Abstract:
Introduction.
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).
Purpose.
To highlight the importance of the multidisciplinary approach to a
patient with systemic sclerodermia.
Material and methods.
Patient with paresthesia at low temperatures, discoloration of the fingers,
dysphagia, arthralgias, thickening and stiffness of the skin, fatigue and
dyspnea was examined clinically and paraclinical.
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. Transthoracic echocardiography: LV diastolic dysfunction, ejection fraction 61%,
severe obstruction of the LV ejection tract. HCM is an autosomal dominant
genetic disorder associated with HLA-DR3 genes, acting with genetic and nongenetic 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.
Conclusions.
This affiliation can be interpreted as two concomitant diseases or a causal
association.
