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Published on: October 28, 2020
Left ventricular morphologic progression in apical hypertrophic cardiomyopathy
Mirae Lee1, Alon Shechter2, Donghee Han3
1Division of Cardiology, Department of Internal Medicine, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Republic of Korea; Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Insights
About half of apical hypertrophic cardiomyopathy (AHC) patients show left ventricular (LV) morphology progression, including pouch or aneurysm formation. Advanced AHC types correlate with increased clinical events and scar burden.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Apical hypertrophic cardiomyopathy (AHC) is a form of hypertrophic cardiomyopathy characterized by thickening of the left ventricular apex.
- The morphologic progression of left ventricular (LV) structures in AHC patients is not well understood.
- Serial echocardiographic evaluation is crucial for assessing cardiac morphology changes.
Purpose of the Study:
- To evaluate serial echocardiographic changes in left ventricular (LV) morphology in patients with apical hypertrophic cardiomyopathy (AHC).
- To investigate the association between LV morphologic progression and adverse clinical events.
- To determine the relationship between different AHC morphologic types and late gadolinium enhancement (LGE) extent.
Main Methods:
- Retrospective analysis of serial echocardiograms from 41 AHC patients.
- Categorization of LV morphology based on apical pouch/aneurysm presence and hypertrophy severity/extent (relative, pure, apical-mid types).
- Evaluation of clinical events and cardiac magnetic resonance (CMR) LGE extent for each morphologic type.
Main Results:
- Morphologic changes were observed in 46% of patients, with 27% progressing to more severe hypertrophy types.
- New apical pouches or aneurysms developed in 12% and 15% of patients, respectively.
- Patients with advanced morphologic types exhibited higher LGE extent (19% for apical-mid) and increased clinical event rates.
Conclusions:
- Approximately half of AHC patients experience LV morphologic progression, including apical changes.
- Advanced AHC morphologic types are linked to greater scar burden and higher rates of adverse clinical events.
- Serial echocardiography is valuable for monitoring AHC progression and risk stratification.
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.
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