Complex venous malformation in an elderly female: A case report

AbstractA 76-year-old female taking oral medications for chronic atrial fibrillation and hypertension was admitted to our hospital for examination of bilateral leg edema. Transthoracic echocardiography showed an enlarged coronary sinus of about 40mm. Contrast-enhanced computed tomography revealed absence of the left innominate vein, a persistent left superior vena cava, and absence of the hepatic segment of the inferior vena cava. The interrupted inferior vena cava connected with the hemiazygos vein. The left superior vena cava also continued to the hemiazygos vein with reflux to the enlarged coronary sinus. A diagnosis of heterotaxy syndrome associated with polysplenia was made. No other congenital cardiac abnormalities were found. The patient was also diagnosed as having right cardiac failure due to atrioventricular valve regurgitation associated with chronic atrial fibrillation. The bilateral leg edema improved rapidly with oral medications. Although complex venous malformations such as those described above are rarely observed in the elderly, absence of the hepatic segment of the inferior vena cava, as in this case, is a particularly pathognomonic malformation in left isomerism in heterotaxy syndrome