Warden Procedure via Vertical Right Thoracotomy in a 7-Year-Old Girl

A 7-year-old, 41 kg, girl presented with murmur and evidence of right-sided cardiac chamber enlargement. She was asymptomatic. Echocardiography and cross sectional imaging demonstrated partial anomalous pulmonary venous connection of the right upper and middle lobar pulmonary veins to the superior vena cava (SVC) and superior vena caval/right atrial junction. There was a moderate size superior sinus venosus atrial septal defect. Due to the high insertion of the anomalous pulmonary veins, a Warden procedure with superior vena caval translocation seemed to be appropriate. Through a vertical muscle-sparing right thoracotomy approach, the anomalous pulmonary veins were visualized. The SVC was dissected and all anomalous connections were identified so as the azygous vein. Central cannulation was performed. Cardiopulmonary bypass was initiated at mild hypothermia. After cardioplegic arrest, both cavae were snared and an oblique right atriotomy was created. The sinus venosus defect was enlarged by resecting the rest of the septum primum. An appropriately sized bovine pericardial patch was then used to baffle the anomalous veins to the left atrium via the atrial septal defect, thus closing the superior vena caval entry into the right atrium. The SVC was then transected above the highest anomalous pulmonary vein insertion and its cardiac end was patched to avoid compromising the pulmonary vein ostium due to its close proximity. The cranial end of the SVC was then connected to the right atrial appendage (RAA) after cutting all the trabeculations to ensure no obstruction to the SVC drainage. The anterior wall of the SVC/RAA anastomosis was patched with another bovine pericardial patch. The right atriotomy is then closed and the patient is weaned off cardiopulmonary bypass in the standard fashion. Transesophageal echocardiogram showed widely patent pulmonary venous baffle and no significant gradient across the SVC/RAA anastomosis. A single chest drain was placed in addition to temporary epicardial atrial pacemaker wires. The chest incision was then closed in layers and the patient was extubated in the operating room. The cardiopulmonary bypass time was 187 minutes and the aortic cross clamp time was 89 minutes. The postoperative course was uneventful apart from transient junctional rhythm that recovered prior to discharge. Pre-discharge transthoracic echocardiogram and computed tomography scan confirmed widely patent pulmonary venous baffle, absence of residual shunts