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Co-Existing Subaortic Stenosis in a Patient With Hypertrophic Obstructive Cardiomyopathy: A Rare and Interesting
Raja S Mushtaque1, Rabia Mushtaque1, Shahbano Baloch2
1Cardiology, National Institute of Cardiovascular Diseases, Karachi, PAK.
Insights
Hypertrophic cardiomyopathy (HCM) can cause left ventricular outflow tract obstruction. A rare co-occurrence with a subaortic membrane presents diagnostic challenges and risks heart failure.
Area of Science:
- Cardiology
- Genetics
- Medical Imaging
Background:
- Hypertrophic cardiomyopathy (HCM) is an inherited condition causing left ventricular outflow tract (LVOT) obstruction, potentially leading to symptoms like chest pain, syncope, or sudden cardiac death.
- Diagnostic tools for HCM include echocardiography, cardiac CT, and cardiac MRI, aiding in identifying characteristic left ventricular hypertrophy.
Observation:
- This case report details a young female with diagnosed HCM presenting with palpitations, chest pain, and shortness of breath.
- Echocardiography revealed severe asymmetric LV hypertrophy, normal LV function, systolic anterior motion of the mitral valve, and a subvalvular aortic membrane.
- Cardiac CT confirmed severe asymmetric septal hypertrophy, a thickened aortic valve without stenosis, and a concentric subaortic membrane.
Findings:
- The co-existence of hypertrophic cardiomyopathy and a subaortic membrane is an uncommon finding.
- This combination poses diagnostic difficulties and can exacerbate LVOT obstruction.
Implications:
- Untreated cases of HCM with subaortic membranes are prone to progressive heart failure and worsening symptoms.
- Comprehensive evaluation and meticulous surgical planning are crucial for successful intervention in such complex cases.
Abstract:
Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder leading to left ventricular outflow tract (LVOT) obstruction. It can present with chest pain, syncope, breathlessness, or it may cause sudden cardiac death in some cases. The echocardiography in most cases while cardiac CT or cardiac MRI in selected cases are the important diagnostic modalities to make the diagnosis of HCM. In this case report, we discuss a case of a young female patient previously diagnosed with HCM and presented with palpitations, chest pain, and shortness of breath. Her echocardiography revealed severe asymmetrically hypertrophied left ventricle (LV) with normal function, the systolic anterior motion of the mitral valve was present and a subvalvular aortic membrane was also seen. The CT was also performed showing severe asymmetrical hypertrophied septum and thickened trileaflet tricommissural aortic valve with no calcification or significant valvular aortic stenosis but there was a subaortic membrane (concentric only sparing anteriorly). The presence of subaortic membrane with HCM is a rare finding and it can be a diagnostic challenge and untreated cases are susceptible to progressive heart failure and worsening of the symptoms by further increasing LVOT obstruction. A thorough investigation and planning before surgical intervention is required to achieve optimal results.
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