Successful Fontan procedure for asplenia with pulmonary atresia and major aortopulmonary collateral arteries

Abstract

Asplenia with a single ventricle and major aortopulmonary collateral arteries (MAPCAs) is a rare congenital cardiac anomaly. In this patient group achievement of the Fontan procedure depends on ample rehabilitation of the pulmonary arteries to secure sufficient pulmonary vascular beds and the prevention of progressive pulmonary vascular obstructive disease. Here we report a successful 1-stage bilateral unifocalization of the pulmonary blood supply followed by a staged Fontan procedure in a patient with a univentricular heart and MAPCAs. Clinical Summary The patient was given a diagnosis of asplenia, univentricular heart (right ventricular type), common atrioventricular orifice, pulmonary atresia, bilateral superior vena cavas, total anomalous pulmonary venous connection (intracardiac type), and MAPCAs on the basis of echocardiography and cardiac catheterization. His neonatal aortography showed a small central pulmonary artery (3 mm in diameter) and bilateral multiple MAPCAs (Figure 1, A). The patient’s arterial oxygen saturation (Sa O2) was around 80%, and his pulmonary/systemic blood flow ratio (Qp/Qs) was 1.6 with stable hemodynamics. Subsequent cardiac catheterization at 6 month of age revealed a good-sized central pulmonary artery (Figure 1, B) and 5 large MAPCAs. His SaO2 was approximately 87%, and his Qp/Qs had increased to 3.7. At 1 year of age, he underwent a primary complete unifocalization of his bilateral pulmonary arteries, total anomalous pulmonary venous connection repair for progressive pulmonary venous obstruction (Figure 2), and a left modified Blalock-Taussig shunt (polytetrafluoroethylene [PTFE],* 5 mm). The postoperative course was uneventful except for transient bilateral phrenic nerve palsy. He subsequently did well, while becoming increasingly cyanotic. A catheterization at 2 years of age revealed peripheral pulmonary stenosis, low pulmonary vascular resistance (1.2 Wood units), and normal pulmonary blood flow (Qp/Qs 1.0). The patient then underwent a bilateral bidirectional cavopulmonary shunt and take down of his left modified Blalock-Taussig shunt without cardiopulmonary bypass. This postoperative course was also uneventful, with an SaO2 of 85% and superior vena caval pressure of 10 mm Hg. Eight months after the bilateral bidirectional cavopulmonary shunt, a cardiac catheterization was performed, demonstrating good-shaped pulmonary arteries (Figure 3), low pulmonary vascular resistance (1.4 R*U), low pulmonary artery pressure (mean, 10 mm Hg), and low pulmonary blood flow (Qp/Qs 0.8). Two weeks before definitive surgical intervention, he underwent balloon angioplasty for peripheral pulmonary stenoses and coil embolization of aortopulmonary collateral arteries. At 3 years of age, a fenestrated modified Fontan procedure was performed. An 18-mm PTFE tube graft with a 4-mm PTFE tube graft anastomosed in end-to-side fashion was used for the extracardiac total cavopulmonary connection. The 4-mm PTFE tube graft was anastomosed to the common atrium as a fenestration. The patient was hemodynamically stable intraoperatively, with superior vena caval and inferior vena caval pressures of 11 mm Hg, a common atrial pressure of 4 mm Hg, and an SaO2 of 94%. The patient returned to the cardiovascular intensive care unit in stable condition and was extubated 3 hours after the operation. His postoperative course was uneventful. He was transferred to the general ward on the first postoperative day and discharged home 13 days later without any complications. Three months after the Fontan procedure, another cardiac catheterization was performed, demonstrating a spontaneously occluded tube graft fenestration, an Sa O2 of 96%, low pulmonary artery pressure (mean, 12 mm Hg), and excellent Fontan circulation.