Yonsei Med J.  2007 Oct;48(5):879-882. 10.3349/ymj.2007.48.5.879.

Mid-Ventricular Obstructive Hypertrophic Cardiomyopathy Associated with an Apical Aneurysm: Evaluation of Possible Causes of Aneurysm Formation

Affiliations
  • 1Department of Cardiology, Nihon University School of Medicine, Tokyo, Japan. yuichis@med.nihon-u.ac.jp
  • 2Department of Cardiovascular and Respiratory Medicine, Shiga University of Medical Science, Seta Otsu, Shiga, Japan.

Abstract

Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) is a rare type of cardiomyopathy, associated with apical aneurysm formation in some cases. We report a patient presenting with ventricular fibrillation, an ECG with an above normal ST segment, and elevated levels of cardiac enzymes but normal coronary arteries. Left ventriculography revealed a left ventricular obstruction without apical aneurysm. There was a significant pressure gradient between the apical and basal sites of the left ventricle. Cine magnetic resonance imaging (MRI), performed on the 10th hospital day, showed asymmetric septal hypertrophy, mid-ventricular obstruction, and an apical aneurysm with a thrombus. The first evaluation by contrast-enhanced imaging showed a subendocardial perfusion defect and delayed enhancement. It was speculated that the intraventricular pressure gradient, due to mid- ventricular obstruction, triggered myocardial infarction, which subsequently resulted in apical aneurysm formation.

Keyword

Mid-ventricular obstructive hypertrophic cardiomyopathy; magnetic resonance imaging

MeSH Terms

Cardiomyopathy, Hypertrophic/complications/*diagnosis
Coronary Angiography
Echocardiography, Doppler
Heart Aneurysm/*diagnosis/etiology
Humans
Hypertrophy, Left Ventricular/complications/*diagnosis
Magnetic Resonance Imaging, Cine
Male
Middle Aged
Myocardial Ischemia/complications/diagnosis

Figure

  • Fig. 1 (A) Coronary aniogram showing normal left (left panel) and right (right panel) coronary arteries. (B) Left ventriculogram in the 30° right anterior oblique projection, showing a small left ventricular cavity during diastole (left panel) and complete obstruction of the apical site during systole (right panel).

  • Fig. 2 Left ventricular pressure in the apex (upper panel) and in the base (lower panel), showing a pressure gradient of 45mmHg

  • Fig. 3 ECG-gated, cine magnetic resonance imaging of the long axis (A) and 4-chamber view (B), showing mid-ventricular obstruction during systole (large arrows) (lower panels), an apical aneurysm (small arrows), and a thrombus (Th, upper panels) during diastole. Contrast-enhanced first-pass myocardial perfusion (C) and delayed enhancement (D) images, showing a subendomyocardial perfusion defect (arrows) and delayed enhancement (arrows), respectively.


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