Uncommon Coronary Abnormalities in a Patient with Discoid Lupus Erythematosus

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

Publication History

Article published online:
12 September 2019

• References

• 1 Moder KG, Miller TD, Tazelaar HD. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc 1999; 74 (03) 275-284
• 2 Tincani A, Rebaioli CB, Taglietti M, Shoenfeld Y. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus. Rheumatology (Oxford) 2006; 45 (04) (Suppl. 04) iv8-iv13
• 3 Maisch B, Ristić AD. Immunological basis of the cardiac conduction and rhythm disorders. Eur Heart J 2001; 22 (10) 813-824
• 4 Yoshikai M, Hamada M, Takarabe K. Coronary artery aneurysm with systemic lupus erythematosus. Jpn J Thorac Cardiovasc Surg 2004; 52 (08) 379-382
• 5 Famularo G, Vallone A, Compagnucci M, Capo G, Minisola G, Gasbarrone L. Tiny bubbles: coronary aneurysms and lupus. Am J Med 2014; 127 (10) 933-935
• 6 Sumino H, Kanda T, Sasaki T, Kanazawa N, Takeuchi H. Myocardial infarction secondary to coronary aneurysm in systemic lupus erythematosus. An autopsy case. Angiology 1995; 46 (06) 527-530
• 7 Hirata K, Yagi N, Wake M. et al. Coronary steal due to ruptured right coronary aneurysm causing myocardial infarction in a patient with systemic lupus erythematosus. Cardiovasc Diagn Ther 2014; 4 (04) 333-336