Department of Pediatrics, Kyushu Hospital, Japan Community Healthcare Organization, 1-8-1, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka, 806-8501, Japan.
Department of Cardiovascular Surgery, Kyushu Hospital, Japan Community Healthcare Organization, Kitakyushu, Japan.
Pediatr Cardiol. 2022 Feb;43(2):435-442. doi: 10.1007/s00246-021-02740-9. Epub 2021 Sep 24.
This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.
这项回顾性队列研究旨在探讨个体化策略在患有肺动脉闭锁伴完整室间隔（PAIVS）患者中的长期疗效。我们分析了生存和再干预率，并确定了 1979 年至 2019 年期间根据个体右心结构接受治疗的 PAIVS 患者的结局的危险因素。本研究纳入了 95 例患者。肺动脉瓣环、三尖瓣环和右心室舒张末期容积的 z 评分分别为-3.30（-15.15 至 1.83）、-0.70（-4.65 至 2.33）和-1.51（-6.35 至 1.18）。15%的患者存在右心室依赖性冠状动脉循环。在首次尝试双心室策略的 63 例患者中，55 例患者实现了双心室循环，3 例患者实现了单心室半循环，4 例患者围手术期死亡。在首次尝试单心室策略的 33 例患者中，10 例患者在 Fontan 手术完成前死亡，17 例（48%）患者完成了 Fontan 手术，5 例患者等待 Fontan 手术。1 例患者转为双心室循环。在 720 人年的随访期间，双心室循环患者的 20 年生存率显著高于单心室循环患者（93% vs. 67%，P<0.001）。双心室循环患者的 20 年免于再干预率显著低于单心室循环患者（29% vs. 72%，P<0.001）。双心室循环患者的肺动脉瓣环 z 评分是再干预的独立危险因素。双心室循环患者具有可接受的生存率，但再干预率较高。同时，单心室循环患者在完成 Fontan 手术前死亡率较高，尽管再干预率相对较低。
