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Apical Hypertrophic Cardiomyopathy Prompting Aneurysm, Thrombus, and Cardiac Arrest in a 56-Year-Old Female
Patrick Biskupski1, Julieta Osella1, Aditya Bhaskaran1
1Internal Medicine, Lincoln Medical Center, New York City, USA.
Insights
Apical hypertrophic cardiomyopathy (apHCM), a rare genetic heart condition, can lead to severe complications like left ventricular aneurysm and cardiac arrest. Early recognition and management are crucial for preventing adverse outcomes in apHCM patients.
Area of Science:
- Cardiology
- Genetics
- Cardiac Imaging
Background:
- Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease.
- Apical hypertrophic cardiomyopathy (apHCM) is a rare, distinct subset of HCM.
- apHCM was previously considered to have a benign clinical course.
Observation:
- A 56-year-old female with known apHCM presented with chest pain, ventricular storm, and cardiac arrest.
- Initial presentation was complicated by suspected cocaine use.
- Cardiac MRI revealed a severe apical aneurysm, thrombosis, and a likely re-entrant circuit.
Findings:
- The patient experienced ventricular tachycardia and cardiac arrest, requiring 10 minutes of resuscitation.
- A severe apical aneurysm and thrombus formation were identified as key contributors to her decompensation.
- Successful management involved antiarrhythmic medications, beta-blockers, and cardioverter-defibrillator (ICD) implantation.
Implications:
- This case highlights the potential for severe, life-threatening complications in apHCM, including aneurysm, thrombosis, and cardiac arrest.
- apHCM requires vigilant monitoring and timely intervention, challenging its prior perception as benign.
- Effective medical and/or surgical management strategies are critical for improving outcomes in apHCM patients.
Abstract:
Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic cardiac disease while apical hypertrophic cardiomyopathy (apHCM) is a rare subset of HCM. The significance of this case report is to present apHCM, its chronological course, and its association with left ventricular aneurysm, thrombosis, and cardiac arrest. We present the case of a 56-year-old female with a past medical history of apHCM who was admitted for substernal chest pain, developed a ventricular storm (VT), and subsequently suffered cardiac arrest; resuscitation of spontaneous circulation (ROSC) was eventually achieved after 10 minutes. It was initially thought that her arrhythmia and hemodynamic decompensation were purely secondary to cocaine use at a party six hours prior to her presentation. During hospitalization, cardiac magnetic resonance imaging demonstrated a severe apHCM apical aneurysm, thrombosis, and a re-entrant circuit as a likely cause of this patient's decompensation and eventual cardiac arrest. After several days of hemodynamic stability and decreased dependence on intravenous antiarrhythmic medication infusions, she was extubated and transitioned to oral amiodarone and beta-blocker therapy with the implantation of a cardioverter-defibrillator (ICD). In this case, we analyze the continuum of apHCM, a rare subset of HCM once thought to be benign but with the emergence of complications, including aneurysm, thrombus formation, resistant ventricular tachycardia, and cardiac arrest. Recognition and management of apHCM with medical and/or surgical intervention are therefore critical to prevent the aforementioned sequela.
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