Key result
The Norwood operation has improved 5-year survival in hypoplastic left heart syndrome to up to 65%, though adult survivors face significant long-term complications requiring specialized surveillance.
Why the study?
Post-operative morbidity and mortality remain high in HLHS, and a growing population of adult survivors with Fontan circulation presents challenging long-term complications requiring specialized ACHD care.
Highlights the need for specialized adult congenital heart disease care and vigilant monitoring for the growing population of HLHS survivors reaching adulthood.
Adult HLHS survivors face persistent challenges; leaves open optimal long-term management strategies.
Hypoplastic left heart syndrome (HLHS) is one of the most complex congenital heart defects (CHD), characterized by a hypoplastic left ventricle (LV), dominant right ventricle (RV) and small left-sided heart structures. The introduction of the Norwood operation has significantly improved outcomes, with 5-year survival reported up to 65 %. Despite these advances, post-operative morbidity and mortality remain high, and the long-term complications in adult survivors represent a challenge. The number of HLHS patients with Fontan circulation is expected to double in the next 20 years, leading to a growing population requiring specialized care from adult congenital heart disease (ACHD) teams. This article reviews current management strategies for HLHS, outlines potential long-term complications, and highlights existing knowledge gaps. Specific considerations in this population include the assessment of the neo-aorta and aortic arch, and systemic RV dysfunction in the setting of a Fontan circulation. The proposed surveillance strategy emphasizes the need for vigilant monitoring and timely intervention to treat the complications unique to this population, ensuring better outcomes for HLHS patients reaching adulthood.
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Kačar et al. (2024) conducted a review in Hypoplastic left heart syndrome (HLHS). The Norwood operation has improved 5-year survival in hypoplastic left heart syndrome to up to 65%, though adult survivors face significant long-term complications requiring specialized surveillance.
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