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[Hypertrophic obstructive cardiomyopathy in the right ventricle presenting in the elderly woman: a case report]
K Yamazaki1, M Horimoto, K Igarashi
1Department of Cardiology, National Sapporo Hospital.
Insights
This study reports an extremely rare case of elderly hypertrophic obstructive cardiomyopathy (HOCM) primarily affecting the right ventricle (RV), with secondary left ventricle (LV) involvement.
Area of Science:
- Cardiology
- Pathology
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) is a genetic heart muscle disease.
- While typically affecting the left ventricle, RV-predominant HOCM is exceptionally rare, especially in the elderly.
Observation:
- A 69-year-old woman presented with a cardiac murmur, cardiomegaly, and imaging showing interventricular septum and RV free wall thickening obstructing RV outflow.
- Cardiac catheterization revealed a significant RV pressure gradient, with normal LV pressures.
- RV angiocardiograms demonstrated severe RV obstruction, while LV angiocardiograms showed mild enlargement and diffuse hypokinesis.
Findings:
- Myocardial biopsy revealed bizarre myocyte hypertrophy and disorganization, more pronounced in the RV.
- The patient was diagnosed with hypertrophic obstructive cardiomyopathy (HOCM) predominantly in the right ventricle (RV), with associated left ventricle (LV) dysfunction.
Implications:
- This case highlights the extreme rarity of RV-predominant HOCM in elderly patients.
- The concomitant LV involvement in this RV-centric HOCM case adds complexity to understanding the disease spectrum and management.
Abstract:
A 69-year-old woman was admitted to the hospital for evaluation of a cardiac murmur, which had been indicated at her childhood. On physical examination, a systolic ejection murmur was heard on the lower left sternal border. Chest roentgenogram revealed a cardiomegaly. Echocardiography and MRI showed a thickening of the interventricular septum and the right ventricular (RV) free wall, which obstructed the RV body. At cardiac catheterization, a systolic pressure gradient of 100 mmHg was shown between the RV outflow tract and the apex. There was no pressure gradient in the left ventricle (LV). RV angiocardiograms disclosed a severe obstruction of the RV body, while LV angiocardiograms showed a slight enlargement of the LV with a diffuse hypokinesis of its wall. Myocardial biopsy of both ventricles revealed a bizarre hypertrophy of myocytes and disorganization. The histological findings were more conspicuous in the RV. Based on these findings, this case was diagnosed as hypertrophic obstructive cardiomyopathy (HOCM) in the RV. HOCM in the RV presenting in the elderly, is extremely rare. Our case is also characterized by a concomitant LV involvement, which demonstrated the slightly enlarged LV and the diffuse hypokinesis of its wall.