Heart block following stage 1 palliation of hypoplastic left heart syndrome

Douglas Y Mah1, Henry Cheng1, Mark E Alexander1

  • 1Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, Mass.

Insights

Heart block after single ventricle palliation is uncommon but significantly increases the risk of death or heart transplantation in hypoplastic left heart syndrome patients. Close monitoring is crucial for these high-risk infants.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Hypoplastic left heart syndrome (HLHS) requires complex surgical palliation.
  • Stage 1 palliation (S1P) is the initial surgical step for HLHS.
  • Heart block (HB) is a potential complication following cardiac procedures.

Purpose of the Study:

  • To determine the prevalence, timing, and risk factors for heart block (HB) after S1P in HLHS patients.
  • To assess the impact of HB on outcomes, including transplantation-free survival.

Main Methods:

  • Analysis of publicly available data from the Pediatric Heart Network's Single Ventricle Reconstruction Trial.
  • Extraction of HB onset, risk factors, and outcome predictors within the first year post-S1P.
  • Multivariable logistic and Cox regression analyses to identify risk factors and survival impact.

Main Results:

  • 6% of 549 HLHS patients developed HB post-S1P, with a median onset of 8 days.
  • Tricuspid valve repair and pre-S1P pulmonary venous obstruction were associated with increased HB risk.
  • 3-year transplantation-free survival was significantly lower in patients with HB (39% vs 65%).

Conclusions:

  • Heart block following S1P in HLHS is rare but associated with significantly poorer outcomes.
  • Patients experiencing HB face increased risks of mortality and heart transplantation.
  • Close surveillance of these patients is recommended.
Abstract

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