Lirong Yan1, Zhimin Wang, Zhimin Xu, Yishi Li, Yongkang Tao, Chaomei Fan. 1. Department of Ultrasound, Cardiovascular Institute and FuWai Hospital, Key Laboratory of Clinical Trial Research in Cardiovascular Drugs Ministry of Health, Beijing, China.
Abstract
BACKGROUND: Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare form of hypertrophic cardiomyopathy (HCM), originally described in Japan and later in the West. Limited information is available on this disease in China. HYPOTHESIS: This study was designed to describe clinical features and prognoses of patients with AHCM in China. METHODS: A retrospective study of 208 consecutive patients with AHCM examined at FuWai Hospital was performed. Clinical features, mortality, and cardiovascular morbidity were analyzed. RESULTS: The 208 patients with AHCM represented 16.0% of all HCM patients. Among them, 64.4% were pure form and 35.6% were mixed form. Compared with the pure group, the mixed group had a significantly larger left atrial diameter and thicker apical thickness. One hundred ninety-nine patients had a mean follow-up of 8.0 ± 3.5 years, cardiovascular mortality was 1.0%, and annual cardiovascular mortality was 0.1%. The 2 cardiovascular deaths were both mixed form. The probability of survival was 97.0 ± 2% at 10 years. Of the patients, 17.8% had 1 or more cardiovascular events. The probability of survival without morbid events at 10 years was 77 ± 4%. Three independent predictors of cardiovascular morbidity were identified: age at diagnosis ≥60 years, left atrial diameter ≥36 mm, and New York Heart Association class ≥III at baseline. CONCLUSIONS: The prevalence of AHCM is relatively high, and it has a benign prognosis in China. However, 17.8% of patients may develop cardiovascular events. It is important to distinguish the 2 phenotypes of AHCM; the mixed form is less common but more serious than the pure form.
BACKGROUND: Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare form of hypertrophic cardiomyopathy (HCM), originally described in Japan and later in the West. Limited information is available on this disease in China. HYPOTHESIS: This study was designed to describe clinical features and prognoses of patients with AHCM in China. METHODS: A retrospective study of 208 consecutive patients with AHCM examined at FuWai Hospital was performed. Clinical features, mortality, and cardiovascular morbidity were analyzed. RESULTS: The 208 patients with AHCM represented 16.0% of all HCM patients. Among them, 64.4% were pure form and 35.6% were mixed form. Compared with the pure group, the mixed group had a significantly larger left atrial diameter and thicker apical thickness. One hundred ninety-nine patients had a mean follow-up of 8.0 ± 3.5 years, cardiovascular mortality was 1.0%, and annual cardiovascular mortality was 0.1%. The 2 cardiovascular deaths were both mixed form. The probability of survival was 97.0 ± 2% at 10 years. Of the patients, 17.8% had 1 or more cardiovascular events. The probability of survival without morbid events at 10 years was 77 ± 4%. Three independent predictors of cardiovascular morbidity were identified: age at diagnosis ≥60 years, left atrial diameter ≥36 mm, and New York Heart Association class ≥III at baseline. CONCLUSIONS: The prevalence of AHCM is relatively high, and it has a benign prognosis in China. However, 17.8% of patients may develop cardiovascular events. It is important to distinguish the 2 phenotypes of AHCM; the mixed form is less common but more serious than the pure form.
Authors: Aurore Lyon; Rina Ariga; Ana Mincholé; Masliza Mahmod; Elizabeth Ormondroyd; Pablo Laguna; Nando de Freitas; Stefan Neubauer; Hugh Watkins; Blanca Rodriguez Journal: Front Physiol Date: 2018-03-13 Impact factor: 4.566