Dumitru Ruxandra, Matsubara Muneaki, Schaeffer Thibault, Osawa Takuya, Palm Jonas, Niedermaier Carolin, Piber Nicole, Heinisch Paul Philipp, Ruf Bettina, Hager Alfred, Ewert Peter, Hörer Jürgen, Ono Masamichi
Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.
Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität München, Munich, Germany.
Interdiscip Cardiovasc Thorac Surg. 2025 Mar 29;40(4). doi: 10.1093/icvts/ivaf072.
This study aimed to investigate the incidence of diaphragm paralysis and its impact on outcomes after the Fontan procedure in patients with single ventricles.
Patients undergoing staged Fontan palliation between 1994 and 2023 were reviewed. Patients who had diaphragm paralysis were identified, and the impact of diaphragm paralysis on outcomes after Fontan completion was evaluated.
Among 601 patients who underwent staged Fontan completion during the study period, diaphragm paralysis was observed in 79 patients (13.1%) before Fontan (33 after stage I palliation and 46 after Glenn) and in 32 patients (5.3%) after the Fontan. Among 111 patients with diaphragm paralysis, 77 had spontaneous recovery, 13 recovered after plication and 21 remained without recovery. Patients with diaphragm paralysis before the Fontan demonstrated higher pulmonary arterial pressure (median 10 vs 9 mmHg, P = 0.045) and lower pulmonary artery symmetry index (median 0.54 vs 0.59, P = 0.046) than those without diaphragm paralysis. The use of an autologous pericardial patch in stage 1 was a risk factor for diaphragm paralysis development (odds ratio: 2.61, P = 0.012). Diaphragm paralysis was associated with an increased risk of protein-losing enteropathy (hazard ratio: 2.31, P = 0.003), particularly in patients without recovery after plication (hazard ratio: 4.85, P = 0.031).
Diaphragm paralysis following Fontan completion significantly increases the risk of protein-losing enteropathy and long-term mortality, particularly in patients who fail to recover after plication. Early identification and appropriate management of diaphragm paralysis may be crucial for optimizing outcomes.
本研究旨在调查单心室患者在Fontan手术术后膈肌麻痹的发生率及其对预后的影响。
回顾了1994年至2023年间接受分期Fontan姑息手术的患者。确定患有膈肌麻痹的患者，并评估膈肌麻痹对Fontan手术完成后预后的影响。
在研究期间接受分期Fontan手术完成的601例患者中，79例（13.1%）在Fontan手术前出现膈肌麻痹（I期姑息手术后33例，Glenn手术后46例），32例（5.3%）在Fontan手术后出现膈肌麻痹。在111例膈肌麻痹患者中，77例自发恢复，13例在折叠术后恢复，21例仍未恢复。Fontan手术前出现膈肌麻痹的患者比未出现膈肌麻痹的患者表现出更高的肺动脉压（中位数10 vs 9 mmHg，P = 0.045）和更低的肺动脉对称指数（中位数0.54 vs 0.59，P = 0.046）。在第1阶段使用自体心包补片是膈肌麻痹发生的危险因素（比值比：2.61，P = 0.012）。膈肌麻痹与蛋白丢失性肠病风险增加相关（风险比：2.31，P = 0.003），特别是在折叠术后未恢复的患者中（风险比：4.85，P = 0.031）。
Fontan手术完成后膈肌麻痹显著增加蛋白丢失性肠病风险和长期死亡率，特别是在折叠术后未恢复的患者中。早期识别和适当处理膈肌麻痹可能对优化预后至关重要。
