Department of Pediatrics (Cardiology) Stanford University Palo Alto CA.
Department of Pediatrics (Cardiology) Medical College of Wisconsin Milwaukee WI USA.
J Am Heart Assoc. 2020 Apr 7;9(7):e015871. doi: 10.1161/JAHA.119.015871. Epub 2020 Mar 19.
Abstracts Because of remarkable advances in survival over the past 40 years, the worldwide population of individuals with single ventricle heart disease living with Fontan circulation has grown to ≈70 000, with nearly half aged >18 years. Survival to at least 30 years of age is now achievable for 75% of Fontan patients. On the other hand, single ventricle patients account for the largest group of the 6000 to 8000 children hospitalized with circulation failure, with or without heart failure annually in the United States, with the highest in-hospital mortality. Because there is little understanding of the underlying mechanisms of heart failure, arrhythmias, pulmonary and lymphatic vascular abnormalities, and other morbidities, there are no specific treatments to maintain long-term myocardial performance or to optimize overall patient outcomes.
摘要 由于过去 40 年来在生存率方面取得了显著进步，患有单心室心脏病并接受 Fontan 循环治疗的全球患者人数已增长至 ≈70000 人，其中近一半患者年龄>18 岁。目前，75%的 Fontan 患者可存活至少 30 年。另一方面，在美国每年因循环衰竭住院的 6000 至 8000 名儿童中，单心室患者占比最大，其中有或没有心力衰竭，住院死亡率最高。由于对心力衰竭、心律失常、肺血管和淋巴血管异常以及其他合并症的潜在机制了解甚少，因此没有特定的治疗方法可以维持长期心肌功能或优化整体患者预后。
