Quantification of end diastolic forward flow in two cases with pulmonary atresia with intact ventricular septum

Similar to patients with repaired tetralogy of Fallot, patients with repaired pulmonary atresia with intact ventricular septum may need a reintervention at a later stage. Although the role of MRI in the long-term follow-up of patients with repaired tetralogy of Fallot has been established, the same has not been established for patients with repaired pulmonary atresia with intact ventricular septum. To define this role, we quantified the end-diastolic forward flow by fractioning it by the total flow of the main pulmonary artery in two cases during their long-term follow up after biventricular repair. In case 1, a male patient had hepatic congestion and a high end-diastolic forward flow fraction and underwent surgical take down to one and one-half ventricle repair at the age of 18 years. In case 2, a female patient, currently 13 years old, has an increasing end-diastolic forward flow fraction. She is under close observation as a potential candidate for one and one-half ventricle repair in the near future. Both patients had a high end-diastolic forward flow fraction of the total right ventricle output, suggesting that end-diastolic forward flow fraction may become a possible become a possible indicator of the adequacy of biventricular repair and the optimal timing for re-intervention.