Patent Ductus Arteriosus Stenting (Transcatheter Potts Shunt) for Palliation of Suprasystemic Pulmonary Arterial Hypertension

Abstract

Idiopathic pulmonary arterial hypertension (IPAH) continues to be a progressive and fatal disease.1 Patients with congenital heart disease and PAH constitute a separate subset with Eisenmenger syndrome where the life expectancy is significantly better than patients with IPAH. Prior surgical series have reported improved survival after converting severe and refractory IPAH into an Eisenmenger physiology using a Potts shunt with direct side-to-side anastomosis.2–4 We describe a similar approach using a transcatheter technique by stenting residual or probe-patent ductus arteriosus (PDA) to establish a stable communication between descending thoracic aorta and left pulmonary artery in patients with severe suprasystemic PAH. During the past 5 years, we have performed cardiac catheterization in 28 IPAH patients and found 4 with a small or probe-patent PDA. One of these patients had infrasystemic PA pressures and did not undergo PDA stenting. The other 3 patients, whose data are summarized in the Table, had the PDA stented. Patient 1 was diagnosed with idiopathic PAH at the age of 6 months. Transthoracic echocardiography at the time of the diagnosis showed an atrial septal defect with left-to-right shunt and a small PDA. He remained stable on oral medications until 9.7 years of age when he presented with recurrent syncope. Severe right ventricular dilatation and bowing of the interventricular septum to the left were noted on transthoracic echocardiogram. Cardiac catheterization confirmed suprasystemic PAH and the presence of a small PDA shunting right-to-left with significant restriction (Figure 1). Figure 1. Angiographic views from the first case. A , Aortography in lateral view, showing …