Paediatric and Congenital Cardiac Service, 36716Starship Children's Hospital, Auckland, New Zealand.
Department of Cardiology, 58991Auckland City Hospital, Auckland, New Zealand.
World J Pediatr Congenit Heart Surg. 2021 Mar;12(2):185-194. doi: 10.1177/2150135120974395.
There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary.
All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle.
A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days ( = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival.
Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.
对于患有完整室间隔的肺动脉闭锁（PAIVS）的婴儿，有许多手术和介入治疗选择。在我们的实践中，我们在新生儿期通过血管造影来确定冠状动静脉瘘和中断，并制定了一种策略，如果需要，可以安全地减轻右心室的压力，并结扎瘘管。
回顾性研究了 1999 年至 2018 年期间年龄<60 天接受 PAIVS 手术的所有婴儿。收集了术前和术后变量，回顾了血管造影，并创建了一个区域评分来分级冠状异常的严重程度。本研究重点关注那些早期进行右心室减压的患者亚组。
共纳入 77 例患者，平均随访 8.6 年。其中 55 例（71%）有冠状动静脉瘘，包括 28 例（36%）有冠状动静脉中断。47 例（60.5%）患者进行了右心室减压（RVD）。接受 RVD 的患者无 30 天死亡率，而未接受 RVD 的患者中有 6 例（20%）在 30 天内死亡（=0.003）。RVD 和非 RVD 的 10 年生存率分别为 97.8%和 73.3%。为了防止冠状动脉窃血，17 例患者在 RV 减压时进行了冠状动静脉瘘结扎，其早期和晚期生存率均为 100%。
如果能够减轻右心室的压力，PAIVS 婴儿的早期和晚期生存率会更好。在选择有大瘘管的患者中，RVD 联合冠状动静脉瘘结扎术已经引入，没有不良后果。
