Use of superior vena cava-right pulmonary artery anastomosis in congenital heart disease with decreased pulmonary blood flow.

Clinical, hemodynamic, and angiocardiographic findings on eight long-term (more than 3 years) survivors of anastomosis of the superior vena cava (SVC) to the right pulmonary artery (RPA) are described. All patients had severe cyanotic congenital heart disease with decreased pulmonary blood flow. Postoperative cardiac catheterization and angiocardiography defined the physiology of the shunt. SVC pressure was elevated after the creation of the SVC-RPA shunt. This waswithout obvious clinical effect in six patients. Mild SVC syndrome developed in the immediate postoperative period but was associated with overall clinical improvement in two cases. A late, and more severe SVC syndrome associated with clinical deterioration developed in three patients with severe pulmonic stenosis as part of their cardiac malformation. Clinical improvement and relief of SVC syndrome followed systemic-pulmonary artery shunt or open heart repair of the underlying cardiac malformation, leaving the SVC-RPA anastomosis intact. Clinical improvement following open heart repair of tetralogy of Fallot has persisted in spite of severe postoperative pulmonic valve insufficiency and elevated mean pressure in the left pulmonary artery. This is believed to be a result of the SVC-RPA shunt, which reduced the venous return into the right heart.