Review
Cardiac resynchronization therapy in congenital heart disease

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Abstract

While cardiac resynchronization therapy (CRT) is of proven benefit in selected patients with severe ischemic or dilated cardiomyopathy, refractory symptoms, and conduction delay, extrapolation to congenital heart disease is not straightforward. This rapidly expanding patient population commonly suffers from heart failure, particularly in the presence of a single or systemic right ventricle. Surgical repair may also contribute to ventricular asynchrony.

In this systematic review, the current state of knowledge regarding CRT in congenital heart disease is presented. Issues specific to congenital heart disease including right bundle branch block, right (pulmonary) ventricular dysfunction, systemic right ventricular dysfunction, and single ventricle dysfunction are explored. Evidence-based CRT applications for each of these particular conditions are reviewed. Initial experience with CRT in the acute postoperative setting and longer-term, including our own, is elaborated.

Unlike standard indications based on multiple randomized clinical trials, supporting evidence for CRT in congenital heart disease is limited to case reports, case series, and small experimental crossover studies in the acute postoperative setting. The heterogeneous patient population, technical limitations from patient size, vascular access issues, and unique forms of ventricular asynchrony further obscure the selection of potential beneficiaries. Despite these limitations, experience thus far has been favorable. Quality of current data precludes definitive evidence-based recommendations, but optimistic initial results suggest that research endeavors in this field should be pursued. Multicenter prospective collaborative efforts are to be encouraged.

Introduction

Cardiac resynchronization therapy (CRT) is effective in selected patients with ischemic and dilated cardiomyopathy and symptomatic heart failure despite optimal medical therapy [1], [2], [3], [4], [5], [6]. Asynchrony, characterized by intraventricular and interventricular conduction delay, may compromise diastolic filling, increase mitral regurgitation, worsen left ventricular dilation, and impair cardiac output [7], [8], [9], [10], [11]. CRT may improve quality of life, New York Heart Association (NYHA) functional class, exercise capacity, and left ventricular systolic function [1], [2], [3], [4], [5], [6]. In patients with advanced heart failure and prolonged QRS duration, CRT has recently been shown to reduce all-cause mortality, particularly when combined with defibrillator capabilities [1], [6].

In congenital heart disease, cardiac dysfunction is a major cause of morbidity and mortality. Yet, issues specific to this population limit the applicability of results from large randomized clinical trials of CRT. For example, in contrast to adults with heart failure and left bundle branch block (LBBB), right ventricular conduction delay and right bundle branch block (RBBB) are more common in congenital heart disease. The morphologic right ventricle may be systemically positioned and more prone to dysfunction. Moreover, only one functional yet failing ventricle may be present, precluding biventricular pacing but not CRT by multisite pacing.

In order to assess the current state of knowledge regarding CRT in congenital heart disease, a systematic review was performed.

Section snippets

Methodology

The MEDLINE database from January 1985 to April 2005 was systematically searched using the following query terms: resynchronization, biventricular pacing, or multisite pacing, combined with congenital heart, tetralogy of Fallot, single ventricle, or systemic right ventricle. The search was not limited to a particular language of publication. This strategy was supplemented by manual retrieval of secondary sources including references from primary articles. All manuscripts addressing CRT in

Literature search

The electronic literature search revealed 10 manuscripts. Searching secondary sources identified 2 additional publications. All manuscripts were case reports, case series, or small experimental crossover studies in the acute postoperative setting. Quantitative pooled estimates were not derived, given the paucity of experimental or observational studies with intra-study comparisons and heterogeneity in study populations, definitions, and outcomes assessment. Analyses were, therefore, limited to

Institutional experience

At our pediatric hospital, CRT was performed in 13 patients with congenital heart disease, age 7.8 ± 5.7 years (range 0.8 to 15.5 years), between May 2002 and December 2004. Indications for CRT were impaired systemic ventricular function in combination with documented conduction abnormality. Data regarding type of conduction abnormality, morphologic systemic ventricle, underlying congenital heart disease, and whether dual chamber pacemakers were upgraded to CRT and/or implanted concomitant with

Candidates for CRT

Although large randomized clinical trials of CRT in ischemic and non-ischemic cardiomyopathy are not uniform in design or results, well-suited candidates may be recommended on the basis of clinical criteria. In a recent report from an expert panel, CRT was deemed indicated in patients with underlying sinus rhythm, left ventricular ejection fraction ≤ 35%, QRS duration ≥ 120 ms, and NYHA class III or IV symptoms despite optimal medical therapy [55]. Two studies demonstrated functional improvement

Conclusion

Unlike standard CRT indications based on multiple randomized clinical trials, evidence supporting this technology in patients with congenital heart disease is limited to case reports, case series, and small experimental crossover studies in the acute postoperative setting. The heterogeneous patient population, technical limitations from patient size, vascular access issues, and unique forms of ventricular asynchrony encountered further obscure the selection of potential beneficiaries. Moreover,

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    This work was supported in part by a Canada Research Chair in Electrophysiology and Adult Congenital Heart Disease (PK).

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