The online version of this article (doi:10.1186/1476-7120-7-26) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
GKE and EP conceived the case report, collected the data, reviewed literature and wrote the manuscript. IHS and GP revised the article for important intellectual content and edited the final version. GKE, DGP and CP performed the ultrasounds and participated in the analysis and interpretation of data. GS performed cardiac MRI. SP implanted the ICD. All authors read and approved the final manuscript.
Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV) apical aneurysms in the absence of coronary artery disease.
We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period.
The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.
Additional file 1: Transthoracic echocardiography movie. This movies shows an apical two chamber view during 3 cardiac circles demonstrating mid-ventricular septal and lateral walls thickening and apical aneurysm formation in case-index 1 patient. (AVI 3 MB)12947_2009_273_MOESM1_ESM.avi
Additional file 2: Cardiac Magnetic Resonance movie. Cardiac MRI movie obtained from case index-2 patient showing systolic midventricular wall thickening and an apical aneurysm. (AVI 859 KB)12947_2009_273_MOESM2_ESM.avi
Additional file 3: Catheterization movie. Catheterization movie revealing systolic left midventricular obstruction and apical aneurysm in case-index 2 patient. (AVI 13 MB)12947_2009_273_MOESM3_ESM.avi
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- Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report
Georgios K Efthimiadis
Efstathios D Pagourelias
Despina G Parcharidou
Ioannis H Styliadis
- BioMed Central
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