Two-Stage Procedure for Pulmonary Vascular Obstructive Disease in Down Syndrome With Congenital Heart Disease

Background Down syndrome patients are characterized by early progression of pulmonary vascular obstructive disease because of insufficient thickness of the pulmonary arterial media. For those with congenital heart disease (CHD) associated with pulmonary hypertension (PH), a 2-stage procedure of pulmonary artery banding (PAB) and then intracardiac repair (ICR) in early infancy is performed to prevent such pulmonary vascular diseases in early infancy. Methods and Results The subjects were 16 patients with Down syndrome who underwent lung biopsy during PAB and ICR. PAB was planned to be performed in early infancy and ICR approximately 1 year later. Efficacy of the 2-stage procedure was retrospectively examined with reference to pulmonary vascular disease and pulmonary diseases. The index of pulmonary vascular disease at PAB fulfilled the indication for ICR, and it was significantly lower at ICR than at PAB (p=0.0469); furthermore, PAB prevented progression of pulmonary diseases. Conclusions The results of the lung biopsies support the safety of the 2-stage procedure and show that it is effective for Down syndrome patients with CHD and PH.