Behcet's Disease Unmasked after Heart Transplantation

Abstract

<h3>Introduction</h3> Behcet's disease (BD) is an autoinflammatory, multisystem vasculitis. Cardiac involvement is rare and clinical diagnosis is challenging. We present a case of Behcet's disease diagnosed after heart transplantation (HTx). <h3>Case Report</h3> A 24 year-old female with a history of morbid obesity, unprovoked venous thrombus and nonischemic cardiomyopathy with biventricular systolic dysfunction who presented in cardiogenic shock. Echocardiogram revealed an EF of 10-15%, LVEDD of 4.9cm, LV apical thrombus and severe RV dysfunction. Right heart catheterization showed RA 14 PA 38/23 (29) and wedge of 24 with calculated cardiac index of 1.6 and PAPi of 1.07. She required VA-ECMO and IABP followed by HM3 BiVAD as bridge to HTx. The explanted heart revealed myocyte hypertrophy and severe interstitial fibrosis, no evidence of vasculitis or inflammation. At 10 months post-transplant she presented with new painful oral and genital ulcers. Prednisone at this time was 2.5 mg daily. Complete infectious and viral work-up was negative and there was no resolution with empiric valganciclovir and antibiotics. Pathergy test was positive and treatment for BD was initiated with dapsone 150 mg twice daily and steroids. Two months, later after tapering steroids, she presented with hematochezia. Colonoscopy revealed multiple, deep, clean-based ulcers in the terminal ileum, a classic finding in BD (Figure 1). <h3>Summary</h3> BD presents a diagnostic challenge in the absence of classic clinical findings. Initial presentation is often thrombosis, which was the case in this patient years preceding her severe heart failure. Cardiac involvement is rare and to our knowledge has only been described in case reports with predominantly LV dysfunction. Long term morbidity and outcomes in BD and HTX is not well known. This is the first case reported of BD unmasked after HTx and describing biventricular failure as a possible manifestation of BD.