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616.5-004.1+616.127-007.61 (1) |
Skin. Common integument. Clinical dermatology. Cutaneous complaints (211) |
Pathology of the circulatory system, blood vessels. Cardiovascular complaints (1025) |
![]() GUȚAN, Inesa, PANFILE, Elena, MAZUR-NICORICI, Lucia, VETRILĂ, Snejana, LÎSÎI, Dorin, MAZUR, Minodora. Systemic sclerodermia and hypertrophic cardiomyopathy - causal or stochastic affiliation. In: Revista de Ştiinţe ale Sănătăţii din Moldova, 2022, nr. 3 An.1(29), p. 200. ISSN 2345-1467. |
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Revista de Ştiinţe ale Sănătăţii din Moldova | |||||||
Numărul 3 An.1(29) / 2022 / ISSN 2345-1467 | |||||||
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CZU: 616.5-004.1+616.127-007.61 | |||||||
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Background. Hypertrophic cardiomyopathy (HCM) is characterized by the presence of left ventricular hypertrophy, which cannot be explained only by ventricular filling abnormalities. HCM has been previously described in a small number of patients with systemic scleroderma (SDS). Objective of the study. Highlighting the importance of a multidisciplinary approach to the diagnostic approach in a patient with SDS. Material and Methods. Patient with paresthesia at low temperatures, discoloration of the fingers, dysphagia, arthralgia, thickening, and stiffness of the skin, fatigue, dyspnea on physical exertion. Results. Clinical and paraclinical parameters: BP-130/80mmHg, HR-74bpm; PCR - 22.9 mg/L, ESR - 21 mm/h, pro-BNP - 2461 ng/ml, positive Scl-70, ANA - 1/5120, HLA-DR3 was positive; ECG - sinus rhythm, LV myocardial hypertrophy. Trans-thoracic echocardiography: LV diastolic dysfunction, ejection fraction 61%, SIV – 17 mm, PPVS - 13 mm with severe obstruction of the LV ejection tract. HCM is an autosomal dominant genetic disorder associated with HLA-DR3 genes, acting with genetic and non-genetic factors, in which the link to SDS is perceived. Diffuse connective tissue disease can be considered a “natural experiment” in the interaction between inflammation and heart disease, which could elucidate the fundamental mechanisms by which inflammation accelerates the development of cardiovascular disease. Thus, in a patient with SDS, the vector of the investigation is redirected to a confluence that includes cardiac involvement. Conclusion. Diagnosis: systemic scleroderma and hypertrophic cardiomyopathy. This affiliation can be interpreted as two concomitant diseases or a causal association |
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Cuvinte-cheie systemic sclerosis, hypertrophic cardiomyopathy., sclerodermie sistemică, cardiomiopatie hipertrofică |
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