Biventricular Noncompaction Associated With Left Ventricular Systolic and Diastolic Dysfunction and Severe Pulmonary Hypertension in a Young Man

Michal Ciurzynski; Barbara Lichodziejewska; Andrzej Tomaszewski; Dorota Piotrowska-Kownacka; Lukasz Kownacki; Zuzanna Rymarczyk; Andrzej Wysokinski; Piotr Pruszczyk

doi:10.1253/circj.CJ-08-0857

Case Reports

Biventricular Noncompaction Associated With Left Ventricular Systolic and Diastolic Dysfunction and Severe Pulmonary Hypertension in a Young Man

Michal Ciurzynski, Barbara Lichodziejewska, Andrzej Tomaszewski, Dorota Piotrowska-Kownacka, Lukasz Kownacki, Zuzanna Rymarczyk, Andrzej Wysokinski, Piotr Pruszczyk

Author information

Michal Ciurzynski
Department of Internal Medicine and Cardiology, Medical University of Warsaw
Barbara Lichodziejewska
Department of Internal Medicine and Cardiology, Medical University of Warsaw
Andrzej Tomaszewski
Department of Cardiology, Medical University of Lublin
Dorota Piotrowska-Kownacka
Laboratory of Biological and Medical Imaging, Medical University of Warsaw
Lukasz Kownacki
Laboratory of Biological and Medical Imaging, Medical University of Warsaw
Zuzanna Rymarczyk
Department of Internal Medicine and Cardiology, Medical University of Warsaw
Andrzej Wysokinski
Department of Cardiology, Medical University of Lublin
Piotr Pruszczyk
Department of Internal Medicine and Cardiology, Medical University of Warsaw

Keywords: Echocardiography, Magnetic resonance imaging, Ventricular noncompaction

JOURNAL FREE ACCESS

2009 Volume 73 Issue 11 Pages 2163-2165

DOI https://doi.org/10.1253/circj.CJ-08-0857

View "Advance Publication" version (February 13, 2009).

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Abstract

Noncompaction of the ventricular myocardium is a recently recognized genetic cardiomyopathy. The left ventricle is the most affected site, but right ventricular involvement has been reported in some cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. The major clinical manifestations are heart failure, arrhythmias and embolic events. A 20-year old man had left and right ventricular noncompaction complicated by severe pulmonary hypertension, which is one of the first cases of biventricular noncompaction associated with severe pulmonary hypertension. Pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic heart dysfunction secondary to noncompaction. (Circ J 2009; 73: 2163-2165)

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