Partial closure of the atrial septal primum defect and biventricular repair for a case of hypoplastic right ventricle with partial atrioventricular canal defect

An eight-year-old girl with partial atrioventricular canal defect and hypoplastic right ventricle was treated successfully by a palliative open-heart surgery. The preoperative right ventricular pressure was 58/7 mmHg (RVP/LVP = 0.67) and morphology of the right ventricle showed severe tricuspid stenosis and small outflow portion. The preoperative RVEDVI was 31 (41% of normal and tricuspid annulus was 17 mm (47% of normal). The right ventricular outflow was reconstructed with insertion of MVOP and the ASD was partially left open (the amplitude of the interatrial communication was 7 mm). Angiocardiogram two and half years after the operation demonstrated significant right ventricular growth with no right to left shunt through interatrial communication. The RVEDVI was 46 (56% of normal) and tricuspid annulus was 36 mm (90% of normal). This technique can be a procedure of choice in patient with right ventricular hypoplasia, who is not candidate for simple right ventricular reconstruction nor Fontan procedure, as the growth of the right ventricule is expected in the future.