Valved reverse Potts shunt in a case of pulmonary hypertension due to pulmonary veno-occlusive disease

Idiopathic pulmonary hypertension has a predictably morbid natural history with an absence of a uniformly successful treatment strategy. We describe our palliative surgical strategy in a symptomatic teenager. A 16-year-old girl, with a recent diagnosis of severe suprasystemic pulmonary hypertension, with severe right ventricular dysfunction, presented with syncope and World Health Organization functional class 4 symptoms. Blood and imaging work up revealed changes suggestive of pulmonary veno-occlusive disease. She failed to improve with oral pulmonary vasodilators and was listed for heart and lung transplant. Pending the transplant, a 10-mm handmade valved tube graft was placed between descending thoracic aorta and the proximal left pulmonary artery, on cardiopulmonary bypass. She had an uneventful recovery period with an early improvement in her symptoms. She was discharged home on aspirin and oral pulmonary vasodilators. At last follow up, 4 months post procedure, her functional capacity and right ventricular function had improved. The valved Potts shunt proved to be helpful in improving her symptomatology and as a bridge to transplant.