Left ventricular morphologic progression in apical hypertrophic cardiomyopathy

Background: Left ventricular (LV) morphologic progression in apical hypertrophic cardiomyopathy (AHC) has not been well studied. We evaluated serial echocardiographic changes in LV morphology. Methods: Serial echocardiograms in AHC patients were assessed. LV morphology was categorized according to the presence of an apical pouch or aneurysm, and LV hypertrophic severity and extent; relative, pure, and apical-mid type defined as mild (<15 mm thickness) apical hypertrophy, significant (≥15 mm) apical hypertrophy, and both apical and midventricular hypertrophy, respectively. Adverse clinical events and late gadolinium enhancement (LGE) extent on cardiac magnetic resonance were evaluated for each morphologic type. Results: In 41 patients, 165 echocardiograms (maximal interval: 4.2 [IQR, 2.3–11.8] years) were evaluated. Morphologic changes were observed in 19 (46%) patients. Eleven (27%) patients displayed the progression of LV hypertrophy toward pure or apical-mid type. Five (12%) and 6 (15%) patients developed new pouches and aneurysms. Patients with progression tended to be younger (50 ± 15.6 vs 59 ± 14.4 years, P = 0.058) and had a longer period of follow-up (12 [5–14] vs 3 [2–4] years, P < 0.001). During a follow-up of 7.6 (IQR 3.0–12.1) years, 21 (51%) experienced clinical events. The relative, pure, and apical-mid types showed different LGE extents (2%, 6%, and 19%, P = 0.004). Patients with severe hypertrophic and apical involvement showed higher clinical event rates. Conclusions: About half of AHC patients had a progression of LV morphology to more hypertrophic involvement and/or an apical pouch or aneurysm formation. Advanced AHC morphologic types were associated with higher event rates and scar burdens.