Isolated left ventricular apical hypoplasia with myocardial non-compaction: a case report

European Heart Journal. Case Reports
Viktoria I SkidanGalina P Nartsissova

Abstract

Isolated left ventricular apical hypoplasia (ILVAH) is a rare congenital cardiac abnormality, which might result in severe symptomatic heart failure (HF) with pulmonary hypertension, atrial fibrillation (AF), or malignant ventricular tachycardia in adults. A 32-years-old man presented with exertional dyspnoea New York Heart Association Class II and persistent AF. Echocardiography and cardiac magnetic resonance showed the presence of (i) spherical remodelling of the left ventricle (LV) with impaired contractile function (three-dimensional ejection fraction, EF 32%); (ii) substitution of apical myocardium by fatty tissue; (iii) abnormal origin of a papillary muscle network; and (iv) an elongated right ventricle, compatible with ILVAH. In addition, non-compacted endomyocardial layer of the LV was observed. Because of a high risk of sudden cardiac death in symptomatic HF patients with reduced EF, an implantable cardioverter-defibrillator was placed which followed by pulmonary vein isolation. After the procedures and restoration of sinus rhythm, the patient demonstrated improvement in HF symptoms and exercise tolerance. This was accompanied by an enhancement of left and right ventricular systolic function by echocardiography. At 6-m...Continue Reading

References

May 16, 2007·Pediatric Radiology·Carlos MarinJuan I Zabala
Nov 23, 2013·Korean Journal of Radiology : Official Journal of the Korean Radiological Society·Jin Il MoonJi Won Lee
Aug 28, 2014·European Heart Journal·Christopher Orsborne, Matthias Schmitt
Sep 30, 2015·Annals of Noninvasive Electrocardiology : the Official Journal of the International Society for Holter and Noninvasive Electrocardiology, Inc·Yonghui ZhaoJing Zhang
Jan 23, 2016·Korean Journal of Radiology : Official Journal of the Korean Radiological Society·Sun Hwa HongHyun Jong Lee

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