Department of Pediatric Cardiology and Adult Congenital Heart Disease, National Cerebral and Cardiovascular Center.
Circ J. 2018 Feb 23;82(3):840-846. doi: 10.1253/circj.CJ-17-0801. Epub 2017 Dec 29.
Heart failure (HF) is an important complication in adults with congenital heart disease (CHD), but because only a few studies have focused on acute HF hospitalization in adults with CHD, we study aimed to define the clinical characteristics of such patients and examine the differences in acute HF between adults with CHD and acquired heart disease.Methods and Results:We retrospectively evaluated 50 adults with CHD admitted for treatment of acute HF and compared their data with those from Japanese HF registries. Patient mean age was 37±15 years and 58% were male. In total, 86% of the patients had complex forms of CHD and 62% had undergone corrective surgery, including the Fontan procedure; 66% of patients showed right heart hemodynamic abnormality. In-hospital mortality was 4%, which was comparable to the Japanese HF registries. Survival rate was 93% at 1 year and 75% at 3 years, which was similarly poor to the rates of HF secondary to acquired heart disease.
We clarified the clinical characteristics of adults with CHD requiring HF hospitalization. Young adults with complex CHD were hospitalized for management of acute right HF. Short-term and mid-term outcomes were similarly poor compared with acute HF secondary to acquired heart disease.
心力衰竭（HF）是成人先天性心脏病（CHD）的重要并发症，但由于仅有少数研究关注成人 CHD 的急性 HF 住院治疗，因此我们的研究旨在确定此类患者的临床特征，并比较 CHD 患者与获得性心脏病患者的急性 HF 之间的差异。
我们回顾性评估了 50 例因急性 HF 住院治疗的 CHD 成人患者，并将其数据与日本 HF 登记处的数据进行比较。患者平均年龄为 37±15 岁，58%为男性。共有 86%的患者患有复杂型 CHD，62%的患者接受过矫正手术，包括 Fontan 手术；66%的患者存在右心血液动力学异常。院内死亡率为 4%，与日本 HF 登记处相似。1 年生存率为 93%，3 年生存率为 75%，与获得性心脏病引起的 HF 相似。
我们阐明了需要 HF 住院治疗的成人 CHD 的临床特征。患有复杂型 CHD 的年轻成人因急性右心 HF 住院治疗。与获得性心脏病引起的急性 HF 相比，短期和中期预后同样较差。
