ArticleJTCVS open2025
The complex and hazardous course for heterotaxy-associated congenital heart disease.
Article in JTCVS open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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8 authors.
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Abstract
Objective: Patients with heterotaxy-associated congenital heart disease often require multiple operations, which may have a cumulative effect on their outcomes. This study aimed to define the cardiac surgical course in a large cohort and identify longitudinal risk factors for death/transplant. Methods: All patients with heterotaxy-associated congenital heart disease who underwent cardiac surgery at one institution from 2005 to 2022 were retrospectively reviewed. Demographics, anatomy, and operative course were summarized. Parametric risk hazard analysis for death/transplant was performed. Operations were analyzed as time-varying covariates. Results: Of 217 patients included, left isomerism occurred in 89 (41%), 139 (64%) were right ventricle dominant, and 43 (20%) had biventricular dominance. Total anomalous pulmonary venous return occurred in 100 patients (46%): 40 (40%) supracardiac, 34 (34%) intracardiac, 18 (18%) infracardiac, 7 (7%) mixed, and 19 (19%) obstructed. Ultimately, 102 patients (47%) reached the Fontan and 29 patients (13%) reached biventricular circulation. Transplant-free survivals at 1, 5, and 10 years were 79.4% ± 3%, 71.0% ± 3%, 63.9% ± 3%, respectively. Multivariable parametric analysis resolved 2 phases of risk for death/transplant. First-phase predictors included coarctation (parameter estimate 0.91 ± 0.55, Conclusions: Operative management of heterotaxy-associated congenital heart disease is high risk, especially when total anomalous pulmonary venous return repair with pulmonary blood flow control is required. Risk after infancy is primarily determined by need for the Fontan or atrioventricular valve reintervention.
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