Sengsim Jantaras, Vijarnsorn Chodchanok, Chanthong Prakul, Chungsomprasong Paweena, Kanjanauthai Supaluck, Thammasate Ploy, Pacharapakornpong Thita, Chaiwangyen Nalin, Bositthipichet Densiri, Soongswang Jarupim, Tocharoenchok Teerapong, Nitiyarom Ekarat, Tantiwongkosri Kriangkrai, Subtaweesin Thaworn, Durongpisitkul Kritvikrom
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Rd., Bangkok, 10700, Thailand.
Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Sci Rep. 2024 Dec 2;14(1):29891. doi: 10.1038/s41598-024-81834-9.
Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. We aimed to investigate the survival of adults who had PAH-CHD with predominantly left-to-right (L-R) shunts with (1) borderline-to-high PVR and (2) treat-and-repair compared with those with Eisenmenger syndrome (ES). From 1995 to 2021, 99 adults with ES (age 34.1 ± 11.2 years) and 118 adults with PAH-CHD with predominantly L-R shunts (age 39.1 ± 13.7 years) were eligible. The PVR in the ES group was 21.0 ± 13.1 WU. Most ES patients (97%) received pulmonary vasodilator therapy. Among the 118 patients with PAH-CHD with predominantly L-R shunts, the baseline PVR was 7.6 ± 4.6 WU, and 78 patients (66.1%) had borderline to high PVR (≥ 5 WU). In the group, 105 patients (88.9%) underwent repair; 84 had defect closure, and 21 had fenestrated closure. Treat-and-repair was used to treat 53 patients with a preoperative final PVR of 3.58 ± 2.63 WU. No early postoperative deaths were reported. At a median follow-up time of 5.4 years (range 0.1-23.6 years), the 10- and 15-year survival rates of adults with borderline PVR were 82.3% and 82.3%, respectively, which were not inferior to the rates for patients with ES, which were 77.8% and 71.2%, respectively (p = 0.41). The survival rate of patients who underwent treat-and-repair was slightly better than that of patients who underwent ES, although the difference was not statistically significant (p = 0.19). Independent mortality risk factors were functional class III-IV at initial presentation (hazard ratio 5.7, 95% CI 1.2-26.6; p = 0.02) and oxygen saturation < 94% at the most recent visit (hazard ratio 9.4, 95% CI 2.1-42.9; p = 0.004).Trial registration: TCTR20200420004.
先天性心脏病相关肺动脉高压（PAH-CHD）是未修复的大型体肺分流的结果。关于患有肺动脉高压且肺血管阻力（PVR）升高的PAH-CHD成年患者的长期数据有限。我们旨在研究主要为左向右（L-R）分流且具有（1）临界至高PVR和（2）治疗并修复的PAH-CHD成年患者与艾森曼格综合征（ES）患者相比的生存率。1995年至2021年，99名ES成年患者（年龄34.1±11.2岁）和118名主要为L-R分流的PAH-CHD成年患者（年龄39.1±13.7岁）符合条件。ES组的PVR为21.0±13.1伍德单位。大多数ES患者（97%）接受了肺血管扩张剂治疗。在118名主要为L-R分流的PAH-CHD患者中，基线PVR为7.6±4.6伍德单位，78名患者（66.1%）的PVR为临界至高（≥5伍德单位）。在该组中，105名患者（88.9%）接受了修复；84例行缺损闭合术,21例行开窗闭合术。53名术前最终PVR为3.58±2.63伍德单位的患者采用了治疗并修复的方法。未报告早期术后死亡病例。在中位随访时间5.4年（范围0.1 - 23.6年）时，临界PVR成年患者的10年和15年生存率分别为82.3%和82.3%，不低于ES患者的生存率，分别为77.8%和71.2%（p = 0.41）。接受治疗并修复的患者的生存率略高于接受ES治疗的患者，尽管差异无统计学意义（p = 0.19）。独立的死亡风险因素为初次就诊时功能分级为III - IV级（风险比5.7，95%置信区间1.2 - 26.6；p = 0.02）和最近一次就诊时氧饱和度<94%（风险比9.4，95%置信区间2.1 - 42.9；p = 0.004）。试验注册号：TCTR20200420004。
