Stroke due to Eisenmenger's syndrome

Medical mystery NEJM 355:23 Dec 7 2006 p. 2493

Case
35 year old woman developed a sudden inferolateral visual field deficit in the left eye. Two days earlier she took a 16 hour plane trip. Opthalmoscopy showed a left macular band of opacification that was diagnostic of a branch retinal artery occlusion, also shown on fluroscein angiography. The patient had digital erythema, cyanosis and severe clubbing of the hands and feet. Auscultation showed a SEM at the RUSB. The HCt was 57.4.

The syndrome is associated with a VSD and PDA. Anticoagulation was instituted.

The clubbing and polycythemia suggest chronic hypoxemia. The long plane flight and murmur suggest a paradoxical embolus.