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Subaortic and mid-ventricular obstructive hypertrophic cardiomyopathy with an apical aneurysm: a case report
Tomás Francisco Cianciulli1, María Cristina Saccheri, Isabel Victoria Konopka
1Department of Cardiology, Hospital del Gobierno de la Ciudad de Buenos Aires Dr, Cosme Argerich, Buenos Aires, Argentina. tcianciulli@fibertel.com.ar
Insights
This rare case report details a patient with hypertrophic cardiomyopathy (HCM) exhibiting both mid-ventricular and apical variants, alongside an apical aneurysm. This unique combination, presenting with myocardial infarction and normal coronary arteries, highlights unusual HCM manifestations.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) commonly presents with asymmetric septal hypertrophy, often leading to dynamic subaortic obstruction in 25% of cases.
- Apical and mid-ventricular HCM are rare variants, sometimes associated with apical aneurysms.
- The coexistence of mid-ventricular and apical HCM is exceptionally infrequent.
Observation:
- A 49-year-old female patient presented with combined septal asymmetric HCM and midventricular HCM.
- She exhibited a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, along with an apical aneurysm.
- Clinical presentation included acute myocardial infarction, normal coronary arteries, and left ventricular hypertrophy.
Findings:
- The patient had a rare combination of obstructive HCM, mid-ventricular HCM, and an apical aneurysm.
- Coronary angiography revealed no coronary artery disease, despite the myocardial infarction.
- Electrocardiogram showed a right bundle branch block without ST-segment elevation or Q waves post-infarction.
Implications:
- This case represents a unique presentation of hypertrophic cardiomyopathy, previously unreported in medical literature.
- It underscores the importance of considering rare HCM variants and their potential complications, such as apical aneurysms.
- Further research into the genetic and pathophysiological mechanisms of these rare HCM presentations is warranted.
Background:
Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction. Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm. An even more rare presentation is the coexistence mid-ventricular and apical HCM. This case is a combination of obstructive HCM with mid-ventricular HCM and an apical aneurysm, which to date, has not been reported in the literature.
Case Presentation:
The patient is a 49 year-old lady who presents a combination of septal asymmetric hypertrophic cardiomyopathy (HCM) and midventricular HCM, a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, plus an apical aneurysm. Her clinical presentation was an acute myocardial infarction in June 2005. One month after hospital discharge, the electrocardiogram (ECG) showed a right bundle branch block (RBBB) with no Q waves or ST segment elevation. Coronary angiography revealed normal coronary arteries, left ventricular hypertrophy and an apical aneurysm.
Conclusion:
This case is a rare example of an asymptomatic patient with subaortic and mid-ventricular hypertrophic cardiomyopathy, who presents a myocardial infarction and normal coronary arteries, and during the course of her disease develops an apical aneurysm.
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