Valved Pulmonary-to-Systemic Shunt for Pulmonary Arterial Hypertension in Two 5-Year-Old Patients

We report the use of a unidirectional-valved pulmonary-to-systemic shunt to manage advanced pulmonary arterial hypertension in two 5-year-old patients. After failure of medical therapy, both patients underwent placement of a valved pulmonary-to-systemic shunt to allow right ventricular unloading at suprasystemic pulmonary pressures. Postoperative imaging demonstrated exclusive, intermittent right-to-left flow through the shunt. Both patients experienced excellent postoperative recovery and were discharged home. Follow-up has demonstrated shunt patency and improved functional status. To our knowledge, these are the youngest patients to have undergone this procedure in the English literature.