Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.

One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome.The cardiac malformations included dextrocardia,double outlet right ventricle,pulmonary stenosis,interrupted inferior vena cava,hemiazygos continuation and total anomalous pulmonary venous return.One-stage correction was performed.The atrial procedure consisted of intra-and extraatrial rerouting of the anomalous systemic and pulmonary venous return.The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit.The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta.The right ventricular outflow was ultimately remodeled using a valved conduit.For better perception of the complex morphology,a three-dimensional model was designed,using CT scan images.This proved to be very useful for surgical planning,especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.

An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

Abernethy malformation （AM） is a rare congenital anomaly in which the splanchnic blood bypasses the liver and drains directly into the systemic veins.It was first described by Abemethy in 1793 and has two types.Type Ⅰ is characterized by a complete portosystemic shunt and an absence of a portal vein （consists of superior mesenteric and splenic veins）,while Type Ⅱ exhibits partial portosystemic shunt and a hypoplastic portal vein.Type Ⅰ is further subclassified into subtype a and b based on the absence or presence of a patent connection between the superior mesenteric vein and the splenic vein.