Department of Pediatrics, Institution for Clinical Sciences. Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Department of Pediatric Cardiology, 59564Skåne University Hospital, Lund, Sweden.
World J Pediatr Congenit Heart Surg. 2021 Jan;12(1):27-34. doi: 10.1177/2150135120958641.
Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years.
A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group).
The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group ( = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) = .0010.
The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.
患有肺动脉闭锁伴完整室间隔的患儿对儿科心脏病专家来说是一个挑战。我们的目的是研究瑞典 36 年间所有患有肺动脉闭锁伴完整室间隔的患儿在形态学方面的生存率变化。
这是一项基于病历和超声心动图检查的回顾性描述性研究，研究对象为 1980 年至 1998 年（早期组）和 1999 年至 2016 年（晚期组）出生的患儿。
该队列由 171 例患儿组成（早期组 86 例，晚期组 85 例），发病率分别为每 10 万活产儿 4.35 例和 4.46 例。早期组患儿的 1 年生存率为 76%，而晚期组为 92%（ =.0004）。对于膜性闭锁的患儿，1 年生存率从 78%增加到 98%，而对于肌性肺动脉闭锁的患儿，生存率从 68%增加到 85%。对于肌性肺动脉闭锁伴冠状动静脉交通的患儿，生存率没有显著提高。死亡的危险因素包括出生于早期时间段（危险比 HR 6；95%可信区间（2.33-14.28）.0002）、出生体重低（HR 1.26；95%可信区间（1.14-1.4） <.0001）和肌性肺动脉闭锁（HR 3.74；95%可信区间（1.71-8.19）.0010）。
在研究期间，肺动脉闭锁伴完整室间隔的发病率保持不变。生存率有所提高，尤其是对于膜性肺动脉闭锁的患儿，而肌性肺动脉闭锁仍然是死亡的危险因素。为了进一步提高生存率，需要更加关注肌性肺动脉闭锁伴冠状动静脉交通的患儿。
