Long-term outcomes in adults with repaired tetralogy of Fallot and pulmonary atresia. (10th August 2019)

• Record Type: Journal Article
• Authors: Egbe, Alexander C.; Crestanello, Juan; Dearani, Joseph A.; Osman, Karim; Banala, Keerthana; Angirekula, Mounika; Najam, Maria; Ammash, Naser M.
• Is Part Of: Cardiology in the young. Volume 29:Number 8(2019)
• Page Start: 1078
• Page End: 1081
• Publication Date: 2019-08-10
• Subjects: Tetralogy of Fallot, -- pulmonary atresia, -- mortality, -- re-operation; Pediatric cardiology -- Periodicals; 618.9212
• DOI: 10.1017/S1047951119001598
• Languages: English
• ISSNs: 1047-9511

Abstract

Abstract: Background: There are limited outcome data in adults with tetralogy of Fallot and pulmonary atresia. The purpose of this study was to describe re-operations and all-cause mortality in adults with tetralogy of Fallot and pulmonary atresia. Methods: Retrospective review of adults with repaired tetralogy of Fallot and pulmonary atresia who received care at the Mayo Adult Congenital Heart Disease Clinic, 1990–2016. All-cause mortality was calculated as events per 100 patient-years from the time of first presentation to the Adult Congenital Heart Disease Clinic. Results: Of the 221 patients, the age at initial tetralogy of Fallot repair was 6 (5–13) years, and the age at first presentation to the clinic was 27 – 8 years. All patients had at least one right ventricular to pulmonary artery conduit re-operation. There were 31 deaths (14%) at mean age of 41 – 14 years. The causes of death were end-stage heart failure (n = 17), sudden cardiac death (n=9), post-operative death after cardiac surgery (n = 2), sepsis with multi-system organ failure (n = 2), and unknown (n = 1). All-cause mortality rate was 1.7 per 100 patient-years. The risk factors for all-cause mortality were older age (>12 years) at the time of repair (hazard ratio 1.41, 95 confidence interval 1.06–2.02, p = 0.033), non-sustained ventricular tachycardia (hazard ratio 1.36, 95 confidence interval 1.17–2.47, p = 0.015), and left ventricular ejection fraction <50% (hazard ratio 1.39, 95 confidence interval 1.08–2.31, p = 0.031). Conclusion: Based on a review of 221 adults with repaired tetralogy of Fallot and pulmonary atresia, all patients had re-operations and all-cause mortality rate was 1.7 events per 100 patient-years. The current study provides important outcomes data for risk stratification in adults with tetralogy of Fallot and pulmonary atresia.