AbdelMassih Antoine Fakhry, Kiraly Laszlo, Badaoui Hazem El, Khan Mohammad, Hetharsi Balazs, Till Judit Noemi, Omelchenko Aleksandr Y, Salah Alaa Ziad, Jburi Farah Tarik Al, Alkhouli Laila, Taher Mina, Alhosani Najah, Youssef Omnia, Iqbal Sumaiya, Allami Zahraa, Jha Neerod Kumar, Hamad Eman Mahmoud, Omar Yasmin, Khan Arshad, Azeez Zafar, Attia Michael, Mina Mariam, Ali Alyaa Al, Afifi Yara Khaled, Shershaby Meryam El, Musleh Afnan
Pediatric Cardiology unit, Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
Pediatric Cardiology Division, Cardiac Sciences' department, Sheikh Khalifa Medical City, Abu Dhabi, UAE.
Glob Cardiol Sci Pract. 2023 Aug 1;2023(3):e202317. doi: 10.21542/gcsp.2023.17.
The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years.
This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation.
Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure ( = 0.012) and the degree of atrio-ventricular valve regurgitation ( = 0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion.
This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.
对于患有单心室心脏的患者，如左心发育不全综合征（HLHS）或其他单心室心脏疾病，Fontan手术是三阶段姑息治疗过程的最后阶段。尽管该手术已被证明对这类病例至关重要，但Fontan循环生理会降低心输出量并升高体静脉压力，进而导致静脉充血，并可能并发蛋白丢失性肠病（PLE）。这项回顾性研究旨在确定过去八年在我们中心（谢赫·哈利法医疗城/SKMC）接受Fontan手术的所有患者中此类并发症的预测因素。
本研究检查了自阿拉伯联合酋长国（UAE）SKMC心脏外科项目启动（2012年1月1日至2020年12月31日）以来在我们中心接受Fontan修复手术的患者的病历。排除标准包括患者档案中缺少以下任何数据。患者分为两组：发生PLE的患者和未发生PLE的患者。对于每组，收集以下数据：人口统计学数据（当前年龄和Fontan手术完成时的年龄）、临床和实验室数据（血氧饱和度、血清白蛋白）、超声心动图数据（原始心脏诊断分类、房室瓣反流程度、心室功能）、血流动力学数据（Fontan手术完成前上腔静脉和肺动脉的平均压力）、手术数据（初始姑息治疗类型、Fontan手术类型、是否存在开窗及其大小）以及Fontan手术完成前是否需要任何心脏干预，如房室瓣修复、外周肺动脉支架置入和主动脉弓球囊扩张。
在纳入的48例患者中，13例（25%）发生了PLE。多变量回归分析证明，PLE的最佳预测因素是上腔静脉平均压力（P = 0.012）和房室瓣反流程度（P = 0.013）。Fontan手术完成前血氧饱和度<83%对预测Fontan手术完成后发生PLE的敏感性为92%。
这是一项关于Fontan手术后PLE预测因素的单中心研究，正如类似研究预期的那样，上腔静脉压力高于11 mmHg和中重度房室瓣反流是Fontan手术失败的预测因素。我们队列中PLE的较高患病率以及可预测并发症的较低上腔静脉压力临界值，可能与手术患者中左心发育不全占主导有关，在过去十年中，该中心一直是阿联酋心脏手术的主要转诊中心。
