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Published on: June 14, 2016
Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy
Georgios K Efthimiadis1, Georgios Giannakoulas, Despina G Parcharidou
1Cardiology Department, AHEPA Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece. efthymos@med.auth.gr
Cardiovascular Ultrasound
|March 14, 2007
Summary
This study presents a rare case of extreme hypertrophic cardiomyopathy (HCM) with both subaortic and midventricular obstruction. The patient received a cardioverter-defibrillator for primary prevention of sudden cardiac death due to severe hypertrophy.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Extreme segmental hypertrophy exceeding typical limits is rare.
- Subaortic and midventricular obstruction are infrequent HCM phenotypes.
Observation:
- A 19-year-old man presented with severe asymmetric left ventricular (LV) hypertrophy (septum 49 mm).
- The patient exhibited LV outflow obstruction and midventricular obstruction.
- Cardiac MRI revealed significant late gadolinium enhancement in the hypertrophied septum.
Findings:
- The patient had an LV outflow tract gradient of 38 mm Hg and a midventricular gradient of 43 mm Hg.
- Despite no syncope or arrhythmias on monitoring, the extreme LV hypertrophy and myocardial enhancement indicated high sudden death risk.
- A cardioverter-defibrillator was recommended for primary prevention.
Implications:
- Midventricular HCM is an uncommon presentation.
- This phenotype may be linked to apical aneurysms and progression to end-stage heart failure.
- Prophylactic cardioverter-defibrillator implantation is crucial for managing high-risk HCM patients.
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