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Published on: July 18, 2014
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.
Objectives:
Publicly available data from the Pediatric Heart Network's Single Ventricle Reconstruction Trial was analyzed to determine the prevalence, timing, risk factors for, and impact of second- and third-degree heart block (HB) on outcomes in patients who underwent stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS).
Methods:
The presence and date of onset of post-S1P HB occurring within the first year of life, potential risk factors for HB, and factors known to predict poor outcomes after S1P were extracted. Multivariable logistic and Cox regression analyses were performed to identify risk factors for HB and to determine the effect of HB on 3-year transplantation-free survival.
Results:
Among the 549 patients in the cohort, 33 (6%) developed HB after S1P. The median interval between S1P and HB was 8 days (interquartile range, 0-133 days). Regression analysis showed that tricuspid valve repair during S1P and obstruction of pulmonary venous drainage requiring pre-S1P intervention were independently associated with HB (adjusted odds ratio [aOR], 11.6, 95% confidence interval [CI] 3.3-40; P < .001 and aOR, 5.1; 95% CI, 1.3-20.6; P = .02, respectively). Transplantation-free survival at 3 years was lower for those with HB (39% vs 65%; P = .004). HB remained associated with transplantation-free survival after controlling for known risk factors (adjusted hazard ratio, 3.1; 95% CI, 1.9-5.0; P < .001). Nine children (27%) had a pacemaker implanted, and 7 of these children (78%) died or underwent heart transplantation.
Conclusions:
HB after S1P is rare but heralds a poor outcome. Careful monitoring of these patients is recommended given their significantly increased risks of death and heart transplantation.
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