A new palliative surgical technique for high risk Total anomalous pulmonary venous connection (Sarmast-Takriti shunt)

BACKGROUND: Total Anomalous Pulmonary Venous Connection (TAPVC) is a rare heterogeneous condition That accounting for 1.5–3% of congenital heart diseases. It is characterized by failure of the Pulmonary Venous Confluence (PVC) to be directly connected to the left atrium in combination with a persistent splanchnic connection to the systemic venous circulation. The most critical status occurs when it is accompanied by pulmonary venous obstruction. Managing of this situation is very difficult and in fact, pulmonary venous obstruction is usually lethal. The real aim of this study is offering a new palliative surgical technique (Sarmast – Takriti Shunt) in order to alleviate the patient’s signs and symptoms until becomes ready for the main surgical correction. CASE PRESENTATION: The study included a 4–day old, low birth weight boy who suffered from Critical Obstructive Total Anomalous Pulmonary Venous Connection. The decision was made to perform the new palliative technique using Gore - Tex (ePTFE). Anastomosis was established without Cardiopulmonary Bypass (CPB) between Pulmonary Venous Confluence (PVC) and the left atrial appendage. Therefore the Sarmast – Takriti Shunt (STS) was taken place. CONCLUSION: After completion of the procedure, the pressure gradient across the venous confluence and the Left innominate vein became zero. Cyanosis, agitation and feeding Problem subsided. Three days later, when he was discharged, arterial oxygen saturation had reached as high as 91%. After 7 months we perfomed the main correction.