Work

Unrepaired most severe form of hypoplastic left heart syndrome in adulthood: a case report of late diagnosis and long-term follow-up

Year: 2025

Type: article

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.

Source: European Heart Journal - Case Reports

DOI: 10.1093/ehjcr/ytaf621

Authors: José Martín Alanís-Naranjo, Stephanie Teresa Angulo-Cruzado, Edgar García-Cruz, Sergio Alfonso Patrón-Chi, Regina de la Mora-Cervantes

Institution: Instituto Nacional de Cardiología

Language: English

Topic: Congenital Heart Disease Studies

Subfield: Epidemiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $815