Introduction: Isolated hypoplasia of the right ventricle is a rare congenital disease whose prevalence is not known in specialized literature, isolated cases being re-ported in literature. This condition is usually associated with foramen ovale communication or ostium secundum septal defect (ASD).
Case presentation: We present the case of a 7-year-old patient who addressed the pediatric cardiology cabinet for evaluation in the context of cyanosis and palpitations. The patient was fully evaluated: clinically, ecocardiographically, by nuclear magnetic resonance. The evaluations have demonstrated 11 mm atrial septal defect with bidirectional shunt, increased end-diastolic left ventricular volume compared with right ventricle (RV), 28 ml/m² versus 60 ml/m², small end diastolic RV 26 mm diameter, RV peak does not reach the apex, diastolic dysfunction, decrease of the the right ventricular ejection fraction to 41%, TAPSE 19 mm. The patient was addressed to surgery to perform partial cavo-pulmonary anastomosis (superior vena cava connected to the right pulmonary artery), closure of the ASD leaving a 4 mm fenestration. The postoperative progression was favourable, keeping the patient asymptomatic at 12 months after surgery with good and stable echocardiographic parameters. RVEF improved from 40% to 50%, obviously without changes in the right ventricle size.
Conclusions: This therapeutic attitude, the partial cavo-pulmonary anastomosis associated with the reduction of the ASD size, aims to prevent progressive and continuous deterioration of the right ventricular systolic function.