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

Abstract:

BACKGROUND:There 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. METHODS:Twenty 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. RESULTS:Mean 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. CONCLUSIONS:Although 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.

journal_name

Int J Cardiol

authors

John AS,Warnes CA

doi

10.1016/j.ijcard.2011.04.026

subject

Has Abstract

pub_date

2012-11-01 00:00:00

pages

13-7

issue

1

eissn

0167-5273

issn

1874-1754

pii

S0167-5273(11)00373-1

journal_volume

161

pub_type

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