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Conditions Leading to Pediatric Consultation in a Tertiary Academic Hospital

Robert L. Geggel, MD

ABSTRACT. Objective. To determine the basis for neonates with aortic arch obstruction. Approximately cardiac consultations for pediatric patients in an aca- two-thirds of arrhythmias were supraventricular in ori- demic hospital setting. gin. The most common arrhythmias requiring treatment Methods. The activities of the cardiology consulta- were supraventricular and atrial flutter/fi- tion service were tabulated for 12 months, from July 2001 brillation, the latter occurring mainly among older pa- to June 2002. Patients were identified from 4 sources, ie, tients with structural disease. Diagnoses made with a monthly log of patient encounters maintained by the fetal echocardiography accounted for 14.3% of newborn consultation service, encounter forms submitted to the consultations and included 83% of patients with cyanotic billing office, consultation notes maintained in a central cardiac disease. Three syndromes accounted for 57% of file, and a departmental list of echocardiography studies. consultations for this indication, ie, VACTERL associa- Patients who required clearance for noncardiac surgical tion (vertebral anomalies, anal atresia, congenital heart procedures were generally evaluated in the cardiology disease, tracheoesophageal fistula, renal abnormality, clinic and not by the consultation service. Patient data and limb anomalies), trisomy 21, and infant of diabetic were obtained from consultation and echocardiography mother. and syncope/dizziness were fre- reports and from hospital computer-based records for quently evaluated in the emergency department and, in discharge summaries for inpatient admissions, emer- this setting, accounted for 13 and 10% of all evaluations gency department encounter summaries, and laboratory and 19 and 25% of evaluations for new patients, respec- reports. For each patient, consultations were tabulated as tively. For patients evaluated for chest pain, the most separate encounters if they occurred on different days in common basis was musculoskeletal/costochondritic the emergency department, during separate admissions, (42%) or idiopathic (22%). There was a cardiac or pericar- or for different clinical concerns during a single admis- dial basis in 11% of cases; these patients either had sion. known heart disease associated with this complication or Results. A total of 2071 consultations were performed systemic symptoms, abnormal cardiac auscultatory find- for 1724 patients. The age at the time of consultation was ings, and electrocardiographic features of pericarditis. years (median: 1.2 years; range: 1 day to 60.6 Syncope/dizziness most commonly had a vasovagal 9.3 ؎ 6.6 years). A total of 1507 patients (87.4%) had a single con- (50.5%) or orthostatic (24.7%) basis. There was a cardiac sultation; 217 patients (12.6%) had multiple encounters, basis in 5.4% of cases; these patients were more likely to ranging from 2 to 9, accounting for 564 consultations have symptoms associated with exercise. Although endo- (27.2%). Clinical concerns included murmurs (18.5%), car- carditis was a frequent clinical concern (91 patients), only diac function (12.7%), arrhythmias (12.7%), intercurrent 3 cases were identified, involving 2 patients with struc- illnesses among cardiac patients (11.3%), cyanosis (6.3%), tural heart disease and 1 neonate with an indwelling syndromes (5.7%), chest pain (5.2%), syncope/dizziness intracardiac catheter. Two other patients had central ve- (4.5%), subacute (4.4%), follow-up evalua- nous lines, intravascular , and fungemia. Ka- tions of fetal diagnoses (4.3%), Kawasaki disease (3.4%), wasaki disease was the most common acquired condition cor pulmonale (3%), recent cardiac surgery or catheteriza- leading to consultation. Cor pulmonale was most com- tion (1.6%), cerebrovascular accidents (1.2%), and miscel- monly screened among patients with congenital dia- laneous conditions. Four diagnoses accounted for 91% of phragmatic hernia, chronic disease of prematurity, murmur evaluations, ie, , ventric- , , or . ular septal defects, innocent murmurs, and pulmonary Patients with recent cardiac surgery or cardiac catheter- branch murmur of infancy. The most common murmur ization typically had postpericardiotomy syndrome or diagnosis in the neonatal intensive care unit was patent complications associated with vascular access. Approxi- ductus arteriosus (68%), in the well-child nursery was mately 20% of cases of cerebrovascular accidents had a ventricular septal defect (64%), and on the medical ward cardiac basis. was innocent murmur (62%). The most common basis for Conclusions. Although a variety of conditions were evaluation of function was oncologic disease. Among assessed, some were encountered more frequently. Fu- patients evaluated for function, there were 3 new diag- ture educational curricula developed for cardiac training noses of structural congenital heart disease, all involving of pediatric residents should appropriately emphasize conditions necessitating consultation. Pediatrics 2004; 114:e409–e417. URL: www.pediatrics.org/cgi/doi/10.1542/ From the Department of Cardiology, Children’s Hospital, and the Depart- ment of Pediatrics, Harvard Medical School, Boston, Massachusetts. peds.2003-0898-L; congenital heart disease, health educa- Accepted for publication Apr 26, 2004. tion, patient care. doi:10.1542/peds.2003-0898-L Address correspondence to Robert L. Geggel, MD, Children’s Hospital, lthough a variety of cardiac conditions and 300 Longwood Ave, Boston, MA 02115. E-mail: robert.geggel@cardio. chboston.org symptoms occur in the pediatric age range, PEDIATRICS (ISSN 0031 4005). Copyright © 2004 by the American Acad- Athe relative frequency of issues addressed in emy of Pediatrics. cardiac consultations in a hospital setting has not

www.pediatrics.org/cgi/doi/10.1542/peds.2003-0898-LDownloaded from www.aappublications.org/news byPEDIATRICS guest on October Vol. 2, 2021 114 No. 4 October 2004 e409 been determined. Education of medical students and ter for a given diagnosis. Chest pain was classified with previously residents in pediatric cardiology is challenging both reported criteria.2 because structural congenital heart disease is uncom- mon, affecting Ͻ1% of children, and because condi- Statistical Analyses tions potentially having a cardiac basis frequently Patient ages are expressed as mean Ϯ SD. Analyses of the have other causes.1–5 To establish the appropriate occurrence of heart disease among patients with supraventricular emphasis for clinical instruction in pediatric resi- tachycardia or atrial fibrillation/atrial flutter and among patients with syncope/dizziness associated with exertion were performed dency training programs, I determined the basis for with Fisher’s exact test. Comparisons of the age at first encounter cardiac consultations in a tertiary academic pediatric between patients with supraventricular tachycardia and those hospital. with atrial fibrillation/atrial flutter and between patients with supraventricular tachycardia with or without congenital heart METHODS disease were made with the Wilcoxon rank sum test. Study Population RESULTS The activities of the cardiology consultation service were tab- ulated from July 1, 2001, to June 30, 2002. Patients were evaluated Number of Consultations in the emergency department, neonatal and pediatric intensive care units, inpatient wards, and occasional outpatient settings at During the study period, 2071 consultations were Children’s Hospital, Boston, and in the neonatal intensive care performed for 1724 patients. The age at the time of units and nurseries at Brigham and Women’s Hospital and Beth consultation was 6.6 Ϯ 9.3 years (median: 1.2 years; Israel-Deaconess Hospital. There is a general cardiology ward and range: 1 day to 60.6 years). A single consultation was a cardiac intensive care unit at Children’s Hospital, Boston, which performed for 1507 patients. Two hundred seventeen are not staffed by the consultation service. Patients were identified from 4 sources, ie, a monthly log of patient encounters maintained patients (12.6%) had multiple encounters, ranging by the consultation service, encounter forms submitted to the from 2 to 9, accounting for 564 consultations (27.2%). billing office, consultation notes maintained in a central file, and a The monthly total of consultations ranged from 154 departmental list of echocardiographic studies. Although cardiol- to 201 (average: 173). Patients were evaluated most ogy consultation is generally required for echocardiography, our departmental policy has permitted independent ordering of echo- often in the neonatal intensive care unit, inpatient cardiographic tests by the oncology service, to evaluate cardiac wards, or the emergency department, for known car- function associated with chemotherapy protocols, and by the neo- diac patients (Fig 1). natology service, to screen for patent ductus arteriosus among premature infants. Patients who required clearance for noncardiac surgical proce- Reasons for Consultations dures were generally evaluated in the cardiology clinic and not by Murmurs the consultation service. The study was approved by the Chil- dren’s Hospital Committee on Clinical Investigation. Evaluation of a murmur represented 18.5% of en- counters (Tables 1 and 2). The median age was 6 days Data Tabulation (range: 1 day to 18.5 years). Echocardiography was Patient data were obtained from consultation and echocardiog- used for all patients except for ϳ50% of patients with raphy reports and Children’s Hospital computer-based records for pulmonary branch murmurs of infancy or other in- discharge summaries for inpatient admissions, emergency depart- nocent murmurs and for 10% of patients with ven- ment encounter summaries, and laboratory reports. Tabulated data included patient age, date, site, and basis of consultation, tricular septal defects, each of which was judged to whether the patient was new or was known to the cardiology be small. For patients evaluated for a murmur, the service, performance of echocardiography, , most common diagnosis or clinical concern in the or chest radiography, and final diagnosis. For each patient, con- neonatal intensive care unit was patent ductus arte- sultations were tabulated as separate encounters if they occurred on different days in the emergency department, during separate riosus (68%), in the well-child nursery was ventricu- admissions, or for a different clinical concern during a single lar septal defect (64%), and on the medical ward was admission. Patient ages were tabulated as age at the first encoun- innocent murmur (62%).

Fig 1. Numbers of consultations performed on a monthly basis at different sites. Numbers in parentheses indicate the total numbers of patients evaluated. “Other” sites included the preoperative clinic, post-anesthesia care unit, radiology department, and infusion center. ER indicates emergency department; NICU, neonatal intensive care unit; PICU, pediatric intensive care unit.

e410 CARDIOLOGY CONSULTATIONDownloaded from www.aappublications.org/news IN AN ACADEMIC HOSPITAL by guest on October 2, 2021 TABLE 1. Sites and Bases for Consultations Basis for consultation No. of Cases ED, Known ED, New NICU Well PICU Medical Other Total Nursery Ward Murmur 17 254 73 3 34 3 384 Function 35 4 20 53 150 2 264 Arrhythmia 106 43 20 8 15 62 8 262 Intercurrent illness 210 2 21 233 Cyanosis (postnatal diagnosis) 13 12 47 5 8 1 86 Syndrome 97 2 4 15 118 Chest pain 57 35 13 2 107 Syncope/dizziness 25 45 22 1 93 Subacute bacterial endocarditis 5 2 9 17 58 91 Fetal diagnosis follow-up Arrhythmia 6 1 7 Cyanotic heart disease 45 45 Acyanotic heart disease 25 25 Normal postnatal status 8 5 13 Kawasaki disease 1 3 1 65 70 PAH/cor pulmonale 11 37 14 62 Recent cardiac surgery 25 25 Recent catheterization 8 8 CVA/TIA 8 3 6 7 24 Other conditions Surgical clearance 2151128 8 4 2 6 20 Abnormal ECG,-nonarrhythmic 4 2 3 8 1 18 4 3 10 17 ALTE 2 1 12 15 Thrombus 3 1 8 12 Systemic hypertension 1 1 1 6 1 10 Vascular ring 1 1 1 6 9 1 6 7 Miscellaneous 9* 4† 2‡ 1§ 2¶ 18 ALTE indicates apparent life-threatening episode; CVA/TIA, cerebrovascular accident/transient ischemic attack; ED, emergency depart- ment; NICU, neonatal intensive care unit; PAH, pulmonary hypertension; PICU, pediatric intensive care unit; ECG, electrocar- diogram. * Five central venous line malfunctions (for prostacyclin or antibiotic administration), 2 hemoptysis in patients with pulmonary vascular obstructive disease, hypercalcemia, and 1 femoral artery pseudoaneurysm (catheterization performed 1 year previously). † Two thoracopagus conjoined twins, 1 sibling with congenital heart disease, and 1 rule out coarctation. ‡ Two siblings with congenital heart disease. § Rule out . ¶ Tachypnea.

TABLE 2. Consultations for Evaluation of a Murmur Diagnosis No. of Cases ED, New NICU Well PICU Medical Other Total Echocardiography Nursery Ward Aortic 1 1 2 4 4 Atrial myxoma 1 1 1 , secundum 1 6 1 1 9 9 Atrioventricular canal, transitional 1 1 1 Innocent or flow murmur 2 19 5 21 1 48 26 Mitral regurgitation 1 1 1 Mitral stenosis 1 1 1 Patent ductus arteriosus 135 5 1 1 1 143 143 Rule out patent ductus arteriosus, not present 37 37 37 Peripheral pulmonary stenosis 4 27 5 1 8 1 46 24 Pulmonary stenosis 3 1 4 4 Truncus arteriosus 1 1 1 Ventricular septal defect 5 21 47 1 2 76 68 Ventricular septal defect/coarctation 1 1 1 Resolved before consultation 7 4 11 0 Abbreviations as in Table 1.

Function oncologic disease (40.5%), congenital heart disease Cardiac function evaluation accounted for 12.7% (13.6%), sepsis (10.6%), postoperative general surgi- of consultations (Table 1). The patient age was 8.7 Ϯ cal procedures (7.2%), neuromuscular disease (6.8%), 8.5 years (median: 6.4 years). Common conditions arteriovenous malformations (3.8%), and hemato- prompting evaluation of cardiac function included logic disease (sickle cell disease or ␤-thalassemia)

Downloaded from www.aappublications.org/newswww.pediatrics.org/cgi/doi/10.1542/peds.2003-0898-L by guest on October 2, 2021 e411 TABLE 3. Consultations for Evaluation of an Arrhythmia Arrhythmia No. of Cases ED, Known ED, New NICU Well Nursery PICU Medical Ward Other Total Atrial Atrial fibrillation 5 1 2 8* Atrial flutter 28 1 1 30† Atrial premature beats 4 2 4 4 1 1 16 Ectopic atrial tachycardia 2 2 1 1 6 Sinus arrhythmia 2 3 1 6 Sinus 2 2 1 3 21 1 30 Sinus tachycardia 4 5 1 7 17‡ Supraventricular tachycardia 34 11 4 1 1 3 54§ Ventricular Ventricular premature beat 3 2 5 2 7 8 2 29 Ventricular tachycardia 3 2 2 7 Ventricular fibrillation 2 2 Junctional Junctional ectopic tachycardia 1 1 1 1 4 Junctional escape rhythm 2 1 3 Junctional premature beat 112 Heart block First-degree heart block 1 1 2 Second-degree heart block 2 1 3 Congenital complete heart block 1 1 Normal 1 1 2 2 1 7 , no identified arrhythmia 7 8 1 16 Pacemaker ICD discharge 6 6 Dual-chamber pacemaker malfunction 3 3 Prolonged OT interval 1 2 2 11 1 17 Values include prenatal and postnatal evaluations. ICD indicates implantable cardioverter-defibrillator; other abbreviations as in Table 1. * Six different patients (congenital heart disease in 3; 1 patient each with Ebstein’s anomaly, status post Fontan, and cardiomyopathy). † Seventeen different patients (congenital heart disease in 15: single ventricle, status post Fontan, n ϭ 9; dextro-transposition of the great , status post atrial switch procedure, n ϭ 2; cardiomyopathy, n ϭ 2; 1 patient each with and levo-transposition of the great arteries). ‡ Fifteen different patients. § Thirty-eight different patients.

(3.4%). There were 3 new diagnoses of structural cemia in 5 cases and hypokalemia in 1 case) or had a congenital heart disease among neonates who were normal interval (n ϭ 6). An associated disease was examined initially in the emergency department or present for 1 patient each with first-degree atrioven- pediatric intensive care unit; 2 had severe coarctation tricular block (anorexia nervosa), transient second- and the other an interrupted aortic arch. degree atrioventricular block (Lyme disease), and neonatal complete heart block (maternal systemic Arrhythmias lupus erythematosus). Evaluation of an arrhythmia accounted for 12.7% of consultations (Tables 1 and 3). Atrial arrhythmias Intercurrent Illnesses represented 63% of consultations for the indication of Evaluation of patients with known cardiac disease arrhythmia. The most common arrhythmias requir- presenting with an intercurrent noncardiac illness ing treatment were supraventricular tachycardia, (mainly viral syndromes) accounted for 11.3% of atrial flutter, and atrial fibrillation. Compared with consultations (Table 1). The patient age was 5.3 Ϯ 7.4 patients with atrial flutter/atrial fibrillation, patients years (median: 2.0 years). with supraventricular tachycardia were younger (7.9 Ϯ 8.5 years; median: 5.4 years; vs 26.1 Ϯ 12.1 years; median: 22.7 years; P Ͻ .001) and had a lower Cyanosis incidence of congenital heart disease (8% vs 78%, P Ͻ Cyanosis formed the basis of consultations for .001). Patients with supraventricular tachycardia 6.3% of patients (Tables 1 and 4). Three-quarters of with congenital heart disease presented at older ages the patients were Ͻ1 week of age. Of 54 patients with than did those without structural defects (median structural cyanotic heart disease, 45 (83%) were di- age: 15.1 years vs 3.1 years; P ϭ .05). agnosed with fetal echocardiography. The mothers Of the 17 patients evaluated for possible pro- of these neonates had delivery scheduled at a nearby longed QT interval, 1 neonate had congenital prolon- hospital. Intercurrent illnesses among patients with gation of the QT interval. The other patients had palliated cyanotic heart disease produced increasing prolongation of the interval resulting from medica- cyanosis if there was fever for patients with system- tion effects (n ϭ 4; clomipramine, nortriptyline, or ic-pulmonary shunts or dehydration for those with pentamidine) or metabolic defects (n ϭ 6; hypocal- intracardiac right-to-left shunts.

e412 CARDIOLOGY CONSULTATIONDownloaded from www.aappublications.org/news IN AN ACADEMIC HOSPITAL by guest on October 2, 2021 TABLE 4. Consultations for Evaluation of Cyanosis Diagnosis No. of Cases ED, Known ED, New NICU PICU Medical Ward Other Total Acrocyanosis 3 6 2 11 Apnea-bradycardia 1 2 1 4 Cyanotic CHD, Follow-up fetal diagnosis 45* 45 Cyanotic CHD, intercurrent illness 9 1 10 Cyanotic CHD, new diagnosis 2† 7‡ 9 Cyanosis, crying only, neonate 2 1 3 Obstructive sleep apnea 11 PPHN 26 3 29 Pulmonary disease§ 18251 17 Rule out tetralogy spell 1 1 Seizure 11 CHD indicates congenital heart disease; PPHN, persistent of newborn; other abbreviations as in Table 1. * Double-outlet right ventricle, 1; hypoplastic left heart syndrome, 17; pulmonary atresia, intact ventricular septum, 5; single ventricle, 4; tetralogy of Fallot, 11; dextro-transposition of the great arteries, 5; tricuspid atresia, 2. † Total anomalous pulmonary venous connection, 2 (1 infradiaphragmatic, 1 supracardiac to innominate ). ‡ Pulmonary atresia, intact ventricular septum, 1; right ventricular endocardial fibroelastosis, 1; tetralogy of Fallot, 1; total anomalous pulmonary venous connection, 1; dextrotransposition of the great arteries, 3. § Pulmonary conditions included associated with gastroesophageal reflux disease and aspiration, , or dysplasia, laryngomalacia, and postoperative bronchospasm.

Syndromes ment evaluations, and 25% of emergency department The evaluation of cardiac involvement in syn- evaluations for new patients. The patient age was dromes prompted consultation for 5.7% of patients 14.4 Ϯ 5.4 years (median: 14.9 years). The most com- (Tables 1 and 5). The median age was 2 days. Three mon causes were vasovagal (47 episodes, 50.5%) or syndromes represented 57% of the consultations for orthostatic (23 episodes, 24.7%). For 5 patients, or- this indication, ie, VACTERL association (vertebral thostasis was related to medication (clonidine, anomalies, anal atresia, congenital heart disease, tra- quetiapine fumarate, or furosemide). Six episodes cheoesophageal fistula, renal abnormality, and limb (6.5%) had an idiopathic basis and 2 had other causes anomalies), trisomy 21, and infant of a diabetic (hyperventilation or menorrhagia). Five patients mother. (5.4%) each demonstrated a cardiac, neurologic, or psychiatric basis. Cardiac diseases included 2 new Chest Pain patients with congenital prolonged QT interval and 1 Chest pain accounted for 5.2% of consultations, known patient each with primary pulmonary hyper- 13% of all emergency department evaluations, and tension, severe subaortic stenosis, and bradycardia 19% of emergency department consultations for new secondary to pacemaker dysfunction. Neurologic patients (Tables 1 and 6). The patient age was 16.1 Ϯ conditions included a seizure disorder, breath-hold- 7.4 years (median: 15.4 years). For this symptom, the ing spell, and migraine headache. Patients with a 2 most common categories were musculoskeletal/ cardiac basis were more likely to have symptoms costochondritic (42%) and idiopathic (22%) condi- associated with exercise (P ϭ .001). Of 13 patients tions. There was a cardiac or pericardial basis in 11% who experienced syncope (n ϭ 8) or dizziness (n ϭ 1) of cases; these patients either had a known cardiac during exercise or syncope shortly after completion history or had pericarditis presenting with systemic of exercise (n ϭ 4), 7 demonstrated a vasovagal basis, symptoms and abnormal cardiac auscultatory or 4 had a cardiac condition, and 1 each exhibited a electrocardiographic findings (6). Chest pain with psychiatric or idiopathic cause. exertion occurred for 2 patients, each with known heart disease. One patient with palliated cyanotic Subacute Endocarditis heart disease and normocytic erythrocytes experi- Evaluation for subacute endocarditis accounted enced resolution of chest pain with reduction of he- for 4.4% of consultations (Tables 1 and 7). The patient matocrit values from 71% to 63%. A chest radiograph age was 7.7 Ϯ 7.3 years (median: 5.2 years). Of 91 was obtained in 92 episodes (86%) and contributed to consultations for this concern, there were 3 cases of the diagnosis for the 13 patients with pulmonary subacute endocarditis (3.3%) (Table 7). Two events abnormalities and the 5 patients with pericarditis. An occurred among known cardiac patients, presenting electrocardiogram was obtained in 106 episodes to the emergency department with either fever and (99%) and differed from baseline tracings or was bacteremia or an indolent course of weight loss and abnormal for 4 patients with pericarditis. Concern anorexia. For each patient, the blood cultures yielded about possible pulmonary embolism was raised for 6 Streptococcus viridans. A premature neonate had an patients, but the results of imaging studies were umbilical venous line, Staphylococcus aureus bactere- normal. mia, and right-sided intracardiac vegetations. Two patients had candidemia and intravascular thrombus Syncope/Dizziness associated with a central venous line. Evaluation for syncope or dizziness accounted for Transthoracic echocardiograms were obtained in 4.5% of consultations, 10% of all emergency depart- 79 consultations (87%) and transesophageal echocar-

Downloaded from www.aappublications.org/newswww.pediatrics.org/cgi/doi/10.1542/peds.2003-0898-L by guest on October 2, 2021 e413 TABLE 5. Consultations for Syndromes or Other Congenital Anomalies Syndrome No. % CHD CHD Lesions VACTERL 28 43 VSD, 6; ASD, 2; bicuspid AoV, 2; TOF, 1; RAA-aberrant LSCA, 3* Trisomy 21 22 45 VSD, 3; CAVC, 2; PDA, 2; TOF, 2 Infant of diabetic mother 17 65 VSD, 1; PS, 1; septal hypertrophy, 9 (2 with LVOT obstruction) Omphalocele 6 17 VSD and PDA, 1 Cleft palate 6 33 VSD, 1; RAA-aberrant LSCA, 1 Twin-twin transfusion 4 25 VSD, 1 Dandy-Walker syndrome 3 67 VSD, 1; bicuspid AoV, 1 Jejunal atresia 3 33 PS Rule out DiGeorge syndrome† 30 Rule out ‡ 30 Rule out tuberous sclerosis§ 3 50 Intracardiac rhabdomyoma, 1 Miscellaneous¶ 20 45 VSD, 4; VSD and ASD, 1; ASD, 2; bicuspid AoV, 1; MR, 1 AoV indicates aortic valve; ASD, atrial septal defect; CAVC, complete atrioventricular canal defect; LSCA, left subclavian artery; LVOT, left ventricular outflow tract; MR, mitral regurgitation; PDA, patent ductus arteriosus; PS, pulmonary stenosis; RAA, right aortic arch; TOF, tetralogy of Fallot; VSD, ventricular septal defect. * Two patients with RAA-aberrant LSCA also had either VSD or ASD. † Evaluated for hypocalcemia; 1 patient had DiGeorge syndrome. ‡ Evaluated for , joint dislocation, or superior mesenteric artery aneurysm; none diagnosed with Marfan syndrome. § Each with seizure disorder; 2 patients diagnosed with tuberous sclerosis. ¶ Chromosome abnormality other than trisomy 21 (3; partial trisomy 9q, 14q duplication, and partial deletion of 18q); or stenosis 2, Klippel-Trenaunay-Weber 2; 1 patient each with Beal’s syndrome, cloacal extrophy, coloboma, extrahepatic biliary atresia, gastroschisis, Holt-Oram syndrome, Kartagener’s syndrome, lobar emphysema, Marden-Walker syndrome, nemoline rod myopathy, Russel-Silver syndrome, Smith-Lemli-Opitz syndrome, and tracheal stenosis.

TABLE 6. Consultations for Evaluation of Chest Pain Diagnosis No. of Cases ED, Known ED, New Medical Ward Other Total Anxiety 4 1 5 Cardiac disease 7* 3† 2‡ 12 Gastroesophageal reflux 1 1 Idiopathic 9 9 4 2 24 Musculoskeletal/costochondritic 27 14 4 45 Polycythemia 1 1 Pulmonary /pleuritis 1 2 3 Pneumonia 2 1 2 5 2 2 Pneumothorax 2 1 3 Skin infection at cvl site 1 1 Toxin, cannabis 2 3 5 cvl indicates central venous line; other abbreviations as in Table 1. * Known patients with pulmonary vascular obstructive disease, 2; recurrent pericarditis, 2; tetralogy of Fallot with systemic level right ventricular pressure produced by an obstructed right ventricular- pulmonary artery conduit, 1; Kawasaki disease with known giant aneurysm of left anterior descend- ing coronary artery with echocardiographic confirmation of new clot in this artery, 1; subclavicular pacemaker pocket infection, 1. † Pericarditis associated with fever and in each patient. ‡ Known patients with Takayasu’s aortitis or severe cardiomyopathy secondary to ␤-thalassemia. diograms in 6 (6.7%). Thrombus or vegetation was of consultations and 14.1% of consultations per- noted only for the patients cited above. Echocardiog- formed in the neonatal intensive care unit and nurs- raphy was deferred for 9 patients who had sterile eries (Table 1). blood cultures and for 3 others who had sterile cul- tures after removal of a central venous catheter and Kawasaki Disease antibiotic therapy for bacteremia. This clinical concern represented 3.4% of all con- The most common clinical background was septi- sultations (Tables 1 and 2). The patient age was 4.3 Ϯ cemia with an indwelling central venous catheter (36 3.3 years (median: 3.7 years). Of the 70 encounters, 51 episodes, 40%). A new murmur was present for 11 patients were diagnosed as having Kawasaki dis- patients, 6 of whom had positive blood cultures. ease. The other 19 patients were diagnosed as having Echocardiograms were obtained for 10 of these pa- viral syndrome (15 patients), urinary tract infection tients. Each murmur was innocent, ie, 6 Still’s mur- or pyelonephritis (1 patient, 2 episodes), hypersensi- murs, 4 flow murmurs associated with anemia, and 1 tivity reaction to dilantin, or necrotizing fasciitis. innocent pulmonary branch murmur of infancy. Pulmonary Artery Hypertension/Cor Pulmonale Follow-up Evaluations of Fetal Diagnoses Concern about pulmonary artery hypertension ac- Ninety neonates had consultations prompted by counted for 3.0% of consultations. The patient age fetal echocardiographic diagnoses, representing 4.3% was 3.7 Ϯ 6.9 years (median: 92 days; range: 1 day to

e414 CARDIOLOGY CONSULTATIONDownloaded from www.aappublications.org/news IN AN ACADEMIC HOSPITAL by guest on October 2, 2021 TABLE 7. Consultations to Rule Out Subacute Bacterial Endocarditis No. of Cases ED, Known ED, New NICU PICU Medical Ward Total Basis for consultation Positive BC 1* 1 7* 13† 35 57‡ Fever 5* 1 3 16† 42 24 New murmur 1 1 1 8 11 New S3 11 Weight loss, anorexia 1† 1 Fatigue 1 1 Embolic event 112 Clinical conditions Repaired/palliated CHD 4* 3 7 Unrepaired CHD 1† 45 Oncologic diseases 31619 General surgery within previous 8 wk 4 5 11 20 Indwelling central venous catheter 5* 10† 33 48 Intravenous drug abuser 1 1 Dental abscess 1 1 BC indicates blood culture; other abbreviations as in Tables 1 and 4. * One patient with each symbol had subacute bacterial endocarditis; see text for details. † Two patients with possible infected intravascular thrombus; see text for details. ‡ Three organisms accounted for 39 (67%) of the 58 episodes of septicemia (57 presenting with septicemia and 1 presenting with constitutional symptoms and subsequently found to have bacteremia). Various species of Candida were isolated in 17 cases, Staphylococcus aureus in 14 episodes, and coagulase-negative Staphylococcus in 8. There were 5 cases of Streptococcus viridans and 2 cases each of Pseudomonas aeruginosa and group A Streptococcus. Ten other bacteria were isolated only once, for different patients.

25.6 years). The most common condition was con- 69 FL). The other 18 patients had a variety of condi- genital diaphragmatic hernia, which was found for tions, ie, protein C, protein S, or antithrombin III 34% of patients evaluated for this concern. Other deficiencies, carotid artery dissection, moyamoya conditions prompting evaluation of pulmonary ar- disease, sickle cell disease, encephalitis, coagulopa- tery pressure included chronic lung disease associ- thy associated with intraventricular hemorrhage, se- ated with premature birth, pneumonitis or reactive vere hyponatremia caused by psychogenic polydip- airway disease, cystic fibrosis, pulmonary hypopla- sia, or idiopathic causes. sia associated with chest wall deformity or cystic adenomatoid malformation, obstructive sleep apnea, Other Conditions pulmonary hemosiderosis, neurologic conditions as- A variety of other conditions were evaluated (Ta- sociated with hypoventilation, and orthotopic lung ble 1). Eight of 20 patients (40%) who underwent transplantation. consultations because of cardiomegaly on chest ra- diographs had heart disease. The cardiologist and Recent Cardiac Surgery or Cardiac Catheterization radiologist judged the cardiac silhouette to be nor- Eight of 25 patients evaluated within 2 months mal in an equal number, 5 of whom had a large after surgery had postpericardiotomy syndrome. thymus. For 4 other patients, echocardiograms Other issues addressed included surgical wound showed normal cardiac dimensions. Infants with an evaluation, pleural effusion, intervening viral illness, apparent life-threatening event had normal QT inter- pain control, and bacterial pericarditis. vals and no other identifiable cardiac disorders; 5 Evaluations after catheterization, with 1 exception, patients demonstrated an idiopathic basis, whereas involved interventional procedures. One patient had the others had apnea, periodic breathing, breath- transient fever after coil occlusion of collateral ves- holding, gastroesophageal reflux, or seizure disor- sels, whereas the others had vascular access prob- der. Systemic hypertension was associated with re- lems consisting of pain (local hematoma, infected nal disease for 4 patients, agitation, headache, or hematoma, or arterial pseudoaneurysm) or coolness postoperative pain for 5, and medication (fludrocor- of an extremity. tisone and midodrine) for 1. Evaluation for a vascu- lar ring was prompted mainly by the symptom of Cerebrovascular Accidents/Transient Ischemic Attacks , which was caused for 2 patients each by Evaluation of a cardiac source for loss of neuro- and laryngomalacia and for 1 patient logic function accounted for 1.2% of consultations each by left main stenosis, subglottic hem- (Table 1). A cardiac basis was identified for 5 of the angioma, innominate artery compression, and uni- 24 patients evaluated for this concern, ie, intracardiac lateral ; 1 patient with an incidental thrombus for 2 patients with a single ventricle and finding of a right aortic arch had a loose vascular bidirectional flow across a patent foramen ovale for 3 ring with an aberrant left subclavian artery. Of 7 patients, including a neonate with a thrombus at the patients evaluated for rheumatic fever, 2 with chorea tip of a central venous line and 2 adolescents. An and mitral regurgitation were diagnosed with this additional adult patient had pulmonary vascular ob- condition. Two other patients with movement disor- structive disease, polycythemia (hematocrit: 62%), ders had either Guillain-Barre´ or antiphospholipid and microcytic anemia (mean corpuscular volume: syndrome. The other 3 patients did not fulfill Jones

Downloaded from www.aappublications.org/newswww.pediatrics.org/cgi/doi/10.1542/peds.2003-0898-L by guest on October 2, 2021 e415 criteria, including 2 with fever and an innocent mur- rence in the general population,1 because delivery of mur. these infants was arranged at a nearby hospital. Such a policy can decrease morbidity and mortality DISCUSSION rates.18 Patients with palliated cyanotic heart disease This review documents for the first time the issues experienced increased cyanosis with intercurrent ill- prompting cardiac consultation in an academic pe- ness in the presence of dehydration, because of in- diatric hospital setting. Although a variety of condi- creased oxygen extraction and lower venous oxygen tions were assessed, some diagnoses were encoun- saturation, in conditions with right-to-left intracar- tered more frequently and should be given emphasis diac shunting or in the presence of fever, because of in future curriculum development for cardiac train- decreased shunt perfusion produced by lower sys- ing for pediatric residents. temic vascular resistance, in conditions with system- Evaluation of a murmur was the most frequent ic-pulmonary artery shunting. The oxygen dissocia- basis for consultation. Cardiac skills in tion curve is steep for arterial oxygen pressure values various training programs, including pediatrics, of Ͻ50 mm Hg, so that a small decrease in oxygen have been documented to be poor.6–8 Four condi- tension produces a large decrease in oxygen satura- tions (patent ductus arteriosus, ventricular septal de- tion.19 fect, innocent murmur, and pulmonary branch mur- Chest pain and syncope were frequently evaluated mur of infancy) accounted for 91% of diagnoses for in the emergency department,3 and each was typi- patients with murmurs. Initially developing stetho- cally associated with a benign condition.2,3,20 A seri- scope skills among pediatric residents for the detec- ous organic cause for chest pain was unusual with- tion of these conditions should fulfill clinical needs out associated symptoms of illness, positive physical frequently. Developing skills for the diagnosis of an examination findings related to the cardiac or respi- innocent murmur should have additional benefits in ratory systems, or symptoms during exertion.21 The reducing parental morbidity if confirmation by a occurrence of syncope during or shortly after exer- cardiologist is subsequently sought, because the abil- tion also was commonly observed among patients ity of the pediatrician to provide greater reassurance with cardiac conditions.3 has been shown to reduce parental anxiety associ- Syndromes have been reported for 8.5% of patients ated with referral.9 with congenital heart disease.22 Recognition of syn- Assessment of function was frequently requested dromes permits thorough patient evaluation, includ- for a variety of clinical conditions. Newly developed ing early detection of heart disease, which may not dysfunction among infants after discharge from the produce clinical symptoms as long as pulmonary birth hospital can represent duct-dependent lesions vascular resistance is elevated in the early neonatal that elude detection while the ductus is widely period. The 3 syndromes evaluated most frequently patent.10 are associated commonly with congenital heart dis- The majority of arrhythmias were atrial in origin. ease.23–25 The most common arrhythmia was supraventricular Endocarditis was uncommon and occurred among tachycardia, which usually was associated with a patients with known congenital heart disease or in- structurally normal heart.11 Atrial fibrillation/atrial dwelling venous catheters.26 Risk factors for trans- flutter occurred among older patients, who typically thoracic echocardiographic evidence of endocarditis had congenital heart disease; the chronic effects of that were identified in each of 2 pediatric reports pressure or volume overload or surgical scars con- included positive blood cultures, physical findings of tribute to the development of these 2 arrhythmias,12 new or changing heart murmur, and congestive which are commonly seen among patients, as in our .27,28 Indications in neonates may be dif- study group, who have undergone atrial baffle repair ferent.26,27 Our experience shows that the feature of a of dextrotransposition of the great arteries (Mustard new murmur should be defined as a new regurgitant or Senning procedures), modified Fontan procedures murmur,29 because a hyperdynamic condition asso- because of a functional single ventricle, or repair of ciated with febrile illness can produce innocent mur- tetralogy of Fallot.12,13 Young adults with repaired murs. congenital heart disease are becoming a larger pa- Kawasaki disease was the most common acquired tient group, because of improved survival rates, and disease leading to consultation. Approximately one- often are cared for at pediatric hospitals; therefore, quarter of patients evaluated for Kawasaki disease familiarity with these arrhythmias is important. Ven- were determined to have other conditions. Consid- tricular arrhythmias were infrequent and mainly ering the diagnosis of Kawasaki disease for children consisted of ventricular premature beats. Prolonged with some features of the illness is warranted, be- QT syndrome usually was associated with a meta- cause atypical presentation has been associated with bolic abnormality or medication-related side effect,14 coronary involvement.30 rather than with congenital prolongation. Heart Various conditions were encountered less fre- block was uncommon and was associated with an- quently. Evaluation of pulmonary hypertension orexia nervosa,15 Lyme disease,16 or maternal sys- mainly involved conditions associated with pulmo- temic lupus erythematosus.17 nary disease. Doppler echocardiography can be used Diagnoses made with fetal echocardiography were to estimate the level of pulmonary systolic pressure a frequent basis for neonatal consultation. Cyanotic and to monitor the hemodynamic effects and dosing lesions were over-represented among the prenatally of nitric oxide or supplemental oxygen in these con- diagnosed patients, in comparison with the occur- ditions.31,32 Consultation after cardiac surgery

e416 CARDIOLOGY CONSULTATIONDownloaded from www.aappublications.org/news IN AN ACADEMIC HOSPITAL by guest on October 2, 2021 chiefly involved evaluation for postpericardiotomy 12. Triedman JK. Arrhythmias in adults with congenital heart disease. syndrome,33 whereas assessment after catheteriza- Heart. 2002;87:383–389 13. Geggel RL. Update on the modified Fontan procedure. Curr Opin Car- tion mainly involved vascular access concerns diol. 1997;12:51–62 among patients who had undergone interventional 14. Viskin S. Long QT syndromes and torsade de pointes. Lancet. 1999;354: procedures (a risk factor for complications).34 Al- 1625–1633 though neurologic deficits usually had a noncardiac 15. Palla B, Litt IF. Medical complications of eating disorders in adoles- 5 cents. Pediatrics. 1988;81:613–623 cause, our patient group confirmed risks associated 16. Steere AC. Lyme disease. N Engl J Med. 345:115–125, 2001 35 15 with cyanotic heart disease, Fontan procedure, 17. Chameides L, Truex RC, Vetter V, Rashkind WJ, Galioto FM Jr, Noonan thrombosis related to the use of an indwelling central JA. Association of maternal systemic lupus erythematosus with congen- venous catheter,36 patent foramen ovale,37 and poly- ital complete heart block. N Engl J Med. 1977;297:1204–1207 cythemia associated with microcytosis.35 Cardiomeg- 18. Allan L. Antenatal diagnosis of heart disease. Heart. 2000;83:367–370 19. Rudolph A. Congenital Diseases of the Heart. Chicago, IL: Year Book aly was not present for 60% of patients evaluated for Medical Publishers; 1974:102 38 this condition. Failure to distinguish a large over- 20. Kapoor WN. Current evaluation and management of syncope. Circula- lying thymus gland from true cardiac enlargement tion. 2002;106:1606–1609 occurred relatively frequently. Systemic hyperten- 21. Geggel RL. Evaluation of pediatric chest pain. In: Rose BD, ed. UpTo- Date. Wellesley, MA: UpToDate; 2003 sion was associated with renal disease, the most 22. Greenwood RD, Rosenthal A, Parisi L, Fyler DC, Nadas AS. Extracar- 39 common cause in pediatrics. Stridor, which can be diac anomalies in children with congenital heart disease. Pediatrics. produced by vascular compression of the airways,40 1975;55:485–492 was associated mainly with airway disease among 23. Zahka KG. Associated abnormalities in children with congenital heart our patients. disease. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gut- gesell HP, eds. Heart Disease in Infants, Children, and Adolescents. 5th ed. Baltimore, MD: Williams & Wilkins; 1995;1:614–628 CONCLUSIONS 24. Lin AE. Congenital heart defects in chromosome abnormality syn- dromes. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gut- Although numerous conditions were evaluated by gesell HP, eds. Heart Disease in Infants, Children, and Adolescents. 5th ed. the cardiac consultation service, some were encoun- Baltimore, MD: Williams & Wilkins; 1995;1:633–643 tered more frequently and should be given emphasis 25. Caddell JL. Metabolic and nutritional diseases. In: Emmanouilides GC, in curricula developed for pediatric residency train- Riemenschneider TA, Allen HD, Gutgesell HP, eds. Heart Disease in ing programs. Future investigations will be needed Infants, Children, and Adolescents. 5th ed. Baltimore, MD: Williams & Wilkins; 1995;1:1453–1486 to determine the effectiveness of acquiring skills for 26. Ferrieri P, Gewitz MH, Gerber MA, et al. Unique features of infective the diagnosis of cardiac symptoms and disease dur- endocarditis in childhood. Circulation. 2002;105:2115–2127 ing pediatric residency. 27. Sable CA, Rome JJ, Martin GR, Patel KM, Karr SS. Indications for echocardiography in the diagnosis of in children. Am J Cardiol. 1995;75:801–804 ACKNOWLEDGMENT 28. Aly AM, Simpson PM, Humes RA. The role of transthoracic echocar- I thank the fellows and attending physicians who helped staff diography in the diagnosis of infective endocarditis in children. Arch the cardiology consultation service during the study period. Pediatr Adolesc Med. 1999;153:950–954 29. Lindner JR, Case A, Dent JM, Abbott RD, Scheld WM, Kaul S. Diagnos- Circulation. REFERENCES tic value of echocardiography in suspected endocarditis. 1996;93:730–736 1. Hoffman JIE. Incidence of congenital heart disease: part I: postnatal 30. Newburger JW, Burns JC. Kawasaki disease. Vasc Med. 1999;4:187–202 incidence. Pediatr Cardiol. 1995;16:103–113 31. Fraser KL, Tullis E, Sasson Z, Hyland RH, Thornley KS, Hanly PJ. 2. Selbst SM, Ruddy RM, Clark BJ, Henretig FM, Santulli T Jr. Pediatric Pulmonary hypertension and cardiac function in adult cystic fibrosis. chest pain: a prospective study. Pediatrics. 1988;82:319–323 Chest. 1999;115:1321–1328 3. 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