Transjugular Balloon Pulmonary Valvuloplasty Through a Bidirectional Glenn Shunt for Dysplastic Pulmonary Valve Stenosis in an 8.7-Year-Old Boy with Inaccessible Femoral Veins

An 8.7-year-old boy was affected by exertional dyspnea with cyanosis of the lip at 6 years old. Oxygen saturation (SpO2) was 66%. A bidirectional Glenn shunt (BGS) was constructed to successfully elevate SpO2 to 88%. Unfortunately, he again experienced exertional dyspnea with flagrant cyanosis of the lip at 8.5 years old. SpO2 decreased to 65%. Echocardiography revealed a dysplastic pulmonary valve with severe stenosis. Considering the potential growth of the right ventricle and the branch pulmonary arteries, transjugular balloon pulmonary valvuloplasty (BPV) through a BGS was performed as a palliative treatment for cyanosis in this boy because of inaccessible femoral veins. After gradational BPV, the opening of the pulmonary valve was dilated from 2.59 mm to 6.65 mm, the pressure gradient decreased from 60 mmHg to 25 mmHg, and the SpO2 increased to 85%. He became physically active and was free of exertional dyspnea at the 12-month follow-up. BGS is irrefutably an alternative vascular access through which transjugular BPV could be performed to ameliorate cyanosis due to dysplastic pulmonary valve stenosis in patients with inaccessible femoral vessels.