Abstract 20517: Coronary Artery Disease in Eisenmenger Syndrome
Background: A long held maxim in congenital heart disease is that patients (pts) with cyanotic congenital defects do not develop coronary artery disease (CAD) due to hypothetical protective effects of hypocholesterolemia, hypoxemia, indirect hyperbilirubinemia, thrombocytopenia, and upregulation of nitric oxide. We aimed to report the first case series of adult pts with Eisenmenger syndrome (ES) who developed CAD.
Methods: Adult pts with ES were identified by searching the Mayo Clinic medical record from 1990-2016. Clinical and catheterization data were collected and analyzed from those pts with ES and CAD.
Results: Overall, 177 adult pts (58 males) with ES were identified. A total of 86 pts underwent cardiac catheterization of whom, 8 pts (9.3%; 4 males) were diagnosed with CAD at a median age of 55 (31-68) years (left main disease, n=3, two-vessel disease, n=2, one-vessel disease, n=3)(Table). Extrinsic ostial left main coronary artery compression from an enlarged main pulmonary artery was the mechanism of left main disease in 2/3 pts. Two pts suffered ST elevation myocardial infarctions. PCI and stent placement was undertaken 4 pts (bare metal stent, n = 3; drug eluting stent, n = 1). Three pts had premature CAD. One or more traditional risk factors for CAD were present in 88% of pts. All pts were on pulmonary vasodilator therapy. The oldest pt with ES to develop CAD was a 68 year-old male with unoperated single ventricle (double-inlet left ventricle with L-transposition).
Conclusion: In contrast to longstanding dictum, pts with ES can develop CAD. Development of CAD in ES pts may be attributed to longevity secondary to pulmonary vasodilator therapies in addition to acquired CAD risk factors. Pulmonary artery coronary compression may be an unique mechanism of left main disease and may play a role in sudden death of these pts. Assessment for CAD should not be ignored in pts with ES. More study is needed to identify risk factors for CAD in ES pts.
Author Disclosures: J.T. Poterucha: None. N.S. Anavekar: None. A.C. Egbe: None.
- © 2016 by American Heart Association, Inc.