Lee Sang-Yun, Song Mi-Kyoung, Kim Gi-Beom, Bae Eun-Jung, Kim Seong-Ho, Jang So-Ick, Cho Sung-Kyu, Kawk Jae-Gun, Kim Woong-Han, Lee Chang-Ha, Kim Hyun-Jeong, Kim Jayoun
Department of Pediatrics, Seoul National University Hospital, 101, Daehak-ro, Jongno-gu, Seoul, Republic of Korea.
Department of Pediatrics, Sejong General Hospital, Bucheon-si, Republic of Korea.
Pediatr Cardiol. 2019 Dec;40(8):1584-1590. doi: 10.1007/s00246-019-02190-4. Epub 2019 Aug 31.
Because Fontan circulation does not have a subpulmonary ventricle, the preload is limited. In Fontan circulation with extracardiac conduit, the size of conduit could be an important factor in determining the preload. We compared exercise capacity with each conduit size and tried to search for optimal conduit size in Fontan circulation. We reviewed the medical record of 677 patients with Fontan circulation. Patients who had other type Fontan circulation (Kawashima, atriopulmonary, lateral tunnel), SpO < 85%, protein losing enteropathy, results of inappropriate exercise test were excluded. As a result, 150 patients were enrolled and classified according to conduit size. We compared with their exercise capacity and analyzed correlation between exercise capacity and conduit size per body surface area (BSA). 97 Males were included and mean age was 17.5 ± 5.1 years old. In cardiac catheterization, central venous pressure (CVP) was 12.4 ± 2.5 mmHg and pulmonary vascular resistance was 1.2 ± 0.5 wu m. In cardiopulmonary exercise test, predictive peak VO2 was 59.1 ± 9.7% and VE/VCO was 36.2 ± 6.9. In analysis using quadratic model, impacts of gender, age at Fontan operation, ventricular morphology, isomerism, and fenestration on exercise capacity were excluded and conduit size per BSA had a significant curved correlation with predictive peak VO and VE/VCO. Our results showed that patients with about 12.5 mm/m conduit per BSA have the best exercise capacity. Patients with larger than smaller-sized conduit were found to be more attenuated in their ability to exercise.
由于Fontan循环没有肺下心室,前负荷受到限制。在采用心外管道的Fontan循环中,管道大小可能是决定前负荷的一个重要因素。我们比较了不同管道大小的运动能力,并试图寻找Fontan循环中的最佳管道大小。我们回顾了677例Fontan循环患者的病历。排除了患有其他类型Fontan循环(川岛型、心房肺型、侧隧道型)、SpO < 85%、蛋白丢失性肠病以及运动试验结果不恰当的患者。结果,150例患者被纳入研究,并根据管道大小进行分类。我们比较了他们的运动能力,并分析了运动能力与每体表面积(BSA)管道大小之间的相关性。纳入97名男性,平均年龄为17.5 ± 5.1岁。在心脏导管检查中,中心静脉压(CVP)为12.4 ± 2.5 mmHg,肺血管阻力为1.2 ± 0.5 wu m。在心肺运动试验中,预测的峰值VO2为59.1 ± 9.7%,VE/VCO为36.2 ± 6.9。在使用二次模型的分析中,排除了性别、Fontan手术时的年龄、心室形态、异构和开窗对运动能力的影响,每BSA的管道大小与预测的峰值VO和VE/VCO有显著的曲线相关性。我们的结果表明,每BSA约12.5 mm/m管道的患者运动能力最佳。发现管道尺寸大于或小于该尺寸的患者运动能力更弱。