Clinical trial NCT05665556: REgistry of Pulmonary Arterial Hypertension Associated With CONNECTIVE Tissue Diseases (RECONNECTIVE)
Official title: REgistry of Pulmonary Arterial Hypertension Associated With CONNECTIVE Tissue Diseases (RECONNECTIVE) at the National Institute of Medical Sciences and Nutrition Salvador Zubirán
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Pulmonary Arterial Hypertension
Connective Tissue Diseases
Keywords: Pulmonary Arterial Hypertension
Connective Tissue Disease
Lead sponsor: Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
Sponsor class: OTHER
First posted: 2022-12-27
Enrollment: 170
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patients with Group I Pulmonary Arterial Hypertension (PAH) associated to Connective Tissue Diseases (CTD) and Group IV Pulmonary Hypertension group (PH) with CTD.

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: Inclusion Criteria:

* Incident and prevalent patients diagnosed with Group I associated with Connective Tissue Diseases (CTD)
* Incident and prevalent patients diagnosed with Group IV Pulmonary Arterial Hypertension (PAH) associated with Connective Tissue Diseases (CTD) with evidence of a chronic thromboembolic pulmonary disease by ventilation/perfusion pulmonary gammagraph or computed tomography pulmonary angiogram with at least three months of total anticoagulation therapy.
* Patient diagnosed with a connective tissue disease according to the classification criteria of the American College of Rheumatology.
* Precapillary pulmonary hypertension confirmed by right heart catheterization (RHC): Mean pulmonary arterial pressure (mPAP) \>20 mm Hg with a pulmonary arterial wedge pressure ≤ 15 mm Hg and Pulmonary vascular resistance (PVR) ≥ 2.0 Wood units

Exclusion Criteria:

* Patients who meet the criteria for another group of pulmonary hypertension (Groups II, III or V).

Primary outcomes:
measure: To characterize the clinical clinical course of Pulmonary Arterial Hypertension (PAH) associated with Connective Tissue Diseases (CTD) in a register of Mexican patients from the first visit and at every 6 months.
timeFrame: 5 years
Secondary outcomes:
measure: World Health Organization functional class (I-IV)
timeFrame: 5 years
measure: Assesment of the right ventricular function by echocardiogram (TAPSE, Right atrial area and pericardial effusion)
timeFrame: 5 years
measure: Concentration of serum brain natriuretic peptide
timeFrame: 5 years
Other outcomes:
Locations:
city: Mexico City
country: Mexico
facility: Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán
Study officials:
affiliation: Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
name: Jose Luis Hernandez Oropeza, PhD
role: PRINCIPAL_INVESTIGATOR
References: