Abstract
BackgroundHypertrophic 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.Case presentationWe 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.ConclusionThe 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.
Highlights
Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease
In this report we present a case-series of HCM patients with midventricular obstruction and apical aneurysm formation in a single family
A cardioverter defibrillator was implanted in index-case 1 patient because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test
Summary
Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease, in terms of clinical course and phenotypic expression [1,2,3]. In this report we present a case-series of HCM patients with midventricular obstruction and apical aneurysm formation in a single family. We studied 3 female patients (case-index 1, 63-y; case-index 2, 56-y; case-index 3, 27-y) (Figure 1) with already diagnosed HCM who were referred to our department for further evaluation. The three female patients we studied (case-index 1, 63-y; case-index 2, 56-y; case-index 3, 27-y) are represented by black colour filled circles in generations II and III. Apical four chamber view showing predominant thickening of midseptal and midlateral regions during systole (blue arrow) and the formation of left ventriculat apical aneurysm (arrow head) in case index-1 patient. A cardioverter defibrillator was implanted in index-case 1 patient 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 rest of the patients are stable after a 2.5-y follow-up period
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
