Abstract

Introduction: Cardiac resynchronization therapy (CRT) is associated with an improvement in survival in selected pediatric and congenital heart disease (CHD) patients. However, there is minimal data to guide the identification of CRT responders in these challenging subgroups, even though implant is more complex and entails a longer lifetime legacy of device-related complications. Hypothesis: Echocardiographic measures of dyssynchrony predict response to CRT in this patient population Methods: This single-center study included all CRT pediatric and/or CHD patients (implant date 2004-2018). Response to CRT was defined as change in systemic ventricle ejection fraction (SVEF) on an ordinal scale (worsened/no change/improved). Multiple established echo indices (conventional and strain-derived) were assessed prior to implant. In addition, a novel ‘CPD-Score’, using an automated assessment of the presence of each of the four Classic Pattern Dyssynchrony (CPD) criteria on strain imaging (septal flash, lateral stretch, early peak septal strain, late peak lateral strain), was created. (Figure) Results: Of 127 CRT recipients, 91 met inclusion criteria (age 11yr (IQR 3-22yr), baseline SVEF 36±15%). In 62 (68%), SVEF improved following CRT (15 (16%) no change, 14 (15%) deteriorated). The presence of a septal flash was the best conventional echo predictor of response (PPV 78%). Conventional, strict, CPD also had high PPV for CRT response (72%) but was identified in only 9 (10%) patients. ‘CPD-Score’ offered a more graded response and higher PPV. A score of ≥3 was observed in 21 patients with PPV 96% (CPD-Score≥2 in 48 patients, PPV 83%). On stepwise multivariate ordinal regression, CPD-Score was independently associated with response (p= 0.02). Conclusion: CRT in pediatric and CHD patients is associated with an improvement in SVEF for the majority of recipients. Those with multiple features of CPD (‘CPD-Score’) are most likely to have an improvement in EF following CRT.

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