20.500.12556/DiRROS-27800 Hypoplastic left heart syndrome (HLHS) becomes of age assessing the young adult with HLHS including the neoaorta/aortic arch 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. hypoplastic left heart syndrome Fontan circulation univentricular heart systemic right ventricle sindrom hipoplasničnega levega srca Fontanov obtok enoprekatni prekat sistemski desni prekat true false false Angleški jezik Slovenski jezik Neznano 2026-02-25 10:35:49 2026-02-25 10:35:49 2026-02-26 03:55:12 0000-00-00 00:00:00 2025 0 0 Nasl. z nasl. zaslona; Opis vira z dne 21. 11. 2025; str. 1-8 Vol. 19, [article no.] 100555 2025 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 0000-00-00 616.1 2666-6685 10.1016/j.ijcchd.2024.100555 258066691 RAZ_Kacar_Polona_2025.pdf RAZ_Kacar_Polona_2025.pdf 1 F59608A0BA69F3972B894CB773FF2F38 2e4a893ace726c99be3bb9db211b65325457374c8955308186d8b90c942230e3 9c248f59-122d-11f1-9cb0-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=40875 https://doi.org/10.1016/j.ijcchd.2024.100555 0 a99bf900-122d-11f1-9cb0-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=40876 Univerzitetni klinični center Ljubljana 0 0 0