Cardiac resynchronisation therapy in paediatric and congenital heart disease: differential effects in various anatomical and functional substrates

Abstract

Background:Cardiac resynchronisation therapy (CRT) is increasingly used in children in a variety of anatomical and pathophysiological conditions, but published data are scarce.Objective:To record current practice and results of CRT in paediatric and congenital heart disease.Design:Retrospective multicentre European survey.Setting:Paediatric cardiology and cardiac surgery centres.Patients:One hundred and nine patients aged 0.24–73.8 (median 16.9) years with structural congenital heart disease (n = 87), congenital atrioventricular block (n = 12) and dilated cardiomyopathy (n = 10) with systemic left (n = 69), right (n = 36) or single (n = 4) ventricular dysfunction and ventricular dyssynchrony during sinus rhythm (n = 25) or associated with pacing (n = 84).Interventions:CRT for a median period of 7.5 months (concurrent cardiac surgery in 16/109).Main outcome measures:Functional improvement and echocardiographic change in systemic ventricular function.Results:The z score of the systemic ventricular end-diastolic dimension decreased by median 1.1 (p<0.001). Ejection fraction (EF) or fractional area of change increased by a mean (SD) of 11.5 (14.3)% (p<0.001) and New York Heart Association (NYHA) class improved by median 1.0 grade (p<0.001). Non-response to CRT (18.5%) was multivariably predicted by the presence of primary dilated cardiomyopathy (p = 0.002) and poor NYHA class (p = 0.003). Presence of a systemic left ventricle was the strongest multivariable predictor of improvement in EF/fractional area of change (p<0.001). Results were independent of the number of patients treated in each contributing centre.Conclusion:Heart failure associated with ventricular pacing is the largest indication for CRT in paediatric and congenital heart disease. CRT efficacy varies widely with the underlying anatomical and pathophysiological substrate.

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

“…Terapia resynchronizująca stała się ważnym uzupełnie-niem leczenia HF u pacjentów pediatrycznych, najczęściej wtedy, gdy występuje również wskazanie do stymulacji chroniącej przed bradykardią [523,524]. U wybranych pacjentów korzystne może być zastosowanie CRT-D, zwłaszcza w populacji pacjentów z wrodzonymi wadami serca po naprawie chirurgicznej, ale dostępnych jest niewiele danych na ten temat.…”

Cardiac Electrical System Involvement in Alström Syndrome: Uncommon Causes of Dilated Cardiomyopathies

“…Ventricular resynchronization has been found to improve functional outcomes and survival in adults with cardiomyopathy, [23][24][25][26][27] and biventricular pacing has improved cardiac function in pediatric patients with cardiomyopathy. [28][29][30] Given that both patients described in this report had early documentation of left bundle branch block, larger cohorts of patients with Alström syndrome should be evaluated with regard to conduction system involvement and possible response rates to cardiac resynchronization therapy. It has been our practice to follow published indications for both ICD and/or biventricular resynchronization therapy based on cardiac dysfunction and QRS duration.…”

Cardiac Conduction System in Congenitally Corrected Transposition of the Great Arteries and Its Clinical Relevance

“…In a multicenter study by the Working Group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Pediatric Cardiology, CRT was assessed in 109 pediatric and CHD patients including 36 patients with a systemic RV (ccTGA present in 20 patients). Reverse ventricular remodeling after CRT was significantly greater in patients with systemic left ventricle (LV) compared with those with systemic RV, and the degree of systemic AV valve regurgitation did not improve in subjects with a systemic RV after CRT 34. Rarely, BiV pacing may actually worsen heart failure symptoms and RVFAC.…”