背景:先天性心脏病最严重的终点是单心室生理机能的患者。它们包括一组异质的先天性心脏畸形,其共同特征是心脏形态不适合维持双心室循环。
方法:这里,我们介绍了一个成年患者在Fontan姑息治疗后,说明在随访期间可能遇到的复杂临床过程和广泛的并发症,强调在这些患者的临床护理中需要多学科方法。
结论:在Fontan外科手术期间,下腔静脉与肺循环相连,在上腔静脉与肺动脉循环之前连接后。由此产生的腔肺连接,因此缺乏肺下心室,提供从全身静脉循环进入肺部的贫氧血液的非脉动被动流动,功能性单室将富氧的肺静脉血液泵入主动脉。手术死亡率<5%,目前30年生存率高达85%,Fontan循环患者的成年人口正在增长。这种生存的增加是,然而,不可避免地伴随着影响多器官系统的长期并发症,导致心血管性能下降。
结论:对于最佳治疗,多学科团队的评估是强制性的,利用团队成员的专业知识,及时发现和解决晚期并发症,并支持生活质量。
BACKGROUND: At the most severe end of the spectrum of congenital heart disease are patients with an univentricular physiology. They comprise a heterogeneous group of congenital heart malformations that have the common characteristic that the cardiac morphology is not equipped for sustaining a biventricular circulation.
METHODS: Here, we present a
case of an adult patient after Fontan palliation, illustrative of the complex clinical course and the broad spectrum of complications that can be encountered during follow-up, highlighting the need for a multidisciplinary approach in the clinical care for these patients.
CONCLUSIONS: During the surgical Fontan procedure, the inferior vena cava is connected to the pulmonary circulation, after prior connection of the superior vena cava to the pulmonary arterial circulation. The resulting cavopulmonary connection, thus lacking a subpulmonic ventricle, provides non-pulsatile passive flow of oxygen-poor blood from the systemic venous circulation into the lungs, and the functional monoventricle pumps the oxygen-rich pulmonary venous return blood into the aorta. With an operative mortality of <5% and current 30-year survival rates up to 85%, the adult population of patients with a Fontan circulation is growing. This increase in survival is, however, inevitably accompanied by long-term complications affecting multiple organ systems, resulting in decline in cardiovascular performance.
CONCLUSIONS: For optimal treatment, the evaluation in a multidisciplinary team is mandatory, using the specific expertise of the team members to timely detect and address late complications and to support quality of life.