Surgical Management of Univentricular Heart with Total Anomalous Pulmonary Venous Drainage and Intrapulmonary Vertical Vein

Abstract

We report a patient with univentricular heart, heterotaxy syndrome with total anomalous pulmonary venous connection, and an intrapulmonary vertical vein draining into the left superior vena cava who underwent a successful bidirectional Glenn shunt without cardiopulmonary bypass. A polytetrafluoroethylene tube interposition graft was used between the superior vena cava and the pulmonary artery. This condition is unusual and physiologic palliation, rather than anatomic correction as described in this report, may be appropriate for managing this condition.

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

“…In 16 patients with left isomerism in Tokyo [12], actuarial survival was 93.8% at 4 months and remained constant. In 11 out of the 16 patients with right isomerism, actuarial survival was 81.8% at 1 month, 63.6% at 6 months and 53.0% at 1 year.…”

Anomalous Pulmonary Venous Connection With an Intraparenchymal Course

“…This finding, or failure to fully delineate the pulmonary venous anatomy, should prompt the use of another imaging modality. Two of the 22 cases 8,9 were diagnosed by computed tomography (CT) and three others by CMRI (two of our cases and one reported by Wang 5 ). Angiography has also been used 10 but low pulmonary vein flow due to obstruction can result in poor visualization.…”

“…We found 19 previously reported cases of anomalous pulmonary venous connection with an intraparenchymal course, 1 –12 including our three 3 makes a total of 22 cases (Table 1). In three of these cases, there was a luminal connection between the pulmonary veins and the left atrium or a common atrium, with some flow of pulmonary venous blood into the atrium.…”

Functionally Univentricular Heart With Right Atrial Isomerism and Mixed Total Anomalous Pulmonary Venous Drainage

“…However, even in the presence of a short length of SVC with an intrapulmonary vertical vein, interposition of a homograft or prosthetic graft may be helpful, as described earlier by us. 8,9 This patient thus demonstrates a rare instance of right atrial isomerism and mixed total anomalous pulmonary venous drainage, which did not require neonatal palliation and could be successfully managed at surgery beyond the first year of life. Postoperative management with pulmonary vasodilators appears to have been helpful.…”

“…However, even in the presence of a short length of SVC with an intrapulmonary vertical vein, interposition of a homograft or prosthetic graft may be helpful, as described earlier by us. 8,9…”