Pulmonary arterial hypertension remains a progressive, life-threatening condition despite advances in medical treatments. We report the first case of the interventional creation of a reverse Potts shunt by stenting a closed ductus arteriosus in a four-year-old child with right ventricular failure due to suprasystemic pulmonary arterial hypertension associated with Von Hippel-Lindau disease, unresponsive to triple anti-pulmonary arterial hypertension therapy. Following the procedure, the patient’s clinical status and echocardiographic systolic and diastolic right ventricular function improved.