Palacio Andres M, Williams William G, Barron David J, Argo Madison B, Jegatheeswaran Anusha, Jacobs Marshall L, Bondarenko Igor, Welke Karl F, Kirklin James K, Karamlou Tara, Alsoufi Bahaaldin, McCrindle Brian W
Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Department of Surgery, University of Wisconsin Hospital and Clinics, Madison, WI, USA.
World J Pediatr Congenit Heart Surg. 2025 Mar;16(2):254-261. doi: 10.1177/21501351241286441. Epub 2024 Nov 18.
BackgroundTricuspid atresia (TA) is the second most common form of functionally univentricular heart. For patients with TA and normally related great arteries (Type I), left ventricular outflow tract obstruction (LVOTO) is rare.MethodsFrom the Congenital Heart Surgeons' Society multi-institutional cohort of 445 patients with Type I TA enrolled from 1999 to 2024 from 42 sites, 14 infants (3%) had interventions for associated LVOTO, either at presentation or after their first TA-related intervention.ResultsOf seven infants initially undergoing Norwood/Damus-Kaye-Stansel (DKS), six survived to Stage II, of whom five survived with one developing pulmonary hypertension and four achieving Fontan. An additional seven infants who were first managed with pulmonary artery band placement subsequently had bidirectional superior cavopulmonary anastomosis (BCPA) and a DKS procedure; there were six survivors, all achieving Fontan. All ten survivors who underwent the Fontan procedure had normal left ventricular and mitral valve function at the latest follow-up. The overall Kaplan-Meier survival estimate at 20 years for these 14 patients was 79% (70% CI, 66%-88%), and the median follow-up was 8.3 years (0.24-21.5).ConclusionsWhile infants with TA and transposition of the great arteries are more likely to have LVOTO, this can also occur in the setting of normally related great arteries. Infants with Type I TA and LVOTO can be managed in the neonatal period with the Norwood procedure ensuring complete arch relief with acceptable outcomes. If LVOTO becomes evident after initial pulmonary artery band placement, a subsequent DKS procedure facilitates satisfactory success to Fontan.
背景
三尖瓣闭锁（TA）是功能性单心室心脏的第二常见形式。对于患有TA且大动脉关系正常（I型）的患者，左心室流出道梗阻（LVOTO）很少见。
方法
从先天性心脏外科医生协会多机构队列中选取了1999年至2024年期间从42个地点招募的445例I型TA患者，其中14例婴儿（3%）因相关的LVOTO在就诊时或首次TA相关干预后接受了干预。
结果
最初接受诺伍德/达姆斯-凯伊-斯坦塞尔（DKS）手术的7例婴儿中，6例存活至二期，其中5例存活，1例出现肺动脉高压，4例完成了Fontan手术。另外7例最初接受肺动脉环扎术治疗的婴儿随后进行了双向腔肺吻合术（BCPA）和DKS手术；有6例幸存者，均完成了Fontan手术。所有接受Fontan手术的10例幸存者在最近一次随访时左心室和二尖瓣功能正常。这14例患者20年的总体Kaplan-Meier生存估计为79%（70%CI，66%-88%），中位随访时间为8.3年（0.24-21.5）。
结论
虽然患有TA和大动脉转位的婴儿更有可能出现LVOTO，但在大动脉关系正常的情况下也可能发生。患有I型TA和LVOTO的婴儿在新生儿期可以通过诺伍德手术进行治疗，确保完全解除主动脉弓梗阻，取得可接受的结果。如果在最初的肺动脉环扎术后LVOTO变得明显，随后的DKS手术有助于顺利成功完成Fontan手术。
