Title: The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: Executive summary
Abstract: These guidelines have been produced to bring together current recommendations on the evaluation and management of pediatric heart failure (HF) and update the previous guideline.1Rosenthal D. Chrisant M.R.K. Edens E. et al.International Society for Heart and Lung Transplantation: practice guidelines for management of heart failure in children.J Heart Lung Transplant. 2004; 23: 1313-1333Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar The writing group was chosen from the membership of the International Society for Heart and Lung Transplantation (ISHLT), the Association of European Pediatric and Congenital Cardiology (AEPC), and the Pediatric and Congenital Electrophysiology Society (PACES) across health care disciplines to achieve representation of HF practice throughout the world. Overall, 90 contributors from 13 counties across 4 continents (Appendix 1) were assigned various aspects of HF according to their expertise. A comprehensive review of the available published evidence for HF management was undertaken. The strength and level of evidence was assessed according to standard practice.2U. S. Task Force StaffGuide to Clinical Preventive Services: report of the U.S. Preventive Services Task Force. Diane Publishing Co, Derby, PA1989: 24Google Scholar The recommendations were achieved by consensus with the contributors; however, it is recognized that the evidence base for many of the recommendations is Level C due to the lack of trials in children. In some areas, there is such a lack of information that no recommendations can be made: it is hoped that by recognizing these deficiencies, research will be stimulated to address them. External review was undertaken by 8 international experts invited from adult advanced HF, pediatric cardiology, and congenital cardiovascular surgery (Appendix 2). The final guidelines were reviewed and approved by the ISHLT Board and Standards & Guidelines Committee, endorsed by the AEPC, and those guidelines pertinent to electrophysiology, by the PACES Executive Committee. The background information for these guidelines and complete references have been published in the monograph series, Volume 8 by the ISHLT.3Richard Kirk Anne I Dipchand D.N.R. ISHLT guidelines for the management of pediatric heart failure. University of Alabama at Birmingham, Birmingham, AL2014Google Scholar The abbreviations used are listed in Appendix 3. Chairs: Richard Kirk, Anne I. Dipchand, and David N. Rosenthal HF in children is a clinical and pathophysiologic syndrome that results from ventricular dysfunction, volume, or pressure overload, alone or in combination. It leads to characteristic signs and symptoms, such as poor growth, feeding difficulties, respiratory distress, exercise intolerance, and fatigue, and is associated with circulatory, neurohormonal, and molecular abnormalities. HF has numerous etiologies that are a consequence of cardiac and non-cardiac disorders, either congenital or acquired. Class I1.The documentation of HF severity and when appropriate, staging facilitates monitoring of disease progression and patient management (Table 1): Level of Evidence CTable 1Heart Failure Severity ClassificationsClassNYHARossINo limitations of physical activityNo limitations or symptomsIIMay experience fatigue, palpitations, dyspnea, or angina during moderate exercise but not during restMild tachypnea or diaphoresis with feedingIIISymptoms with minimal exertion that interfere with normal daily activityInfants with growth failure and marked tachypnea or diaphoresis with feedings, older children with marked dyspnea on exertionIVUnable to carry out any physical activity because they typically have symptoms of HF at rest that worsens with any exertionSymptoms at rest such as tachypnea, retractions, grunting, or diaphoresisExpansions for the abbreviations used in Table 1 are provided in Appendix 3. Open table in a new tab Expansions for the abbreviations used in Table 1 are provided in Appendix 3. Chairs: Richard Kirk and Jeffery Towbin Class I1.Comprehensive or targeted DCM genetic testing (LMNA and SCN5) is recommended for patients with DCM and significant cardiac conduction disease (i.e., first-, second-, or third-degree heart block) and/or a family history of premature unexpected sudden death. Level of Evidence C2.Mutation-specific genetic testing is recommended for first-degree family members after the identification of a DCM-causative mutation in the index case. Level of Evidence C Class IIa1.Genetic testing can be useful for patients with familial DCM to confirm the diagnosis, to recognize those who are at highest risk of arrhythmia and syndromic features, to facilitate cascade screening within the family, and to help with family planning. Level of Evidence C Class IIa1.Genetic testing is indicated in the most clinically affected individual to facilitate screening. Level of Evidence A2.Mutation-specific genetic testing is recommended for first-degree family members after the identification of an HCM-causative mutation in the index case. Level of Evidence A Class I1.Mutation-specific genetic testing is recommended for first-degree family members after the identification of an RCM-causative mutation in the index case. Level of Evidence C Class IIb1.RCM genetic testing may be considered for patients in whom a cardiologist has established a clinical index of suspicion for RCM based on examination of the patient’s clinical history, family history, and ECG/echocardiographic phenotype. Level of Evidence C Class I1.Mutation-specific genetic testing is recommended for first-degree family members after the identification of an LVNC-causative mutation in the index case. Level of Evidence C Class IIa1.LVNC genetic testing can be useful for patients in whom a cardiologist has established a clinical diagnosis of LVNC based on an examination of the patient’s clinical history, family history, and ECG/echocardiographic phenotype. Level of Evidence C Class I1.Mutation-specific genetic testing is recommended for first-degree family members after the identification of the AVC-causative mutation in an index case. Level of Evidence C Class IIa1.Comprehensive or targeted AVC genetic testing (DSC2, DSG2, DSP, JUP, PKP2, and TMEM43) can be useful for patients who satisfy task force diagnostic criteria for AVC. Level of Evidence C Chairs: Anne I. Dipchand, Michael Burch, and Luc Mertens Class IIb1.BNP/NT-proBNP can be used as an adjunctive marker in the integrated evaluation and monitoring of patients with known HF to further define severity, response to therapy, and its progression. Level of Evidence B2.BNP/NT-proBNP can be used as an adjunctive marker, not a stand-alone test, to aid in the diagnosis of new HF in symptomatic patients. Level of Evidence B Table 2 compares the imaging modalities.Table 2Comparison of Imaging ModalitiesModalityAdvantagesLimitationsUse in pediatric HFEchocardiographyFirst-line technique for all patients in acute/chronic HF; anatomic + functional assessment possibleLimited by acoustic windowsFirst-line technique in the assessment of HFM-modeHigh temporal resolutionOnly works if global remodeling/dysfunctionSerial measurement of LV dimensions, wall thickness, and fractional shorteningNormal pediatric values availableNot valid if paradoxical septal motion2-D echocardiographyAssessment of 2-D anatomy, identification of structural diseaseDependent on acoustic windowsIdentification of structural disease as cause for HF2-D EFGood parameter for global performanceGeometrical assumptionsRecommended technique for assessment of LV performanceCan be calculated in patients with regional dysfunctionLoad dependency3-D EFNo geometric assumptionsHighly dependent on acoustic windowsEmergent technique replacing 2-D EFLower temporal resolutionBlood Doppler techniques (MPI, dP/dt, S/D ratio)Geometry-independentVariability of measuring time intervalsLimited use in heart failure patientsHigh temporal resolutionNo spatial informationLoad dependency of different indicesTDIGeometry-independentAngle-dependentAssessment of longitudinal function, early detection of dysfunction, assessment of diastolic functionHigh temporal resolutionLimited information on its use in childrenDyssynchrony detectionQuantification of regional functionLoad dependencyStrain imagingGeometry-independentSoftware dependency and differences between vendorsEmergent techniqueImages myocardial mechanicsLoad dependencyDyssynchrony assessmentLimited normal dataCardiac MRINot limited by imaging windows, no radiation, good for imaging extracardiac structures, flow quantification, tissue characterization (fibrosis imaging)Accessibility, requirement for general anesthesia, cost, not compatible with many devices (pacemaker, assist devices)Limited use in HF patients; quantification of RV function, patients with limited acoustic windows, fibrosis detectionSteady-state free precession imagingClinical reference technique for quantification of LV and RV volumes + massLower temporal resolution (ECG-gated)Mainly calculation of volumes and EFPhase contrastReference technique for quantifying flowsLower temporal resolution than Doppler techniquesCalculation of CO, quantification of valve regurgitationMR taggingAllows quantifying myocardial mechanicsDifficult post-processing; lower frames rates compared with echocardiographyLimited clinical use in heart failure patients. Research toolLate gadolinium enhancementIdentification of regional fibrosisLimited spatial resolution, only detects regional fibrosisClinical use still uncertain; possible prognostic value in HCM, detection of EFE in DCM or obstructive lesionsAngiographyDetection of extracardiac abnormalitiesSpatial resolution is less good than cardiac CT (coronary imaging)Can be used to detect extracardiac abnormalities causing heart failure (arch, coronary anomalies)Cardiac CTNot limited by imaging windows; high resolution, allows studying spatial relationships between cardiovascular structures and the airway, anesthesia often not requiredExposure to radiation, limited functional information, no flow quantification, coronary imaging influenced by high heart ratesMainly used for coronary artery imaging if uncertain about coronary artery origins on echocardiography; limited role in follow-upExpansions for the abbreviations used in Table 2 are provided in Appendix 3. Open table in a new tab Expansions for the abbreviations used in Table 2 are provided in Appendix 3. Class I1.Measurement of LV dimensions and LV wall thickness is an essential part of every echocardiographic functional LV assessment in patients with HF. The recent American Society of Echocardiography pediatric recommendations propose the use 2-D imaging instead of M-mode, but this was not based on actual data proving its clinical superiority. Normal values are more readily available for M-mode measurements. Level of Evidence B2.LV remodeling should be monitored during serial follow-up. This consists of measuring cavity dimensions, wall thickness, and where clinically relevant, LV mass. Level of Evidence B3.For patients with HF, assessing LV function by calculating LVEF based on a well-standardized 2-D method (biplane Simpson’s or area-length method) should be undertaken. Level of Evidence B Class IIa1.Fractional shortening can be used for sequential assessment of LV function, although caution is required in patients with abnormal or paradoxical septal motion. Level of Evidence C2.No recommendations can be made about the use of automated 2-D methods or 3-D echocardiography. These are emerging techniques for EF calculation that still need further validation in pediatric HF. Level of Evidence C Class IIb1.LVEF is load-dependent. The use of methods correcting for afterload have been proposed that do not improve diagnostic accuracy and predictive value. Level of Evidence C2.Although measured and reported, the value of blood pool Doppler parameters, such as dP/dt, myocardial performance index, and the ratio of systolic-to-diastolic duration, in the assessment of LV systolic function in pediatric patients with HF is probably limited. (Problems with reliability of measuring time intervals, loading, and heart rate dependency of the methods, and their uncertain prognostic value, all limit their clinical use.) Level of Evidence C3.Although measured and reported, the role of tissue Doppler imaging (TDI) and strain imaging in pediatric patients with HF is still uncertain. Pulsed TDI at the annulus has the benefit of published normal ranges and may assist in the early detection of myocardial dysfunction. Apart from the potential additional predictive information, its use in the description of mechanical dyssynchrony and identification of candidates for CRT seems to be important but requires further investigation. Level of Evidence C Class IIa1.Diastolic function, including mitral inflow patterns, mitral annular TDI, and pulmonary venous Doppler flow patterns should be assessed by echocardiography in children with HF. The tracings should be interpreted to define the type of diastolic abnormality (relaxation abnormality, reduced compliance, restrictive filling) as well as to attempt to diagnose the presence of elevated filling pressure (Table 3 summarizes typical changes in progressive LV diastolic dysfunction). Level of Evidence CTable 3Summary of Typical Changes in Progressive Left Ventricular Diastolic DysfunctionVariableEarly relaxation abnormalityProgressive decreasing complianceRestrictive fillingMitral e′↓Persistent ↓↓↓Mitral E velocity↓↑↑Mitral A velocity↑↔↓Mitral E/A ratio↓Pseudonormalizes↑Deceleration time↑Progressive ↓↓↓Pulmonary vein D↓↑Pulmonary vein S↑↓↓↓OtherAbnormal pulmonary vein S/D ratio; increased pulmonary vein A wave reversalProminent A reversal in pulmonary veinsD, diastole; S, systole. Expansions for the other abbreviations used in Table 3 are provided in Appendix 3. Open table in a new tab D, diastole; S, systole. Expansions for the other abbreviations used in Table 3 are provided in Appendix 3. Class IIb1.Cardiac MRI can be used to assess LV function in children. Use can be limited due to the requirement for general anesthesia in younger children, the presence of arrhythmia, and the availability and cost associated with cardiac MRI. Level of Evidence C2.Balanced steady-state-free precession cine analysis has become the reference standard for volumetric and myocardial mass assessment of the LV. Where feasible, it should be used to assess children with HF. However, accuracy depends on standardization of analysis protocol and acquisition of adequate temporal and spatial resolution specific to the patient’s heart rate and size. Level of Evidence C Class IIa1.For the quantitative assessment of RV function by 2-D echocardiography, it is reasonable to use fractional area of change from the apical 4-chamber view together with tricuspid annular planar systolic excursion. Level of Evidence C2.TDI assessment of tricuspid annular motion is a useful technique for the assessment of RV longitudinal function and should be added in the quantitative assessment of RV function. Level of Evidence C Class IIb1.Due to methodologic variability and the load-dependency, the clinical use of RV myocardial performance index in the assessment of right HF is limited. Level of Evidence C2.RV strain analysis of RV longitudinal deformation is an emerging technique and requires further validation before routine use for the assessment of RV function. Level of Evidence C Class IIa1.Assessment of RV size and EF by MRI is considered the clinical reference for the assessment of RV function in patients with RV failure. Restricted access, the need for general anesthesia or sedation in infants and young children, and costs remain important limitations. Level of Evidence C Class IIb1.Doppler echocardiography can be used for the assessment of RV diastolic function, although criteria for grading and assessment of filling pressures are still poorly validated. Level of Evidence C2.Different MRI techniques are available for studying RV diastolic dysfunction, but their clinical utility still needs to be evaluated. Level of Evidence C Class IIb1.Quantitative techniques, such as fractional area of change, EF, annular excursion, and TDI for serial follow-up may be used, but good data regarding the prognostic value of observed changes are not available. Level of Evidence C Class IIa1.Cardiac MRI is the reference standard for volumetric analysis of the single ventricle. Larger studies are required to define acceptable ranges of end-diastolic or end-systolic volume in these patients. Level of Evidence B Class IIa1.Metabolic exercise testing with a measurement of peak Vo2 consumption2, if feasible, should be part of the assessment of cardiomyopathy patients with HF. Level of Evidence C2.A peak Vo2 consumption of < 50% predicted for age and sex in patients with Stage C HF associated with severe limitation in exercise and activity may form the basis for consideration of heart transplantation. Level of Evidence C Class IIa1.Children with HF and a history consistent with sleep apnea should undergo evaluation for sleep disorder breathing. Level of Evidence C Class I1.In a pediatric HF patient who presents with palpitations or syncope, some form of ambulatory monitoring should be considered to achieve a specific diagnosis and drive further management decisions. Less frequent symptoms may require use of longer-term event monitors. Level of Evidence C Class IIa1.In pediatric HF patients with a high risk of developing atrial or ventricular arrhythmias or heart block, regular ambulatory monitoring should be considered. This would include patients after a Fontan palliation, any form of atrial switch procedure, heterotaxy (isomeric) syndromes, congenitally corrected transposition of the great arteries, or cardiomyopathy (HCM, DCM, RCM, and LVNC). Level of Evidence C2.In asymptomatic pediatric HF patients, there are no data to support the timing and frequency of regular ambulatory monitoring for arrhythmias; however, intermittent screening for asymptomatic arrhythmias should be considered. Level of Evidence C Class IIa1.EMB should not be performed in clinically diagnosed myocarditis with minimal symptoms and mild dysfunction or rapid normalization of function. Level of Evidence C2.It is reasonable for cardiac catheterization to be performed if increased pulmonary resistance is suspected to measure the PVR and reversibility of pulmonary hypertension in patients with CHD and HF. Level of Evidence B Class IIb1.Cardiac catheterization and EMB can be considered for the assessment of unexplained cardiomyopathy or myocarditis if non-invasive testing does not yield a diagnosis. Level of Evidence C2.Cardiac catheterization and EMB is reasonable in the setting of suspected AVC. Level of Evidence C3.Cardiac catheterization and EMB is reasonable in pediatric patients presenting with HF when a specific diagnosis is suspected that would influence therapy. Level of Evidence C4.Cardiac catheterization may be considered to determine PVR and reversibility after medical therapy has been optimized in patients with cardiomyopathy if they are stable enough to undergo testing, but the necessity is controversial outside of consideration for transplantation. Level of Evidence C5.It is reasonable for EMB to be performed in the setting of unexplained new-onset HF associated with hemodynamic compromise, ventricular arrhythmia, or heart block where there is failure to respond to medical therapy. Level of Evidence B Class IIa1.Doppler-derived estimations of right heart pressures (right ventricular systolic pressure and pulmonary artery pressures) can be obtained for assessment and longitudinal follow-up of HF patients to monitor response to treatment, progression of disease, and contribute to decision making about more invasive assessment of PVR for the purpose of decision-making around medical and/or surgical interventions including transplantation. Level of Evidence C2.It is reasonable for cardiac catheterization to be performed to assess PVR and reversibility in patients with CHD and HF. Level of Evidence B Class IIb1.The role of MRI in the assessment of PVR in children with HF requires further study. Level of Evidence C Class IIa1.Electrophysiology testing or long-term monitoring can be useful in pediatric patients with presyncope or syncope with at least moderately impaired LV function. Level of Evidence C2.Tachycardia-induced cardiomyopathy should be considered in any patient presenting with DCM because it is potentially reversible with appropriate intervention. Level of Evidence B Class IIb1.Programmed ventricular stimulation may be helpful in specific situations, such as in patients with HF and syncope, but does not appear to have a routine role in risk stratification in the pediatric patient with HF. Level of Evidence C Chairs: David N. Rosenthal and Robert Shaddy Class I1.Patients with fluid retention associated with ventricular dysfunction (HF Stage C) should be treated with diuretics to achieve a euvolemic state. Level of Evidence C Class11.For the treatment of symptomatic left ventricular dysfunction (HF Stage C), ACE inhibitors should be routinely used unless there is a specific contraindication. These medications should be started at low doses and should be up-titrated to a maximum tolerated safe dose. Level of Evidence B Class IIa1.For the treatment of asymptomatic left ventricular dysfunction (HF Stage B), ACE inhibitors should be routinely used unless there is a specific contraindication. Level of Evidence B2.ACE inhibitor therapy should be considered for individuals with a diagnosis of Duchenne muscular dystrophy unless there is a specific contraindication, although the optimal age of institution of therapy is unclear. Level of Evidence B Class IIb1.ACE inhibitor therapy should not be routinely instituted for all patients with single-ventricle CHD, but could be considered in specific cases such as in situations of valve regurgitation or ventricular dysfunction. Level of Evidence B Class IIa1.Following adult HF guidelines, it is reasonable to consider β-blockers in symptomatic children with systemic LV systolic dysfunction, particularly if the systemic ventricle has a LV morphology. Therapy should start at a small dose and slowly up-titrate. Level of Evidence B2.Following adult HF guidelines, it is reasonable to consider β-blockers in asymptomatic children with systemic LV systolic dysfunction. Therapy should start at a small dose and slowly up-titrate. Level of Evidence B Class I1.Following adult HF guidelines, it is reasonable to consider aldosterone antagonists in children with systemic LV dysfunction. Level of Evidence C Class IIa1.Similar to adults, angiotensin receptor blockers are generally reserved for those children with systemic ventricular systolic dysfunction who would benefit from renin-angiotensin-aldosterone– system blockade but are intolerant of ACE inhibitors. Level of Evidence C Class I1.Digoxin is not recommended for children with asymptomatic LV dysfunction because no survival benefit was seen with digoxin in adults with HF and low EF. Level of Evidence C Class IIa1.Digoxin may be used to relieve symptoms in children with symptomatic HF and low EF. Doses targeting lower serum digoxin concentrations (e.g., 0.5–0.9 ng/ml) should be considered with attention to dose reductions in patients on carvedilol and amiodarone or those who have or are at risk for renal dysfunction. Level of Evidence C Class III1.The use of combination therapy of hydralazine and isosorbide dinitrate is not recommended in pediatric HF. Level of Evidence C Class IIb1.The use of anti-arrhythmic medications may be warranted in select cases where arrhythmias persist after normalization of electrolyte disturbances or metabolic issues (i.e., hyperthyroidism) and the arrhythmias are poorly tolerated. Level of Evidence C Class III1.Anti-arrhythmic medications should not be used routinely in the management of children with HF with low EF. Level of Evidence C Class III1.Treatment of HF with statin therapy is not indicated in pediatric HF patients. Level of Evidence C Class III1.Direct renin inhibitors cannot be recommended for the treatment of HF in children. Level of Evidence C Class I1.Patients with intracardiac thrombus should receive anticoagulation with heparin or warfarin. Level of Evidence B Class IIa1.For patients with a history of thrombus or a thromboembolic event who have an EF < 25% (fractional shortening < 15%), anti-coagulation with heparin or warfarin should be considered. Level of Evidence C2.Extrapolating from the strong data in the adult HF population, children with low EF and persistent or uncontrolled paroxysmal atrial fibrillation or flutter should receive anti-coagulation with heparin or warfarin. Level of Evidence C Class III1.No recommendation can be made regarding the use of anti-coagulation or anti-platelet therapy in patients with reduced EF and no history of thrombus or thromboembolic event, because there is insufficient evidence to justify a recommendation. Level of Evidence C Class IIb1.The use of nesiritide cannot be recommended for routine use in acute HF in children, although it may be considered in select situations where other interventions to lower central venous pressure have been unsuccessful. Level of Evidence C Class IIa1.Inotropic therapy may be considered for symptomatic relief in the palliative setting. Level of Evidence C Class III1.On the basis of a lack of any pediatric data and lack of data supporting improved outcomes in adults, use of intermittent or chronic inotropic therapy, other than as a bridge to transplant, is not recommended. Level of Evidence C Class III1.Vasopressin receptor antagonists cannot be recommended for the routine treatment of chronic HF in children. Level of Evidence C Chairs: David N Rosenthal and Robert Weintraub Class I1.Use of diuretics to establish a clinically euvolemic state is recommended for children with HFpEF. Level of Evidence C2.In patients with HFpEF, close monitoring of renal function and blood pressure should be performed during initiation and up-titration of diuretic therapy. Level of Evidence C Class IIa1.Treatment of systemic hypertension in patients with HFpEF is recommended to prevent disease progression. Although no particular class of medication is favored, diuretics may be considered for this purpose. Level of Evidence C Class IIb1.Routine use of renin-angiotensin antagonists is not recommended in HFpEF, unless there is an additional indication for use of these classes of medications such as hypertension. Level of Evidence C2.Renin-angiotensin antagonists may be used for control of hypertension in HFpEF, but careful monitoring of hemodynamics and renal function is indicated due to the enhanced risk of hypotension and renal toxicity. Level of Evidence C Class III1.Use of calcium channel blockers is not recommended for treatment of HFpEF in children, unless there is an additional indication. Level of Evidence C Class IIb1.In children with HFpEF, aldosterone blockade with either spironolactone or eplerenone is not recommended. Level of Evidence C Class IIb1.Use of phosphodiesterase inhibitors is not recommended for treatment of HFpEF in children, unless there is an additional indication for use of these classes of medications such as pulmonary hypertension. Level of Evidence C Class III1.Use of digoxin is not recommended for treatment of HFpEF in children, unless there is an additional indication such as arrhythmia requiring atrial rate control. Level of Evidence C Class III1.Intravenous β-agonists, such as dopamine, dobutamine, and epinephrine, are not indicated for treatment of HFpEF. Level of Evidence C Class III1.The use of prostaglandins and endothelin receptor antagonists to treat secondary pulmonary hypertension in children with HFpEF is not supported by current evidence. Level of Evidence C Chairs: David N. Rosenthal and Anne M. Dubin Class I1.Permanent pacemaker implantation is recommended for advanced second- or third-degree atrioventricular block associated with ventricular dysfunction. Level of Evidence B Class IIa1.LV apical pacing can be useful in epicardial ventricular pacing systems. Technical considerations may require alternate ventricular lead placement. Level of Evidence B Class IIa1.CRT can be useful for pediatric patients with a systemic LV with an EF < 35%, complete left bundle branch block pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-IV on GDMT. Level of Evidence B Class IIb1.CRT may be considered for pediatric patients with a systemic RV, with an EF < 35%, complete right bundle branch block pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-IV on GDMT. Level of Evidence C2.CRT may be considered for pediatric patients with a single ventricle, with an EF < 35%, complete bundle branch pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-V on GDMT. Level of Evidence C Class I1.ICD implantation is recommended in the pediatric survivor of cardiac arrest after evaluation to define the cause of the event and to exclude any reversible/treatable causes. Level of Evidence B Class IIa1.ICD implantation can be useful in the pediatric patient with unexplained syncope and at least moderate LV dysfunction and DCM. Level of Evidence C2.ICD implantation can be useful for adolescent patients with HCM who have 1 or more major risk factors for SCD. In younger patients, the risk/benefit ratio must be considered due to technical considerations. Level of Evidence C3.ICD implantation can be useful for the prevention of SCD in adolescent patients with AVC who have 1 or more risk factors for SCD. In younger patients, the risk/b