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Cardiomyopathy in Celiac Disease: Carnitine Behind?

Nada Boutrid, Hakim Rahmoune , Mounira Amrane
International Journal of Celiac Disease. 2020, 8(4), 124-125. DOI: 10.12691/ijcd-8-4-3
Received August 04, 2020; Revised September 05, 2020; Accepted December 01, 2020

Abstract

In Volume 8, Issue 2 of the International Journal of Celiac Disease, Shah et al. presented a peculiar celiac disease presenting as cardiomyopathy in an adult female. We would highlight the importance of carnitine deficiency in such cases, both at diagnosis as well as during follow up; carnitine being widely reported as a cause of cardiomyopathy in celiac and non-celiac population, and supplementing these patients by carnitine might reverse this heart dysfunction.

1. Introduction

Among the interesting case reports recently published in the International Journal of Celiac Disease, Shah et al. presented a peculiar celiac disease (CD) presenting as a dilated cardiomyopathy in an Indian adult female 1.

We address the cornerstone role of carnitine deficiency in such cases, both at diagnosis as well as during follow up. Carnitine is more and more reported as a cause of cardiomyopathy in celiac and non-celiac population, and supplementing these patients by carnitine might reverse this heart dysfunction 2, 3, 4, 5.

In this article, we give a glance of dilated cardiomyopathy due to carnitine deficiency in celiac patients.

2. Carnitine Deficiency and Cardio-myopathy

Carnitine is needed for the transfer of long-chain fatty acids across the inner mitochondrial membrane for subsequent β-oxidation. Carnitine can be synthesized by the body and is also obtained in the diet through the consumption of meat and dairy products 6.

Levo-carnitine is a natural and biologically active amino -acid derivatives and a micronutrient that plays a crucial role in human lipid metabolism and also in mitochondrial defence. It contributes to a myriad of physiological activities and has been proposed for treating an expanding range of cardiac problems including myocardial injury and cardiomyopathies 7, 8.

It is well-known that cellular metabolism of fatty acids does require both intra-cytosol carnitine cycle and intra-mitochondrial β-oxidation cycle; and carnitine is primordial for translocation of long-chain acyl-CoA across the inner mitochondrial membrane; and thus plays a pivotal role in cardiac cells’ contraction 9, 10.

3. Carnitine and Cardiomyopathy in Celiac Population

Several cases of celiac patients presenting a dilated cardiomyopathy due to a secondary carnitine deficiency in CD related malabsorption are reported in the scientific literature, sometimes as a unique extra-intestinal manifestation 11, 12, 13, 14, 15.

Notably, most of CD associated cardiomyopathy cases are reported in childhood 16, probably as a result of the profound deficiency in the pediatric population.

For example, a monocentric Brazilian study found that CD prevalence in pediatric patients with dilated cardiomyopathy or myocarditis was as high as 1.8% 17.

Biologically, patients presenting a dilated cardiomyopathy with CD show a deeper decrease in serum carnitine levels than those presenting an isolated dilated cardiomyopathy and gluten-free diet leads to a progressive restoration of normal carnitine concentrations 18.

A recent Saudi investigational study reported a good correlation between the cardiac functions and the degree of intestinal mucosal injury and advocating for the use of Doppler tissue imaging (rather than conventional two-dimensional echocardiography) to detect early myocardial dysfunction in children with CD. 19

A strict gluten withdrawal in these cases would restore a normal intestinal permeability and normalize the carnitine serum and then cellular levels, allowing a progressive reversal of the cardiomyopathy 20.

Of course, carnitine supplementation should be prescribed at first-line if a clear deficiency is documented or even in highly suspected cases (if carnitine dosage is not available).

In sum, a dilated cardiomyopathy due to carnitine deficiency may occur in CD patients and carnitine deficiency may present not only at the time of diagnosis of the CD but it may also develop during the gluten-free diet, particularly in patients with fast weight gain and without carnitine supplementation. 21.

4. Conclusion

Co-occurrence of dilated cardiomyopathy and CD should emphasize the prompt diagnosis of carnitine deficiency, even without systemic symptoms (digestive manifestations of CD and/or muscular signs of carnitine deficiency). Strict, long-life gluten-free diet with adapted carnitine supplementation reverse this heart dysfunction. Close management and follow up are mandatory.

Acknowledgements

N. Boutrid, H. Rahmoune, M. Amrane (from LMCVGN laboratory) are supported by the Directorate-General for Scientific Research and Technological Development (DGRSDT), MESRS, Algeria.

The sponsor had no involvement in the collection, analysis and interpretation of data or the writing of the manuscript.

References

[1]  Bohra S, Shah A. Celiac Disease Presenting as Cardiomyopathy - A Rare Extra Intestinal Manifestation. International Journal of Celiac Disease 2020: 8(2): 55-56.
In article      
 
[2]  Kothari SS, Sharma M. L-carnitine in children with idiopathic dilated cardiomyopathy. Indian heart journal. 1998; 50(1): 59.
In article      
 
[3]  Zales VR, Benson DW. Reversible cardiomyopathy due to carnitine deficiency from renal tubular wasting. Pediatric Cardiology. 1995 Mar 1; 16(2): 76-8.
In article      View Article  PubMed
 
[4]  Baragou S, Pio M, Di Bernardo S, et al. Une cause de cardiomyopathie dilatée chez l'enfant: le déficit primaire en carnitine [A cause of dilated cardiomyopathy in a child: primary carnitine deficiency]. Ann Cardiol Angeiol (Paris). 2014; 63(2): 107-110.
In article      View Article  PubMed
 
[5]  Mormile R. Dilated cardiomyopathy in celiac disease: what is the plot of the story?. Minerva pediatrica. 2017 Oct; 69(5): 455.
In article      
 
[6]  Longo N. Primary carnitine deficiency and newborn screening for disorders of the carnitine cycle. Annals of Nutrition and Metabolism. 2016; 68(Suppl. 3): 5-9.
In article      View Article  PubMed
 
[7]  Wang ZY, Liu YY, Liu GH, Lu HB, Mao CY. l-Carnitine and heart disease. Life sciences. 2018 Feb 1; 194: 88-97.
In article      View Article  PubMed
 
[8]  M.A. Arsenian, Carnitine and its derivatives in cardiovascular disease, Prog.Cardiovasc. Dis. 40 (1997) 265-286.
In article      View Article
 
[9]  Adeva‐Andany MM, Calvo‐Castro I, Fernández‐Fernández C, Donapetry‐García C, Pedre‐Piñeiro AM. Significance of l‐carnitine for human health. IUBMB life. 2017 Aug; 69(8): 578-94.
In article      View Article  PubMed
 
[10]  Longo N, Frigeni M, Pasquali M: Carnitine transport and fatty acid oxidation. Biochim Biophys Acta 2016; pii:S0167-4889(16)30013-1.
In article      
 
[11]  Lodha A, Haran M, Hollander G, Frankel R, Shani J. Celiac disease associated with dilated cardiomyopathy. Southern medical journal. 2009 Oct; 102(10): 1052-4.
In article      View Article  PubMed
 
[12]  De Bem RS, Utiyama SR, Nisihara RM, Carmes ER, Souza RA, Pisani JC, Amarante HM. Celiac disease prevalence in Brazilian dilated cardiomyopathy patients. Digestive diseases and sciences. 2006 May 1; 51(5): 1016-9.
In article      View Article  PubMed
 
[13]  Milisavljević N, Cvetković M, Nikolić G, Filipović B, Milinić N. Dilated cardiomiopathy associated with celiac disease: Case report and literature review. Srpski arhiv za celokupno lekarstvo. 2012; 140(9-10): 641-3.
In article      View Article  PubMed
 
[14]  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 Journal of Gastroenterology: WJG. 2010 May 14; 16(18): 2302.
In article      View Article  PubMed
 
[15]  Patel P, Smith F, Kilcullen N, Artis N. Dilated cardiomyopathy as the first presentation of coeliac disease: association or causation?. Clinical Medicine. 2018 Apr; 18(2): 177.
In article      View Article  PubMed
 
[16]  Boskovic A, Kitic I, Prokic D, Stankovic I. Cardiomyopathy associated with celiac disease in childhood. Case reports in gastrointestinal medicine. 2012; 2012.
In article      View Article  PubMed
 
[17]  Motta ME. Celiac disease prevalence in children and adolescents with myocarditis and dilated cardiomiopathy. Jornal de pediatria. 2012; 88(5): 439-42.
In article      View Article  PubMed
 
[18]  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. Nutrition, Metabolism and Cardiovascular Diseases. 2005 Aug 1; 15(4): 279-83.
In article      View Article  PubMed
 
[19]  Fathy A, Abo-Haded HM, Al-Ahmadi N, El-Sonbaty MM. Cardiac functions assessment in children with celiac disease and its correlation with the degree of mucosal injury: Doppler tissue imaging study. Saudi journal of gastroenterology: official journal of the Saudi Gastroenterology Association. 2016 Nov; 22(6): 441.
In article      View Article  PubMed
 
[20]  McGrath S, Thomas A, Gorard DA. Cardiomyopathy responsive to gluten withdrawal in a patient with coeliac disease. Case Reports. 2016 Mar 14; 2016: bcr2015213301.
In article      View Article  PubMed
 
[21]  Uslu N, Demir H, Karagöz TE, Saltik-Temizel IN. Dilated cardiomyopathy in celiac disease: role of carnitine deficiency. Acta Gastro-Enterologica Belgica. 2010 Oct 1; 73(4): 530-1.
In article      
 

Published with license by Science and Education Publishing, Copyright © 2020 Nada Boutrid, Hakim Rahmoune and Mounira Amrane

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

Cite this article:

Normal Style
Nada Boutrid, Hakim Rahmoune, Mounira Amrane. Cardiomyopathy in Celiac Disease: Carnitine Behind?. International Journal of Celiac Disease. Vol. 8, No. 4, 2020, pp 124-125. http://pubs.sciepub.com/ijcd/8/4/3
MLA Style
Boutrid, Nada, Hakim Rahmoune, and Mounira Amrane. "Cardiomyopathy in Celiac Disease: Carnitine Behind?." International Journal of Celiac Disease 8.4 (2020): 124-125.
APA Style
Boutrid, N. , Rahmoune, H. , & Amrane, M. (2020). Cardiomyopathy in Celiac Disease: Carnitine Behind?. International Journal of Celiac Disease, 8(4), 124-125.
Chicago Style
Boutrid, Nada, Hakim Rahmoune, and Mounira Amrane. "Cardiomyopathy in Celiac Disease: Carnitine Behind?." International Journal of Celiac Disease 8, no. 4 (2020): 124-125.
Share
[1]  Bohra S, Shah A. Celiac Disease Presenting as Cardiomyopathy - A Rare Extra Intestinal Manifestation. International Journal of Celiac Disease 2020: 8(2): 55-56.
In article      
 
[2]  Kothari SS, Sharma M. L-carnitine in children with idiopathic dilated cardiomyopathy. Indian heart journal. 1998; 50(1): 59.
In article      
 
[3]  Zales VR, Benson DW. Reversible cardiomyopathy due to carnitine deficiency from renal tubular wasting. Pediatric Cardiology. 1995 Mar 1; 16(2): 76-8.
In article      View Article  PubMed
 
[4]  Baragou S, Pio M, Di Bernardo S, et al. Une cause de cardiomyopathie dilatée chez l'enfant: le déficit primaire en carnitine [A cause of dilated cardiomyopathy in a child: primary carnitine deficiency]. Ann Cardiol Angeiol (Paris). 2014; 63(2): 107-110.
In article      View Article  PubMed
 
[5]  Mormile R. Dilated cardiomyopathy in celiac disease: what is the plot of the story?. Minerva pediatrica. 2017 Oct; 69(5): 455.
In article      
 
[6]  Longo N. Primary carnitine deficiency and newborn screening for disorders of the carnitine cycle. Annals of Nutrition and Metabolism. 2016; 68(Suppl. 3): 5-9.
In article      View Article  PubMed
 
[7]  Wang ZY, Liu YY, Liu GH, Lu HB, Mao CY. l-Carnitine and heart disease. Life sciences. 2018 Feb 1; 194: 88-97.
In article      View Article  PubMed
 
[8]  M.A. Arsenian, Carnitine and its derivatives in cardiovascular disease, Prog.Cardiovasc. Dis. 40 (1997) 265-286.
In article      View Article
 
[9]  Adeva‐Andany MM, Calvo‐Castro I, Fernández‐Fernández C, Donapetry‐García C, Pedre‐Piñeiro AM. Significance of l‐carnitine for human health. IUBMB life. 2017 Aug; 69(8): 578-94.
In article      View Article  PubMed
 
[10]  Longo N, Frigeni M, Pasquali M: Carnitine transport and fatty acid oxidation. Biochim Biophys Acta 2016; pii:S0167-4889(16)30013-1.
In article      
 
[11]  Lodha A, Haran M, Hollander G, Frankel R, Shani J. Celiac disease associated with dilated cardiomyopathy. Southern medical journal. 2009 Oct; 102(10): 1052-4.
In article      View Article  PubMed
 
[12]  De Bem RS, Utiyama SR, Nisihara RM, Carmes ER, Souza RA, Pisani JC, Amarante HM. Celiac disease prevalence in Brazilian dilated cardiomyopathy patients. Digestive diseases and sciences. 2006 May 1; 51(5): 1016-9.
In article      View Article  PubMed
 
[13]  Milisavljević N, Cvetković M, Nikolić G, Filipović B, Milinić N. Dilated cardiomiopathy associated with celiac disease: Case report and literature review. Srpski arhiv za celokupno lekarstvo. 2012; 140(9-10): 641-3.
In article      View Article  PubMed
 
[14]  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 Journal of Gastroenterology: WJG. 2010 May 14; 16(18): 2302.
In article      View Article  PubMed
 
[15]  Patel P, Smith F, Kilcullen N, Artis N. Dilated cardiomyopathy as the first presentation of coeliac disease: association or causation?. Clinical Medicine. 2018 Apr; 18(2): 177.
In article      View Article  PubMed
 
[16]  Boskovic A, Kitic I, Prokic D, Stankovic I. Cardiomyopathy associated with celiac disease in childhood. Case reports in gastrointestinal medicine. 2012; 2012.
In article      View Article  PubMed
 
[17]  Motta ME. Celiac disease prevalence in children and adolescents with myocarditis and dilated cardiomiopathy. Jornal de pediatria. 2012; 88(5): 439-42.
In article      View Article  PubMed
 
[18]  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. Nutrition, Metabolism and Cardiovascular Diseases. 2005 Aug 1; 15(4): 279-83.
In article      View Article  PubMed
 
[19]  Fathy A, Abo-Haded HM, Al-Ahmadi N, El-Sonbaty MM. Cardiac functions assessment in children with celiac disease and its correlation with the degree of mucosal injury: Doppler tissue imaging study. Saudi journal of gastroenterology: official journal of the Saudi Gastroenterology Association. 2016 Nov; 22(6): 441.
In article      View Article  PubMed
 
[20]  McGrath S, Thomas A, Gorard DA. Cardiomyopathy responsive to gluten withdrawal in a patient with coeliac disease. Case Reports. 2016 Mar 14; 2016: bcr2015213301.
In article      View Article  PubMed
 
[21]  Uslu N, Demir H, Karagöz TE, Saltik-Temizel IN. Dilated cardiomyopathy in celiac disease: role of carnitine deficiency. Acta Gastro-Enterologica Belgica. 2010 Oct 1; 73(4): 530-1.
In article