AI Article Synopsis

  • Hypoplastic left heart syndrome (HLHS) is a serious congenital heart defect marked by an underdeveloped left ventricle and a severely narrowed ascending aorta, which disrupts normal blood flow.
  • Key features include problems with the aortic and mitral valves, such as atresia or stenosis, and associated conditions like endocardial fibroelastosis.
  • Additional complications may involve the interatrial septum, anomalous pulmonary venous drainage, variations in systemic veins, and often a coarctation of the aorta.

Article Abstract

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect characterized by several abnormalities that result in a significantly underdeveloped left ventricle and severe hypoplasia of the ascending aorta, often leading to retrograde perfusion. These abnormalities include aortic valve atresia or severe stenosis, accompanied by a severely hypoplastic aortic valve annulus (Fig. 59.1). Mitral valve atresia, hypoplasia, and/or stenosis with a hypoplastic valve annulus with or without a ventricular septal defect can also contribute to the development of HLHS. Endocardial fibroelastosis and sinusoids may be present as well. The interatrial septum can either be closed or the foramen ovale severely stenotic. Other malformations, such as anomalous pulmonary venous drainage or variations of the systemic veins, may coexist. It is also common to observe a coarctation of the aorta in these cases.

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http://dx.doi.org/10.1007/978-3-031-44087-8_59DOI Listing

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