Author + information
- Ashish H. Shah1,
- Andrew Leventhal1,
- Yvonne Bach1,
- Mark Osten1,
- William Wilson1,
- Lee Benson2 and
- Eric M. Horlick1
Platypnea - orthodeoxia syndrome (POS) is a rare disorder characterized by dyspnea and hypoxia, precipitated by upright position with relief of symptoms in a supine position. Abnormal intra-cardiac right-to-left shunting (RLS) can occur either through a patent foramen ovale (PFO) or an atrial septal defect (ASD). Percutaneous closure of such a defect relieves the symptoms and is accepted as the treatment of choice. Septal characteristics, device type to close the defect, and the underlying mechanisms remain poorly explained.
Fifty-two patients were treated with percutaneous closure of an interatrial communication after presentation with POS at the Toronto General Hospital between January 1997 and July 2015. Their clinical, procedural, and outcomes data were analyzed. Septal/device angulation to the midline was calculated in patients who underwent a cardiac/thorax CT post-procedure, and it was compared with patients who underwent PFO closure for a non-POS indication.
All patients had a PFO; however, nearly a quarter of the patients required a change device from a PFO to a non-PFO closure device to achieve optimal PFO occlusion. Interestingly, none of the patient undergoing PFO closure for non-POS indications (n=886) required a change of device to achieve a satisfactory result. Patients requiring non-PFO device were characterized by an aneurysmal septum, shorter membranous primum septal overlap with secundum septum, and higher septal angulation with the midline. Post PFO closure, all patients demonstrated improvement in their oxygen saturation and the majority reported symptomatic relief. No major adverse events occurred during the procedure or during follow-up.
All the patients presenting with POS due to RLS through a PFO were successfully treated with percutaneous intervention using a variety of devices aimed at achieving complete occlusion of the defect. Those requiring a non-PFO device had a higher prevalence of an aneurysmal septum, and shorter membranous primum septal overlap with the secundum septum. An improvement in oxygen saturation was recorded in all patients, despite some having residual flow. The majority of patients had excellent intermediate and long-term results.