cardiac repercussion of systemic sclerodermias

Conditions: Systemic Scleroderma

Summary: systemic sclerodermia is a connectivity characterized by multiple visceral impairments, in particular pulmonary, which can lead to the development of a pulmonary arterial hypertension (paht). in one hand, this paht is an evolutionary turn in symptomatology and prognosis, and on the other hand, the tracking and the analysis of its effects on the right ventricular function are difficult with the conventional techniques. so, the analysis of the right ventricular function appears capital, because: * it is recognized like an essential determinant of the symptoms and effort capacity, * its prevalence, physiopathology and prognostic values remain unknown in this pathology, * its interest in the starting of the treatment remains to be specified. the aim of this trial is to identify in a population of 150 patients presenting a systemic scleroderma without paht: * the incidence of a right ventricular dysfonction, evaluated by the analysis of the myocardic regional function with myocardial tissular doppler mode, * the physiopathology of this damage by correlation with the tests of respiratory function and the not invasive hemodynamic datas at rest and exercise. * the prognosis value of the abnormalities of the right ventricular function by a follow-up of these patients over a 5 years period. this trial should allowed to define the place of the new right ventricular function markers in the evaluation of the functional consequences, the forecast and perhaps the care of systemic sclerodermic patients.


Eligibility criteria:
inclusion criteria: age between 18 years and 75 years * having a maximum speed of it \< 2.8 m/sec. * having read and understood the information note and having signed the informed consent form. * and : * either carrying a systemic sclerodermia satisfying the criteria of american college of rheumatology (the presence of a major criteria or two minor criterias is required for the systemic diagnosis of sclerodermia): * major criteria: proximal sclerodermia defined by a bilateral cutaneous sclerosis extending beyond the metacarpophalangeal and metatarsophalangeal joint. * minor criteria: pulpar sclerodactylia, pulpar canker or scars, pulmonary fibrosis on thoracic radiography. * or presenting crest syndrome, with at least 4 of the 5 following criterias : * subcutaneous calcinosis, * syndrome of raynaud, * attack oesophagienne, * sclerodactylia, * telangiectasis. exclusion criteria: * over 75 years * having a maximum speed of ti \> 2.8 m/sec. * patient affected by a connectivitis other than a sclerodermia : mixed connectivity, disseminated erythematous lupus, inflammatory idiopathic myopathy, rheumatoïd polyarthritis. * patient carrying a sclerodermia complicated by: * renal failure (clearance \> 30 ml - cockraft) * recent heart failure (\< 2 months) * cardiac valvular attack, dilated, hypertrophic or restrictive cardiomyopathy, constrictive pericarditis, chronic pulmonary heart or antecedent of myocarditis * coronaropathy objectified by stenosis or simple coronary irregularities on the coronarography or antecedent of myocardial infarction * chronic respiratory handicap with another origin than sclerodermia * unsigned informed consent form * patient with mental or psychiatric disorders, unable or unwilling to comply with protocol requirements. * patient treated with intraveinous derived of prostacyclin within 1 month before inclusion

Keywords: systemic scleroderma echocardiography