2018 Guideline for the Management of Adults with Congenital Heart Disease

2018 Guideline for the Management of Adults with Congenital Heart Disease GUIDELINES MADE SIMPLE A Selection of Tables and Figures ACC.org/GMSACHD 2018 Guideline for the Management of Adults with Congenital Heart Disease A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines Writing Committee: Karen K. Stout, MD, FACC, Chair Curt J. Daniels, MD, FACC, Vice Chair Jamil A. Aboulhosn, MD, FACC, FSCAI Biykem Bozkurt, MD, PhD, FACC, FAHA Craig S. Broberg, MD, FACC Jack M. Colman, MD, FACC Stephen R. Crumb, DNP, AAC Joseph A. Dearani, MD, FACC Stephanie Fuller, MD, MS, FACC Michelle Gurvitz, MD, FACC Paul Khairy, MD, PhD Michael J. Landzberg, MD, FACC Arwa Saidi, MB, BCH, FACC Anne Marie Valente, MD, FACC, FAHA, FASE George F. Van Hare, MD The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease”, which was the first U.S. guideline to be published on the topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and growing ACHD expertise to develop recommendations. The following resource contains tables and figures from the 2018 Guideline for the Management of Adults with Congenital Heart Disease. The resource is only an excerpt from the Guideline and the full publication should be reviewed for more tables and figures as well as important context. CITATION: J Am Coll Cardiol. Aug 2018, 25255; DOI 10.1016/j.jacc.2018.08.1029 2018 Guideline for the Management of Adults with Congenital Heart Disease GUIDELINES MADE SIMPLE Selected Table or Figure Page Physiological Variables as Used in ACHD AP Classification ………………………………………………… 4-7 ACHD Anatomic and Physiological (AP) Classification (CHD Anatomy + Physiological Stage = ACHD AP Classification ………………………………………… 8-10 Circumstances Where CMR, CCT, TEE, and/or Cardiac Catheterization May be Superior to TTE ……… 11 Comparison of Imaging Modalities Useful in ACHD Evaluation …………………………………………… 11 Specific Management Practices for Cyanotic CH …………………………………………………………… 12 Secundum ASD ………………………………………………………………………………………………… 13 Hemodynamically Significant Ventricular Level Shunt ……………………………………………………… 14 Isolated PR after Repair of PS ………………………………………………………………………………… 15 Pulmonary Valve Replacement in Patients With TOF Repair and PR ……………………………………… 16 Anomalous Aortic Origin of the Coronary Artery …………………………………………………………… 17 Routine Follow-Up and Testing Intervals for Specific Conditions: ASD …………………………………………………………………………………………………… 18 VSD …………………………………………………………………………………………………… 18 AVSD ………………………………………………………………………………………………… 18 PDA …………………………………………………………………………………………………… 18 Congenital Mitral Stenosis ………………………………………………………………………… 19 SubAS ………………………………………………………………………………………………… 19 Supravalvular Aortic Stenosis ……………………………………………………………………… 20 CoA …………………………………………………………………………………………………… 20 Valvular PS …………………………………………………………………………………………… 20 Branch and Peripheral PS …………………………………………………………………………… 21 Double-Chambered Right Ventricle ………………………………………………………………… 21 Ebstein Anomaly ……………………………………………………………………………………… 21 TOF …………………………………………………………………………………………………… 22 Right Ventricle-to-PA Conduit ……………………………………………………………………… 22 d-TGA with Atrial Switch …………………………………………………………………………… 23 d-TGA with Arterial Switch …………………………………………………………………………… 23 CCTGA ………………………………………………………………………………………………… 24 Fontan Palliation …………………………………………………………………………………… 24 Pulmonary Hypertension and Eisenmenger Syndrome …………………………………………… 25 Routine Follow-Up and Testing Intervals for Congenital Aortic Stenosis ………………………………… 26 GUIDELINES MADE SIMPLE Back to Table of Contents ACHD 2018 Guideline for the Management of Adults with Congenital Heart Disease Physiological Variables as Used in ACHD AP Classification (1 of 4) Variable Description Aortopathy Aortic enlargement is common in some types of CHD and after some repairs. Aortic enlargement may be progressive over a lifetime. There is no universally accepted threshold or repair, nor is the role of indexing to body size clearly dened in adults, as is done in pediatric populations. For purposes of categorization and timing of follow-up imaging: • Mild aortic enlargement is dened as maximum diameter 3.5–3.9 cm • Moderate aortic enlargement is dened as maximum diameter 4.0–4.9 cm • Severe aortic enlargement is dened as maximum diameter ≥5.0 cm Arrhythmia Arrhythmias are very common in patients with ACHD and may be both the cause and consequence of deteriorating hemodynamics, valvular dysfunction, or ventricular dysfunction. Arrhythmias are associated with symptoms, outcomes, and prognosis, thus are categorized based on presence and response to treatment. • No arrhythmia–no documented clinically relevant atrial or ventricular tachyarrhythmias •Arrhythmia not requiring treatment–bradyarrhythmia, atrial or ventricular tachyarrhythmia not requiring antiarrhythmic therapy, cardioversion, or ablation • Arrhythmia controlled with therapy: • Bradyarrhythmia requiring pacemaker implantation • Atrial or ventricular tachyarrhythmia requiring antiarrhythmic therapy, cardioversion, or ablation • AF and controlled ventricular response • Patients with an ICD • Refractory arrhythmias: • Atrial or ventricular tachyarrhythmia not currently responsive to or refractory to antiarrhythmic therapy or ablation Concomitant Severity dened according to the 2014 VHD guideline VHD • Mild VHD • Moderate VHD • Severe VHD Table 3 is continued in the next page. For abbreviations please refer to page 7. Table 3 4 GUIDELINES MADE SIMPLE Back to Table of Contents ACHD 2018 Guideline for the Management of Adults with Congenital Heart Disease Physiological Variables as Used in ACHD AP Classification (2 of 4) Variable Description End-organ Clinical and/or laboratory evidence of end-organ dysfunction including dysfunction • Renal (kidney) • Hepatic (liver) • Pulmonary (lung Exercise Patients with ACHD are often asymptomatic notwithstanding exercise limitations capacity demonstrated as diminished exercise capacity when evaluated objectively. Thus, assessment of both subjective and objective exercise capacity is important (see NYHA classication system below). Exercise capacity is associated with prognosis. • Abnormal objective cardiac limitation to exercise is dened as an exercise maximum ventilatory equivalent of oxygen below the range expected for the specic CHD anatomic diagnosis. • Expected norms for CPET values should take into account age, sex, and underlying congenital diagnosis. Published studies with institution-specic norms can be used as guides, bearing in mind variability among institutional norms and ranges. Hypoxemia/ See Section 3.16 for detailed denition of cyanosis. hypoxia/ cyanosis • Hypoxemia is dened as oxygen saturation measured by pulse oximetry at rest ≤90%. • Severe hypoxemia is dened as oxygen saturation at rest <85%. • In patients with normal or high hemoglobin concentrations, severe hypoxemia will be associated with visible cyanosis (which requires ≥5g/L desaturated hemoglobin to be appreciated). • The terms cyanosis and hypoxemia (or hypoxia) are sometimes used interchangeably. Such interchangeability would not apply; however, in the presence of anemia, when severe hypoxemia can be present without visible cyanosis. Table 3 is continued in the next page. For abbreviations please refer to page 7. Table 3 5 GUIDELINES MADE SIMPLE Back to Table of Contents ACHD 2018 Guideline for the Management of Adults with Congenital Heart Disease Physiological Variables as Used in ACHD AP Classification (3 of 4) Variable Description HA Class Functional Capacity functional classification I Patients with cardiac disease but resulting in no limitation of physical activity. system Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain. II Patients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity results in fatigue, palpitation, dyspnea, or anginal pain. III Patients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes fatigue, palpitation, dyspnea, or anginal pain. IV Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of HF or the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort increases. Pulmonary Pulmonary hypertension is a broad term that encompasses pulmonary arterial hypertension, hypertension which is pulmonary hypertension with increased pulmonary vascular resistance. This document denes PH and PAH as they are used in the eld of pulmonary hypertension. Pulmonary hypertension is dened: Mean PA pressure by right heart catheterization ≥25 mm Hg. PAH is dened: Mean PA pressure by right heart catheterization ≥25 mm Hg and a pulmonary capillary wedge pressure ≤15 mm Hg and pulmonary vascular resistance ≥3 Wood units Table 3 is continued in the next page. For abbreviations please refer to page 7. Table 3 6 GUIDELINES MADE SIMPLE Back to Table of Contents ACHD 2018 Guideline for the Management of Adults with Congenital Heart Disease Physiological Variables as Used in ACHD AP Classification (4 of 4) Variable Description Shunt An intracardiac shunt is hemodynamically signicant if: (hemo- There is evidence of chamber enlargement distal to the shunt dynamically significant • And/or evidence of sustained Qp:Qs ≥1.5:1 shunt) • An intracardiac shunt not meeting

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