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Long-term Follow-up of a Fenestrated Amplatzer Atrial Septal Occluder in Pulmonary Arterial Hypertension^*

Till F. Althoff, MD; Fabian Knebel, MD; Alexander Panda, MD; John McArdle, MD; Volker Gliech, MD; Ines Franke, MD; Christian Witt, MD; Gert Baumann, MD and Adrian C. Borges, MD

^* From the Departments of Cardiology and Angiology (Drs. Althoff, Knebel, Franke, Baumann, and Borges) and Pulmology and Infectiology (Dr. Witt), Charité Universitätsmedizin Berlin, Berlin, Germany; Pulmonary & Critical Care Medicine Section (Drs. Panda and McArdle), Department of Internal Medicine, Yale University School of Medicine, New Haven, CT; and Department of Cardiology (Dr. Gliech), HUMAINE Klinikum Bad Saarow/Fürstenwalde, Bad Saarow, Germany. These authors contributed equally to this work.

Correspondence to: Till F. Althoff, MD, Department of Cardiology and Angiology, Charité Universitätsmedizin Berlin, Campus Mitte, Charité-Platz 1, 10177 Berlin, Germany; e-mail: till.althoff{at}charite.de

Pulmonary arterial hypertension (PAH) is a progressive disease, with right-heart failure being the main cause of death. In patients refractory to conventional drug therapy, atrial septostomy can serve as palliative treatment or as a bridge to transplantation. A 41-year-old woman with a 15-year history of PAH associated with a corrected atrial septal defect presented with severe deterioration of symptoms. Echocardiography confirmed reocclusion of an atrial septal stoma that had been created several months before. After performing a repeat atrial septostomy, we implanted a custom-made atrial septostomy device, an Amplatzer septal occluder that had been fenestrated to serve as a custom-made atrial septostomy device. This resulted in an improvement in cardiac output and a marked symptomatic relief. During the 6-year follow-up, the patient was clinically stable with limited but constant exercise tolerance, under specific medical therapy. Repeated echocardiography confirmed long-term patency of the device.

Key Words: Amplatzer atrial septal occluder • atrial septostomy • atrial septostomy device • pulmonary arterial hypertension