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.
This is a 1-month-old male diagnosed with transposition of big vessels, treated with ASA and carvedilol; he was sent to a multispecialty hospital when he was 11 years old. Here, a CT angiogram was donde revealing an ambiguous situs with levocardia, two bilobed lungs, left liver, right stomach, right polysplenia, interruption of the IVC with hemiazygos continuation, four pulmonary veins reached the left atrium, presenting two SVC that reached the RA and IVC interruption with hemiazygos continuation to the left SVC. An ASD was observed, the left atrium communicated with a small and inferiorly situated ventricle, the RA communicated with a larger ventricle of superior situation, with an interventricular communication that can be taken as a single ventricle, transposition of great vessels, the aorta in front of the pulmonary artery. The suprahepatic veins drained directly with a common trunk to the RA. ().\nWith these findings, a pulmonary artery–left vena cava anastomosis was performed; his postoperative course was uneventful and was discharged home.\nFour years later, the patient consulted because of cyanosis, growth retardation and pulse oximetry of 70%; therefore, a second surgery was decided after right SVC ligation pulse oximetry raised to 90%.