Celiac Cardiomyopathy

Abstract

Purpose: Celiac disease (CD) is an autoimmune condition primarily affecting the small intestine with a prevalence of 1 percent in the general U.S. population. Gluten in wheat provokes a reaction in genetically predisposed individuals resulting in villous loss and mucosal damage. In addition to malabsorption and diarrhea, CD is often associated with diverse extra intestinal manifestations. We hereby present a patient with CD and non-ischemic dilated cardiomyopathy (NIDCM) in whom cardiac disease had progressed despite standard medical therapy but initiation of a gluten free diet led to improvement in cardiac function. A 66-year-old male with hypertension, hypercholesterolemia and family history of coronary artery disease presented with a history of pre-syncopal episodes. He denied tobacco or alcohol use. His vitals and physical exam were normal. In evaluation, an echocardiogram showed mild left ventricular enlargement with global left ventricular hypokinesis, with an estimated ejection fraction of 25%. Exercise stress test was negative for ischemia. Holter monitor showed frequent isolated ventricular premature beats without sustained tachyarrythmias, prolonged pauses or heart block. Patient was started on a beta-blocker and an ACE inhibitor. Serial echocardiograms persistently showed a low ejection fraction and he was referred to undergo an internal cardiac defibrillator (ICD) placement. A few months before the ICD placement, he underwent an evaluation for diarrhea. Serologic testing revealed a tisse transglutaminase (tTG) antibody level of 120 (normal<20). The diagnosis of CD was confirmed with duodenal biopsies and he started a strict gluten free diet. Just prior to ICD placement, a repeat echocardiogram showed an improved ejection fraction and ICD placement was deferred. Another echocardiogram (3 months after diagnosis of celiac disease and concurrent with a drop in Ttg antibody levels to 31) showed high normal LV size, normal LV wall thickness, and low normal LV function (50-55%) without wall motion abnormalities. There is good evidence for the association of CD with multiple extra intestinal complications including NIDCM. The prevalence of CD in patients with NIDCM has been reported to be from 1.9% to 5.3%. Possible mechanisms include nutritional deficiencies and increased intestinal permeability leading to increased systemic absorption of luminal antigens followed by formation of antibodies against myocardial transglutaminase proteins that are highly homologous to those found in the small intestine. While the exact pathogenesis of celiac disease related cardiomyopathy remains to be elucidated, it is reasonable to consider the diagnosis of celiac disease in patients with otherwise unexplained NIDCM.