Connective tissue diseases represents an important risk factor for pulmonary hypertension, either due to vasculopathy or chronic pulmonary thromboembolism. These chronic conditions are characterized by being disabling, progressive and fatal in a short time and are caused by multiple mechanisms that results in remodeling of the pulmonary microvasculature and right heart failure.~In recent years, due to international registries, we have acquired a better understanding of high-risk subpopulations, their poor prognostic factors, and the results of specific combination therapies. In Mexico, the National Institute of Medical Sciences and Nutrition Salvador Zubirán (INCMNSZ) is a referral center for connective tissue diseases and pathologies of the pulmonary circulation. The RECONNECTIVE registry is a five years follow-up cohort that offers the possibility to obtain data regarding to the clinical characteristics and hemodynamic assesment of pulmonary circulation. The main objective is to evaluate the clinical course of the PAH in this subgroup of patients and their specific therapy for PAH.
The RECONNECTIVE Registry is an observational single center study, focused on the subgroup of precapillary pulmonary hypertension related to connective tissue diseases. All patients will have hemodynamic confirmation by right heart catheterization and will be follow-up for at least 5 years from admission. All patients diagnosed with Group I Pulmonary Arterial Hypertension (PAH) associated with Connective Tissue Diseases (CTD) and Group IV Pulmonary Hypertension (PH) with CTD will be included. The purpose of the registry is to learn and understand the clinical outcomes and natural history of the pulmonary arterial hypertension in this subgroup of patients to improve the medical care and treatment.