Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation in Pulmonary Arterial Hypertension

• Register: ClinicalTrials.gov
• Last refreshed on: 12 December 2020
• Main ID: NCT01054105
• Date of registration: 21/01/2010
• Prospective Registration: Yes
• Primary sponsor: Gachon University Gil Medical Center
• Public title: Effect of BMPR-2 Gene Mutations on Hemodynamic Response by Iloprost Inhalation in Pulmonary Arterial Hypertension PILGRIM
• Scientific title: The Prevalence of BMPR-2 Gene Mutations in Korean Patients With Pulmonary Arterial Hypertension (PAH) and the Effects of Gene Mutations on Hemodynamic Response by Drug Therapy
• Date of first enrolment: October 2010
• Target sample size: 73
• Recruitment status: Completed
• URL: https://clinicaltrials.gov/show/NCT01054105
• Study type: Observational

Countries of recruitment

Korea, Republic of

Contacts

• Name: Wook-Jin Chung, MD,PhD
• Affiliation: Gachon University Gil Medical Center

Key inclusion & exclusion criteria

Inclusion Criteria:

1. The patients aged from 20 to 80 years

2. Newly diagnosed WHO category I PAH patients: Patients who meet the following criteria
within 3 months obtained by right heart catheterization (mean PAP of more than 25 mm
Hg at rest and mean pulmonary arterial wedge pressure (PCWP) or left ventricular
end-diastolic pressure of 15 mm Hg or less) or echocardiography (peak PAP of more than
40mmHg and mean PAP more than 30mmHg).

3. Previously diagnosed PAH patients who refractory to conventional treatment except
iloprost inhalation solution (Ventavis): Patients meet the echo criteria (peak PAP of
more than 40mmHg and mean PAP more than 30mmHg) who have been treated with PAH
medications except iloprost inhalation solution (Ventavis) after diagnosed as WHO
Group 1 PAH based on prior RHC data (above criteria) but refractory to them.

4. The patients who are able to undergo low intensity exercise test (low dose bicycle or
walking)

Exclusion Criteria:

1. The patients with other left heart disease (category II in WHO classification of
pulmonary hypertension); ex. Congestive HF, cardiomyopathy, significant valvular heart
disease, significant arrhythmia, suspicious elevated PCWP.

2. The patients with category III,IV and V in WHO classification of pulmonary
hypertension:

- Pulmonary hypertension with lung disease and/or hypoxemia

- Chronic obstructive pulmonary disease

- Interstitial lung disease

- Sleep disorder breathing

- Alveolar hyperventilation disorders

- Chronic exposure to high altitude

- Developmental abnormalities

- Pulmonary hypertension due to chronic thrombotic and/or embolic disease

- Thromboembolic obstruction of the proximal pulmonary arteries

- Thromboembolic obstruction of the distal pulmonary arteries

- Non-thrombotic pulmonary embolism (e.g. tumor or parasitic)

- Miscellaneous disorders affecting the pulmonary vasculature

- Patients with contraindication to Ventavis;(Hypersensitive to Ventavis, High risk
of bleeding, which can be increased by use of Ventavis (e.g. active peptic ulcer,
trauma, intracranial hemorrhage)

- Severe coronary disease

- Unstable angina

- History of Acute myocardial infarction within 6 months

- Uncompensated heart failure not under close medical monitoring

- Severe arrhythmia

- Suspected pulmonary congestion

- Cerebrovascular disease within 3 months (e.g. transient ischemic attack, stoke)

- Pulmonary hypertension due to venous occlusive disease, valvular defect with
dysfunction of cardiac muscle, which is independent of pulmonary hypertension)

- Pregnancy

- Women with high probability of pregnancy

- Breast feeding

- Renal failure (creatinine clearance: less than 30mL/min)

3. The patients concurrently using other pulmonary vasodilator (ex. Inhaled NO,
endothelin antagonists) except PDE5 inhibitor

4. The patients with poor echo window which is unavailable to accept the echo data

5. The patients who cannot do any exercise

6. The patients who changes medication administered during ventavis treatment

7. The patients with allergic reaction to ventavis

8. The patients with other systemic disease (ex. Leukemia, MM, Sickle cell anemia,
significant liver disease)



Age minimum: 20 Years
Age maximum: 80 Years
Gender: All

Health Condition(s) or Problem(s) studied

Pulmonary Arterial Hypertension

Intervention(s)

Drug: Iloprost

Primary Outcome(s)

Cardiopulmonary exercise test parameters [Time Frame: after 3 months active follow-up]

Secondary Outcome(s)

Major cardiovascular Events (cardiovascular mortality, all cause mortality, hospitalization) [Time Frame: After 2 years follow-up]

NT-proBNP [Time Frame: After 3 months active follow-up]

WHO/NYHA class [Time Frame: After 3 months active follow-up]

Echo parameters [Time Frame: After 3 months active follow-up]

Six-minutes walking test [Time Frame: After 3 months active follow-up]

Secondary ID(s)

WJC-IIT-1001

GIRBA 2278

Source(s) of Monetary Support

Please refer to primary and secondary sponsors

Secondary Sponsor(s)

The Catholic University of Korea

Seoul National University Bundang Hospital

Bayer

Seoul National University Hospital