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Totally Endoscopic Repair Of Common Atrium With Partial Atrioventricular Canal And Unroofed Coronary Sinus In Severe Pulmonary Hypertension
Xiaohua Li, Zhanhao Su, Jie Jian, Miao Tian.
Guangdong Provincial People's Hospital, Guangzhou, China.
BACKGROUND: Adults with unrepaired complex congenital heart disease complicated by long-standing severe pulmonary arterial hypertension (PAH) and exercise capacity pose major challenges in operability assessment and perioperative risk control. A treat-and-repair strategy using PAH-targeted therapy may improve hemodynamics sufficiently to permit definitive correction; however, surgical risk remains substantial, and minimally invasive endoscopic repair in this setting is rarely reported.
METHODS:A 28-year-old male patient with common atrium, partial atrioventricular septal defect (partial AVSD), and unroofed coronary sinus (UCS) presented with severe PAH and cyanosis (resting SpO
2 89%) with limited functional capacity (WHO functional class level 3). The patient was deemed not suitable for repair at first presentation, and received triple PAH-targeted therapy for 6 months and underwent repeat clinical and hemodynamic reassessment for operability (PVR 5.88 Wood units→1.44 Wood units; Qp:Qs 0.74→1.68). Thereafter, a single-stage totally endoscopic repair was performed on cardiopulmonary bypass with femoral cannulation, including atrial septation using bovine pericardial patch, AV valve repair (modified double-orifice technique), and tailored intra-atrial venous baffle rerouting procedure for UCS.
RESULTS: The procedure was completed totally endoscopically without conversion (CPB: 218 min; aortic cross-clamp: 142 min). Intraoperative and early postoperative echocardiography demonstrated effective septation without residual shunt, satisfactory AV valve competence, and unobstructed systemic venous and coronary sinus drainage. The postoperative course was uneventful and the patient was discharged home ten days after surgery, with early improvement in oxygenation and functional status. Follow-up at three months and six months after surgery showed minimal left AV regurgitation and mild PAH.
CONCLUSIONS: This case highlights key technical challenges and solutions in a treat-and-repair strategy for severe PAH in adult CHD patients and demonstrates the feasibility and safety of totally endoscopic repair for complex congenital anatomy after PAH-targeted therapy.
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