Extracardiac Venous Heterotaxy Syndrome

Abstract

A 20-year-old man was referred for the assessment of palpitations and associated dyspnea during exertion. He had no other associated cardiovascular symptoms. Physical examination revealed a healthy male with stable vital signs and a normal cardio respiratory examination. ECG, ( Figure 1A), chest x-ray ( Figure 1B), and blood counts as well as electrolyte panel were normal.Transthoracic echocardiography revealed a dilated coronary sinus ( Figure 2); in addition, a persistent left superior vena cava was noted (Figure 3). A small coronary-pulmonary arterial fistula was noted (Figure 4). Two-dimensional subcostal echocardiography view demonstrated absence of the hepatic portion of the inferior vena cava with the hepatic veins draining directly into the right atrium (interrupted inferior vena cava). The right suprasternal view revealed venous flow into the right superior vena cava from a dilated azygous vein ( Figure 5). Left and right ventricles were normal in size and function. From the Mayo Clinic Arizona, Scottsdale, Ariz. The online-only Data Supplement, which includes movies, is available with this article at http://circ.ahajournals.org/cgi/content/full/117/e498/DC1.

A retrospective analysis of cases with left atrial isomerism

“…[1][2][3][4] İki taraflı sol atriyal morfoloji nedeniyle, hemen hemen tüm olgularda inferior kaval ven (İVK)-sağ atriyum bağlantısı eksiktir. [5][6][7] Tek ventrikül fizyolojisine sahip sol atriyal izomerizm olgularında uygulanan ve Kawashima ameliyatı olarak adlandırılan kavopulmoner anastomoz ile hepatik venler dışındaki tüm sistemik venöz dönüş pulmoner artere yönlendirilmektedir.…”