Outcome after surgery for pulmonary atresia with ventricular septal defect, a long-term follow-up study

Aim To study the long-term outcome after surgery for pulmonary atresia and ventricular septal defect (PA-VSD), and to determine association between the contribution of major aorto-pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. Methods Patients who had undergone surgery for PA-VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross-checked against the Swedish National Population Register. Results Seventy patients were identified, giving an incidence of 5.3 newborns per 100 000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11-microdeletion syndrome (23%). Survival at follow-up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow-up time of 14.3 years (3.2-41.8 years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease. Conclusion Long-term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome.

Automated summary

The aim of the study was to investigate the long-term outcomes of surgery for pulmonary atresia and ventricular septal defect (PA-VSD), specifically looking at the contribution of major aorto-pulmonary collateral arteries (MAPCAs) to pulmonary blood flow, comorbidities, and causes of death. Retrospective analysis of patients who underwent surgery for PA-VSD between 1994 and 2017 revealed that 59% of patients had pulmonary blood flow originating from a patent ductus arteriosus (PDA), while 41% had contribution from MAPCAs. The study found that long-term survival did not differ between patients with and without MAPCAs, nor was there a difference in mortality based on the presence of syndromes or extracardiac diseases.