Minimally Invasive Repair of Sinus Venosus Defect With PAPVC in an Adult Patient

The patient was a 43-year-old man with a background in endurance sports who presented with exertional chest pain. His medical history included a cervical disc herniation in 2020, anxiety disorder, and depression. Computed tomography angiography (CTA) revealed a peripheral pulmonary embolism and a sinus venosus defect (SVD) with partial anomalous pulmonary venous connection (PAPVC), with the right upper pulmonary vein (RUPV) draining into the superior vena cava (SVC). Transesophageal echocardiography (TEE) demonstrated a hemodynamically significant left-to-right shunt with right atrial (RA) and right ventricular (RV) enlargement. Biventricular systolic function was normal.

Following standard femoral cannulation and cannulation of the right internal jugular vein, a right axillary minithoracotomy was performed. After the pericardium was opened, the RUPV was identified and carefully dissected. The aorta was cross-clamped, and cardiac arrest was achieved using antegrade cardioplegia. The right atrium was then opened, stay sutures were placed, and the anatomy was inspected. The fossa ovalis was incised, and a vent was inserted.

A bovine pericardial patch was used to redirect the RUPV through the atrial septal defect (ASD) into the left atrium. To prevent stenosis of the SVC, the vessel was incised laterally, away from the sinus node, and enlarged with a second bovine pericardial patch. Following completion of the proximal suture line, the heart was deaired and the aortic cross-clamp was removed. The rest of the suture line was completed on a beating heart.

The right atrium was closed, and the patient was successfully weaned from cardiopulmonary bypass. Intraoperative TEE demonstrated no residual ASD and unobstructed pulmonary venous drainage without gradients in the SVC or the intra-atrial baffle. The patient was extubated in the operating room and transferred to the intensive care unit in sinus rhythm and without catecholamine support. After an uneventful postoperative course, the patient was discharged home on postoperative day seven.

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