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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

Completed
Conditions
Pulmonary Arterial Hypertension
Interventions
Registration Number
NCT03863990
Lead Sponsor
Bayer
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.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
104
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.
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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.
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Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
Patients with PAHPAH medicationAdult male and female patients from Argentina diagnosed with pulmonary arterial hypertension (PAH) of WHO functional class I between 01-Jan-2012 and 31-Dec-2017 and with at least one year of follow-up.
Primary Outcome Measures
NameTimeMethod
SexRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Age at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
EthnicityRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Descriptive analysis of comorbidities at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Overall survival rateRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
PAH-subgroup at baseline as assessed by physicianRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

PAH-subgroups may be idiopathic, heritable, drug- or toxin-induced, or associated PAH (with CTD or HIV or portopulmonary hypertension or repaired congenital heart disease).

Right atrial pressure at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary artery pressure at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary vascular resistance at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary artery wedge pressure (PAWP) at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Mixed venous oxygen saturation (SvO2) at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Cardiac Index (CI) at baseline by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pericardial effusion at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patients may have no, mild, moderate or severe pericardial effusion.

Right ventricular function at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patients may have a normal, mild, moderate and severe right ventricular function.

6-minute walking distance at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Right atrial area at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Tricuspid annular plane systolic excursion (TAPSE) at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary artery systolic pressure at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Left ventricular ejection fraction at baseline by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary hypertension functional class according to WHO classification at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Four functional classes ranging from Class I (Pulmonary hypertension without limited physical activity) to Class IV (Pulmonary hypertension with strongly limited physical activity).

Syncope frequency at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

No, occasional or repeated syncope

Systolic blood pressure at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Heart rate at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Body weight at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary vasoreactivity at baseline by pulmonary artery pressureRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Yes / No - variable

Peak oxygen consumption by cardiopulmonary exercise test at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Time from onset of diagnostic symptoms to PAH-diagnosisRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Body mass index at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Concentration of diagnostic markers for heart failure in blood at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Used diagnostic marker are either Brain natriuretic Peptide (BNP) or N-terminal pro b-type Natriuretic Peptide (NT-proBNP).

Drug class of supportive PAH treatmentRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Supportive treatments for PAH are assigned to four drug classes: diuretics, anticoagulants, oxygen and other.

Symptoms progression at baseline assessed by physicianRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patient may display no, a slow or rapid progression of symptoms.

Drug class of PAH-treatment after diagnosisRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

PAH-treatments are assigned to six drug classes: endothelin receptor antagonists (ERA), PDE5 inhibitors, prostanoides, prostacyclin receptor agonists, soluble guanylate cyclase (sGC) stimulants and calcium blockers.

PAH risk status at baseline according to ESC/ERS 2015 guidelinesRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patients may have a low, intermediate or high risk for PAH according to the European Society of Cardiology and European Respiratory Society 2015 guidelines.

Body height at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Time from diagnosis to death from any causeRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Secondary Outcome Measures
NameTimeMethod
Survival rate of patients with low risk for PAHRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary artery wedge pressure (PAWP) at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Cardiac Index (CI) at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Right atrial pressure at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary artery pressure at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary vascular resistance at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Mixed venous oxygen saturation (SvO2) at follow-up by right heart catheterization hemodynamicsRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Right ventricular function at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Left ventricular ejection fraction at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Cause of deathRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Time from diagnosis to death from any causeRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Survival rate of patients with intermediate or high risk for PAHRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
PAH risk status of patients without transplant being alive at the date of latest medical records according to ESC/ERS 2015 guidelinesRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patients may have low, intermediate and high risk for PAH according to the European Society of Cardiology and European Respiratory Society 2015 guidelines.

Right atrial area at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pericardial effusion at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Peak oxygen consumption by cardiopulmonary exercise test at follow-upRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Pulmonary hypertension functional class according to WHO classification at baselineRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Four functional classes ranging from Class I (Pulmonary hypertension without limited physical activity) to Class IV (Pulmonary hypertension with strongly limited physical activity).

Symptoms progression at follow-upRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Syncope frequency at follow-upRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
PAH risk status at follow-up according to ESC/ERS 2015 guidelinesRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Patients may have low, intermediate and high risk for PAH according to the European Society of Cardiology and European Respiratory Society 2015 guidelines.

Time from diagnosis to pulmonary transplantRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Number of hospitalizations per year due to PAH-progressionRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Tricuspid annular plane systolic excursion (TAPSE) at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Concentration of diagnostic markers for heart failure in blood at follow-upRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Used diagnostic marker are either Brain natriuretic Peptide (BNP) or N-terminal pro b-type Natriuretic Peptide (NT-proBNP).

Pulmonary transplantRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Yes or No

Proportion of patients with low PAH riskRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Risk status is assessed according to the Society of Cardiology and European Respiratory Society (ESC/ERS) 2015 guidelines.

Pulmonary artery systolic pressure at follow-up by echocardiographyRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
6-minute walking distance at follow-upRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018
Proportion of patients with intermediate+high PAH riskRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Risk status is assessed according to the Society of Cardiology and European Respiratory Society (ESC/ERS) 2015 guidelines.

Time from diagnosis to first hospitalization due to PAH-progressionRetrospective analysis of data from 01-Jan-2012 to 31-Dec-2018

Trial Locations

Locations (1)

Many facilities

🇦🇷

Multiple Locations, Argentina

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