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Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Apical hypertrophic cardiomyopathy: clinical and two-dimensional echocardiographic assessment
Insights
Apical hypertrophic cardiomyopathy is a rare, nonobstructive form of hypertrophic cardiomyopathy. This study found it uncommon in a North American population, with distinct features from previously described Japanese cases.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Apical hypertrophic cardiomyopathy (AHC) is a morphologic variant.
- Previous studies primarily described AHC in Japanese populations.
Purpose of the Study:
- To evaluate the prevalence and characteristics of AHC in a North American cohort.
- To compare AHC findings in a North American population with those previously reported in Japanese patients.
Main Methods:
- Retrospective review of 965 patients with HCM evaluated by echocardiography.
- Analysis of morphologic features, clinical presentation, and electrocardiographic findings.
Main Results:
- Only 23 patients (2%) had nonobstructive AHC, with predominant apical wall thickening.
- Patients were predominantly male (mean age 37 years) and white.
- Fifteen patients experienced functional limitations, mainly exertional dyspnea and fatigue.
- Only 3 patients exhibited AHC morphology similar to Japanese descriptions; 2 had giant negative T waves.
Conclusions:
- Nonobstructive AHC is uncommon in this North American HCM population.
- The morphologic and clinical features of AHC in this cohort differed from those previously reported in Japanese patients.
- Apical hypertrophy predominantly in the distal left ventricle is rare in North American patients with HCM.
Abstract:
Of 965 patients with hypertrophic cardiomyopathy evaluated by echocardiography at the National Institutes of Health during a 7-year period, 23 (2%) had a nonobstructive morphologic form, in which wall thickening occurred predominantly in the apical (distal) portion of the left ventricle. The patients ranged in age from 15 to 69 years (mean, 37) and were predominantly male (14 patients) and white (only 1 was of oriental descent). Fifteen patients had significant functional limitation, which was usually caused by exertional dyspnea and fatigue. Several electrocardiographic patterns were identified in the study group, but only 4 of these patients showed "giant" negative T waves. Only 3 patients had a morphologic expression of apical hypertrophy that closely resembled that described in Japanese patients--that is, hypertrophy that was particularly localized and confined to the true left ventricular apex (2 of these patients had giant negative T waves). Hence, hypertrophy located predominantly in the distal left ventricle was uncommon in our primarily North American patient population with hypertrophic cardiomyopathy. Most of our patients showed morphologic and clinical features that were dissimilar to those found previously in Japanese patients with apical hypertrophy.
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