American Journal of Medical Case Reports. 2022, 10(9), 239-243
DOI: 10.12691/AJMCR-10-9-7
A Rare form of Hypertrophic Cardiomyopathy with Mid-cavity Obstruction
Muhammad Shabbir Ijaz1 and H.K. Reddy2,
1Department of Medicine, Poplar Bluff Regional Medical Center, Poplar Bluff, Missouri, USA
2Cardiovascular Institute of Southern Missouri, Poplar Bluff Regional Medical Center, Poplar Bluff, Missouri, USA
Pub. Date: September 20, 2022
Cite this paper
Muhammad Shabbir Ijaz and H.K. Reddy. A Rare form of Hypertrophic Cardiomyopathy with Mid-cavity Obstruction.
American Journal of Medical Case Reports. 2022; 10(9):239-243. doi: 10.12691/AJMCR-10-9-7
Abstract
Hypertrophic cardiomyopathy (HCM) is a genetic disorder of cardiac myocytes with variable penetrance and heterogenous phenotypic expression leading to different morphologies of left ventricular (LV) hypertrophy which are usually associated with dynamic left ventricular outflow tract (LVOT) obstruction. Up to 2 percent of cases of HCM have a morphology of mid-cavity or mid-ventricular obstruction in which LV cavity is divided into two cavities by apposition of the hypertrophied septum and free wall, and is many times associated with pressure gradients between both cavities usually in the range of 50-70 mmHg but as high as 110 mmHg has been reported (3). We present a case of this rare form of mid-cavity obstruction with an exceptionally high intraventricular pressure gradient of 154 mmHg and apical aneurysm formation.
Keywords
Hypertrophic cardiomyopathy, left ventricular outflow tract obstruction, intraventricular pressure gradient
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References
[1] | Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation. 1995; 92(7): 1680-1692. |
|
[2] | Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation. 1995; 92(4): 785-789. |
|
[3] | Tse HF, Ho HH. Sudden cardiac death caused by hypertrophic cardiomyopathy associated with midventricular obstruction and apical aneurysm. Heart. 2003; 89(2): 178. |
|
[4] | Maron MS, Olivotto I, Zenovich AG, et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006; 114(21): 2232-2239. |
|
[5] | Shah JS, Esteban MT, Thaman R, et al. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy. Heart. 2008; 94(10): 1288-1294. |
|
[6] | Maron MS. Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy. J Cardiovasc Magn Reson. 2012; 14(1): 13. Published 2012 Feb 1. |
|
[7] | Adabag AS, Maron BJ, Appelbaum E, et al. Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance. J Am Coll Cardiol. 2008; 51(14): 1369-1374. |
|
[8] | Veselka J, Anavekar NS, Charron P. Hypertrophic obstructive cardiomyopathy. Lancet 2017; 389:1253. |
|
[9] | ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011; 124(24): 2761-2796. |
|
[10] | Nistri S, Olivotto I, Maron MS, et al. β Blockers for prevention of exercise-induced left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2012; 110(5): 715-719. |
|