Clinical trial NCT03863990: Study Performed at Various Medical Centers to Learn More About Survival and Expected Course of Pulmonary Arterial Hypertension, a Type of High Blood Pressure in the Lungs Related to the Narrowing of the Small Blood Vessels in the Lungs
Official title: Survival and Prognostic Factors in Pulmonary Arterial Hypertension. A Multicenter Observational Registry (START)
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Pulmonary Arterial Hypertension
Keywords: PAH prognosis
PAH outcomes
PAH survival
PAH risk status
Lead sponsor: Bayer
Sponsor class: INDUSTRY
First posted: 2019-03-05
Enrollment: 104
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Adults patients with pulmonary arterial hypertension from tertiary care centers in Argentina

Brief summary: In this study researchers want to learn more about Pulmonary Arterial Hypertension, a type of high blood pressure in the lungs related to the narrowing of the small blood vessels in the lungs (group 1 according to WHO classification). Goal of the study is to describe the signs and risk factors of the illness at study start and the chances of survival.

Detailed description: The primary objective of the study is to describe baseline clinical characteristics and overall survival in a cohort of patients with pulmonary arterial hypertension (PAH) of WHO functional class I in Argentina.

Secondary objectives are to study the discriminatory ability of the risk assessment tool presented in the European Society of Cardiology and European Respiratory Society (ESC/ERS) 2015 guidelines and to explore the potential prognostic advantage of a low-risk profile at follow-up as treatment goal.

Eligibility criteria: Inclusion Criteria:

* Consecutive newly diagnosed patients by Right heart catheterization (RHC) from 01-Jan-2012 to 31-Dec-2017, belonging to one of the following of Group 1 PAH subgroups: Idiopathic (IPAH), or Heritable (HPAH), or Drug or toxin induced, or Associated (APAH) with one of the following: Connective tissue disease; Congenital heart disease with simple systemic to pulmonary shunt at least 1 year after surgical repair; Portal Hypertension or HIV infection.
* Diagnosis of PAH by RHC exhibiting a mean pulmonary artery pressure (MPAP) ≥ 25 mmHg and a pulmonary artery wedge pressure (PAWP) ≤15 mmHg at normal or reduced cardiac output, according to European Society of Cardiology and European Respiratory Society (ESC/ERS) 2009 guidelines or MPAP ≥ 25 mmHg and a PAWP ≤15 mmHg and a pulmonary vascular resistance (PVR) \> 3 WU according to ESC/ERS 2015 guidelines.
* Patients with at least one year documented follow up or that have died or received transplant before 1 year of follow up after baseline RHC and that have initiated treatment with a PAH-targeted medication.

Exclusion Criteria:

* Patients with severe concomitant left heart disease (left ventricular ejection fraction \<35%).
* Patients with restrictive lung disease (Forced vital capacity (FVC) \<60% predicted) other than connective tissue disease or obstructive lung disease (forced expiratory volume (FEV) \<60% predicted, with FEV1/FVC\<70%).
* Clinical or radiological evidence of Pulmo-Veno-Occlusive Disease (PVOD) or Pulmonary Capillary Haemangiomatosis (PCH).
* Hypertrophic obstructive cardiomyopathy.
* Severe proven or suspected coronary artery disease.
* Congenital or acquired valvular or myocardial disease if clinically significant apart from tricuspid valvular insufficiency due to pulmonary hypertension.
* Underlying medical disorders at baseline with an anticipated life expectancy below 2 years (e.g. active cancer disease with localized and/or metastasized tumor mass) or Clinical relevant hepatic dysfunction (Child-Pugh B and C) or Renal insufficiency (glomerular filtration rate \<30 mL/min).
* Diagnosis of a pulmonary hypertension from WHO groups 2, 3, 4 or 5.

Primary outcomes:
measure: Age at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Sex
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Ethnicity
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Descriptive analysis of comorbidities at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: PAH-subgroup at baseline as assessed by physician
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Right atrial pressure at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery pressure at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary vascular resistance at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery wedge pressure (PAWP) at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Cardiac Index (CI) at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Mixed venous oxygen saturation (SvO2) at baseline by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary vasoreactivity at baseline by pulmonary artery pressure
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Peak oxygen consumption by cardiopulmonary exercise test at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Right atrial area at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pericardial effusion at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Right ventricular function at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Tricuspid annular plane systolic excursion (TAPSE) at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery systolic pressure at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Left ventricular ejection fraction at baseline by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: 6-minute walking distance at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary hypertension functional class according to WHO classification at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Time from onset of diagnostic symptoms to PAH-diagnosis
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Symptoms progression at baseline assessed by physician
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Syncope frequency at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Systolic blood pressure at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Heart rate at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Body weight at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Body height at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Body mass index at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Concentration of diagnostic markers for heart failure in blood at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Drug class of supportive PAH treatment
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Drug class of PAH-treatment after diagnosis
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: PAH risk status at baseline according to ESC/ERS 2015 guidelines
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Overall survival rate
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Time from diagnosis to death from any cause
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Secondary outcomes:
measure: Right atrial pressure at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery pressure at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary vascular resistance at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery wedge pressure (PAWP) at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Cardiac Index (CI) at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Mixed venous oxygen saturation (SvO2) at follow-up by right heart catheterization hemodynamics
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Peak oxygen consumption by cardiopulmonary exercise test at follow-up
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Right atrial area at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pericardial effusion at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Right ventricular function at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Tricuspid annular plane systolic excursion (TAPSE) at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary artery systolic pressure at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Left ventricular ejection fraction at follow-up by echocardiography
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: 6-minute walking distance at follow-up
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary hypertension functional class according to WHO classification at baseline
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Symptoms progression at follow-up
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Syncope frequency at follow-up
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Concentration of diagnostic markers for heart failure in blood at follow-up
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: PAH risk status at follow-up according to ESC/ERS 2015 guidelines
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Cause of death
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Time from diagnosis to death from any cause
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Pulmonary transplant
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Time from diagnosis to pulmonary transplant
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Time from diagnosis to first hospitalization due to PAH-progression
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Number of hospitalizations per year due to PAH-progression
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Proportion of patients with low PAH risk
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Proportion of patients with intermediate+high PAH risk
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Survival rate of patients with low risk for PAH
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: Survival rate of patients with intermediate or high risk for PAH
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
measure: PAH risk status of patients without transplant being alive at the date of latest medical records according to ESC/ERS 2015 guidelines
timeFrame: Retrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Other outcomes:
Locations:
city: Multiple Locations
country: Argentina
facility: Many facilities
Study officials:
References: