Association between celiac disease and idiopathic dilated cardiomyopathy: a case report

Abstract

for the persistence at home of watery diarrhoea and abdominal pain over the previous 10 days.For these symptoms, he had taken rifaximine and loperamide without benefit. He denied fever, blood or mucus in the stools. He had neither family history of inflammatory bowel disease, nor had travelled abroad over the prior months. In his medical history, in 1997 an idiopathic dilated cardiomyopathy was diagnosed when the patient was admitted to the hospital for stroke. Since then he had taken warfarin, along with digoxin, ramipril, furosemide, lansoprazole; he reported intolerance to beta blockers.Upon admission, the patient was conscious and co-operative, blood pressure 100/60 mmHg, heart rate 90 beats/ min., and normal O 2 saturation. Clinical examination was substantially normal; except for the presence of bilateral leg edema. BMI was 24. We performed an ECG ( Fig. 1) that showed normal sinus rhythm with left bundle branch block, and a short run of ventricular tachycardia; an X-ray of the abdomen was normal, whereas a chest radiography revealed ''cardiomegaly'' with initial apical flow distribution.Biochemistry showed PTL 496,000/mm 3 , the prothrombin time exceeding the therapeutic range, severe protein deficiency with 1.85 g/dl albumin, elevation of CRP (3.56 mg/dl), mild hypokalemia, severe iron deficiency (lowered serum iron and serum ferritin without transferrin elevation).Mild rehydration and albumin supplementation were started during the initial days, then replaced by total parenteral nutrition. DiagnosisDr. Boldrini, Dr. Romagnoli: Thyroid function was normal and stool culture negative; both tissue transglutaminase (100 U/ll) IgA endomysial antibodies were positive, with total serum IgA in the normal range.In order to perform upper gastrointestinal (UGI) endoscopy, warfarin was discontinued and replaced by enoxaparin: UGI endoscopy showed appreciable reduction with scalloped configuration; histopathology examination documented partial villous atrophy with increased number of intra-epithelial lymphocytes (Lesion 3A, Marsh Classification).Echocardiography showed severe dilatation and global hypokinesis of left ventricle (ejection fraction 25%) with functional mitral insufficiency, severe left atrial enlargement, presence of ventricular dyssynchrony.Twelve hours after the upper GI endoscopy the patient presented confusion and dysarthria: a head CT scan showed an acute ischemic lesion in the left cerebellar hemisphere whereas carotid Doppler scan was normal. The symptoms disappeared on the following day. The patient was discharged after 20 days with a diagnosis of ''celiac disease with severe malnutrition, cerebellar ischemic stroke (probably cardioembolic) in patient with idiopathic dilated cardiomyopathy''.

Cardiovascular involvement in celiac disease

“…Dilated cardiomyopathy may evolve due to carnitine deficiency[ 126 , 127 ], which is related to CD, but may also develop after onset of the GFD, particularly in patients lacking carnitine supplementation[ 126 ]. Idiopathic dilated cardiomyopathy may have an autoimmune mechanism[ 110 , 128 ]. The tTG-positive serology in relatives with echocardiographic abnormalities suggests that immune-mediated mechanisms are at work in subsets of these patients and their families[ 106 ].…”

“…The GFD has been shown to have a beneficial effect on cardiac performance in CD patients with dilated cardiomyopathy[ 132 ]. After start of the GFD, abnormal left ventricular dimensions, and diminished cardiac function, including decreased ejection fraction, can improve markedly and may even be completely reversible[ 103 , 108 , 118 , 128 , 133 ]. After two years on a GFD, patients presenting with dilated cardiomyopathy associated with CD show progressive increase in mean serum carnitine levels as compared to values observed prior to the diet[ 127 ].…”

Celiac Disease and Autoimmune-Associated Conditions

“…CD causes abnormalities of intestinal permeability, leading to an increased systemic absorption of various luminal antigens and infectious agents, which may cause myocardial damage through immune-mediated mechanisms. Myocardial injury can also be attributed to immune response cross-reactivity against antigens present in the small intestine and the myocardium [180, 182, 183]. …”

Cardiac syncope in a woman with undiagnosed celiac disease

“…These include nutritional deficiencies, abnor- mal intestinal permeability increasing the absorption of luminal antigens, and a direct autoimmune reaction targeting antigens present in both the myocardium and the small intestine. 12 A gluten-free diet is the mainstay of treatment in classic celiac disease. Patients usually experience near total resolution of gastrointestinal symptoms within weeks to months.…”