What is the clinical evidence from this study?

Study design: Observational. Population: Hypertrophic cardiomyopathy with MyBPC gene mutations (n=27). Intervention: Elderly age (≥65 years) vs. Young age (<65 years). Primary outcome: Prevalence of crescent-shaped LV cavity with reversed septal curvature (p=NS).

January 1, 2006Open Access

Morphologic Characteristics of Hypertrophic Cardiomyopathy of the Elderly With Cardiac Myosin-Binding Protein C Gene Mutations

Q: What are the key findings of this study?

An abnormal crescent-shaped left ventricular cavity was similarly prominent in both elderly (75%) and young (95%) patients with hypertrophic cardiomyopathy caused by MyBPC mutations.

Q: What does this research mean for the field?

An abnormal crescent-shaped left ventricular cavity is similarly prominent in both elderly and young patients with hypertrophic cardiomyopathy caused by MyBPC mutations. Novelty: ClaimNovelty.CONTRADICTORY. Consensus alignment: ConsensusAlignment.CHALLENGES_CONSENSUS.

Key Result

An abnormal crescent-shaped left ventricular cavity was similarly prominent in both elderly (75%) and young (95%) patients with hypertrophic cardiomyopathy caused by MyBPC mutations.

Study Design

Type

Observational (n=27)

Multicenter

Structured PICO

Do elderly patients with HCM caused by MyBPC gene mutations have different morphologic characteristics compared to young patients?

Population

27 patients with hypertrophic cardiomyopathy (HCM) caused by cardiac myosin-binding protein C (MyBPC) gene abnormality, divided into an elderly group (>= 65 years of age, n=8) and a young group (< 65 years of age, n=19).

Comparator

Young patients (< 65 years of age) with HCM caused by MyBPC gene abnormality

Outcome

Morphologic characteristics including maximum LV wall thickness, Wigle score, and presence of crescent-shaped LV cavitysurrogate

Elderly patients with HCM caused by MyBPC mutations frequently exhibit an abnormal crescent-shaped LV cavity similar to young patients, despite having milder LV hypertrophy.

Main Result

Absolute Event Rate: 75% vs 95%

p-value: p=NS

Limitations

The number of study patients was small.
Gene testing was performed only for mutations of MyBPC and other genes encoding sarcomeric proteins were not analyzed.

Abstract

BACKGROUND: Several morphologic distinctions between elderly and young patients with hypertrophic cardiomyopathy (HCM) have been reported. In particular, a crescent-shaped left ventricular (LV) cavity with reversed septal curvature, which is often seen in young patients, is rare in elderly patients. However, those studies were carried out before gene testing became available and heterogeneous causes or age-related changes may have been included. The purpose of this study was to determine the morphologic characteristics of elderly patients with HCM definitely caused by a mutation in the cardiac myosin-binding protein C (MyBPC). METHODS AND RESULTS: Twenty-seven patients with HCM caused by MyBPC gene abnormality were evaluated. Patients were divided into an elderly group (> or = 65 years of age, n = 8) and a young group (< 65 years of age, n = 19). LV hypertrophy was milder in the elderly than in the young for maximum LV wall thickness (18 +/- 5 mm vs 24 +/- 6 mm, p = 0.008) and Wigle score (5.7 +/- 1.5 vs 7.6 +/- 1.6, p < 0.005). However, an abnormal crescent-shaped LV was similarly prominent in both groups (75% in the elderly vs 95% in the young, p = NS). None of the elderly patients showed a proximal septal bulge. CONCLUSIONS: An abnormal crescent-shaped LV cavity was frequently present in the elderly as in the young when there are MyBPC mutations. It is possible that this morphologic feature could become useful for determining the etiology of HCM in elderly patients.

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