The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair. (1st April 2019)

• Record Type: Journal Article
• Authors: Montanaro, Claudia; Merola, Assunta; Kempny, Aleksander; Alvarez-Alvarez, Belen; Alonso-Gonzalez, Rafael; Swan, Lorna; Uebing, Anselm; Li, Wei; Babu-Narayan, Sonya V.; Gatzoulis, Michael A.; Dimopoulos, Konstantinos
• Is Part Of: International journal of cardiology. Volume 280(2019)
• Page Start: 61
• Page End: 66
• Publication Date: 2019-04-01
• Subjects: PA pulmonary atresia -- VSD ventricular septal defect -- RV right ventricle -- TCPC total cavo-pulmonary connection -- NYHA New York Heart Association -- MAPCAs major aortopulmonary collateral arteries -- PH pulmonary hypertension -- PPM permanent pacemaker -- AICD Automated Implantable Cardioverter-Defibrillator -- AF atrial fibrillation -- AV atrio-ventricular -- VA ventriculo-arterial; Pulmonary atresia -- Adult -- Outcome -- Congenital heart disease -- Cyanosis; Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2018.11.011
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: Objectives: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. Background: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. Methods: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). Results: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure ( n = 21, 42.8%) and sudden cardiac death ( n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups ( p = 0.12) or between repaired and unrepaired patients in Group 1 ( p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. Conclusions: Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care. Highlights: An increasing number of patients born with complex CHD reach adult life. Patients with pulmonary atresia are a clinically heterogeneous population. Mortality and morbidity in adult are high even after surgical repair. Heart failure is a common cause of death and sudden cardiac death remains a concern. Numerous reinterventions are required, especially in those with prior repair.