (ECG), chest X-ray, or genetic 536 483 panelists’ ratings fell in both the Appropriate and the testing, when appropriate will be considered prior to 537 484 Rarely Appropriate categories. Any indication having ordering a TTE. 538 485 disagreement was categorized as May Be Appropriate 6. All standard TTE techniques for image acquisition are 539 486 regardless of the final median score. available for each indication and have a sensitivity 540

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541 595 542 596 543 597 544 598 Improved 545 599 Patient Patient Image Image Results Patient Care 546 Selection Acquisition Interpretation Communication 600 (Outcomes) 547 601 548 602 Imaging Process 549 603 550 604 551 Laboratory Structure 605 552 606 553 607 FIGURE 2 Factors Influencing Outcomes of an Imaging Study (16) 554 608 555 609 556 610 557 and specificity similar to those found in the published 12. If the reason for a test can be assigned to more than 611 558 literature. one indication, it is classified under the most clinically 612 559 7. The test is performed and interpreted by qualified significant indication. 613 560 individual(s) in a facility that is in compliance with 13. The term family history in this document refers to 614 561 national standards for performing pediatric echocar- first-degree relatives only. 615 562 diograms (4). 14. Cost is considered implicitly in the appropriate use 616 563 8. AUC is one aspect of quality for imaging procedures determination. Clinical benefits should always be 617 564 occurring at the time of patient selection. Several considered first, and costs should be considered in 618 565 additional factors should be addressed to support relationship to these benefitsinordertobetterconvey 619 566 high-quality results (see Figure 2). These other factors net value. For example, a procedure with moderate 620 567 are important but are not covered in this document. clinical efficacy for a given AUC indication should not 621 568 9. The range of potential indications for echocardiogra- be scored as more appropriate than a procedure with 622 569 phy is quite large, particularly in comparison with high clinical efficacy solely due to its lower cost. When 623 570 other cardiovascular imaging tests. Thus, the in- scientific evidence exists to support clinical benefit, 624 571 dications are, at times, purposefully broad to cover an cost efficiency and cost effectiveness should be 625 572 array of cardiovascular and to considered for any indication. 626 573 account for the ordering physician’sbestjudgmentas 15. For each indication, the rating reflects whether 627 574 to the presence of cardiovascular abnormalities. the echocardiogram is reasonable for the patient ac- 628 575 Additionally, there are likely clinical scenarios that cording to the appropriate use definition, not whether 629 576 are not covered in this document. the test is preferred over another modality. It is not 630 577 10. A qualified clinician has obtained a complete clinical assumed that the decision to perform a diagnostic test 631 578 history and performed the physical examination such has already been made. The level of appropriateness 632 579 that the clinical status of the patient can be assumed also does not consider issues of local availability or 633 580 to be valid as stated in the indication (e.g., an skill for any modality. 634 581 asymptomatic patient is truly asymptomatic for the 16. The category of May Be Appropriate is used when 635 582 condition in question and sufficient questioning of insufficient data are available for a definitive catego- 636 583 the patient has been undertaken). rization or when there is substantial disagreement 637 584 11. Some indications address whether or not an ECG regarding the appropriateness of that indication. The 638 585 has been obtained and whether or not it reveals designation May Be Appropriate should not be used as 639 586 any abnormalities as influencing the appropriateness grounds for denial of reimbursement. 640 587 of additional echocardiographic assessment. It is 17.Thismanuscriptdoesnotaddresswhetheracardiol- 641 588 beyond the scope of this document to define every ogy consultation is required prior to the echocardio- 642 589 possible clinical scenario involving specificECG gram unless specified in the indication. 643 590 abnormalities. Therefore, the term “abnormal ECG” 644 591 refers to only clinically pertinent ECG findings. 4. DEFINITIONS 645 592 Criteria for “abnormal ECG” will be based upon stan- 646 593 dard published ECG normal values in pediatric pa- Abnormal electrocardiogram (ECG): Electrocardiographic 647 594 tients (12–15). findings regarded as probably or definitely abnormal 648

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649 according to age as well as clinically significant, and murmur, pulmonary flow murmur, physiologic pe- 703 650 including but not limited to ventricular hypertrophy, ripheral pulmonary stenosis, supraclavicular arterial 704 651 atrial enlargement, complete bundle branch block, atrio- , and venous hum; most innocent murmurs are 705 652 ventricular block, prolonged QTc, abnormal T waves or soft (less than or equal to grade 2/6), heard in early 706 653 ST-T wave segments, Wolff-Parkinson-White syndrome, systole, characterized as crescendo-decrescendo 707 654 premature atrial contractions (PACs), premature ventric- type, and may vary with position 708 655 ular contractions (PVCs), supraventricular , Pathologic murmur: Murmur that is suggestive of the 709 656 ventricular tachycardia, and Brugada syndrome presence of a cardiovascular abnormality (not clearly 710 657 Arrhythmia: Documented irregular and/or abnormal innocent sounding), including but not limited to 711 658 heart rate or rhythm (Patients with palpitations do not diastolic murmurs, holosystolic murmurs, late sys- 712 659 necessarily have an arrhythmia, and patients with an tolic murmurs, grade 3/6 systolic murmur or louder, 713 660 arrhythmia do not necessarily experience palpitations) continuous murmurs other than venous hums, harsh 714 661 Cardiomyopathy: Disease affecting the structure and/or murmurs, and murmurs that are provoked or be- 715 662 function of the myocardium, including but not limited to come louder with changes in position (from squat- 716 663 hypertrophic, dilated, or restrictive cardiomyopathy, left ting to standing) or during the strain phase of a 717 664 ventricular non-compaction, or arrhythmogenic right Valsalva maneuver 718 665 ventricular cardiomyopathy 719 666 Channelopathy: A clinical syndrome involving a genetic Neonate: A child that is less than or equal to 28 days old 720 667 mutation or acquired malfunction of the proteins forming Neurocardiogenic syncope: A type of syncope typically 721 668 the myocardial ion channels (including but not limited to occurring in the upright position, in which the triggering 722 669 Naþ,Kþ,andCa2þ) of the cardiovascular electrical sys- of a neural reflex results in a usually self-limited episode 723 670 tem, including but not limited to long QT syndrome, short of systemic hypotension and/or or asystole 724 671 QT syndrome, catecholaminergic polymorphic ventricular Palpitations: An unpleasant sensation of rapid, irreg- 725 672 tachycardia, and Brugada syndrome ular, and/or forceful beating of the heart 726 673 Chest pain: Physical discomfort in the anterior thoracic Pre-Syncope: A state of experiencing lightheadedness, 727 674 region dizziness, weakness, visual changes (such as spots, tunnel 728 675 Congestive heart failure: A condition in which the heart vision, or loss of vision), auditory changes (ringing, 729 676 is unable to pump enough blood to meet the body’s buzzing, or muffled hearing), or feeling hot or cold 730 677 metabolic demands without loss of consciousness 731 678 Cyanosis: Bluish discoloration of the skin and mucous Syncope: Sudden temporary loss of consciousness asso- 732 679 membranes ciated with a loss of postural tone and with spontaneous 733 680 Desaturation: For pediatric patients other than new- recovery that does not require electrical or chemical 734 681 borns, an oxygen saturation <95% as measured by pulse cardioversion 735 682 oximeter; for newborns $24 hours of age, an oxygen 736 683 saturation that is (a) <90% in the initial screen or in repeat 5. ABBREVIATIONS 737 684 screens, (b) <95% in the right hand and foot on 3 mea- 738 685 > 739 sures, each separated by 1 hour, or (c) a 3% absolute AUC ¼ Appropriate Use Criteria 686 740 difference in oxygen saturation between the right hand ECG ¼ electrocardiogram 687 741 and foot on 3 measures, each separated by 1 hour (17) PAC ¼ premature atrial contraction 688 742 Echogenic focus: Small bright spot(s) frequently seen on PVC ¼ premature ventricular contraction 689 743 a fetal echocardiogram, usually related to the ventricular TTE ¼ transthoracic echocardiogram 690 papillary muscles and chordae and generally considered a 744 691 benign finding 745 6. RESULTS 692 Hypertension: Average systolic and/or diastolic blood 746 693 pressure that is $95th percentile for gender, age, and 747 The final ratings for pediatric echocardiography are listed 694 height on 3 or more occasions 748 by indication in Tables 1 to 9.Thefinal score for each 695 Murmur: Additional heart or vascular sound due to 749 indication reflects the median score of the 15 Rating 696 normal or abnormal turbulent blood flow heard during 750 Panel members and has been labeled according to the 697 751 categories of Appropriate (median 7 to 9), May Be 698 752 Innocent murmur: Murmur that is consistent with Appropriate (median 4 to 6), or Rarely Appropriate (me- 699 753 normal blood flow and is determined not to be dian 1 to 3). In the tables, the final score for each indi- 700 754 related to any structural abnormalities of the heart or cation is shown in parentheses with the ratings. Out of 701 755 great vessels, including but not limited to Still’s 113 total indications, 53 were considered Appropriate 702 756

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757 (47%), 28 were considered May Be Appropriate (25%), AUC score is attained. Likewise, Figure 7 (a-d) in the 811 758 and 32 were considered Rarely Appropriate (28%). To see Online Appendix shows flow diagrams grouped by clin- 812 759 the indications listed by Appropriate Use rating, see the ical presentation, such as family history and test 813 760 Online Appendix. The Discussion section highlights findings. 814 761 further trends in scoring. 815 7. TRANSTHORACIC ECHOCARDIOGRAPHY IN 762 Figures3,4,5,and6illustrate flow diagrams based on 816 OUTPATIENT PEDIATRIC CARDIOLOGY: 763 common patient symptoms (chest pain, syncope, palpi- 817 APPROPRIATE USE CRITERIA (BY INDICATION) 764 tations and arrhythmias, and murmur) that the clinician 818 765 can use to narrow down patient information until the 819 766 820 767 821 TABLE 1 Palpitations and Arrhythmias 768 822 769 Indication Appropriate Use Rating 823 770 Palpitations 824 771 1. Palpitations with no other symptoms or signs of cardiovascular disease, a benign family history, and no recent ECG R (2) 825 772 2. Palpitations with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG R (1) 826 773 3. Palpitations with abnormal ECG M (6) 827 774 4. Palpitations with family history of a channelopathy R (3) 828

775 5. Palpitations in a patient with known channelopathy M (4) 829

776 6. Palpitations with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death A (7) 830 777 and/or pacemaker or implantable defibrillator placement 831 778 7. Palpitations with family history of cardiomyopathy A (9) 832 779 8. Palpitations in a patient with known cardiomyopathy A (9) 833 780 ECG Findings 834

781 9. PACs in the prenatal or neonatal period R (3) 835

782 10. PACs after the neonatal period R (3) 836

783 11. Supraventricular tachycardia A (7) 837 784 838 12. PVCs in the prenatal or neonatal period M (6) 785 839 13. PVCs after the neonatal period M (6) 786 840 14. Ventricular tachycardia A (9) 787 841 15. Sinus bradycardia R (2) 788 842 16. Sinus arrhythmia R (1) 789 843 790 The number in parentheses next to the rating reflects the median score for that indication. 844 ¼ ¼ ¼ ¼ ¼ ¼ 791 Abbreviations: A Appropriate; M May Be Appropriate; R Rarely Appropriate; ECG electrocardiogram; PACs premature atrial contractions; PVCs premature ven- 845 tricular contractions. 792 846 793 847

794 TABLE 2 Syncope 848 795 849 Indication Appropriate Use Rating 796 850 17. Syncope with or without palpitations and with no recent ECG R (3) 797 851 798 18. Syncope with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG R (2) 852 799 19. Syncope with abnormal ECG A (7) 853 800 20. Syncope with family history of channelopathy M (5) 854 801 21. Syncope with family history at a young age (before the age of 50 years) of sudden cardiac arrest or death and/or A (9) 855 pacemaker or implantable defibrillator placement 802 856 22. Syncope with family history of cardiomyopathy A (9) 803 857 804 23. Probable neurocardiogenic (vasovagal) syncope R (2) 858 805 24. Unexplained pre-syncope M (4) 859 806 25. Exertional syncope A (9) 860 807 26. Unexplained post-exertional syncope A (7) 861 808 27. Syncope or pre-syncope with a known non-cardiovascular cause R (2) 862

809 The number in parenthesis next to the rating reflects the median score for that indication. 863 810 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram. 864

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865 919 TABLE 3 Chest Pain 866 920 Indication Appropriate Use Rating 867 921 868 28. Chest pain with no other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG R (2) 922 869 29. Chest pain with other symptoms or signs of cardiovascular disease, a benign family history, and a normal ECG M (6) 923 870 30. Exertional chest pain A (8) 924 871 31. Non-exertional chest pain with no recent ECG R (3) 925 872 32. Non-exertional chest pain with normal ECG R (1) 926 873 33. Non-exertional chest pain with abnormal ECG A (7) 927

874 34. Chest pain with family history of sudden unexplained death or cardiomyopathy A (8) 928

875 35. Chest pain with family history of premature coronary artery disease M (4) 929

876 36. Chest pain with recent onset of fever M (6) 930 877 931 37. Reproducible chest pain with palpation or deep inspiration R (1) 878 932 38. Chest pain with recent illicit drug use M (6) 879 933 fl 880 The number in parenthesis next to the rating re ects the median score for that indication. 934 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram. 881 935 882 936 883 937 884 938 885 939 886 940 887 941 888 942 889 943 890 TABLE 4 Murmur 944

891 Indication Appropriate Use Rating 945

892 39. Presumptively innocent murmur with no symptoms, signs, or findings of cardiovascular disease and a benign R (1) 946 893 family history 947 894 40. Presumptively innocent murmur with signs, symptoms, or findings of cardiovascular disease A (7) 948 895 41. Pathologic murmur A (9) 949

896 The number in parenthesis next to the rating reflects the median score for that indication. 950 897 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram. 951 898 952 899 953 900 954 901 955 902 956 903 957 904 958 905 959 Other Symptoms and Signs 906 TABLE 5 960 907 Indication Appropriate Use Rating 961 908 42. Symptoms and/or signs suggestive of congestive heart failure, including but not limited to respiratory distress, A (9) 962 fi 909 poor peripheral , feeding dif culty, decreased urine output, edema, and/or hepatomegaly 963 910 43. Chest wall deformities and scoliosis pre-operatively M (6) 964 911 44. Fatigue with no other signs and symptoms of cardiovascular disease, a normal ECG, and a benign family history R (3) 965 912 45. Signs and symptoms of in the absence of blood culture data or a negative blood culture A (8) 966 913 46. Unexplained fever without other evidence for cardiovascular or systemic involvement M (5) 967 914 47. Central cyanosis A (8) 968

915 48. Isolated acrocyanosis R (1) 969 916 970 The number in parenthesis next to the rating reflects the median score for that indication. 917 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram. 971 918 972

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973 1027 TABLE 6 PriorTestResults 974 1028 Indication Appropriate Use Rating 975 1029 976 49. Known channelopathy M (4) 1030 977 50. Genotype positive for cardiomyopathy A (9) 1031 978 51. Abnormal chest X-ray findings suggestive of cardiovascular disease A (9) 1032 979 52. Abnormal ECG without symptoms A (7) 1033 980 53. Desaturation based on oximetry A (9) 1034 981 54. Previously normal echocardiogram with no change in cardiovascular status or family history R (1) 1035

982 55. Previously normal echocardiogram with a change in cardiovascular status and/or a new family history suggestive of A (7) 1036 983 heritable heart disease 1037 984 56. Elevated anti-streptolysin O titers without suspicion for R (3) 1038 985 57. Chromosomal abnormality known to be associated with cardiovascular disease A (9) 1039 986 58. Chromosomal abnormality with undefined risk for cardiovascular disease M (5) 1040

987 59. Positive blood cultures suggestive of infective endocarditis A (9) 1041

988 60. Abnormal cardiac biomarkers A (9) 1042

989 61. Abnormal barium swallow or bronchoscopy suggesting vascular ring A (7) 1043 990 1044 The number in parenthesis next to the rating reflects the median score for that indication. 991 1045 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram. 992 1046 993 1047 994 TABLE 7 Systemic Disorders 1048

995 Indication Appropriate Use Rating 1049

996 62. Cancer without chemotherapy M (5) 1050

997 63. Prior to or during chemotherapy in cancer A (8) 1051 998 1052 64. Sickle cell disease and other hemoglobinopathies A (8) 999 1053 65. Connective tissue disorder such as Marfan, Loeys Dietz, and other aortopathy syndromes A (9) 1000 1054 66. Suspected connective tissue disorder A (7) 1001 1055 67. Clinically suspected syndrome or extracardiac congenital anomaly known to be associated with congenital heart disease A (9) 1002 1056 68. Human immunodeficiency virus infection A (8) 1003 1057 fi 1004 69. Suspected or con rmed Kawasaki disease A (9) 1058 1005 70. Suspected or confirmed Takayasu arteritis A (9) 1059 1006 71. Suspected or confirmed acute rheumatic fever A (9) 1060 1007 72. Systemic lupus erythematosis and autoimmune disorders A (7) 1061 1008 73. Muscular dystrophy A (9) 1062 1009 74. Systemic hypertension A (9) 1063

1010 75. Renal failure A (7) 1064

1011 76. Obesity without other cardiovascular risk factors R (2) 1065

1012 77. Obesity with obstructive sleep apnea M (6) 1066 1013 1067 78. Obesity with other cardiovascular risk factors M (6) 1014 1068 79. Diabetes mellitus R (3) 1015 1069 80. Lipid disorders R (3) 1016 1070 81. Stroke A (8) 1017 1071 82. Seizures, other neurologic disorders, or psychiatric disorders R (2) 1018 1072 1019 83. Suspected pulmonary hypertension A (9) 1073 1020 84. Gastrointestinal disorders, not otherwise specified R (2) 1074 1021 85. Hepatic disorders M (4) 1075 1022 86. Failure to thrive M (5) 1076 1023 87. Storage diseases, mitochondrial and metabolic disorders A (8) 1077 1024 88. Abnormalities of visceral or cardiac situs A (9) 1078 1025 1079 The number in parenthesis next to the rating reflects the median score for that indication. 1026 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate. 1080

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1081 1135 Family History of Cardiovascular Disease in Patients Without Signs or Symptoms and Without Confirmed TABLE 8 1082 Cardiac Diagnosis 1136 1083 1137 Indication Appropriate Use Rating 1084 1138 89. Unexplained sudden death before the age of 50 years M (6) 1085 1139 90. Premature coronary artery disease before the age of 50 years R (2) 1086 1140 1087 91. Channelopathy R (3) 1141 1088 92. Hypertrophic cardiomyopathy A (9) 1142 1089 93. Non-ischemic dilated cardiomyopathy A (9) 1143 1090 94. Other cardiomyopathies A (8) 1144 1091 95. Unspecified cardiovascular disease R (3) 1145 1092 96. Disease at high risk for cardiovascular involvement, including but not limited to diabetes, systemic hypertension, R (2) 1146 1093 obesity, stroke, and peripheral 1147 1094 97. Genetic disorder at high risk for cardiovascular involvement A (7) 1148 1095 98. Marfan or Loeys Dietz syndrome A (7) 1149 1096 99. Connective tissue disorder other than Marfan or Loeys Dietz syndrome M (6) 1150 1097 100. Congenital left-sided heart lesion, including but not limited to mitral stenosis, left ventricular outflow tract M (6) 1151 obstruction, bicuspid aortic valve, aortic coarctation, and/or hypoplastic left heart syndrome 1098 1152 1099 101. Congenital heart disease other than the congenital left-sided heart lesions M (5) 1153 1100 102. Idiopathic pulmonary arterial hypertension M (5) 1154 1101 103. Heritable pulmonary arterial hypertension A (8) 1155 1102 104. Pulmonary arterial hypertension other than idiopathic and heritable R (3) 1156 1103 105. Consanguinity R (3) 1157

1104 The number in parenthesis next to the rating reflects the median score for that indication. 1158 1105 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate. 1159 1106 1160 1107 1161 1108 1162 1109 1163 1110 1164 TABLE 9 Outpatient Neonates Without Post-Natal Cardiology Evaluation 1111 1165 1112 Indication Appropriate Use Rating 1166 1113 106. Suspected cardiovascular abnormality on fetal echocardiogram A (9) 1167 1114 107. Isolated echogenic focus on fetal ultrasound R (2) 1168 1115 108. Maternal infection during pregnancy or delivery with potential fetal/neonatal cardiac sequelae A (7) 1169 1116 109. Maternal diabetes with no prior fetal echocardiogram M (6) 1170 1117 110. Maternal diabetes with a normal fetal echocardiogram M (4) 1171

1118 111. Maternal phenylketonuria A (7) 1172

1119 112. Maternal autoimmune disorder M (5) 1173

1120 113. Maternal teratogen exposure M (6) 1174 1121 1175 The number in parenthesis next to the rating reflects the median score for that indication. 1122 1176 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate. 1123 1177 1124 1178 1125 1179 1126 1180 1127 1181 1128 1182 1129 1183 1130 1184 1131 1185 1132 1186 1133 1187 1134 1188

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1189 8. FLOW DIAGRAMS FOR COMMON 1243 1190 PATIENT SYMPTOMS 1244 1191 1245 1192 1246 1193 1247 1194 1248 1195 1249 1196 1250 1197 1251 1198 1252 1199 1253 1200 1254 1201 1255 1202 1256 1203 1257 1204 1258 1205 1259 1206 1260 1207 1261 1208 1262 1209 1263 FPO 1210 = 1264 1211 1265 1212 1266 print & web 4C 1213 FIGURE 3 Chest Pain 1267 1214 1268 1215 Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. *See 1269 ¼ ¼ ¼ ¼ 1216 Discussion section. Abbreviations: A Appropriate; M May Be Appropriate; R Rarely Appropriate; ECG Electrocardiogram. 1270 1217 1271 1218 1272 1219 1273 1220 1274 1221 1275 1222 1276 1223 1277 1224 1278 1225 1279 1226 1280 1227 1281 1228 1282 1229 1283 1230 1284 1231 1285 1232 1286 1233 1287 1234 1288 FPO

1235 = 1289 1236 1290 1237 1291

1238 print & web 4C 1292 FIGURE 4 Syncope 1239 1293

1240 Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. 1294 1241 Abbreviations: A ¼ Appropriate; M ¼ May Be Appropriate; R ¼ Rarely Appropriate; ECG ¼ Electrocardiogram; ICD ¼ Implantable Cardioverter Defibrillator. 1295 1242 1296

PGL 5.2.0 DTD JAC20491_proof 12 September 2014 3:25 pm ce 1350 1349 1348 1347 1346 1345 1344 1343 1342 1341 1340 1339 1338 1337 1336 1335 1334 1333 1332 1331 1330 1329 1328 1327 1326 1325 1324 1323 1322 1321 1320 1319 1318 1317 1316 1315 1314 1313 1312 1311 1310 1309 1308 1307 1306 1305 1304 1303 1302 1301 1300 1299 1298 1297

print & web 4C=FPO print & web 4C=FPO - VOL. JACC ,2014: ahidcto speee ihanme in h aigo ,M rRi hnfloe ytemda cr nprnhssfrta atclrindicatio particular that for parenthesis in score median the by followed then is R or M, A, of rating The sign. number A a Abbreviations: with preceded is indication Each 6 FIGURE indicatio particular that for parenthesis in score median the by followed then is De R dioverter or M, A, A of Abbreviations: rating section. The sign. Discussion number a with preceded is indication Each 5 FIGURE - - – - ,NO. fi apttosadArrhythmias and Palpitations Murmur rlao;PACs brillator; ¼ - prpit;M Appropriate; ,2014 ¼ rmtr tilCnrcin;PVCs Contractions; Atrial Premature ¼ ¼ G .. DTD 5.2.0 PGL a eAporae R Appropriate; Be May prpit;M Appropriate; ¼ a eAporae R Appropriate; Be May A241pof1 etme 0432 mce pm 3:25 2014 September 12 JAC20491_proof ¼ aeyAppropriate. Rarely ¼ rmtr etiua Contractions. Ventricular Premature ¼ aeyAporae ECG Appropriate; Rarely ¼ lcrcriga;ICD Electrocardiogram; U o eiti Echocardiography Pediatric for AUC ¼ mlnal Car- Implantable Campbell .*See n. n. tal et . 13 1400 1404 1403 1402 1380 1384 1388 1390 1360 1394 1364 1398 1386 1389 1368 1399 1396 1369 1366 1401 1370 1354 1385 1383 1382 1374 1358 1387 1378 1395 1393 1392 1365 1363 1362 1359 1356 1397 1379 1367 1376 1355 1353 1352 1381 1375 1373 1372 1357 1377 1391 1361 1351 1371 14 Campbell et al. JACC VOL. - ,NO.- ,2014 AUC for Pediatric Echocardiography - ,2014:- – -

1405 9. DISCUSSION test for an individual patient should not be undermined 1459 1406 because there may be reasons other than those listed in 1460 1407 This is the first report by the American College of Cardi- this document that preclude application of the AUC. The 1461 1408 ology addressing appropriate use in the field of pediatric AUC may also not be applicable if another diagnostic 1462 1409 cardiology. Although the use of AUC for various areas of modality has already proven the diagnosis for which an 1463 1410 cardiovascular imaging in adult cardiology has been echocardiogram was intended. For example, if a vascular 1464 1411 established since 2005, there has not been a tool to ring is confirmed by cardiac magnetic resonance imaging 1465 1412 guide practice in pediatric cardiology (1,18).Giventhe (MRI), then an echocardiogram will not provide any 1466 1413 high level of utilization of echocardiography in the additional critical information. Even though this indica- 1467 1414 outpatientsetting,thistopicwaschosenasthesubject tion is rated as Appropriate in this document, clinical 1468 1415 for the first pediatric AUC, and was intentionally judgment in such scenarios will definitely supersede the 1469 1416 restricted to initial, outpatient, and transthoracic echo- AUC rating. 1470 1417 cardiographic evaluation. Of the various diagnostic mo- The definitions provided in this document were final- 1471 1418 dalities, echocardiography remains the most readily ized by the writing group after it had given due consid- 1472 1419 available, non-invasive and highly diagnostic tool for eration to the current literature and views provided by the 1473 1420 assessing cardiac structure, function and hemodynamics external reviewers and the rating panel. The users of this 1474 1421 in those with suspected cardiac disease. This report will document should be well versed in these assumptions 1475 1422 help us establish the infrastructure precedent for and definitions prior to implementing the AUC. 1476 1423 expanding AUC for echocardiography in pediatric pa- 1477 1424 tients as well as AUC for other diagnostic modalities and Indications and Ratings 1478 1425 procedures used in this field. The indications presented in this report were finalized 1479 1426 It is important to note the differences between clinical after incorporating the suggestions by the external re- 1480 1427 practice guidelines and AUC (19). The American College of viewers, and the members of the rating panel rated the 1481 1428 Cardiology guidelines have been developed by leaders in indications independently. The median score for each 1482 1429 the field of cardiovascular medicine using evidence- indication became the final rating. In general, the in- 1483 1430 baseddocumentsandexpertopinionandareingeneral dications rated as Appropriate included evaluation of 1484 1431 quitebroad.EventhoughAUCareevidencebased, new cardiac symptoms or clinical scenarios known to be 1485 1432 they are created around possible clinical scenarios associated with congenital or acquired heart disease in 1486 1433 that are encountered in everyday practice rather than the pediatric population. The indications ranked as 1487 1434 starting with options based on current evidence. Rarely Appropriate clustered around broad systemic 1488 1435 Echocardiography is the most common imaging modality diseases and family history of conditions that are 1489 1436 used in cardiology, but there is evidence that it may not generally not known to be associated with structural or 1490 1437 be a cost-effective or high-yield diagnostic test for some functional abnormalities detectable by echocardiogra- 1491 1438 indications included in this document (20–29).TheAUC phy. Scenarios that were rated as May Be Appropriate, in 1492 1439 address a reasonable role of echocardiography. Each general, involved uncertainty or required additional 1493 1440 individualpatientisuniqueandthepossibleuseof clinical information to better define the appropriateness 1494 1441 echocardiography deserves to be considered in full of the test. 1495 1442 clinical context. It is noteworthy that there are no recent In the pediatric population, chest pain, syncope and 1496 1443 practice guidelines for indications of echocardiography murmur are 3 common reasons for referral of an echo- 1497 1444 in pediatric patients and this report may become a cardiogram in the outpatient setting. For this reason, 1498 1445 clinically useful guide for practitioners (30). tables dedicated to each of these conditions with various 1499 1446 clinical scenarios were included in the current report. 1500 fi 1447 Assumptions and De nitions Although a murmur is one of the most common indica- 1501 1448 Some of the assumptions used while writing this report tions for obtaining an echocardiogram in the pediatric 1502 1449 are important to emphasize. It is assumed that a thor- population, it is well known that a large number of pa- 1503 1450 ough history and physical examination has been per- tients are referred with an innocent murmur that does 1504 1451 formed by a qualified clinician and that use of other not require evaluation with an echocardiogram. The 1505 1452 more diagnostic tests has been considered prior to current document presumes that the clinician has made 1506 1453 ordering an echocardiogram. It is also assumed that the every effort to determine whether the murmur is inno- 1507 1454 echocardiogram is performed and interpreted by quali- cent or not prior to considering the use of an echocar- 1508 1455 fied individuals. Although the AUC ratings listed in this diogram (21,31). Echocardiographic screening for 1509 1456 report provide general guidance for when transthoracic presumably or clearly innocent murmur has been rated as 1510 1457 echocardiography may be useful in a specificpatient Rarely Appropriate in this document. This rating is sup- 1511 1458 population, the role of clinical judgment in ordering the ported by prior publications reporting that examination 1512

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1513 by a pediatric cardiologist is quite accurate in dis- more than one indication listed in this document could 1567 1514 tinguishing between innocent and pathologic murmurs be applied, clinicians need to use their judgment in 1568 1515 (21,32,33). Pathologic murmurs (including those that are picking the scenario that most closely fits the individual 1569 1516 not clearly innocent after evaluation), along with pre- patient. 1570 1517 sumably innocent murmurs with other signs, symptoms 1571 1518 or findings of cardiovascular disease, were found to be Comparison With the Adult Cardiology AUC 1572 1519 Appropriate for an echocardiogram, since these situa- The current adult cardiology AUC for echocardiography 1573 1520 tions suggest the possibility of a cardiovascular abnor- includes initial and follow-up evaluation in the inpatient 1574 1521 mality as their underlying cause. Of course, the ability to and outpatient setting using transthoracic, trans- 1575 1522 make a final diagnosis of innocent murmur after an esophageal, and stress echocardiography (3). In contrast, 1576 1523 echocardiogram for patients meeting either of these this current document is limited to initial outpatient 1577 1524 appropriate indications does not imply that the rationale transthoracic echocardiography. The initial adult car- 1578 1525 for using an echocardiogram to rule out a cardiovascular diology AUC for transthoracic and transesophageal 1579 1526 abnormality was not appropriate. echocardiography were published in 2007 (1).After 1580 1527 Chest pain and syncope are 2 other common pre- practical application of these AUC, a revised version was 1581 1528 sentations in the pediatric age group. The etiology for published in 2011. This revised version which is currently 1582 1529 these is generally benign and echocardiography has in use included many more indications and now provides 1583 1530 been shown to be low-yield, unlike in adult patients a more complete range of clinical scenarios (3).Studies 1584 1531 (25–29). For this reason, the indications and their ratings comparing the application of these two AUC in adult 1585 1532 related to chest pain in this document are very different cardiology clinical practice have demonstrated significant 1586 1533 from those in the adult AUC. Syncope with no other improvement in the ability to classify the various clinical 1587 1534 symptoms or signs of cardiac disease has been rated as scenarios using the revised version (34,35). This current 1588 1535 Appropriate in the adult AUC (3),butratedasRarely report for pediatric patients has certainly benefited from 1589 1536 Appropriate for pediatric patients (Indication #18), the maturational process and experience gained by the 1590 1537 albeit with additional qualifiers of a benign family AUC in adults (36). Implementation studies in the 1591 1538 history and a normal ECG. The reasonableness of using pediatric population will help us to identify any missing 1592 1539 an echocardiogram as a primary screen versus using an or ambiguous indications that could be addressed in 1593 1540 echocardiographic assessment only after a pediatric future revisions. 1594 1541 cardiology consultation for evaluation of a murmur, chest In comparing the ratings of various indications in 1595 1542 pain, syncope, or any other indication, depends on many the current document with those in the adult AUC, there 1596 1543 factorsandneedstobegivendueconsiderationona were many indications that were rated similarly (3). 1597 1544 case-by-case basis. For example, isolated PACs and sinus bradycardia 1598 1545 Given the complexity of clinical presentations, it is were rated as Rarely Appropriate indications in both 1599 1546 likely that there will be some overlap between the in- documents, while SVT, VT, pathologic murmurs, initial 1600 1547 dications in this document. Several indications share evaluation of suspected pulmonary hypertension, sys- 1601 1548 identical accompanying findings, signs or symptoms, but temic hypertension, and suspected endocarditis were 1602 1549 differ as to the primary patient complaint. As such, the rated as Appropriate in both. However, there were some 1603 1550 ratings were driven in these scenarios by the prevalence striking differences in the ratings of some indications 1604 1551 of the primary presentation and the likelihood of it being such as syncope and chest pain due to variations in the 1605 1552 cardiac-related. For example, non-exertional chest pain most common underlying causes in pediatric versus adult 1606 1553 with abnormal ECG (A [7] #33) and palpitations with patients. 1607 1554 abnormal ECG (M [6] #3) have been rated slightly There are also differences in format. In this report, 1608 1555 differently by the panel even though they both relate to priortestresultsforwhichasubsequentechocardiogram 1609 1556 an abnormal ECG. Given the broad definition of an may be ordered are listed separately in Table 7 with 1610 1557 abnormal ECG described in this paper, it is not unex- individual ratings; but in the adult AUC report they are 1611 1558 pected that the ratings for palpitations that may lumped together under one indication (‘Prior testing 1612 1559 accompany more benign ECG findings were a bit lower. that is concerning for heart disease or structural 1613 1560 Similarly, ratings for indications related to symptoms abnormality including but not limited to chest X-ray, 1614 1561 or signs of cardiovascular disease changed slightly baseline scout images for stress echocardiogram, ECG, or 1615 1562 depending on other presenting factors described in the cardiac biomarkers’ (3)), and are rated as Appropriate. 1616 1563 scenarios (#29 – chest pain and signs and symptoms – M The current report also includes a broad list of systemic 1617 1564 [6], #40 – presumptively innocent murmur with signs disorders (Table 7) and scenarios related to family 1618 1565 and symptoms – A[7],and#42– congestive heart failure history (Table 8) that are not covered in the adult AUC 1619 1566 with signs and symptoms – A [9]). In applying the AUC, if report. 1620

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1621 Limitations of this category lies more in recognition of a pattern of 1675 fi 1622 The current AUC report is not fully inclusive of all ordering where a signi cantly higher number of echo- 1676 1623 possible clinical scenarios and does not include in- cardiograms are requested fortheRarelyAppropriatein- 1677 1624 dications for follow-up or inpatient echocardiography. In dications by an individual provider compared with their 1678 1625 addition, it is restricted to the first use of transthoracic peers. Indications rated as May Be Appropriate could be 1679 1626 echocardiography and does not include indications for considered reasonable for obtaining an echocardiogram, 1680 1627 fetal or transesophageal echocardiography. Some of the particularly if the physician taking care of the patient 1681 1628 indications have been purposefully kept broad either determines that it would provide helpful information. 1682 1629 because it was beyond the scope of this report to list each These two categories should not be considered as the 1683 1630 and every possible scenario, or because they were basis for denying insurance coverage or reimbursement 1684 1631 considered fairly uncommon in routine practice. Exam- for the procedure, as individual decision making is 1685 1632 ples of these broad indications include use of illicit drugs, required to determine what is best for each patient. 1686 1633 chest wall deformities, chromosomal abnormalities with Nevertheless, it is important for the clinicians taking care 1687 1634 undefined risk of cardiovascular disease, suspected con- of pediatric patients to recognize that healthcare facil- 1688 1635 nective tissue disorders, neurologic or psychiatric disor- ities, accreditation bodies, or payers for these tests may 1689 1636 ders, gastrointestinal and hepatic disorders and several use this document to ensure quality care and appropriate 1690 fi 1637 indications related to family history. use of nancial resources. 1691 1638 Though we have attempted to cover a broad range of Ideally, this document will also serve as an educational 1692 1639 clinical scenarios in this document, we realize that by no and quality improvement tool for addressing the high 1693 1640 means is this list exhaustive. Given the experience with number of Rarely Appropriate referrals for echocardio- 1694 1641 the adult cardiology AUC, it would not be surprising for us grams by individual providers. Experience with the adult 1695 1642 to have missed some common indications. We also echocardiography AUC has shown that physician 1696 1643 recognizethatthisdocumentdoesnotaddressthe engagement in quality improvement programs, and 1697 1644 appropriateness, or lack thereof, of not performing echo- tracking and benchmarking of test ordering behavior, has 1698 1645 cardiograms. This underutilization of echocardiography reduced the percentage of inappropriate testing (37). 1699 1646 could result from a lack of availability (equipment, so- Further, lab accreditation organizations such as the 1700 1647 nographer or interpreting cardiologist), denial by payers Intersocietal Accreditation Commission (IAC) require 1701 1648 or lack of insurance, alteration of the management plan attention to AUC as part of their quality improvement 1702 1649 following expert consultation, or lack of sound clinical process (38).Finally,theAUCmayprovidethebasisfor 1703 1650 judgment. evaluation of the impact of using AUC, especially as 1704 1651 accessed by online tools, instead of more onerous and 1705 1652 Use of AUC to Improve Care less physician-driven administrative controls on imaging 1706 1653 use. 1707 We foresee several important applications of these AUC in 1654 1708 the pediatric population. The most obvious use of this 1655 1709 document will be to support the clinical decision making 10. CONCLUSIONS 1656 1710 of a provider as to the appropriateness of care that they 1657 1711 deliver to an individual pediatric patient. It is important This AUC report provides a helpful guide to clinicians in 1658 1712 to keep in mind that an Appropriate rating in this docu- determining the reasonable role of initial transthoracic 1659 1713 ment should not be misinterpreted as a recommendation echocardiography in the evaluation of pediatric patients 1660 1714 to perform an echocardiogram in every patient that meets in an outpatient setting. It also lays the foundation for 1661 1715 the indications described herein. Rather, it should be developing AUC in other areas of pediatric cardiology. 1662 1716 interpreted as something that would be reasonable to do Furthermore, it can form the basis of designing educa- 1663 1717 if the information obtained will help in caring for the tional and quality improvement projects to improve 1664 1718 patient. On the other hand, a Rarely Appropriate rating quality of care. Future studies to evaluate implementa- 1665 1719 should not be misinterpreted as one in which an echo- tion of these AUC in clinical care will be helpful not only 1666 1720 cardiogram should absolutely not be performed. This in identifying any deficiencies in the current document, 1667 1721 category was termed as “Inappropriate” in the initial AUC but also in defining ordering patterns for individual 1668 1722 documents, but due to significant misperceptions, the practitioners and understanding variations in delivery of 1669 1723 AUC Task Force changed the terminology from Inappro- care. We expect that there will be a continued need for 1670 1724 priate to Rarely Appropriate to emphasize that individual refinement of these AUC based on any gaps identified 1671 1725 patient circumstances do exist where an echocardiogram through this initial effort, changes in evidence-based 1672 1726 would be reasonable to perform. Instead of precluding an medicine, and availability of technical and financial 1673 1727 echocardiogram in an individual patient, the importance resources. 1674 1728

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1729 ACC PRESIDENT AND STAFF Z. Jenissa Haidari, MPH, CPHQ, Senior Research Specialist, 1783 1730 Appropriate Use Criteria 1784 1731 Patrick T. O’Gara, MD, FACC, President Lara M. Gold, MA, Senior Research Specialist, Appropriate 1785 1732 Shalom Jacobovitz, Chief Executive Officer Use Criteria 1786 1733 William J. Oetgen, MD, FACC, Executive Vice President, Amelia Scholtz, PhD, Publications Manager, Clinical 1787 1734 Science, Education, and Quality Policy and Pathways 1788 1735 Joseph M. Allen, MA, Senior Director, Clinical Policy and 1789 1736 Pathways 1790 1737 1791 1738 1792 REFERENCES 1739 1793 1740 1. Douglas PS, Khandheria B, Stainback RF, et al. ACCF/ 6. Patel MR, Spertus JA, Brindis RG, et al. ACCF pro- 14. Surawicz B, Childers R, Deal BJ, et al. AHA/ACCF/ 1794 1741 ASE/ACEP/ASNC/SCAI/SCCT/SCMR 2007 appropriate- posed method for evaluating the appropriateness of HRS recommendations for the standardization and 1795 ness criteria for transthoracic and transesophageal cardiovascular imaging. J Am Coll Cardiol 2005;46: interpretation of the electrocardiogram: part III: intra- 1742 echocardiography: a report of the American College of 1606–13. ventricular conduction disturbances: a scientific 1796 Cardiology Foundation Quality Strategic Directions statement from the American Heart Association 1743 7. Hendel RC, Patel MR, Allen JM, et al. Appropriate 1797 Committee Appropriateness Criteria Working Group, and Arrhythmias Committee, 1744 use of cardiovascular technology: 2013 ACCF appro- 1798 American Society of Echocardiography, American Col- Council on Clinical Cardiology; the American College priate use criteria methodology update: a report of the 1745 lege of Emergency Physicians, American Society of of Cardiology Foundation; and the Heart Rhythm 1799 American College of Cardiology Foundation appro- Nuclear Cardiology, Society for Cardiovascular Angi- Society. Endorsed by the International Society for 1746 priate use criteria task force. J Am Coll Cardiol 2013;61: 1800 ography and Interventions, Society of Cardiovascular Computerized Electrocardiology. J Am Coll Cardiol 1747 1305–17. 1801 Computed Tomography, and the Society for Cardio- 2009;53:976–81. 1748 vascular Magnetic Resonance endorsed by the Amer- 8. Fitch K, Bernstein S, Aguilar M, et al. The RAND/ 1802 15. Wagner GS, Macfarlane P, Wellens H, et al. AHA/ ican College of Chest Physicians and the Society of UCLA Appropriateness Method User’s Manual. Arling- 1749 ACCF/HRS recommendations for the standardization 1803 Critical Care Medicine. J Am Coll Cardiol 2007;50: ton, VA: RAND Corporation, 2001. 1750 and interpretation of the electrocardiogram: part VI: 1804 187–204. 9. Quinones MA, Otto CM, Stoddard M, et al. Recom- acute ischemia/infarction: a scientific statement from 1751 fi 1805 2. Douglas PS, Khandheria B, Stainback RF, et al. ACCF/ mendations for quanti cation of Doppler echocardi- the American Heart Association Electrocardiography fi 1752 ASE/ACEP/AHA/ASNC/SCAI/SCCT/SCMR 2008 appro- ography: a report from the Doppler Quanti cation Task and Arrhythmias Committee, Council on Clinical Car- 1806 1753 priateness criteria for stress echocardiography: a report Force of the Nomenclature and Standards Committee diology; the American College of Cardiology Founda- 1807 of the American College of Cardiology Foundation of the American Society of Echocardiography. J Am Soc tion; and the Heart Rhythm Society. Endorsed by the 1754 – 1808 Appropriateness Criteria Task Force, American Society Echocardiogr 2002;15:167 84. International Society for Computerized Electro- 1755 cardiology. J Am Coll Cardiol 2009;53:1003–11. 1809 of Echocardiography, American College of Emergency 10. Thomas JD, Adams DB, Devries S, et al. Guidelines 1756 Physicians, American Heart Association, American So- and recommendations for digital echocardiography. 16. Douglas P, Iskandrian AE, Krumholz HM, et al. 1810 1757 ciety of Nuclear Cardiology, Society for Cardiovascular J Am Soc Echocardiogr 2005;18:287–97. Achieving quality in cardiovascular imaging: pro- 1811 Angiography and Interventions, Society of Cardiovas- ceedings from the American College of Cardiology- 1758 11. Lang RM, Bierig M, Devereux RB, et al. Recom- 1812 cular Computed Tomography, and Society for Cardio- Duke University Medical Center Think Tank on Quality mendations for chamber quantification: a report from 1759 vascular Magnetic Resonance endorsed by the Heart in Cardiovascular Imaging. J Am Coll Cardiol 2006;48: 1813 the American Society of Echocardiography’s Guidelines Rhythm Society and the Society of Critical Care Medi- – 1760 fi 2141 51. 1814 cine. J Am Coll Cardiol 2008;51:1127–47. and Standards Committee and the Chamber Quanti - 1761 cation Writing Group, developed in conjunction with 17. Kemper AR, Mahle WT, Martin GR, et al. Strategies 1815 1762 3. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/ the European Association of Echocardiography, a for implementing screening for critical congenital heart 1816 AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 branch of the European Society of Cardiology. J Am disease. Pediatrics 2011;128:e1259–67. 1763 1817 Appropriate Use Criteria for Echocardiography. A Soc Echocardiogr 2005;18:1440–63. 18. Brindis RG, Douglas PS, Hendel RC, et al. ACCF/ 1764 Report of the American College of Cardiology Foun- 1818 12. Hancock EW, Deal BJ, Mirvis DM, et al. AHA/ ASNC appropriateness criteria for single-photon dation Appropriate Use Criteria Task Force, American 1765 ACCF/HRS recommendations for the standardization emission computed tomography myocardial perfusion 1819 Society of Echocardiography, American Heart Associa- and interpretation of the electrocardiogram: part V: imaging (SPECT MPI): a report of the American College 1766 tion, American Society of Nuclear Cardiology, Heart 1820 electrocardiogram changes associated with cardiac of Cardiology Foundation Quality Strategic Directions 1767 Failure Society of America, Heart Rhythm Society, So- 1821 chamber hypertrophy: a scientific statement from Committee Appropriateness Criteria Working Group ciety for Cardiovascular Angiography and In- 1768 the American Heart Association Electrocardiography and the American Society of Nuclear Cardiology 1822 terventions, Society of Critical Care Medicine, Society and Arrhythmias Committee, Council on Clinical endorsed by the American Heart Association. J Am Coll 1769 of Cardiovascular Computed Tomography, and Society 1823 Cardiology; the American College of Cardiology Cardiol 2005;46:1587–605. 1770 for Cardiovascular Magnetic Resonance Endorsed by 1824 Foundation; and the Heart Rhythm Society. the American College of Chest Physicians. J Am Coll 19. Antman EM, Peterson ED. Tools for guiding clinical 1771 Endorsed by the International Society for Computer- 1825 Cardiol 2011;57:1126–66. practice from the american heart association and the 1772 ized Electrocardiology. J Am Coll Cardiol 2009;53: american college of cardiology: what are they and 1826 4. Lai WW, Geva T, Shirali GS, et al. Guidelines and – 992 1002. how should clinicians use them? Circulation 2009;119: 1773 standards for performance of a pediatric echocardio- 1827 13. Rautaharju PM, Surawicz B, Gettes LS, et al. AHA/ 1180–5. 1774 gram: a report from the Task Force of the Pediatric 1828 ACCF/HRS recommendations for the standardization Council of the American Society of Echocardiography. 20. Sable CA, Rome JJ, Martin GR, et al. Indications for 1775 and interpretation of the electrocardiogram: part IV: 1829 J Am Soc Echocardiogr 2006;19:1413–30. echocardiography in the diagnosis of infective endo- 1776 the ST segment, T and U waves, and the QT interval: a carditis in children. Am J Cardiol 1995;75:801–4. 1830 5. Lopez L, Colan SD, Frommelt PC, et al. Recom- scientific statement from the American Heart Associa- 1777 21. Danford DA, Nasir A, Gumbiner C. Cost assessment 1831 mendations for quantification methods during the tion Electrocardiography and Arrhythmias Committee, of the evaluation of heart murmurs in children. 1778 performance of a pediatric echocardiogram: a report Council on Clinical Cardiology; the American College of 1832 Pediatrics 1993;91:365–8. 1779 from the Pediatric Measurements Writing Group of the Cardiology Foundation; and the Heart Rhythm Society. 1833 American Society of Echocardiography Pediatric and 1780 Endorsed by the International Society for Computer- 22. Yi MS, Kimball TR, Tsevat J, et al. Evaluation of 1834 Congenital Heart Disease Council. J Am Soc Echo- ized Electrocardiology. J Am Coll Cardiol 2009;53: heart murmurs in children: cost-effectiveness and 1781 cardiogr 2010;23:465–95. 982–91. practical implications. J Pediatr 2002;141:504–11. 1835 1782 1836

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1837 23. Friedman KG, Kane DA, Rathod RH, et al. 29. Verghese GR, Friedman KG, Rathod RH, et al. 33. Geva T, Hegesh J, Frand M. Reappraisal of the 1891 1838 Management of pediatric chest pain using a standard- Resource Utilization Reduction for Evaluation of Chest approach to the child with heart murmurs: is echo- 1892 ized assessment and management plan. Pediatrics Pain in Pediatrics Using a Novel Standardized Clinical cardiography mandatory? Int J Cardiol 1988;19:107–13. 1839 2011;128:239–45. Assessment and Management Plan (SCAMP). Journal 1893 34. Bhatia RS, Carne DM, Picard MH, et al. Comparison 1840 of the American Heart Assocation 2012;1. 1894 24. McCrindle BW, Shaffer KM, Kan JS, et al. Cardinal of the 2007 and 2011 appropriate use criteria for 1841 clinical signs in the differentiation of heart murmurs in 30. Cheitlin MD, Alpert JS, Armstrong WF, et al. ACC/ transthoracic echocardiography in various clinical set- 1895 – 1842 children. Arch Pediatr Adolesc Med 1996;150:169 74. AHA Guidelines for the Clinical Application of Echo- tings. J Am Soc Echocardiogr 2012;25:1162–9. 1896 cardiography. A report of the American College of 25. Drossner DM, Hirsh DA, Sturm JJ, et al. Cardiac 35. Mansour IN, Razi RR, Bhave NM, et al. Comparison 1843 Cardiology/American Heart Association Task Force on 1897 disease in pediatric patients presenting to a pediatric of the updated 2011 appropriate use criteria for echo- 1844 – Practice Guidelines (Committee on Clinical Application 1898 ED with chest pain. Am J Emerg Med 2011;29:632 8. cardiography to the original criteria for transthoracic, of Echocardiography). Developed in collaboration with 1845 26. Massin MM, Bourguignont A, Coremans C, et al. transesophageal, and stress echocardiography. J Am 1899 the American Society of Echocardiography. Circulation 1846 Chest pain in pediatric patients presenting to an Soc Echocardiogr 2012;25:1153–61. 1900 1997;95:1686–744. emergency department or to a cardiac clinic. Clin 1847 36. Douglas PS. Appropriate use criteria: past, present, 1901 Pediatr (Phila) 2004;43:231–8. 31. Rosenthal A. How to distinguish between innocent future. J Am Soc Echocardiogr 2012;25:1176–8. 1848 and pathologic murmurs in childhood. Pediatr Clin 1902 27. Ritter S, Tani LY, Etheridge SP, et al. What is the 1849 North Am 1984;31:1229–40. 37. Imaging in “FOCUS”. Available at: http://www. 1903 yield of screening echocardiography in pediatric syn- cardiosource.org/focus. Accessed January 24, 2014. 1850 cope? Pediatrics 2000;105:E58. 32. Newburger JW, Rosenthal A, Williams RG, et al. 1904 Noninvasive tests in the initial evaluation of 38. Echocardiography/ ICAEL: Accreditation Evolved. 1851 28. Steinberg LA, Knilans TK. Syncope in children: 1905 heart murmurs in children. N Engl J Med 1983;308: Available at: http://www.intersocietal.org/echo/. diagnostic tests have a high cost and low yield. 1852 61–4. Accessed January 24, 2014. 1906 J Pediatr 2005;146:355–8. 1853 1907 1854 1908 1855 1909 APPENDIX A. APPROPRIATE USE CRITERIA FOR Montefiore; and Associate Professor of Clinical Pediatrics, 1856 1910 INITIAL TRANSTHORACIC ECHOCARDIOGRAPHY Albert Einstein College of Medicine, New York, NY 1857 1911 IN OUTPATIENT PEDIATRIC CARDIOLOGY: RituSachdeva,MD,FACC,FAAP,FASE—Associate 1858 1912 PARTICIPANTS Professor, Emory University; and Director, Cardiovascular 1859 1913 Imaging Research Core, Children’sHealthcareofAtlanta, 1860 1914 Writing Group Sibley Heart Center, Atlanta, GA 1861 1915 — 1862 Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS 1916 ’ Rating Panel 1863 Chief, Children s Healthcare of Atlanta Sibley Heart 1917 1864 Center, Professor and Division Director of Cardiology, Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, 1918 — ’ 1865 Department of Pediatrics, Emory University School of Writing Committee Liaison Chief, Children sHealthcare 1919 1866 Medicine, Atlanta, GA of Atlanta Sibley Heart Center; Professor and Division 1920 — 1867 Pamela S. Douglas, MD, MACC, FAHA, FASE Past Director of Cardiology, Department of Pediatrics, Emory 1921 1868 President, American College of Cardiology; Past Presi- University School of Medicine, Atlanta, GA 1922 1869 dent, American Society of Echocardiography, and Ursula PamelaS.Douglas,MD,MACC,FAHA,FASE,Moder- 1923 — 1870 Geller Professor of Research in Cardiovascular Diseases, ator Past President, American College of Cardiology; 1924 1871 Duke University Medical Center, Durham, NC Past President, American Society of Echocardiography; 1925 — 1872 Benjamin W. Eidem, MD, FACC, FASE Past President, and Ursula Geller Professor of Research in Cardio- 1926 1873 Society of Pediatric Echocardiography; Past Chair, vascular Diseases, Duke University Medical Center, 1927 1874 Pediatric & Congenital Heart Disease Council; Past mem- Durham, NC 1928 — 1875 ber, Board of Directors, American Society of Echocardiog- Louis I. Bezold, MD, FACC, FAAP, FASE UK Health 1929 1876 raphy; and Professor of Medicine and Pediatrics, Division Care Enterprise Quality Director, Jennifer Gill Roberts 1930 1877 of Pediatric Cardiology, Department of Pediatric and Professor in Pediatric Cardiology, and Vice Chair of Pedi- 1931 1878 Adolescent Medicine, Mayo Graduate School of Medicine, atrics, University of Kentucky, Lexington, KY 1932 — 1879 Mayo Clinic College of Medicine, Rochester, MN WilliamB.Blanchard,MD,FACC,FAAP,FAHA Past 1933 — ’ 1880 WymanW.Lai,MD,MPH,FACC,FASE Chair, Pediatric Medical Director of Nemours Children sClinicinPensa- 1934 1881 and Congenital Council Board, American Society of cola, FL; Past President, American Heart Association 1935 fi 1882 Echocardiography; Director of Non-invasive Cardiac Florida/Puerto Rico Af liate; Statewide Associate Pediat- 1936 ’ 1883 Imaging, Pediatric Echocardiography Laboratory, and ric Cardiology Consultant for Children s Medical Services, 1937 1884 Congenital Cardiac MRI Program, Division of Pediatric FL; and Medical Director, Florida Association of Chil- 1938 ’ 1885 Cardiology, Department of Pediatrics, New York Presby- dren sHospitals,FL 1939 ’ — 1886 terian Morgan Stanley Children sHospital,NewYork,NY JeffreyR.Boris,MD,FACC,FAAP Clinical Associate 1940 — 1887 LeoLopez,MD,FACC,FAAP,FASEChair, Pedia- Professor of Pediatrics; Director, Postural Orthostatic 1941 1888 tric and Congenital Heart Disease Council, American Tachycardia Syndrome Program; and Pediatric Cardiolo- 1942 ’ 1889 Society of Echocardiography; Director, Pediatric Cardiac gist, Division of Cardiology, Children s Hospital of Phila- 1943 ’ 1890 Non-invasive Imaging at the Children sHospitalat delphia, Philadelphia, PA 1944

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1945 Bryan Cannon, MD—Associate Professor of Pediatrics, Michigan Congenital Heart Center, University of Michigan 1999 1946 and Director, Pediatric Arrhythmia and Pacing Service, Medical School, Ann Arbor, MI 2000 1947 Mayo Clinic, Rochester, MN Robert Wiskind, MD, FAAP—President, Georgia Chapter 2001 1948 Gregory J. Ensing, MD, FACC, FASE—Member of the of the American Academy of Pediatrics; Managing Part- 2002 1949 ASE Pediatric Council Board, and Professor of Pediatrics ner, Peachtree Park Pediatrics, Atlanta, GA 2003 1950 and Pediatric Cardiology, University of Michigan CS Mott 2004 1951 Hospital for Children, Ann Arbor, MI Reviewers 2005 1952 Craig E. Fleishman, MD, FACC, FASE—President, Soci- MerylS.Cohen,MD—Medical Director, Non-Invasive 2006 1953 ety of Pediatric Echocardiography; Medical Director, Non- Cardiovascular Laboratory, The Children’sHospitalof 2007 1954 Invasive Cardiac Imaging, The Heart Center at Arnold Philadelphia, Philadelphia, PA 2008 1955 Palmer Hospital for Children; and Associate Professor of Mario J. Garcia, MD, FACC—Chief, Division of Cardiol- 2009 1956 Pediatrics, University of Central Florida College of Medi- ogy, Professor of Medicine and Radiology, and Co-Director, 2010 1957 cine,Orlando,FL Montefiore-Einstein Center for Heart and Vascular Care, 2011 1958 Mark A. Fogel, MD, FACC, FAHA, FAAP—Professor of Montefiore Medical Center, The University Hospital for 2012 1959 Cardiology and Radiology, University of Pennsylvania the Albert Einstein College of Medicine, Bronx, NY 2013 1960 School of Medicine; Past member, Board of Trustees, Michael Gewitz, MD, FACC, FAAP, FAHA—Professor 2014 1961 Society for Cardiovascular Magnetic Resonance; Past and Physician-in-Chief, Pediatric Cardiology, Maria Fareri 2015 1962 member, American College of Cardiology Imaging Coun- Children’s Hospital at Westchester Medical Center; and 2016 1963 cil; Member, Board of Scientific Counselors, National Professor and Vice Chairman, Department of Pediatrics, 2017 1964 Heart Lung and Blood Institute of the National Institute of New York Medical College, Valhalla, NY 2018 1965 Health; and Director of Cardiac Magnetic Resonance, The Willem A. Helbing, MD—Professor of Pediatric Cardiol- 2019 1966 Children’s Hospital of Philadelphia, Division of Cardiol- ogy, Erasmus University Medical Center, Sophia Chil- 2020 1967 ogy, Philadelphia, PA dren’s Hospital, Rotterdam, The Netherlands 2021 1968 B. Kelly Han, MD, FACC—Director of Congenital Cardiac Alexander J. Javois, MD, FACC, FAAP, FSCAI—Assistant 2022 1969 Imaging, Minneapolis Heart Institute and the Children’s Clinical Professor of Pediatrics, University of Illinois 2023 1970 HeartClinicattheChildren’s Hospitals and Clinics of Medical Center, Advocate Children’sHospital,OakLawn, 2024 1971 Minnesota, Minneapolis, MN IL 2025 1972 Shabnam Jain, MD, MPH, FAAP—Associate Professor of Walter H. Johnson, Jr, MD—Professor of Pediatrics, Di- 2026 1973 Pediatrics and Emergency Medicine; Director for Quality, vision of Pediatric Cardiology, Alabama Congenital Heart 2027 1974 Pediatric Emergency Medicine, Emory University; Medi- Disease Center, University of Alabama at Birmingham & 2028 1975 cal Director for Clinical Effectiveness, Children’sHealth- Children’s of Alabama, Birmingham, AL 2029 1976 care of Atlanta, Atlanta, GA Ann Kavanaugh-McHugh, MD—Associate Professor of 2030 1977 Mark B. Lewin, MD—Professor and Chief, Division of Pediatrics, and Director of Pediatric Cardiology Imaging 2031 1978 Pediatric Cardiology, University of Washington School of Laboratory, Division of Pediatric Cardiology, Vanderbilt 2032 1979 Medicine; and Co-Director, Heart Center, Seattle Chil- University Medical Center 2033 1980 dren’s Hospital, Seattle, WA H. Helen Ko, BS, RDMS, RDCS, FASE—Technical 2034 1981 Richard H. Lockwood, MD—Associate Medical Director, Director/Operations Manager, Pediatric Echocardiogra- 2035 1982 Excellus Blue Cross Blue Shield, Syracuse, NY phy, Mount Sinai Medical Center, New York, NY 2036 1983 G. Paul Matherne, MD, MBA, FACC, FAHA—Dammann Seema Mital, MD, FACC, FAHA, FRCPC—Professor of 2037 1984 Professor of Pediatrics, Vice Chair for Clinical Affairs, and Pediatrics, Hospital for Sick Children, University of Tor- 2038 1985 Associate Chief Medical Officer, University of Virginia onto,Toronto,Ontario,Canada 2039 1986 Children’s Hospital, Charlottesville, VA Andrew J. Powell, MD—Associate Professor of Pediat- 2040 1987 David Nykanen, MD, FACC, FRCPC, FSCAI—Member, rics, Harvard Medical School; Senior Associate in Cardi- 2041 1988 Executive Committee, Congenital Heart Disease Council, ology, Department of Cardiology, Boston Children’s 2042 1989 Society for Cardiovascular Angiography and Inter- Hospital, Boston, MA 2043 1990 ventions; Co-Director, The Heart Center; and Chief, Car- J. Carter Ralphe, MD—Assistant Professor of Pediatrics, 2044 1991 diology and Cardiac Catheterization, Arnold Palmer and Chief, Pediatric Cardiology, University of Wisconsin 2045 1992 Hospital for Children, Orlando, FL School of Medicine & Public Health, Madison, WI 2046 1993 CatherineL.Webb,MD,FACC,FAHA,FASE—Past Chair, Arno AW Roest, MD, PhD—Pediatric Cardiologist, Division 2047 1994 Council on Cardiovascular Disease in the Young, Amer- of Pediatric Cardiology, Department of Pediatrics, Leiden 2048 1995 ican Heart Association; Past Co-Chair, Congenital Heart University Medical Center, Leiden, The Netherlands 2049 1996 Public Health Consortium; Past Board Member, Sub-board JenniferN.A.Silva,MD—Pediatric Electrophysiology, 2050 1997 of Pediatric Cardiology, American Board of Pediatrics; and Washington University School of Medicine, St. Louis 2051 1998 Professor of Pediatrics and Communicable Diseases, Children’s Hospital, St. Louis, MO 2052

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2053 Julia Steinberger, MD, MS—Professor of Pediatrics, and Associate Professor of Medicine, Baylor College of Medi- 2107 2054 Dwan Chair of Pediatric Cardiology, University of Min- cine, Houston, TX 2108 2055 nesota Amplatz Children’s Hospital, Minneapolis, MN L. Samuel Wann, MD – Columbia St. Mary’s Healthcare, 2109 2056 Milwaukee, WI 2110 2057 ACC Appropriate Use Criteria Task Force Joseph M. Allen, MA—Senior Director, American Col- 2111 2058 Steven R. Bailey, MD, FACC, FSCAI, FAHA—Chair, Divi- lege of Cardiology, Washington, DC 2112 2059 sion of Cardiology, Professor of Medicine and Radiology, 2113 2060 Janey Briscoe Distinguished Chair, University of Texas 2114 2061 Health Sciences Center, San Antonio, TX APPENDIX B. RELATIONSHIPS WITH INDUSTRY 2115 2062 Alan S. Brown, MD, FACC—Medical Director, Midwest (RWI) AND OTHER ENTITIES 2116 2063 Heart Disease Prevention Center, Midwest Heart Special- 2117 2064 ists,EdwardHeartHospital,Naperville,IL The College and its partnering organizations rigorously 2118 2065 John U. Doherty, MD, FACC, FAHA—Professor of Med- avoid any actual, perceived, or potential conflicts of in- 2119 2066 icine, Jefferson Medical College of Thomas Jefferson terest that might arise as a result of an outside relation- 2120 2067 University, Philadelphia, PA ship or personal interest of a member of the rating panel. 2121 2068 Pamela S. Douglas, MD, MACC, FAHA, FASE—Past Specifically, all panelists are asked to provide disclosure 2122 2069 President, American College of Cardiology; Past Presi- statements of all relationships that might be perceived 2123 2070 dent, American Society of Echocardiography; and Ursula as real or potential conflicts of interest. These statements 2124 2071 Geller Professor of Research in Cardiovascular Diseases, were reviewed by the Appropriate Use Criteria Task 2125 2072 Duke University Medical Center, Durham, NC Force, discussed with all members of the rating panel at 2126 2073 Robert C. Hendel, MD, FACC, FAHA, FASNC—Chair, the face-to-face meeting, and updated and reviewed as 2127 2074 Appropriate Use Criteria for Radionuclide Imaging necessary. A table of relevant disclosures by the rating 2128 2075 Writing Group; Director of Cardiac Imaging and Outpa- panel and oversight working group members can be found 2129 2076 tient Services, Division of Cardiology, Miami University below. In addition, to ensure complete transparency, 2130 2077 School of Medicine, Miami, FL a full list of disclosure information—including relation- 2131 2078 Christopher M. Kramer, MD, FACC, FAHA—Co-Chair, ships not pertinent to this document—is available in the 2132 2079 AUC Task Force, Ruth C. Heede Professor of Cardiology and Online Appendix. 2133 2080 Radiology, and Director, Cardiovascular Imaging Center, 2134 2081 University of Virginia Health System, Charlottesville, VA 2135 2082 Bruce D. Lindsay, MD, FACC—Professor of Cardiology, Appropriate Use Criteria for Initial Transthoracic 2136 2083 Cleveland Clinic Foundation of Cardiovascular Medicine, Echocardiography in Outpatient Pediatric Cardiology: Members 2137 2084 Cleveland, OH of the Writing Group, Rating Panel, Indication Reviewers, and 2138 — 2085 Manesh R. Patel, MD, FACC— Chair, AUC Task Force, AUC Task Force Relationships with Industry and Other Entities 2139 2086 Assistant Professor of Medicine, Division of Cardiology, (Relevant) 2140 2087 Duke University Medical Center, Durham, NC Note: A standard exemption to the ACC RWI policy is 2141 2088 Leslee Shaw, PhD, FACC, FASNC— Professor of Medi- extended to Appropriate Use Criteria writing groups, 2142 2089 cine,EmoryUniversitySchoolofMedicine,Atlanta,GA since they do not make recommendations but rather 2143 2090 Raymond F. Stainback, MD, FACC, FASE—Medical Di- prepare background materials and typical clinical sce- 2144 2091 rector of Non-invasive Cardiac Imaging, Texas Heart narios/indications that are rated independently by a 2145 2092 Institute at St. Luke’s Episcopal Hospital; Clinical separate panel of experts. 2146 2093 2147 2094 2148 2095 2149 2096 2150 2097 2151 2098 2152 2099 2153 2100 2154 2101 2155 2102 2156 2103 2157 2104 2158 2105 2159 2106 2160

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2161 APPENDIX B. CONTINUED 2215 2162 2216 2163 Institutional, 2217 Ownership/ Organizational, 2164 Speakers Partnership/ or Other Financial Expert 2218 fi 2165 Participant Consultant Bureau Principal Personal Research Bene t Witness 2219 2166 Writing Group 2220 2167 Robert M. Campbell None None None None None None 2221 2168 Pamela S. Douglas None None None None None None 2222 2169 Benjamin W. Eidem None None None None None None 2223 2170 Wyman W. Lai None None None None None None 2224

2171 Leo Lopez None None None None None None 2225

2172 Ritu Sachdeva None None None None None None 2226

2173 Rating Panel 2227 2174 2228 Louis I. Bezold None None None None None None 2175 2229 William B. Blanchard None None None None None None 2176 2230 Jeffrey R. Boris None None None None None None 2177 2231 Bryan Cannon None None None None None None 2178 2232 Gregory J. Ensing None None None None None None 2179 2233 2180 Craig E. Fleishman Gore Medical None None None None None 2234 Supplies 2181 2235 Mark A. Fogel None None None Siemens Medical None None 2182 Systems 2236

2183 B. Kelly Han None None None Siemens Medical None None 2237 2184 Systems 2238 2185 Shabnam Jain None None None None None None 2239 2186 Mark B. Lewin None None None None None None 2240 2187 Richard H. Lockwood None None None None Excellus BCBS* None 2241

2188 G. Paul Matherne None None None None None None 2242

2189 David Nykanen None None None None None None 2243

2190 Catherine L. Webb None None None None None None 2244 2191 2245 Robert Wiskind None None None None None None 2192 2246 Reviewers 2193 2247 Meryl S. Cohen None None None None None None 2194 2248 Mario J. Garcia None None None None None None 2195 2249 Michael Gewitz None None None None None None 2196 2250 2197 Willem A. Helbing None None None None None None 2251 2198 Alexander J. Javois None None None None None None 2252 2199 Walter H. Johnson None None None None None None 2253 2200 Ann Kavanaugh-McHugh None None None None None None 2254 2201 Hyun-Sook Helen Ko None None None None None None 2255 2202 Seema Mital None None None None None None 2256

2203 Andrew J. Powell None None None None None None 2257

2204 J. Carter Ralphe None None None None None None 2258

2205 Arno AW Roest None None None None None None 2259 2206 2260 Jennifer Silva None None None None None None 2207 2261 Julia Steinberger None None None None None None 2208 2262 (continued on the next page) 2209 2263 2210 2264 2211 2265 2212 2266 2213 2267 2214 2268

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2269 APPENDIX B. CONTINUED 2323 2270 2324 Institutional, 2271 Ownership/ Organizational, 2325 2272 Speakers Partnership/ or Other Financial Expert 2326 Participant Consultant Bureau Principal Personal Research Benefit Witness 2273 2327 Appropriate Use Criteria Task Force 2274 2328 Steven R. Bailey None None None None None None 2275 2329 2276 Alan S. Brown None None None None None None 2330 2277 John U. Doherty None None None None None None 2331 2278 Pamela S. Douglas None None None None None None 2332 2279 Robert C. Hendel None None None None None None 2333 2280 Christopher M. Kramer None None None Siemens Medical None None 2334 Solutions 2281 2335 2282 Bruce D. Lindsay None None None None None None 2336 2283 Manesh R. Patel None None None None None None 2337 2284 Leslee J. Shaw None None None None None None 2338 2285 Raymond Stainback None None None None None None 2339 2286 L. Samuel Wann None None None None None None 2340 2287 Joseph M. Allen None None None None None None 2341 2288 2342 This table represents the relevant relationships with industry and other entities that were disclosed by participants at the time of participation. It does not necessarily reflect re- 2289 lationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of 5% or more of the voting stock 2343 2290 or share of the business entity, or ownership of $10,000 or more of the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% 2344 of the person’s gross income for the previous year. A relationship is considered to be modest if it is less than significant under the preceding definition. Relationships in this table are 2291 modest unless otherwise noted. Names are listed in alphabetical order within each category of review. Participation does not imply endorsement of this document. 2345 2292 *Significant (greater than $10,000) relationship. 2346 2293 2347 2294 2348 2295 2349 2296 2350 2297 2351 2298 2352 2299 2353 2300 2354 2301 2355 2302 2356 2303 2357 2304 2358 2305 2359 2306 2360 2307 2361 2308 2362 2309 2363 2310 2364 2311 2365 2312 2366 2313 2367 2314 2368 2315 2369 2316 2370 2317 2371 2318 2372 2319 2373 2320 2374 2321 2375 2322 2376

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