Cardiac Sarcoidosis: A Contemporary Concept of Forgotten Granulomatosis
Sarcoidosis is a complex multisystem inflammatory granulomatous disease that can affect any organ, with a wide range of clinical presentations. A significant number of patients with systemic sarcoidosis may also have cardiac involvement. Clinical manifestations of cardiac sarcoidosis can include var...
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Published in: | ARYA atherosclerosis Vol. 19; no. 5; pp. 52 - 256 |
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Isfahan Cardiovascular Research Center
01-09-2023
Cardiovascular research institute, Isfahan University of Medical Sciences Vesnu Publications |
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Abstract | Sarcoidosis is a complex multisystem inflammatory granulomatous disease that can affect any organ, with a wide range of clinical presentations. A significant number of patients with systemic sarcoidosis may also have cardiac involvement. Clinical manifestations of cardiac sarcoidosis can include various rhythm and conduction disturbances, as well as heart failure. The structure of sarcoid granulomas is similar to that of tuberculous granulomas, but in contrast, they lack caseous necrosis. Tissue changes in sarcoidosis tissues depend on the stage of development of the disease, progressing from pathological process: macrophage-lymphocytic infiltration to epithelioid cell granuloma formation, and fibrosis. Granulomas can be found in any part of the myocardium, with the most common locations being the free wall of the left ventricle, the basal part of the interventricular septum, and the interatrial septum. Vasculopathy of the pulmonary circulation and coronary arteries is often observed. Advancements in diagnostic imaging techniques, such as computer tomography and magnetic resonance imaging, have facilitated the verification of cardiac sarcoidosis. This article presents an analysis of updated information on cardiac sarcoidosis by a multidisciplinary working group. |
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AbstractList | Sarcoidosis is a complex multisystem inflammatory granulomatous disease that can affect any organ, with a wide range of clinical presentations. A significant number of patients with systemic sarcoidosis may also have cardiac involvement. Clinical manifestations of cardiac sarcoidosis can include various rhythm and conduction disturbances, as well as heart failure.
The structure of sarcoid granulomas is similar to that of tuberculous granulomas, but in contrast, they lack caseous necrosis. Tissue changes in sarcoidosis tissues depend on the stage of development of the disease, progressing from pathological process: macrophage-lymphocytic infiltration to epithelioid cell granuloma formation, and fibrosis. Granulomas can be found in any part of the myocardium, with the most common locations being the free wall of the left ventricle, the basal part of the interventricular septum, and the interatrial septum. Vasculopathy of the pulmonary circulation and coronary arteries is often observed.
Advancements in diagnostic imaging techniques, such as computer tomography and magnetic resonance imaging, have facilitated the verification of cardiac sarcoidosis. This article presents an analysis of updated information on cardiac sarcoidosis by a multidisciplinary working group. Sarcoidosis is a complex multisystem inflammatory granulomatous disease that can affect any organ, with a wide range of clinical presentations. A significant number of patients with systemic sarcoidosis may also have cardiac involvement. Clinical manifestations of cardiac sarcoidosis can include various rhythm and conduction disturbances, as well as heart failure. The structure of sarcoid granulomas is similar to that of tuberculous granulomas, but in contrast, they lack caseous necrosis. Tissue changes in sarcoidosis tissues depend on the stage of development of the disease, progressing from pathological process: macrophage-lymphocytic infiltration to epithelioid cell granuloma formation, and fibrosis. Granulomas can be found in any part of the myocardium, with the most common locations being the free wall of the left ventricle, the basal part of the interventricular septum, and the interatrial septum. Vasculopathy of the pulmonary circulation and coronary arteries is often observed. Advancements in diagnostic imaging techniques, such as computer tomography and magnetic resonance imaging, have facilitated the verification of cardiac sarcoidosis. This article presents an analysis of updated information on cardiac sarcoidosis by a multidisciplinary working group. Diffuse granulomatous alteration of the myocardium leads to a decrease in systolic function of the heart, however, if this indicator does not fall below 35%, then with timely maintenance therapy (pharmacological, hardware) and timely treatment of the underlying disease, normalization of the ejection fraction index can be achieved [8]. [...]arrhythmogenic lesions of the myocardium are associated with granulomatous infiltration in the areas of the cardiac conduction system's structural and functional components 9,10. During the inflammatory process, hypoxia of cardiomyocytes occurs followed by HIF stimulation and thereby many growth factors with profibrotic activity 9. Because of the difference in the properties of electrical conductivity of a healthy myocardium (striated cardiac muscle tissue), as well as adipose and fibrous tissues, a potential difference arises, which is clinically manifested by a high risk of developing multiple diffuse ectopic foci of the macro- and micro-re-entry type, i.e., there is a syndrome of electrophysiological unstable myocardium. Changes in the electrical and contractile properties of the atrial myocardium lead to a change in the volume and composition of the extracellular matrix and, as a result, to the development of myocardial fibrosis, which, in turn, contributes to the onset and maintenance of arrhythmia by a decrease in the elastic properties of the myocardium, in contractile function and pattern of coronary vasculature, which ultimately leads to the heart failure formation 14. |
Author | Ginosyan, Knarik Ionov, Alexey Avagimyan, Ashot Gogiashvili, Liana Mkrtchyan, Lusine Sarrafzadegan, Nizal Jndoyan, Zinaida Sahakyan, Karmen Shafie, Davood Pavluchenko, Ivan Navasardyan, Grizelda Aznauryan, Anahit Bairamyan, Tamara |
AuthorAffiliation | 7 Histology Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 3 Department of Rheumatology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 5 Department of Pathophysiology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 9 Biology Department with a course of Medical Genetics, Kuban State Medical University, Krasnodar, Russia 8 Internal Deceases Propaedeutic Department, Kuban State Medical University, Krasnodar, Russia 1 Department of Anatomical Pathology and Clinical Morphology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 2 Department of Cardiology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 4 Internal Deceases Propaedeutic Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 6 Rheumatology Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia 10 Experimental and Clinical Pathology Department, Al. Natishvili Institute of Morp |
AuthorAffiliation_xml | – name: 11 Heart Failure Research Centre, Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran – name: 4 Internal Deceases Propaedeutic Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 1 Department of Anatomical Pathology and Clinical Morphology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 5 Department of Pathophysiology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 12 Isfahan Cardiovascular Research Center , Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran – name: 10 Experimental and Clinical Pathology Department, Al. Natishvili Institute of Morphology, Iv. Javakhishvili Tbilisi State University, Tbilisi, Georgia – name: 2 Department of Cardiology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 6 Rheumatology Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 3 Department of Rheumatology, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia – name: 9 Biology Department with a course of Medical Genetics, Kuban State Medical University, Krasnodar, Russia – name: 8 Internal Deceases Propaedeutic Department, Kuban State Medical University, Krasnodar, Russia – name: 7 Histology Department, Yerevan State Medical University after M. Heratsi, Yerevan, Armenia |
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Keywords | Heart Heart failure Myocardium Sarcoidosis Biopsy |
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SubjectTerms | Angina pectoris Biopsy Cardiac arrhythmia Cardiomyocytes Cardiomyopathy Cardiovascular disease Extracellular matrix Granulomas heart Heart failure Magnetic resonance imaging Morphology myocardium Review Sarcoidosis Viruses |
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Title | Cardiac Sarcoidosis: A Contemporary Concept of Forgotten Granulomatosis |
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