Ashraf Syed Faaz, Da Silva Jose Pedro, Castro-Medina Mario, Viegas Melita, Alsaied Tarek, Seese Laura, Morell Victor O, Da Fonseca Da Silva Luciana
Division of Pediatric Cardiothoracic Surgery, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa.
Pittsburgh Children's Hospital Medical Center and Department of Pediatrics, The Heart and Vascular Institute, University of Pittsburgh, Pittsburgh, Pa.
J Thorac Cardiovasc Surg. 2025 Feb;169(2):354-361.e3. doi: 10.1016/j.jtcvs.2024.08.024. Epub 2024 Aug 22.
We present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology.
This is a retrospective database analysis from 2 institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR).
Eleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation at a median age of 27 months (IQR, 20.5 months). All patients were critically ill before their Starnes procedure, and 4 were on extracorporeal membrane oxygenation. Before the Cone repair, the median preoperative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR, 1.3 m/s). During the Cone procedure, 9 patients required a concomitant pulmonary valve repair, of whom 3 needed a transannular monocusp patch. Four patients were successfully rerouted to a 2-ventricle repair, and 7 patients with a previous Glenn achieved 1.5-ventricle circulation. There were no cases of heart block and no deaths. Seven patients had trivial, 3 patients had mild, and 1 patient had moderate tricuspid regurgitation (TR) at a median follow-up of 11 months (IQR, 21.5 months). There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in 1 patient.
After the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TR at a midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single-ventricle physiology.
我们展示了一组患有埃布斯坦畸形（EA）的患者在接受斯塔恩斯手术后进行右心室（RV）康复治疗的病例系列，采用三尖瓣（TV）的圆锥修复术以实现双心室或1.5心室生理功能。
这是一项来自北美两家机构的回顾性数据库分析。我们纳入了所有在斯塔恩斯手术后连续进行圆锥修复的病例。数据以中位数和四分位间距（IQR）表示。
11例患者在2019年至2023年期间，于最初接受斯塔恩斯姑息手术后接受了RV康复治疗，中位年龄为27个月（IQR，20.5个月）。所有患者在接受斯塔恩斯手术前病情都很严重，4例患者接受了体外膜肺氧合治疗。在进行圆锥修复之前，斯塔恩斯补片处术前反流速度的中位数为1.65米/秒（IQR，1.3米/秒）。在圆锥手术过程中，9例患者需要同时进行肺动脉瓣修复，其中3例需要跨环单瓣补片。4例患者成功改道进行双心室修复，7例先前接受格林手术的患者实现了1.5心室循环。没有发生心脏传导阻滞病例，也没有死亡病例。在中位随访11个月（IQR，21.5个月）时，7例患者有轻微三尖瓣反流，3例患者有轻度反流，1例患者有中度三尖瓣反流（TR）。没有明显的TV狭窄；尽管1例患者存在严重的RV功能障碍，但所有患者在最后一次随访时功能状态良好。
在斯塔恩斯手术后，圆锥修复术实现了RV康复，在中期随访时导致轻微或轻度TR。斯塔恩斯手术是一种可重复的技术，不再使患者终身处于单心室生理状态。
