Cardiomyopathy, Idiopathic Dilated

Enlarged heart showing dilated cardiomyopathy at autopsy. 

What Is Idiopathic Dilated Cardiomyopathy?

Idiopathic dilated cardiomyopathy (IDCM) is a disorder of myocardial (heart muscle) function characterized by dilation or enlargement of the cardiac chambers and reduction in the ability of the ventricles (lower chambers) to contract with the required force needed to pump blood out of the heart to the body.

Idiopathic means the cause is not known.

Q: What is the prognosis for idiopathic dilated cardiomyopathy?

A: This disorder often results in symptomatic heart failure due to the inability of the heart to pump blood as required to supply the body with oxygen and meet metabolic needs.

What Is Idiopathic Dilated Cardiomyopathy In Celiac Disease and/or Gluten Sensitivity?

• Relationship between idiopathic dilated cardiomyopathy and celiac disease. Idiopathic dilated cardiomyopathy is an associated disorder of celiac disease.

• Relationship between idiopathic dilated cardiomyopathy and malabsorption. Idiopathic dilated cardiomyopathy and celiac disease are two pathological conditions which may lead, by different mechanisms, to malabsorption of various micronutrients, including carnitine, an amino acid which is active in cardiac metabolism.1

• Relationship between idiopathic dilated cardiomyopathy and carnitine deficiency. Dilated cardiomyopathy due to carnitine deficiency may occur in celiac disease patients and carnitine deficiency may present not only at the time of diagnosis of celiac disease but it may also develop during gluten-free diet, particularly in patients with fast weight gain and without carnitine supplementation.2

• Relationship between idiopathic dilated cardiomyopathy and echocardiograms in patients with celiac disease. A study investigating cardiac functions by Tissue Doppler Echocardiography in 45 pediatric patients with celiac disease detected subclinical systolic dysfunction of the left ventricle in children with celiac disease in whom serum IgA antiendomysial antibody reactivity is prominent.3

How Prevalent Is Idiopathic Dilated Cardiomyopathy In Celiac Disease and/or Gluten Sensitivity?

Celiac disease was found in 5.7% patients with cardiomyopathy.4 and IDCM affects untreated celiac disease patients and those who do not strictly follow gluten free diet.5

What Are The Symptoms Of Idiopathic Dilated Cardiomyopathy?

Idiopathic dilated cardiomyopathy is marked by these symptoms:

• Weakness advancing to inability to get out of bed.
• Dyspnea (shortness of breath) advancing to inability to breathe lying down.
• Tachycardia (rapid heart beat).
• Edema (swelling) of the lower legs advancing bodywide and/or pulmonary edema.

How Does Idiopathic Dilated Cardiomyopathy In Celiac Disease and/or Gluten Sensitivity Develop?

• Idiopathic dilated cardiomyopathy results from unclear etiology involving gluten exposure in celiac disease and carnitine deficiency (an amino acid).2

Does Idiopathic Dilated Cardiomyopathy Respond To Gluten-Free Diet?

Yes. Celiac disease-related ICDM responds to a strict gluten free diet.6

Dilated cardiomyopathy due to carnitine deficiency may also develop during gluten-free diet, particularly in patients with fast weight gain and without carnitine supplementation.2

6 Steps To Improve Idiopathic Dilated Cardiomyopathy Related to Celiac Disease and/or Gluten Sensitivity:

• 1Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:

• 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

• 4 Manage Your Medications Safely:

• 5Nutritional Supplements To Help Correct Deficiencies:

• 6Manage Natural Remedies: 

What Do Medical Research Studies Tell About ICDM In Celiac Disease and/or Gluten Sensitivity?

RESEARCH STUDY SUMMARIES

“Cardiac functions in children with coeliac disease during follow-up: insights from tissue Doppler imaging.“ This study investigating cardiac functions by Tissue Doppler Echocardiography in pediatric patients with celiac disease detected subclinical systolic dysfunction of the left ventricle in children with celiac disease in whom serum IgA antiendomysial antibody reactivity is prominent.

Forty-five clinically stable patients were studied; twenty-five patients with positive serum IgA antiendomysial antibody levels (Group 1), twenty patients with negative serum IgA antiendomysial antibody levels (Group 2) at the time of echocardiographic study. Control group consisted of 30 healthy children free of any disease.

Myocardial systolic wave velocity of the mitral annulus was significantly lower, myocardial precontraction and contraction time were slightly longer in Group 2 when compared control group. There was a negative correlation between the serum IgA antiendomysial antibody levels titers and myocardial systolic wave levels of all patients included in the study. A myocardial systolic wave velocity of <8.9 cm/s had a 92% sensitivity and 80% specificity in predicting serum IgA antiendomysial antibody levels positive patients.

Tissue Doppler echocardiography provides a quantifiable indicator useful for cardiac monitoring of disease during follow up.3

“Celiac disease prevalence in Brazilian dilated cardiomyopathy patients.” This study evaluating the prevalence of celiac disease among 74 south Brazilian precardiac transplant patients with advanced cardiomyopathy found that two individuals (2.63%) were positive for IgA-EmA and 5 (6.75%) for IgA anti-h-tTG; 1 (1.35%) had both tests positive. Histologic confirmation of celiac disease occurred only in the IgA-EmA positive patients. The presence of anti-endomisial antibody (IgA-EmA) was determined by indirect immunofluorescence and for the anti-transglutaminase antibody (IgA anti-h-tTG) by ELISA. Serologically positive patients were submitted to upper endoscopy with intestinal biopsy. Two individuals (2.63%) were positive for IgA-EmA and 5 (6.75%) for IgA anti-h-tTG; 1 (1.35%) had both tests positive. Histologic confirmation of celiac disease occurred only in the IgA-EmA positive patients.9

“Carnitine deficiency in patients with celiac disease and idiopathic dilated cardiomyopathy.” This study investigated the differences in serum concentrations of total carnitine between idiopathic dilated cardiomyopathy (IDCM patients and patients with IDCM associated with celiac disease and then also evaluated, in the latter, the effect of a gluten-free diet on serum concentrations of total carnitine. Study found that patients presenting IDCM associated with celiac disease show a greater decrease in serum total carnitine levels than patients presenting the isolated form of IDCM. A gluten-free diet, in these patients, leads to a progressive increase in serum levels of this substance.

Serum carnitine was determined by enzymatic spectrophotometric assay in three groups of individuals: group A, 10 patients (5 males, 5 females), mean age 46.5+/-10.8 years, presenting isolated IDCM; group B, 3 patients (2 males, 1 female), mean age 34+/-8 years, with IDCM+Celiac Disease; and group C, 10 healthy subjects (5 males, 5 females), mean age 38.6+/-11.1 years. All patients with IDCM belonged to class NYHA I-II. Mean concentrations of total serum carnitine in the group of patients with isolated IDCM (group A) were found to be lower than in the controls (group C). The concentrations in patients with IDCM associated with celiac disease (group B) were lower than in the control group and also lower than in the isolated IDCM (group A). After 2 years on a gluten-free diet, patients presenting IDCM associated with celiac disease showed a progressive increase in mean serum carnitine levels compared to values observed prior to the diet.1

CASE REPORT SUMMARIES

“Transient small bowel intussusception in adults: an overlooked feature of celiac disease.” This case report describes diagnosing celiac disease in a 46-year-old woman with known dilated cardiomyopathy and pulmonary hypertension, who presented with non-specific abdominal symptoms that were not initially attributed to gastrointestinal disease.

Radiological investigations of the abdomen demonstrated transient small bowel intussusception without other abnormality, leading to the suggestion of celiac disease as a cause, which was subsequently confirmed as the diagnosis.10

“Celiac disease with pulmonary hemosiderosis and cardiomyopathy.” This case report describes celiac disease presenting in a patient with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vitamin A deficiency was detected. This case suggests that celiac disease should be considered in patients with cardiomyopathy and/or pulmonary haemosiderosis regardless of the intestinal symptoms of celiac disease.11

“Dilated cardiomyopathy in celiac disease: role of carnitine deficiency.” This case report describes the course of dilated cardiomyopathy in a patient with celiac disease and secondary carnitine deficiency. Dilated cardiomyopathy due to carnitine deficiency may occur in celiac disease patients and carnitine deficiency may present not only at the time of diagnosis of celiac disease but it may also develop during gluten-free diet, particularly in patients with fast weight gain and without carnitine supplementation.2

“Stroke and dilated cardiomyopathy associated with celiac disease.” This case report describes diagnosis of celiac disease in an 8-year-old girl who presented with rarely seen dilated cardiomyopathy and stroke. The girl was admitted with left side weakness. Her medical history indicated abdominal distention, chronic diarrhea, failure to thrive, and geophagia (from vitamin B deficiency). On physical examination, short stature, pale skin and a grade 2 of 6 systolic murmur were detected. Muscle strength was 0/5 on the left side, and 5/5 on the right side.

Coagulation examinations were normal. Brain magnetic resonance imaging (MRI) and cerebral angiography showed an infarction area at the basal ganglia level. Examinations of serologic markers and intestinal biopsy revealed celiac disease. Anti-tissue transglutaminase IgA and IgG, and anti-endomysium IgA were found at a highly positive rate. Duodenal biopsy showed villous atrophy with hyperplasia of the crypts and an increased intraepithelial lymphocyte count (above 40%).

A gluten-free diet and nadroparin calcium treatment were initiated and physiotherapy was performed. At the 18th day of hospitalization, the patient, whose symptoms had regressed, was discharged with a gluten-free diet, nadroparin calcium, co-enzyme Q and salicylate treatment. Symptoms resolved by the following 7th wk. Muscle strength at the left upper and lower extremities was 5/5, and other neurologic examinations were normal.

Because celiac disease is a potentially treatable cause of cerebral vasculopathy, serology (specifically for anti-tissue transglutaminase antibodies) should be included in the evaluation of cryptogenic stroke in childhood, even in the absence of typical gut symptoms.

In conclusion, the cause of ischemic stroke in this case is thought to be multifactorial. “We suggest that celiac disease was a primary factor in its etiology, secondary to a contribution from dilated cardiomyopathy.”12

“Dilated cardiomyopathy in celiac disease: report of one case.” This case report describes a 4 7 year-old female presenting with heart failure secondary to dilated cardiomyopathy of unknown etiology. During the five months following the first hospitalization the patient had multiple hospital admissions due to decompensate heart failure. Due to a history of intermittent diarrhea and weight loss, a celiac disease was suspected. Antiendomysial antibodies were positive and there was a villous atrophy in duodenal mucosa. A gluten free diet was started with a concomitant recovery of her functional capacity. After one month of gluten free diet a new echocardiogram showed a normal left ventricle and systolic function.13

“Idiopathic dilated cardiomyopathy associated with coeliac disease: the effect of a gluten-free diet on cardiac performance.” This case report of 3 patients with celiac disease and cardiomyopathy who underwent clinical and laboratory evaluation to establish the effect of a gluten free diet on cardiac performance showed that the gluten free diet had a beneficial effect on cardiac performance in patients with celiac disease who kept a strict diet.

Two patients who observed the gluten free diet regimen very strictly, after a 28 month follow-up period, showed an improvement in echocardiographic parameters as well as of cardiological features and quality of life. The third patient did not observe the gluten free diet and presented a worsening in the echocardiographic parameters and cardiological symptoms which required supplementary drug therapy.6

1. Curione M, Danese C, Viola F, Di Bona S, Anastasia A, Cugini P, Barbato M. Carnitine deficiency in patients with coeliac disease and idiopathic dilated cardiomyopathy. Nutr Metab Cardiovasc Dis. 2005 Aug;15(4):279-83. [↩] [↩]
2. Uslu N, Demir H, Karagöz T, Saltik-Temizel IN. Dilated cardiomyopathy in celiac disease: role of carnitine deficiency. Acta Gastroenterol Belg. 2010 Oct-Dec;73(4):530-1. [↩] [↩] [↩] [↩]
3. Polat TB, Urganci N, Yalcin Y, Zeybek C, Akdeniz C, Erdem A, Imanov E, Celebi A. Cardiac functions in children with coeliac disease during follow-up: insights from tissue Doppler imaging. Dig Liver Dis. 2008 Mar;40(3):182-7. doi: 10.1016/j.dld.2007.11.014. [↩] [↩]
4. Curione M, Barbato M, De Biase L, Viola F, Lo Russo L, Cardi E. Prevalence of coeliac disease in idiopathic dilated cardiomyopathy. Lancet. Jul 17, 1999;354(9174):222-3. [↩]
5. Curione M, Barbato M, Viola F, Francia P, De Biase L, Cucchiara S. Idiopathic dilated cardiomyopathy associated with coeliac disease: the effect of a gluten-free diet on cardiac performance. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver. Dec 2002;34(12):866-9. [↩]
6. Curione M, Barbato M, Viola F, Francia P, De Biase L, Cucchiara S. Idiopathic dilated cardiomyopathy associated with coeliac disease: the effect of a gluten-free diet on cardiac performance. Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver. Dec 2002;34(12):866-9. [↩] [↩]
7. Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. [↩]
8. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩] [↩] [↩] [↩] [↩] [↩]
9. De Bem RS, Da Ro Sa Utiyama SR, Nisihara RM, Fortunato JA, Tondo JA, Carmes ER, Souza RA, Pisani JC, Amarante HM. Celiac disease prevalence in Brazilian dilated cardiomyopathy patients. Dig Dis Sci. 2006 May;51(5):1016-9. [↩]
10. Briggs JH, McKean D, Palmer JS, Bungay H. Transient small bowel intussusception in adults: an overlooked feature of coeliac disease. BMJ Case Rep. 2014 Jun 20;2014. pii: bcr2013203156. doi: 10.1136/bcr-2013-203156. [↩]
11. Işikay S, Yilmaz K, Kilinç M. Celiac disease with pulmonary haemosiderosis and cardiomyopathy. BMJ Case Rep. 2012 Nov 20;2012. pii: bcr2012007262. doi: 10.1136/bcr-2012-007262. [↩]
12. Doğan M, Peker E, Cagan E, Akbayram S, Acikgoz M, Caksen H, Uner A, Cesur Y. Stroke and dilated cardiomyopathy associated with celiac disease. World J Gastroenterol. 2010 May 14;16(18):2302-4. [↩]
13. Winter Del R JL, Gabrielli N L, Greig D, Inchauste G, Quezada F, Torres M J, Castro G P. Dilated cardiomyopathy in celiac disease: report of one case. Rev Med Chil. 2009 Nov;137(11):1469-73. doi: /S0034-98872009001100009. [↩]