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Long-Term Outcomes of Patients With Apical Hypertrophic Cardiomyopathy Utilizing a New Risk Score
Adel Hajj-Ali1, Andrew Gaballa1, Emmanuel Akintoye1
1Hypertrophic Cardiomyopathy Center, Heart Vascular Thoracic Institute, Cleveland Clinic, Cleveland, Ohio, USA.
JACC. Advances
|November 8, 2024
Summary
Apical hypertrophic cardiomyopathy (aHCM) is a distinct heart condition. A new risk score effectively predicts adverse events in aHCM patients, aiding clinical management.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Apical hypertrophic cardiomyopathy (aHCM) is a specific form of hypertrophic cardiomyopathy.
- It is characterized by hypertrophy predominantly affecting the apex of the left ventricle.
Purpose of the Study:
- To characterize patients with aHCM.
- To develop and validate a risk score for predicting adverse outcomes in aHCM patients.
Main Methods:
- A cohort of 462 patients diagnosed with aHCM was analyzed.
- The primary endpoint included death, defibrillator discharge, or cardiac transplantation.
- An aHCM-specific risk score was developed using relevant clinical and imaging variables.
Main Results:
- The study included 462 patients (58 ± 15 years, 68% male).
- A composite event occurred in 17% of patients over a mean follow-up of 6.3 years.
- The developed aHCM risk score showed good discrimination (C-statistic = 0.75) for predicting the primary endpoint.
Conclusions:
- aHCM represents 6.8% of the hypertrophic cardiomyopathy cohort, with a 2.8% annual composite event rate.
- The aHCM risk score effectively predicts major adverse events, with higher scores indicating increased risk.
