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Published on: August 8, 2022
Apical hypertrophic cardiomyopathy
Syed Wamique Yusuf1, Jaya D Bathina, Jose Banchs
1Syed Wamique Yusuf, Jaya D Bathina, Jose Banchs, Elie N Mouhayar, Iyad N Daher, Department of Cardiology, University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States.
Insights
Apical hypertrophic cardiomyopathy (AHCM) can be asymptomatic but may lead to cardiac arrhythmias. Early diagnosis via echocardiography and advanced imaging, alongside appropriate medical or device therapy, is crucial for managing this rare condition.
Area of Science:
- Cardiology
- Genetics
Background:
- Apical hypertrophic cardiomyopathy (AHCM) is a rare variant of hypertrophic cardiomyopathy affecting the left ventricular apex.
- It can present asymptomatically or with diverse cardiac symptoms including arrhythmias and heart failure.
Purpose of the Study:
- To describe a case of asymptomatic AHCM progressing to cardiac arrhythmias.
- To review diagnostic modalities, differential diagnoses, and treatment options for AHCM.
Main Methods:
- Case report of a patient with AHCM.
- Review of diagnostic tools: electrocardiogram (ECG), transthoracic echocardiogram (TTE), cardiac MRI, CT, and left ventriculography.
- Discussion of pharmacologic and device-based treatments.
Main Results:
- The patient initially asymptomatic, later developed cardiac arrhythmias.
- Classic ECG findings include giant negative T-waves and left ventricular hypertrophy.
- TTE is the initial diagnostic tool, revealing apical hypertrophy; advanced imaging aids in differential diagnosis.
Conclusions:
- AHCM diagnosis requires a combination of clinical presentation and imaging.
- Management involves risk stratification and tailored therapy, including medications and potentially an implantable cardioverter-defibrillator for high-risk individuals.
Abstract:
We describe a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who later developed cardiac arrhythmias, and briefly discuss the diagnostic modalities, differential diagnosis and treatment option for this condition. AHCM is a rare form of hypertrophic cardiomyopathy which classically involves the apex of the left ventricle. AHCM can be an incidental finding, or patients may present with chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation and congestive heart failure. AHCM is frequently sporadic, but autosomal dominant inheritance has been reported in few families. The most frequent and classic electrocardiogram findings are giant negative T-waves in the precordial leads which are found in the majority of the patients followed by left ventricular (LV) hypertrophy. A transthoracic echocardiogram is the initial diagnostic tool in the evaluation of AHCM and shows hypertrophy of the LV apex. AHCM may mimic other conditions such as LV apical cardiac tumors, LV apical thrombus, isolated ventricular non-compaction, endomyocardial fibrosis and coronary artery disease. Other modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings are also valuable tools and are frequently used to differentiate AHCH from other conditions. Medications used to treat symptomatic patients with AHCM include verapamil, beta-blockers and antiarrhythmic agents such as amiodarone and procainamide. An implantable cardioverter defibrillator is recommended for high risk patients.
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