Miyamoto Yuya, Kubo Toru, Ochi Yuri, Baba Yuichi, Hirota Takayoshi, Yamasaki Naohito, Kawai Kazuya, Yamamoto Katsuhito, Kondo Fumiaki, Bando Kanji, Yamada Eisuke, Furuno Takashi, Yabe Toshikazu, Doi Yoshinori L, Kitaoka Hiroaki
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University.
Circ J. 2022 Nov 25;86(12):1934-1940. doi: 10.1253/circj.CJ-22-0068. Epub 2022 Jun 7.
The clinical features of heart failure (HF) in patients with hypertrophic cardiomyopathy (HCM) in Japan have not been fully elucidated.
In 293 patients with HCM (median age at registration, 65 (57-72) years) in a prospective cardiomyopathy registration network in Kochi Prefecture (Kochi RYOMA study), HF events (HF death or hospitalization for HF) occurred in 35 patients (11.9%) (median age, 76 (69-80) years), including 11 HF deaths during a median follow-up of 6.1 years. The 5-year HF events rate was 9.6%. Atrial fibrillation, low percentage of fractional shortening, and high B-type natriuretic peptide level at registration were predictors of HF events. The combination of these 3 factors had a relatively high positive predictive value (55%) for HF events and none of them had a high negative predictive value (99%). There were 4 types of HF profile: left ventricular (LV) systolic dysfunction (40%), severe LV diastolic dysfunction (34%), LV outflow tract obstruction (LVOTO) (20%), and primary mitral regurgitation (MR) (6%). HF deaths occurred in patients with LV systolic dysfunction or LV diastolic dysfunction, but none of patients with LVOTO or primary MR due to additional invasive therapies.
In a Japanese HCM cohort, HF was an important complication, requiring careful follow-up and appropriate treatment.
日本肥厚型心肌病（HCM）患者心力衰竭（HF）的临床特征尚未完全阐明。
在高知县前瞻性心肌病注册网络中的293例HCM患者（注册时的中位年龄为65（57 - 72）岁）（高知龙马研究）中，35例患者（11.9%）发生了HF事件（HF死亡或因HF住院）（中位年龄76（69 - 80）岁），包括在中位随访6.1年期间的11例HF死亡。5年HF事件发生率为9.6%。注册时的心房颤动、低射血分数缩短率和高B型利钠肽水平是HF事件的预测因素。这3个因素的组合对HF事件具有相对较高的阳性预测值（55%），且它们均无较高的阴性预测值（99%）。有4种HF类型：左心室（LV）收缩功能障碍（40%）、严重LV舒张功能障碍（34%）、LV流出道梗阻（LVOTO）（20%）和原发性二尖瓣反流（MR）（6%）。LV收缩功能障碍或LV舒张功能障碍患者发生HF死亡，但LVOTO或原发性MR患者因额外的侵入性治疗均未发生HF死亡。
在日本HCM队列中，HF是一种重要的并发症，需要密切随访和适当治疗。
