Sakamoto Takahiko, Nagashima Mitsugi, Hiramatsu Takeshi, Matsumura Goki, Park In-Sam, Yamazaki Kenji
Division of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan
Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
Asian Cardiovasc Thorac Ann. 2016 Oct;24(8):765-771. doi: 10.1177/0218492316667771. Epub 2016 Aug 24.
This study aimed to evaluate Fontan circulation which was observed over 30 years.
Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2-32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6).
Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL, and Holter monitoring revealed sinus rhythm in 10 patients. New York Heart Association class was I in 12 patients, II in 6, and III in 2.
For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important.
本研究旨在评估观察30年以上的Fontan循环。
对1974年至1986年间接受Fontan手术且存活（S组，n = 20）或晚期死亡（LD组，n = 20）的40例患者进行评估。手术时的中位年龄为10岁（范围2 - 32岁）。诊断为三尖瓣闭锁21例，单心室9例，其他10例。Fontan手术方式为右心房 - 肺动脉吻合术2例，心房 - 肺动脉连接术28例，右心房 - 右心室吻合术10例（Björk术式4例，移植术6例）。
晚期死亡原因包括充血性心力衰竭6例，猝死4例，心律失常4例，其他6例。16例患者在Fontan手术后23.3±6.5年接受了再次手术。在1个月和12年时进行了心导管检查。两组之间的中心静脉压、心室射血分数或肺血管阻力无显著差异。然而，心室舒张末期容积的变化提示LD组存在容量和压力超负荷。在S组，最新的胸部X线片显示心胸比率为51.3%±7.6%，氧饱和度为94%±2%，脑钠肽为153±111 pgċmL，动态心电图监测显示10例患者为窦性心律。纽约心脏协会心功能分级I级12例，II级6例，III级2例。
对于Fontan手术的长期存活，及时进行全腔静脉 - 肺动脉连接转换以及使用药物降低心室容量和压力负荷可能很重要。
