A 41-year-old male patient with a history of IVC anomaly and interatrial communication, diagnosed when he was 15 years old, was asymptomatic, with no hemodynamic repercussion and good exercise tolerance. Recently presented syncopal episodes and progressive dyspnea due to moderate pulmonary hypertension; despite clinical treatment with sildenafil, symptoms persisted, so he consulted a cardiothoracic surgeon who requested complementary tests. Echocardiogram reported pulmonary artery pressure of 36 mmHg (on Sildenafil) and a left ventricular ejection fraction of 72%. Angiography showed an anomalous distal venous drainage with an absence of the IVC and the persistence of the azygos vein draining distal blood to the superior vena cava (SVC) ().\nWith these findings, surgical closure of the atrial septal defect (ASD) was decided; however, due to the IVC anomaly, the surgeon had to modify the surgical technique.\nMedian sternotomy and systemic heparinization were performed with extracorporeal circulation cannulating the aorta and SVC directing the tip of this cannula to the azygos vein. Right atrium (RA) was opened, the suprahepatic venous system drained into the atrial cavity, complicating the surgical exposure due to the large amounts of blood draining; therefore, these veins were cannulated from the inside of the RA, leaving a bloodless field (). Chambers were explored, finding a 2-cm ASD and a separate partial anomalous pulmonary venous drainage from the two right pulmonary veins to the RA. The ASD was enlarged with resection of a septal segment; then, correction of the septal communication was performed doing a baffle technique with bovine pericardium creating a tunnel to redirect the flow from the right pulmonary veins to the left atrium (). Finally, he was removed from extracorporeal circulation and was sent to ICU for postoperative care.
A 14-year-old male, without relevant history, consulted the urologist for enuresis and was diagnosed with the right varicocele. CT angiography was performed, showing an IVC anomaly, with partial hypoplasia of the upper segment, persistence of the left SVC to which the left and right renal veins drain, the left renal vein with a retro-aortic segment showing signs of aorto-vertebral impingement, a right dilated and tortuous testicular vein ending in the right renal vein and continuation of the azygos in the visualized thoracic segment. ().