Clinical characteristics and long-term outcomes in patients with apical hypertrophic cardiomyopathy
Meng Guo1,2, Chuanfen Liu1,2, Jingjing Ye3
1Department of Cardiology, Peking University People's Hospital, Beijing, China.
Insights
Apical hypertrophic cardiomyopathy (ApHCM) patients show a better prognosis than non-ApHCM patients, with fewer adverse cardiac events. Left ventricular ejection fraction (LVEF) and age are key predictors of long-term outcomes in ApHCM.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Apical hypertrophic cardiomyopathy (ApHCM) is a distinct subtype of hypertrophic cardiomyopathy (HCM) with incompletely understood natural history and prognosis.
- Characterizing ApHCM is crucial for accurate diagnosis and risk stratification.
Purpose of the Study:
- To delineate the clinical characteristics and long-term outcomes of ApHCM patients.
- To identify independent predictors of adverse cardiac events in ApHCM.
Main Methods:
- A single-center retrospective study involving 479 HCM patients, categorized into ApHCM and non-ApHCM groups.
- Comparison of clinical, electrocardiographic, echocardiographic, and survival data.
- Primary outcome: major adverse cardiac events during hospitalization and follow-up.
Main Results:
- ApHCM patients (n=109) were older at diagnosis and exhibited more electrocardiographic abnormalities but had lower brain natriuretic peptide levels and better left ventricular ejection fraction (LVEF) compared to non-ApHCM patients (n=370).
- ApHCM patients experienced significantly fewer major adverse cardiac events (11.4% vs. 27.2%) during a median follow-up of 5.59 years.
- Reduced all-cause death and fatal ventricular arrhythmias were observed in ApHCM. LVEF and age independently predicted adverse outcomes in ApHCM.
Conclusions:
- Apical HCM is associated with a potentially better long-term prognosis compared to other forms of HCM.
- Left ventricular ejection fraction (LVEF) and patient age are significant independent predictors of adverse outcomes in ApHCM.
Aims:
As a special type of hypertrophic cardiomyopathy (HCM), apical HCM (ApHCM) has different clinical characteristics while its nature history and prognosis are not well recognized. We aimed to describe the characteristics and outcomes of ApHCM and identify predictors of adverse outcomes.
Methods:
In this single-centre retrospective study, we included 479 patients with HCM and divided them into ApHCM and non-ApHCM groups. Clinical, electrocardiographic, echocardiographic and survival data were compared between the groups. The primary outcome was major adverse cardiac events in hospital and during follow-up. A two-sided P-value < 0.05 was considered statistically significant.
Results:
A total of 109 ApHCM patients and 370 non-ApHCM patients were analysed and 379 patients completed the follow-up among them. The age of enrolled patients was 61.0 (50.0-69.0) years, and 289 (60.3%) were male. Compared with non-ApHCM patients, ApHCM patients were older at diagnosis [55.0 (45.0-64.0) vs. 50.0 (40.0-61.0) years, P = 0.006] and had less positive family history for HCM [3 (2.8%) vs. 34 (9.2%), P = 0.027], more electrocardiographic abnormalities [101 (92.7%) vs. 287 (77.6%), P < 0.001], lower brain natriuretic peptide level [135.5 (60.8-272.8) vs. 422.5 (182.8-888.2) pg/mL, P < 0.001] and better left ventricular ejection fraction (LVEF) [69.00 (64.00-73.87) vs. 67.00 (60.24-73.45) %, P = 0.048] at baseline. During a median follow-up of 5.59 (2.33-10.30) years, the primary outcome occurred less frequently in ApHCM patients [11.4% vs 27.2%; hazard ratio (HR)adj 0.360 (95% confidence interval, CI: 0.187-0.696), P = 0.002; log rank P = 0.001]. Specifically, ApHCM was characterized by fewer all-cause death (HRadj 0.545, 95% CI: 0.305-0.975; P = 0.041) and fatal ventricular arrhythmia or appropriate implantable cardioverter defibrillator intervention (HRadj 0.099, 95% CI: 0.013-0.724; P = 0.023). LVEF (HRadj 0.861, 95% CI: 0.763-0.971; P = 0.015) and age (HRadj 1.247, 95% CI: 1.095-1.419; P = 0.001) were identified as independent predictors of the composite outcome in ApHCM.
Conclusions:
Patients with ApHCM may have better prognosis. LVEF and age were independent predictors of long-term outcomes in ApHCM.
