Heart transplantation in a case of cava vein absence

pp. 212-216

Abstract

Absence of the right upper cava vein with persistent left upper cava vein is a rare anomaly of heart venous return. Here we report the case of a patient with dilated cardiomyopathy, functional class IV (NYHA), who underwent orthotopic heart transplantation. Although we discovered this anomaly during the surgery we could perform the procedure without problems. Afterwards we extensively reviewed worldwide case reports and the embryological evolution of this patholy. As there are no hemodynamic alterations in these patients, diagnosis is not easy before surgery.  However there are differents signs (ECG, Rx, ultrasonographic, tomographic and angiographic) that may alert us about the presence of this anomaly preoperatively. The importance of the diagnosis before surgery is that modifications in the procedure of ablation of donor's heart can be performed to allow the reconstruction of right upper vein as a useful structure to obtain postoperatory biopsies and control immunosupression.