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The purpose of this study is to assess the value in terms of sensitivity, specificity and likelihood ratio of the stress echocardiography in the screening of pulmonary arterial hypertension in patients with systemic sclerosis and indirect signs of pulmonary arterial hypertension.
Pulmonary artery catheterization (rest and exertion) and treadmill stress echocardiography will be done to all patients of the study.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| systemic sclerosis patients | Every patient will have a rest echocardiography, a stress echocardiography, a right heart catheterization, a blood specimen, and a pulmonary function test. |
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| Measure | Description | Time Frame |
|---|---|---|
| Correlation of a 20 mmhg increase in the pulmonary artery pressures (PAP) during stress echocardiography and PAP using right heart catheterization. | Every patient will have both procedures; stress echocardiography and right heart catheterization. A positive stress echocardiography is defined as >= 20 mmhg increase in the systolic pulmonary artery pressure (SPAP) (between rest and stress) or an absolute value >= 55 mmhg. A positive right heart catheterization at rest is defined as a PAPm >25mmhg, wedge < 18 and pulmonary vascular resistances >3 wood units. Stress catheterization will also be perform and is defined as a PAPm > 30mmhg and wedge <18 mm hg. | 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Correlation of a 20 mmhg increase in the PAP during stress echocardiography and elevated NT-proBNP. | 5 years | |
| Function of the left ventricle (left ventricular ejection fraction) at rest and at stress. | We hypothesize that patients who do not increase their left ventricular ejection fraction at stress have a worst clinical outcome in the follow up. |
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Inclusion Criteria:
Exclusion Criteria:
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Systemic slerosis patients with indirect signs of arterial pulmonary hypertension:
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| Name | Affiliation | Role |
|---|---|---|
| Paul Farand, md, msc | Centre de recherche du Centre hospitalier universitaire de Sherbrooke | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre hospitalier universitaire de Sherbrooke | Sherbrooke | Quebec | J1H 5N4 | Canada |
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| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D008171 | Lung Diseases |
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whole blood
| Follow up every year X 5 |
| Diastolic function at rest and at stress | We hypothesize that patients with diastolic dysfunction manifesting at stress have a worst clinical outcome in the follow up. | follow up every year X 5 |
| Function of the right ventricle | We hypothesise that in patients with or without pulmonary hypertension, right ventricular dysfunction is associated with a worse clinical outcome | Follow up every year X 5 |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |