ArticleEuropean heart journal. Case reports2025
Unrepaired most severe form of hypoplastic left heart syndrome in adulthood: a case report of late diagnosis and long-term follow-up.
Article in European heart journal. Case reports, 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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Abstract
Background: Hypoplastic left heart syndrome (HLHS) is a rare, severe congenital heart disease (CHD) characterized by the underdevelopment of the left-sided cardiac structures, typically necessitating neonatal surgical intervention. Survival beyond infancy without surgical palliation is exceptionally uncommon. Case summary: We report the case of an 17-year-old woman with a late diagnosis of unrepaired HLHS, comprising mitral and aortic atresia, a hypoplastic ascending aorta, and a rudimentary left ventricle. Cardiac imaging provided a comprehensive anatomical assessment. Remarkably, despite the absence of surgical intervention, the patient remained clinically stable over a 10-year follow-up under exclusive medical management. Discussion: This case illustrates extraordinary natural survival in unrepaired HLHS and emphasizes the importance of specialized CHD centres in long-term management, clinical surveillance, and imaging-based evaluation of complex univentricular physiology.
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