Surgical Outcomes in Patients Undergoing a Double Switch Operation for Corrected Transposition.

BACKGROUND: Congenitally corrected transposition of the great arteries (CC-TGA) is a rare and complex form of congenital heart disease. Results of physiologic repair proved disappointing due to late right ventricular dysfunction and/or tricuspid regurgitation. The current study was performed to evaluate surgical outcomes in patients undergoing a double switch for CC-TGA. METHODS: This was a retrospective review of 121 patients who underwent a double switch over a 2-decade time frame (2002-2023). Patients were a median age of 32 months. Before the double switch, 49 of 121 patients (40%) had undergone left ventricular retraining. RESULTS: Sixty-seven patients underwent an arterial switch, and 54 underwent a Rastelli procedure. There were 4 in-hospital deaths (3.3%), including 3 who had a Rastelli procedure (5.6%) and 1 who had an arterial switch (1.5%). At a median follow-up of 30 months, there were 4 late deaths (2 Rastelli and 2 arterial switch). Combined early and late mortality was 9.3% for the Rastelli and 4.5% for arterial switch. Combined mortality was 2.0% for patients who required left ventricular retraining vs 9.7% for those who did not. For the 117 patients discharged from the hospital, 93% have normal or low-normal left ventricular function, and 96% have mild or less neoaortic insufficiency. CONCLUSIONS: Surgical outcomes in patients undergoing a double switch procedure have been excellent both in the short- and midterm. However, the Rastelli procedure was associated with a more than 2-fold increase in mortality risk compared with the arterial switch.

A Deep Dive Into Retroesophageal Major Aortopulmonary Collateral Arteries.

Background: The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retroesophageal) and bronchus before entering the lung parenchyma. The purpose of this paper was to review the anatomy, physiology, and surgical characteristics of retroesophageal MAPCAs. Methods: This manuscript summarizes the data from a series of three papers that have focused on the subject of retroesophageal MAPCAs from our institution over the past ten years. Results: Two-thirds of patients evaluated had a retroesophageal MAPCA identified at surgery. Retroesophageal major aortopulmonary collateral arteries (REMs) were more common with a left arch (77%) compared with a right arch (53%). Of all REMs evaluated, 83% were single supply, 13% were dual supply with an inadequate connection, and 4% were dual supply with an adequate connection. Based on these findings, 96% of retroesophageal MAPCAs were unifocalized. Follow-up catheterization was performed at a median of 17 months after surgery; 75% of unifocalized MAPCAs were widely patent, 20% were patent but stenotic, and 5% were occluded. Conclusions: The data demonstrate that retroesophageal MAPCAs are relatively common and almost always require unifocalization. At mid-term follow-up, 95% of unifocalized MAPCAs were found to be patent.

Analysis of risk factors associated with extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenoses.

Objective: Acute lung injury is a known complication of pulmonary artery reconstruction for peripheral pulmonary artery stenosis. Severe cases may require support with extracorporeal membrane oxygenation. The purpose of this study was to evaluate the characteristics of patients requiring extracorporeal membrane oxygenation after pulmonary artery reconstruction. Methods: This was a retrospective study of 150 patients who underwent surgical repair of peripheral pulmonary artery stenosis at our institution from 2002 to 2022. Underlying diagnoses included Williams syndrome (n = 44), Alagille syndrome (n = 43), elastin arteriopathy (n = 21), tetralogy of Fallot (n = 21), and other (n = 21). Characteristics of patients who required extracorporeal membrane oxygenation were compared with those who did not require extracorporeal membrane oxygenation. Results: Eleven of the 150 patients undergoing pulmonary artery reconstruction (7.3%) required postoperative extracorporeal membrane oxygenation support (10 for acute lung injury and 1 for cardiac insufficiency). Four patients receiving extracorporeal membrane oxygenation had Williams syndrome, 3 patients had Alagille, and 4 patients had tetralogy of Fallot. Patients requiring extracorporeal membrane oxygenation had a higher preoperative right ventricle to aortic peak systolic pressure ratios (mean 1.14 vs 0.95), greater number of pulmonary artery ostial interventions (median, 23 vs 17), and longer duration of cardiopulmonary bypass (median, 597 vs 400 minutes). There were 3 in-hospital deaths (2.0%), 2 of whom required postoperative extracorporeal membrane oxygenation support. Conclusions: The data demonstrate multiple differences between patients who did and did not require extracorporeal membrane oxygenation after surgical repair of peripheral pulmonary artery stenosis. These results suggest that the preoperative extent of disease may predispose to the development of acute lung injury requiring extracorporeal membrane oxygenation support.

Surgical repair of peripheral pulmonary artery stenosis: A 2-decade experience with 145 patients.

BACKGROUND: Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy. This disease is characterized by stenoses at nearly all lobar and segmental ostia and results in systemic-level right ventricular pressures. The current study summarizes our experience with the surgical treatment of PPAS. METHODS: This was a retrospective review of 145 patients who underwent surgical repair of PPAS. This included 43 patients with Williams syndrome, 39 with Alagille syndrome, and 21 with elastin arteriopathy. Other diagnoses include tetralogy of Fallot with PPAS (n = 21), truncus arteriosus (n = 5), transposition (n = 3), double-outlet right ventricle (n = 2), arterial tortuosity syndrome (n = 3), and other (n = 8). RESULTS: The median preoperative right ventricle to aortic peak systolic pressure ratio was 1.01 (range, 0.50-1.60) which was reduced to 0.30 (range, 0.17-0.60) postoperatively. The median number of ostial repairs was 17 (range, 6-34) and median duration of cardiopulmonary bypass was 398 minutes (range, 92-844). There were 3 in-hospital deaths (2.1%). The median duration of follow-up was 26 months (range, 1-220) with 4 late deaths (2.9%). Eighty-two patients have subsequently undergone catheterization and 74 had a pressure ratio <0.50. CONCLUSIONS: The surgical treatment of PPAS resulted in a 70% reduction in right ventricular pressures. At 3 years, freedom from death was 94% and 90% of those evaluated maintained low pressures. These results suggest that the surgical treatment of PPAS is highly effective in most patients.

Impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.

OBJECTIVES: Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery. METHODS: This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n = 29), tetralogy of Fallot with PPAS (n = 14), tetralogy with PA (n = 3), PA with ventricular septal defect and major aortopulmonary collateral arteries (n = 21) and one each with D-transposition and supravalvar aortic stenosis. RESULTS: The median age at surgery was 16 months (range 0-228 months). Procedures performed included PPAS repair (n = 43), tetralogy with PA repair (n = 3), unifocalization procedures (n = 21) and other (n = 2). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345 min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P < 0.05). CONCLUSIONS: These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery.