Clinical outcomes of adult survivors of pulmonary atresia with intact ventricular septum

BackgroundThere are no studies on the long term clinical outcomes and complications in the adult patient with pulmonary atresia with intact ventricular septum (PA/IVS). This study reviews our experience with a limited group of adult survivors of PA/IVS seen in our adult congenital clinics.MethodsTwenty adult patients with PA/IVS (1998 to 2009) were identified from Mayo Clinic adult congenital heart disease databases. Surgical history and clinical outcomes were reviewed.ResultsMean age at last evaluation was 29 years (19–39 years). There were five deaths within the study period (1998–2009). Median age at death was 32 years (30–37 years). Seven patients underwent the Fontan operation, eight patients had a biventricular repair, and five patients remained with palliative shunts. All patients required re-interventions in adulthood. Tricuspid valve (TV) (n = 5), pulmonary valve (PV)/conduit (n = 6), and mitral valve (n = 2) replacements were the most frequent re-intervention in the biventricular repair subset. Atrial arrhythmias were present in 80% of the total cohort, the highest rate among Fontan repairs (n = 7) and biventricular repairs (n = 7). Ventricular arrhythmias occurred in 15% of the cohort.ConclusionsAlthough limited in number, the adult PA/IVS patients in this series continue to have high rates of morbidity and mortality, with arrhythmias and need for re-operations as the major causes. Patients with biventricular repairs had the highest re-intervention rate in adulthood. While this subset of patients might not be representative of all adult PA/IVS survivors, continued follow-up at centers with expertise in adult congenital cardiology is recommended for all patients.