Abstract
A 43-year-old male presented with shortness of breath and palpitations. Physical examination
was significant for skin lichenification, an erythematous maculopapular rash with
annular plaques, a fissured tongue, and digital clubbing. Electrocardiogram captured
a supraventricular tachycardia and right bundle branch block. Left heart catheterization
found several proximal left coronary artery branch aneurysms, a right coronary–pulmonary
artery fistula, venous–luminal vessels between the right heart chambers and cardiac
veins, and thebesian venous lakes. Functional cardiac capacity was reduced, with left
ventricular ejection fraction of 35 to 40%, on ventriculogram. A diagnosis of discoid
lupus erythematosus was established after skin biopsies revealed systemic lupus erythematosus-like
features but negative anti-nuclear, anti-Smith, anti-ds-deoxyribonucleic acid, anti-Ro,
and anti-La antibodies. The case reported is unique, in that while our patient had
pathologic electrophysiologic changes typically seen with autoimmune disease, the
patient had many anatomical cardiac anomalies without atherosclerotic coronary disease.
It is uncertain whether these findings are purely incidental or are related to an
underlying genetic disorder.
Keywords
coronary aneurysm - discoid lupus erythematosus - thebesian venous lakes - fistula
- venous–luminal vessels - coronary–pulmonary fistula - coronary steal
