Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 Sep 1;3(10):101235.
doi: 10.1016/j.jacadv.2024.101235. eCollection 2024 Oct.

Long-Term Outcomes of Patients With Apical Hypertrophic Cardiomyopathy Utilizing a New Risk Score

Adel Hajj-Ali  1 , Andrew Gaballa  1 , Emmanuel Akintoye  1 , Shada Jadam  1 , Jay Ramchand  1 , Bo Xu  1 , Susan Ospina  1 , Maran Thamilarasan  1 , Nicholas G Smedira  1 , Zoran B Popovic  1 , Milind Y Desai  1
Affiliations

Long-Term Outcomes of Patients With Apical Hypertrophic Cardiomyopathy Utilizing a New Risk Score

Adel Hajj-Ali et al. JACC Adv. .

Abstract

Background: Apical hypertrophic cardiomyopathy (aHCM) is a distinct variant characterized by predominant hypertrophy of the left ventricle apex.

Objectives: This study sought to describe aHCM patients' characteristics and develop a risk score for aHCM patients.

Methods: A total of 462 patients (age 58 ± 15 years, 68% male) diagnosed with aHCM were included. The primary endpoint was death, appropriate defibrillator discharge, or need for cardiac transplantation. Variables showing potential association with the composite endpoint were considered to develop an aHCM-specific risk score.

Results: At baseline, 67% patients were asymptomatic and 69% had no risk factors for sudden death. On echocardiography, the mean left ventricle ejection fraction, left atrial volume index, and right ventricular systolic pressure were 64% ± 8%, 36 ± 15 ml/m2, and 32 ± 10 mm Hg, respectively, with 51(11%) demonstrating an apical aneurysm. Baseline cardiac magnetic resonance, performed in 246 (53%) patients, demonstrated delayed gadolinium enhancement in 170 (71%) patients (mean percentage of 4.9% ± 6.6%). At age 6.3 ± 4.8 years, the composite events occurred in 80 (17%, death in 62 [13%]) patients. The aHCM-specific risk score, incorporating age, apical aneurysm, left atrial volume index, serum creatinine, and right ventricular systolic pressure, demonstrated good discrimination (C-statistic = 0.75) with an expected to observed ratio of 1.02 and a calibration slope of 0.91. The risk score ranged between 0 and 8 points, with a higher score associated with higher composite events.

Conclusions: aHCM constituted 6.8% of our overall HCM cohort with a composite event rate of 2.8%/year. The aHCM risk score provided good discrimination in predicting the composite primary endpoint, with a higher score associated with a higher rate of events.

Keywords: apical hypertrophic cardiomyopathy; prognosis; risk score.

PubMed Disclaimer

Conflict of interest statement

The current study was funded by unrestricted philanthropic gifts by the Ratner family, Stinson family, and Anderson family for Dr Desai’s research. Dr Hajj-Ali, Dr Gaballa, Mrs Ospina, and Dr Jadam have received salary support from unrestricted philanthropic gifts by the Haslam family, Ratner family, Stinson family, and Anderson family. Dr Desai is a consultant and has research agreements with Bristol Myers Squibb, Cytokinetics, Tenaya, Viz-AI, and Edgewise. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Central Illustration
Central Illustration
Long-Term Outcomes in Patients With Apical Hypertrophic Cardiomyopathy: A New Risk Score Key details of the study, including the study population, representative images, follow-up, endpoints, and key findings. LAVI = left atrial volume index; RVSP = right ventricular systolic pressure; SCD = sudden cardiac death; other abbreviation as in Figure 2.
Figure 1
Figure 1
Model Performance for Original Dataset and Internal Validation (A) Graph of model performance in the original data set. The thick blue line represents a calibration plot of observed and expected event-free survival probabilities based on the adaptive linear spline method (calibration intercept was −0.005 (95% CI: −0.06-0.05). The dashed blue line represents a hypothetical perfect calibration. The E:O ratio is the ratio of the expected and observed event-free survival probabilities (ideal value = 1); the slope refers to the model fit (ideal value = 1). (B) Model performance with internal validation. Graph of model performance with internal validation using the bootstrapping method. The thick blue line represents a calibration plot of observed and expected event-free survival probabilities based on the adaptive linear spline method (Calibration intercept was −0.006 (95% CI: −0.05-0.05). The dashed blue line represents a hypothetical perfect calibration. E:O ratio is the ratio of the expected and observed event-free survival probabilities (ideal value = 1); slope refers to the model fit (Ideal value = 1).
Figure 2
Figure 2
Kaplan-Meier Survival Analysis for Primary Composite Events Separated on Basis of Increasing Apical Hypertrophic Cardiomyopathy Risk Score
See this image and copyright information in PMC

References

    1. Arbelo E., Protonotarios A., Gimeno J.R., et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44:3503–3626. - PubMed
    1. Ommen S.R., Mital S., Burke M.A., et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American college of cardiology/American heart association joint committee on clinical practice guidelines. J Am Coll Cardiol. 2020;76:e159–e240. - PubMed
    1. Maron B.J., Desai M.Y., Nishimura R.A., et al. Diagnosis and evaluation of hypertrophic cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol. 2022;79:372–389. - PubMed
    1. Sakamoto T., Tei C., Murayama M., et al. Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle echocardiographic and ultrasono-cardiotomographic study. Jpn Heart J. 1976;17:611–629. - PubMed
    1. Klarich K.W., Attenhofer Jost C.H., Binder J., et al. Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy. Am J Cardiol. 2013;111:1784–1791. - PubMed

LinkOut - more resources