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Phenotypic spectrum and clinical characteristics of apical hypertrophic cardiomyopathy: multicenter echo-Doppler
Eui-Young Choi1, Se-Joong Rim, Jong-Won Ha
1Yonsei University College of Medicine, Seoul, Republic of Korea.
Insights
Apical hypertrophic cardiomyopathy (ApHCM) presents three distinct subtypes: pure focal, pure diffuse, and mixed. Subtyping ApHCM is crucial for predicting atrial fibrillation risk and assessing left ventricular function.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Apical hypertrophic cardiomyopathy (ApHCM) is a variant of hypertrophic cardiomyopathy characterized by hypertrophy predominantly affecting the apex of the left ventricle.
- Understanding the phenotypic spectrum and clinical implications of ApHCM subtypes is essential for patient management and risk stratification.
Purpose of the Study:
- To define the phenotypic spectrum of apical hypertrophic cardiomyopathy (ApHCM).
- To identify clinical characteristics associated with different ApHCM subtypes.
- To investigate the predictive value of ApHCM subtyping for clinical outcomes.
Main Methods:
- 182 consecutive ApHCM patients with preserved ejection fraction (>50%) were analyzed.
- Left ventricular end-diastolic wall thickness was measured in all 16 segments to classify hypertrophy patterns.
- Echo-Doppler parameters, electrocardiography, and clinical findings were assessed to correlate with hypertrophy subtypes.
Main Results:
- Three ApHCM subtypes were identified: pure focal (n=81), pure diffuse (n=70), and mixed (n=31).
- Significant differences were observed in atrial fibrillation incidence (5% vs. 11% vs. 23%, p<0.05) and left atrial volume index (30.9 vs. 35.7 vs. 41.3 ml/m², p<0.001) among subtypes.
- Subtypes also showed significant variations in peak systolic and diastolic mitral annular velocities and E/E' ratios (p<0.05).
Conclusions:
- ApHCM encompasses three morphologically distinct phenotypes.
- Detailed subtyping of ApHCM is important for predicting atrial fibrillation development.
- Subtyping aids in assessing left atrial volume index and left ventricular longitudinal function.
Objectives:
The aim of this study was to define the phenotypic spectrum of apical hypertrophic cardiomyopathy (ApHCM) and clinical characteristics pertaining to identified subtypes.
Methods:
In 182 consecutive ApHCM patients (58.9 +/- 11.2 years; 142 men) with left ventricular ejection fraction > or =50%, we measured end-diastolic wall thickness of all 16 left ventricular segments to determine patterns of hypertrophy. Echo-Doppler parameters, electrocardiography patterns, and clinical findings were analyzed.
Results:
ApHCM was classified into three types as pure focal (n = 81), pure diffuse (n = 70) and mixed type (n = 31) according to patterns of hypertrophy. Incidence of atrial fibrillation (5% for pure focal vs. 11% for pure diffuse vs. 23% for mixed type, p < 0.05) and left atrial volume index (30.9 +/- 11.8, 35.7 +/- 14.8, and 41.3 +/- 15.9 ml/m(2), respectively, p < 0.001) were significantly different among subtypes. Peak systolic (6.6 +/- 1.0 vs. 6.3 +/- 1.2 vs. 5.9 +/- 1.1 cm/s, respectively, p < 0.05), diastolic (5.1 +/- 1.8 vs. 5.0 +/- 1.2 vs. 4.1 +/- 1.3 cm/s, respectively, p < 0.05) mitral annular velocity, E/E' (13.3 +/- 4.2 vs. 13.7 +/- 5.4 vs. 16.1 +/- 6.1, respectively, p < 0.05) were also significantly different.
Conclusions:
ApHCM contains three morphologically distinct phenotypes and detailed subtyping is important in the prediction of development of atrial fibrillation, left atrial volume index and left ventricular longitudinal function.
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