Cardiac Repercussion of Systemic Sclerodermias

Hospices Civils de Lyon148 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

DIAGNOSTIC

Masking

NONE

Enrollment

148

Conditions

Systemic Scleroderma

Eligibility

Sex: ALLMin age: 18 YearsMax age: 75 Years

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

To measure the incidence of right ventricular dysfonction evaluated by analysis of the regional myocardic function with myocardial tissular Doppler mode

Time frame: each year for 5 years

Secondary Outcomes

To define the potential place of new regional myocardic function markers in the evaluation of the cardiac repercussion of systemic scleroderma and to correlate these markers with clinical, functional and hemodynamic parameters.

Time frame: each year for five years

To detect an eventual prognostic role of regional myocardic function parameters which could allow to an earlier screening of the patients being able to receive a pulmonary vasodilator treatment before the rest PAHT occurrence .