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Diagnostic Assessment of Hypoplastic Left Heart Syndrome

Echocardiographic Imaging Before and After Staged Surgical Reconstruction

  • Chapter
Hypoplastic Left Heart Syndrome

Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 246))

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Abstract

The phrase “hypoplastic left heart syndrome” (HLHS) first coined by Lev in 1952 [1] and further defined by Noonan and Nadas in 1958 [2] describes a spectrum of abnormalities in which the left ventricle and other left sided structures are poorly formed. In this anomaly, the left ventricle is of inadequate size and cannot independently provide for a sufficient cardiac output that is life sustaining. The anatomical findings include: 1) hypoplasia of the left ventricle, 2) aortic atresia or severe stenosis/hypoplasia, and 3) mitral atresia or stenosis/hypoplasia. Hypoplasia of the ascending aorta is usually associated, unless there is a ventricular septal defect, in which case the aorta may be normal in size. Some have included patients with unbalanced common atrioventricular canal defect in which the atrioventricular valve is positioned predominantly over the right ventricle resulting in limitation to the left-sided inlet and a diminutive left ventricle [3–5]. The term excludes patients with aortic atresia and large ventricular septal defect who may have a normal mitral valve and a normal size left ventricle [6]. In addition, the term excludes patients with transposition of the great arteries that share similar physiology of “single ventricle” with obstruction to systemic outflow.

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  1. Division of Cardiology, The Children’s Hospital of Philadelphia, USA

    Jack Rychik M.D.

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  1. Jack Rychik M.D.
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  1. Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, USA

    Jack Rychik M.D.  & Gil Wernovsky M.D.  & 

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Rychik, J. (2003). Diagnostic Assessment of Hypoplastic Left Heart Syndrome. In: Rychik, J., Wernovsky, G. (eds) Hypoplastic Left Heart Syndrome. Developments in Cardiovascular Medicine, vol 246. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0253-1_4

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  • DOI: https://doi.org/10.1007/978-1-4615-0253-1_4

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-4988-4

  • Online ISBN: 978-1-4615-0253-1

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