A 24-year-old female with no significant past medical history presented with progressive exercise intolerance.
Further diagnostic evaluation revealed a sinus venosus defect (SVD) associated with partial anomalous pulmonary venous connection (PAPVC) of the right upper (RUPV) and right middle pulmonary vein (RMPV) draining into the superior vena cava (SVC), as well as a persistent left superior vena cava (PLSVC).
Transesophageal echocardiography (TEE) demonstrated a significant left-to-right shunt with right atrial and right ventricular enlargement, while biventricular systolic function was preserved. Given the cranial insertion of both anomalous pulmonary veins, a Warden procedure was planned.
Following cannulation of the right internal jugular vein and the femoral vessels, a right anterior minithoracotomy was performed and a camera port was inserted. The pericardium was opened, stay sutures were placed, and the vessels were carefully dissected and mobilized.
Before initiating cardiac arrest, the SVC and the right atrial appendage (RAA) were marked at the intended anastomotic site to ensure proper orientation and to avoid torsion or tension following reconstruction. The superior and inferior vena cava were both snared with tourniquets.
After aortic cross-clamping, cardiac arrest was achieved with antegrade cardioplegia, and the right atrium was opened. To ensure unobstructed pulmonary venous drainage, the atrial septum was incised, enlarging the atrial septal defect to nearly twice its original size.
The SVC was transected proximal to the entry of the RUPV, and the distal caval stump was closed with an autologous pericardial patch. The anomalous pulmonary veins were then baffled through the enlarged ASD into the left atrium using a bovine pericardial patch.
Finally, the anastomosis between the proximal SVC and the right atrial appendage was performed.
The right atrium was then closed, and the patient was successfully weaned from cardiopulmonary bypass.
Intraoperative TEE demonstrated no residual atrial septal defect and no evidence of obstruction or elevated pressure gradients across either the SVC–RAA anastomosis or the intracardiac baffle.
The patient was extubated in the operating room and transferred to the intensive care unit without catecholamine support. The postoperative course was notable for severe and persistent nausea and vomiting, which prolonged the patient’s stay on the general ward. The patient was ultimately discharged home on postoperative day 10.
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