NATURAL HISTORY- SCLERODERMA & PULMONARY HYPERTENSION

这个子项目是众多研究子项目之一

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This is a registry study of scleroderma patients to determine the time of pathologic progression of pulmonary hypertension from pre-pulmonary hypertension, to diagnosable pulmonary hypertension, to clinical worsening of disease. Patients entered into the registry will be followed in prospective fashion noting the clinical course of disease by both scheduled and event driven follow up. Inclusion Criteria includes Exercise echocardiogram with a resting sPAP of >35mmHg or pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLco ratio >1.6. For diagnosis of definite pulmonary hypertension, patients with symptomatic pulmonary hypertension with a right heart catheterization showing a mean PA pressure > 25mmHg. diagnosed in the past 6 months.