Bieling Frederike, Cesnjevar Robert A, Cuomo Michela, Weigelt Annika, Dittrich Sven, Purbojo Ariawan
Department of Pediatric Surgery, Friedrich-Alexander-University Erlangen/Nürnberg, University Hospital Erlangen, Erlangen, Germany.
Department of Pediatric Cardiac Surgery, University Children's Hospital Zurich-Eleonorenstiftung, Zurich, Switzerland.
Thorac Cardiovasc Surg. 2025 Jan;73(S 03):e21-e30. doi: 10.1055/a-2531-3126. Epub 2025 Mar 28.
Left superior vena cava (LSVC)-related obstruction of mitral inflow is a rare condition in patients with complex cardiac anomalies like hypoplastic left heart complex. We report on the impact of establishing LSVC to right superior vena cava (RSVC) continuity on the growth of borderline hypoplastic left ventricular structures as an addendum to our previously published work.Twenty-two patients underwent LSVC to RSVC anastomosis, whereas six had LSVC ligation ( = 4) or clip closure ( = 2), all alongside congenital heart defect correction. The indication was LSVC-related obstruction of left ventricular inflow due to a dilated coronary sinus. Clinical data were systematically reviewed, with regular follow-up. Left ventricular end diastolic diameters (LVEDD), aortic valve diameters, and left ventricular outflow tract (LVOT) diameters were recorded using echocardiography.Follow-up showed 90% survival at 3.3 ± 0.4 years. Mean LVEDD Z-scores improved from -2.19 ± 0.35 to -1.24 ± 0.26 after repair ( < 0.01) and to -1.33 ± 0.56 at 6-month follow-up. In patients without mitral repair, LVEDD Z-scores improved from -2.11 ± 0.62 preoperatively to -1.85 ± 0.88 postoperatively ( < 0.05). LVOT Z-scores increased from -2.49 ± 0.48 to -0.87 ± 0.75 ( < 0.05) and aortic valve Z-scores improved from -1.08 ± 0.57 to 0.5 ± 0.39 over 24 months ( < 0.05).Anatomical LSVC correction may improve left ventricular filling and growth of the left ventricle, aortic valve, and LVOT in patients with borderline left ventricles and could be considered without as a potential recruitment strategy.
左上腔静脉（LSVC）相关的二尖瓣流入道梗阻在诸如左心发育不全综合征等复杂心脏畸形患者中是一种罕见情况。作为我们之前发表工作的补充，我们报告建立LSVC与右上腔静脉（RSVC）连续性对临界性左心室发育不良结构生长的影响。22例患者接受了LSVC至RSVC吻合术，而6例患者进行了LSVC结扎（n = 4）或夹闭（n = 2），所有这些均与先天性心脏缺陷矫正同时进行。指征为由于冠状窦扩张导致的与LSVC相关的左心室流入道梗阻。系统回顾临床数据并进行定期随访。使用超声心动图记录左心室舒张末期直径（LVEDD）、主动脉瓣直径和左心室流出道（LVOT）直径。随访显示3.3±0.4年时生存率为90%。修复后平均LVEDD Z评分从 -2.19±0.35改善至 -1.24±0.26（P < 0.01），6个月随访时为 -1.33±0.56。在未进行二尖瓣修复的患者中，LVEDD Z评分从术前的 -2.11±0.62改善至术后的 -1.85±0.88（P < 0.05）。LVOT Z评分在24个月内从 -2.49±0.48增加至 -0.87±0.75（P < 0.05），主动脉瓣Z评分从 -1.08±0.57改善至0.5±0.39（P < 0.05）。解剖学上的LSVC矫正可能改善临界性左心室患者的左心室充盈以及左心室、主动脉瓣和LVOT的生长，可作为一种潜在的治疗策略加以考虑。
