Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Efthimiadis
Georgios K
GK
Cardiology Department, Aristotle University of Thessaloniki, Greece. efthymos@med.auth.gr
Pliakos
Christodoulos
C
Pagourelias
Efstathios D
ED
Parcharidou
Despina G
DG
Spanos
Georgios
G
Paraskevaidis
Stylianos
S
Styliadis
Ioannis H
IH
Parcharidis
Georgios
G
D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000000981:diagnostic imaging*; D005190:Family; D005260:Female; D006322:Heart Aneurysm / Q000150:complications* / Q000000981:diagnostic imaging*; D006801:Humans; D008875:Middle Aged; D014463:Ultrasonography; D014694:Ventricular Outflow Obstruction / Q000150:complications* / Q000000981:diagnostic imaging*