Restrictive Cardiomyopathy Revealing Systemic Sclerodermia

Soukaina Safir; Rim Ouazzane; Amal Moukhliss; Hanane Choukrani; Abdenacer Drighil; Leila Azzouzi; Rachida Habbal

doi:doi:10.11648/j.ajim.20200802.13

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Restrictive Cardiomyopathy Revealing Systemic Sclerodermia

Soukaina Safir, Rim Ouazzane, Amal Moukhliss, Hanane Choukrani, Abdenacer Drighil, Leila Azzouzi, Rachida Habbal

Published in American Journal of Internal Medicine (Volume 8, Issue 2)

Received: 14 January 2020 Accepted: 6 February 2020 Published: 10 March 2020

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Abstract

Primary myocardial involvement is common in systemic sclerosis. There is growing evidence strongly suggesting that this involvement is linked to repeated focal ischemic lesions causing irreversible myocardial fibrosis. It can affect all heart structures. We report the case of a 65 years old male patient, without any personal medical history, admitted for inaugural right heart decompensation. The diagnosis of restrictive cardiomyopathy is based mainly on the following criteria: on echocardiography, hypertrohy of the right ventricle with alteration of the longitudinal systolic function, dilation of the right atrium, with good overall contractility. On right cardiac catheterization: elevation and equalization of the telesystolic pressures of the right atrium, right ventricle, and pulmonary artery with appearance of dip plateau on the right ventricle curve. Scleroderma was evoked considering following statements: the presence of Raynaud's phenomenon, unknown by the patient (presence of dermal sclerosis with pericapillary oedema and capillary dystrophy on capillaroscopy), cutaneous involvement (presence of perivascular and perineural mononuclear infiltrates, with collagen fibers increased in number and thickness), pulmonary involvement (diffuse bilateral interstitial lung disease) and renal involvement (moderately impaired function and positive proteinuria). The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer non-invasive imaging techniques. Therfore, it would be necessary to search the cardiac involvement especially subclinical one in this type of system disease, as well as to confirm the systemic origin in front of certain forms of cardiopathies in particular restrictive cardiomyopathy.

Published in	American Journal of Internal Medicine (Volume 8, Issue 2)
DOI	10.11648/j.ajim.20200802.13
Page(s)	62-65
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Systemic Sclerodermia, Restrictive Cardiomyopathy, Echocardiography, Right Cardiac Catheterization

References

[1]	Allanore Y, Avouac J, Kahan A. Systemic sclerosis: an update in 2008. Joint Bone Spine. 2008; 75: 650–655.
[2]	Varga J, Denton CP, Wigley FM. Scleroderma. New York: Springer; 2012. pp. 361–371; 373-395.
[3]	Ferri C, Emdin M, Nielsen H, Bruhlmann P. Assessment of heart involvement. Clin Exp Rheumatol. 2003; 21: S24–S28.
[4]	Kahan A, Allanore Y. Primary myocardial involvement in systemic sclerosis. Rheumatology (Oxford) 2006; 45 Suppl 4: iv14–iv17.
[5]	Steen VD, Medsger TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000; 43: 2437–2444.
[6]	Groote P, Gressin V, Hachulla E, Carpentier P, Guillevin L, Kahan A, Cabane J, Francès C, Lamblin N, Diot E, Patat F, Sibilia J, Petit H, Cracowski JL, Clerson P, Humbert M, ItinerAIR-Scleroderma Investigators. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis. 2008 Jan; 67 (1): 31-6.
[7]	Allanore Y, Meune C, Vonk MC, Airo P, Hachulla E, Caramaschi P, Riemekasten G, Cozzi F, Beretta L, Derk CT, Komócsi A, Farge D, Balbir A, Riccieri V, Distler O, Chialà A, Del Papa N, Simic KP, Ghio M, Stamenkovic B, Rednic S, Host N, Pellerito R, Zegers E, Kahan A, Walker UA, Matucci-Cerinic M, EUSTAR co-authors. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis. Ann Rheum Dis. 2010 Jan; 69 (1): 218-21.
[8]	Hachulla AL, Launay D, Gaxotte V, de Groote P, Lamblin N, Devos P, Hatron PY, Beregi JP, Hachulla E. Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis. 2009 Dec; 68 (12): 1878-84.
[9]	Ferri C, Giuggioli D, Sebastiani M, Colaci M, Emdin M. Heart involvement and systemic sclerosis. Lupus. 2005; 14: 702–707.
[10]	McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009; 53: 1573–1619.
[11]	Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992; 327: 76–81.
[12]	Vachiéry JL, Coghlan G. Screening for pulmonary arterial hypertension in systemic sclerosis. Eur Respir Rev. 2009; 18: 162–169.
[13]	Schwaiger JP, Khanna D, Gerry Coghlan J. Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at-risk populations. Eur Respir Rev. 2013; 22: 515–525.

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APA Style

Soukaina Safir, Rim Ouazzane, Amal Moukhliss, Hanane Choukrani, Abdenacer Drighil, et al. (2020). Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. American Journal of Internal Medicine, 8(2), 62-65. https://doi.org/10.11648/j.ajim.20200802.13

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ACS Style

Soukaina Safir; Rim Ouazzane; Amal Moukhliss; Hanane Choukrani; Abdenacer Drighil, et al. Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. Am. J. Intern. Med. 2020, 8(2), 62-65. doi: 10.11648/j.ajim.20200802.13

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AMA Style

Soukaina Safir, Rim Ouazzane, Amal Moukhliss, Hanane Choukrani, Abdenacer Drighil, et al. Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. Am J Intern Med. 2020;8(2):62-65. doi: 10.11648/j.ajim.20200802.13

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@article{10.11648/j.ajim.20200802.13,
  author = {Soukaina Safir and Rim Ouazzane and Amal Moukhliss and Hanane Choukrani and Abdenacer Drighil and Leila Azzouzi and Rachida Habbal},
  title = {Restrictive Cardiomyopathy Revealing Systemic Sclerodermia},
  journal = {American Journal of Internal Medicine},
  volume = {8},
  number = {2},
  pages = {62-65},
  doi = {10.11648/j.ajim.20200802.13},
  url = {https://doi.org/10.11648/j.ajim.20200802.13},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20200802.13},
  abstract = {Primary myocardial involvement is common in systemic sclerosis. There is growing evidence strongly suggesting that this involvement is linked to repeated focal ischemic lesions causing irreversible myocardial fibrosis. It can affect all heart structures. We report the case of a 65 years old male patient, without any personal medical history, admitted for inaugural right heart decompensation. The diagnosis of restrictive cardiomyopathy is based mainly on the following criteria: on echocardiography, hypertrohy of the right ventricle with alteration of the longitudinal systolic function, dilation of the right atrium, with good overall contractility. On right cardiac catheterization: elevation and equalization of the telesystolic pressures of the right atrium, right ventricle, and pulmonary artery with appearance of dip plateau on the right ventricle curve. Scleroderma was evoked considering following statements: the presence of Raynaud's phenomenon, unknown by the patient (presence of dermal sclerosis with pericapillary oedema and capillary dystrophy on capillaroscopy), cutaneous involvement (presence of perivascular and perineural mononuclear infiltrates, with collagen fibers increased in number and thickness), pulmonary involvement (diffuse bilateral interstitial lung disease) and renal involvement (moderately impaired function and positive proteinuria). The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer non-invasive imaging techniques. Therfore, it would be necessary to search the cardiac involvement especially subclinical one in this type of system disease, as well as to confirm the systemic origin in front of certain forms of cardiopathies in particular restrictive cardiomyopathy.},
 year = {2020}
}

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TY  - JOUR
T1  - Restrictive Cardiomyopathy Revealing Systemic Sclerodermia
AU  - Soukaina Safir
AU  - Rim Ouazzane
AU  - Amal Moukhliss
AU  - Hanane Choukrani
AU  - Abdenacer Drighil
AU  - Leila Azzouzi
AU  - Rachida Habbal
Y1  - 2020/03/10
PY  - 2020
N1  - https://doi.org/10.11648/j.ajim.20200802.13
DO  - 10.11648/j.ajim.20200802.13
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 62
EP  - 65
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20200802.13
AB  - Primary myocardial involvement is common in systemic sclerosis. There is growing evidence strongly suggesting that this involvement is linked to repeated focal ischemic lesions causing irreversible myocardial fibrosis. It can affect all heart structures. We report the case of a 65 years old male patient, without any personal medical history, admitted for inaugural right heart decompensation. The diagnosis of restrictive cardiomyopathy is based mainly on the following criteria: on echocardiography, hypertrohy of the right ventricle with alteration of the longitudinal systolic function, dilation of the right atrium, with good overall contractility. On right cardiac catheterization: elevation and equalization of the telesystolic pressures of the right atrium, right ventricle, and pulmonary artery with appearance of dip plateau on the right ventricle curve. Scleroderma was evoked considering following statements: the presence of Raynaud's phenomenon, unknown by the patient (presence of dermal sclerosis with pericapillary oedema and capillary dystrophy on capillaroscopy), cutaneous involvement (presence of perivascular and perineural mononuclear infiltrates, with collagen fibers increased in number and thickness), pulmonary involvement (diffuse bilateral interstitial lung disease) and renal involvement (moderately impaired function and positive proteinuria). The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer non-invasive imaging techniques. Therfore, it would be necessary to search the cardiac involvement especially subclinical one in this type of system disease, as well as to confirm the systemic origin in front of certain forms of cardiopathies in particular restrictive cardiomyopathy.
VL  - 8
IS  - 2
ER  -

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Author Information

Soukaina Safir

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Rim Ouazzane

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Amal Moukhliss

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Hanane Choukrani

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Abdenacer Drighil

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Leila Azzouzi

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco
Rachida Habbal

Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco

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APA Style

Soukaina Safir, Rim Ouazzane, Amal Moukhliss, Hanane Choukrani, Abdenacer Drighil, et al. (2020). Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. American Journal of Internal Medicine, 8(2), 62-65. https://doi.org/10.11648/j.ajim.20200802.13

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ACS Style

Soukaina Safir; Rim Ouazzane; Amal Moukhliss; Hanane Choukrani; Abdenacer Drighil, et al. Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. Am. J. Intern. Med. 2020, 8(2), 62-65. doi: 10.11648/j.ajim.20200802.13

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AMA Style

Soukaina Safir, Rim Ouazzane, Amal Moukhliss, Hanane Choukrani, Abdenacer Drighil, et al. Restrictive Cardiomyopathy Revealing Systemic Sclerodermia. Am J Intern Med. 2020;8(2):62-65. doi: 10.11648/j.ajim.20200802.13

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@article{10.11648/j.ajim.20200802.13,
  author = {Soukaina Safir and Rim Ouazzane and Amal Moukhliss and Hanane Choukrani and Abdenacer Drighil and Leila Azzouzi and Rachida Habbal},
  title = {Restrictive Cardiomyopathy Revealing Systemic Sclerodermia},
  journal = {American Journal of Internal Medicine},
  volume = {8},
  number = {2},
  pages = {62-65},
  doi = {10.11648/j.ajim.20200802.13},
  url = {https://doi.org/10.11648/j.ajim.20200802.13},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajim.20200802.13},
  abstract = {Primary myocardial involvement is common in systemic sclerosis. There is growing evidence strongly suggesting that this involvement is linked to repeated focal ischemic lesions causing irreversible myocardial fibrosis. It can affect all heart structures. We report the case of a 65 years old male patient, without any personal medical history, admitted for inaugural right heart decompensation. The diagnosis of restrictive cardiomyopathy is based mainly on the following criteria: on echocardiography, hypertrohy of the right ventricle with alteration of the longitudinal systolic function, dilation of the right atrium, with good overall contractility. On right cardiac catheterization: elevation and equalization of the telesystolic pressures of the right atrium, right ventricle, and pulmonary artery with appearance of dip plateau on the right ventricle curve. Scleroderma was evoked considering following statements: the presence of Raynaud's phenomenon, unknown by the patient (presence of dermal sclerosis with pericapillary oedema and capillary dystrophy on capillaroscopy), cutaneous involvement (presence of perivascular and perineural mononuclear infiltrates, with collagen fibers increased in number and thickness), pulmonary involvement (diffuse bilateral interstitial lung disease) and renal involvement (moderately impaired function and positive proteinuria). The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer non-invasive imaging techniques. Therfore, it would be necessary to search the cardiac involvement especially subclinical one in this type of system disease, as well as to confirm the systemic origin in front of certain forms of cardiopathies in particular restrictive cardiomyopathy.},
 year = {2020}
}

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TY  - JOUR
T1  - Restrictive Cardiomyopathy Revealing Systemic Sclerodermia
AU  - Soukaina Safir
AU  - Rim Ouazzane
AU  - Amal Moukhliss
AU  - Hanane Choukrani
AU  - Abdenacer Drighil
AU  - Leila Azzouzi
AU  - Rachida Habbal
Y1  - 2020/03/10
PY  - 2020
N1  - https://doi.org/10.11648/j.ajim.20200802.13
DO  - 10.11648/j.ajim.20200802.13
T2  - American Journal of Internal Medicine
JF  - American Journal of Internal Medicine
JO  - American Journal of Internal Medicine
SP  - 62
EP  - 65
PB  - Science Publishing Group
SN  - 2330-4324
UR  - https://doi.org/10.11648/j.ajim.20200802.13
AB  - Primary myocardial involvement is common in systemic sclerosis. There is growing evidence strongly suggesting that this involvement is linked to repeated focal ischemic lesions causing irreversible myocardial fibrosis. It can affect all heart structures. We report the case of a 65 years old male patient, without any personal medical history, admitted for inaugural right heart decompensation. The diagnosis of restrictive cardiomyopathy is based mainly on the following criteria: on echocardiography, hypertrohy of the right ventricle with alteration of the longitudinal systolic function, dilation of the right atrium, with good overall contractility. On right cardiac catheterization: elevation and equalization of the telesystolic pressures of the right atrium, right ventricle, and pulmonary artery with appearance of dip plateau on the right ventricle curve. Scleroderma was evoked considering following statements: the presence of Raynaud's phenomenon, unknown by the patient (presence of dermal sclerosis with pericapillary oedema and capillary dystrophy on capillaroscopy), cutaneous involvement (presence of perivascular and perineural mononuclear infiltrates, with collagen fibers increased in number and thickness), pulmonary involvement (diffuse bilateral interstitial lung disease) and renal involvement (moderately impaired function and positive proteinuria). The concept of cardiac dysfunction in scleroderma and other rheumatologic conditions has received new interest with the advent of newer non-invasive imaging techniques. Therfore, it would be necessary to search the cardiac involvement especially subclinical one in this type of system disease, as well as to confirm the systemic origin in front of certain forms of cardiopathies in particular restrictive cardiomyopathy.
VL  - 8
IS  - 2
ER  -

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