P493Long term follow-up after biventricular repair of pulmonary atresia with intact ventricular septum by magnetic resonance imaging

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Purposes: Pulmonary atresia with intact ventricular septum (PAIVS) is readily associated with hypertrophy and fibrosis of the right ventricle. Long term outcome of the right ventricle function and hypertrophy are not known. We sought to determine the right (RV) and left ventricular (LV) function in patients with PAIVS late after biventricular repair.Methods: Magnetic resonance imaging (MRI) assessment of 5 consecutive patients with normally developed right ventricle, at a median time of 12 (9- 24) years after biventricular repair with transannular patch of the right ventricular outflow tract.Results: Both right and left ventricular systolic functions were within normal range. Right ventricular volume was increased in 4 patients at a median range of 111 (107- 179) ml/m2. The pulmonary forward flow was normal in 4 cases (>2.6 l/min/m2). Only one patient had normal right ventricular volume, decreased net pulmonary forward flow (2,3 l/min/m2 ) , extensive sub-endocardial late enhancement of the right ventricle free wall. Two patients had limited infundibular late enhancement. Median pulmonary regurgitation fraction was 34 (30- 45) %. No patient had late atrial enhancement.Conclusions: Although fibrosis and restrictive right ventricular physiology potentially prevent right ventricular dilatation, such condition seems deleterious for pulmonary flow. The optimal timing for valvulation is difficult to determine given the restrictive right ventricular physiology that limits right ventricular dilatation.

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