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(Circulation. 2008;117:e319-e321.)
© 2008 American Heart Association, Inc.

Images in Cardiovascular Medicine

Hypoplastic Left Heart Syndrome With Left Ventricular Myocardial Sinusoids: Echocardiographic and Angiographic Findings in the First Neonate Surviving the Norwood I and II Procedure

Harald Bertram, MD; Marc-Phillip Hitz, MD; Masamichi Ono, MD; Michael Sasse, MD; Armin Wessel, MD, PhD; Thomas Breymann, MD; T. Mesud Yelbuz, MD, PhD

From the Department of Pediatric Cardiology and Intensive Care Medicine (H.B., M.-P.H., M.S., A.W., T.M.Y.) and the Division of Thoracic and Cardiovascular Surgery (M.O., T.B.), Hannover Medical School, Hannover, Germany.

Correspondence to T. Mesud Yelbuz, MD, PhD, Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany. E-mail Yelbuz.Mesud@mh-hannover.de

An extract of the first 250 words of the full text is provided, because this article has no abstract.

Hypoplastic left heart syndrome (HLHS) encompasses a broad spectrum of congenital cardiac anomalies. It is characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle, including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch.¹

A 25-year-old woman gave birth to her first child in the 40th week of gestation via caesarean delivery because of a pathological cardiotocogram. The neonate was transferred to the pediatric intensive care unit. Although the chest x-ray was unsuspicious on the first day of life (Figure 1A), the ECG exhibited ST elevation in left precordial leads indicating myocardial damage (Figure 1B), an unusual finding even in HLHS. Echocardiography confirmed the already prenatally diagnosed HLHS with aortic atresia and severe mitral hypoplasia (Figure 2A; online-only Data Supplement Movie I). The most interesting and critical finding was the presence of several marked myocardial sinusoids in the thickened left ventricular myocardium (Figure 2B and 2C; online-only Data Supplement Movies II and III). To clarify the anatomy, cardiac catheterization and angiography were performed. HLHS with aortic atresia and an extreme hypoplastic mitral valve with trivial incompetence and a small left ventricle with suprasystemic blood pressure were confirmed (Figure 3A; online-only Data Supplement Movie IV). The defect within the restrictive atrial septum was dilated by using a 4-mm cutting balloon and subsequently a 6-mm high pressure balloon. Selective left ventricular angiography demonstrated mycocardial . . . [Full Text of this Article]