Pulmonary artERial hypertenSion: bonE morPHOgeNEtic protein signaling out of control (PERSEPHONE)

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 100

Inclusion Criteria

Aim 1: BMP10 secretion by right atrial cardiomyocytes
Inclusion criteria patients:
- Patients with chronic thrombo-embolic pulmonary hypertension (CTEPH)
- Undergoing pulmonary endarterectomy, Inclusion criteria controls:
- Patients undergoing cardiac surgery and put on the heart-lung machine, Aim 2:
BMP10 release as a bio-assay of right heart failure, Inclusion criteria
patients:
- Idiopathic PAH (Invasively assessed mean pulmonary artery pressure >25 mmHg,
pulmonary artery wedge pressure <15 mmHg)
- Right heart catheterization and magnetic resonance imaging < 1 year before
participation
- Age > 18 years, Inclusion criteria controls:
- Age >18 years
- Age and sex-matched to patients
- We will include 10 subjects with a BMPR2 mutation without Pulmonary
Hypertension

Exclusion Criteria

Aim 1: BMP10 secretion by right atrial cardiomyocytes, Exclusion criteria
patients:
- Age < 18 years, Exclusion criteria controls:
- Pulmonary hypertension (according to European Society of Cardiology
guidelines: echocardiography, tricuspid regurgitation peak velocity * 2.8
m/sec, estimated systolic pulmonary artery pressure * 36 mmHg and no additional
echocardiographic signs of pulmonary hypertension)(13)
- RV dysfunction (TAPSE < 16 mm)(13)
- RV (annulus) dilatation (RV end-diastolic diameter > 42 mm (base))(13)
- Tricuspid valve replacement or tricuspid annuloplasty
- Dilated right atrium or RA dysfunction (e.g. related to atrial fibrillation,
congenital abnormalities; cut-off values: area > 18 cm2)
- Age < 18 years, Aim 2: BMP10 release as a bio-assay of right heart failure,
Exclusion criteria patients:
- Pregnancy
- Claustrophobia
- Pacemaker, Exclusion criteria controls:
- Pulmonary hypertension (Invasively assessed mean pulmonary artery pressure >
25 mmHg)
- Increased pulmonary artery wedge pressure (>15 mmHg)
- Tricuspid valve dysfunction
- RV dysfunction
- Dilated right atrium or RA dysfunction (e.g. related to atrial fibrillation,
congenital abnormalities)
- Previous cardiac surgery
- Age < 18 years

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>BMP10 levels measured in bloodsamples and conditioned medium of right atrial<br /><br>cardiomyocytes </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>* BMP10 activity plasma vs. serum<br /><br>* Importance of location of the blood sampling (right atrium vs. venous<br /><br>puncture) on BMP10 activity</p><br>