Ventricular Reversed Remodeling After LTX in PAH Patients

Study Overview
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Brief Summary
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The investigators will evaluate ventricular reversed remodelling after double lung transplantation (LTX) in patients with pulmonary arterial hypertension (PAH), measured with cardiac magnetic resonance imaging (MRI). Reversed remodelling will be compared with control patients without PAH (e.g. Cystic Fibrosis) who will also undergo LTX.

Detailed Description
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In this study, pre-LTX and six-months post-LTX measurements will be compared with each other and between the primary and control group. Pre- and post-LTX measurements include: Past medical history: Including basic diagnosis; interventions, surgery and transplant related complications (re-operations, hospitalizations, infections) and medication history; These data will be collected by studying the medical files including surgical reports. Present medical history: Including NYHA class. Physical examination: Including length and weight. Cardiac Magnetic Resonance Imaging: Ventricular volume, function and mass measurements Flow measurements of the pulmonary artery and aorta Disease specific measurements (e.g. septal bowing, RV trabecularisation, etc.) T1-mapping Transthoracic Echocardiography Resting ECG: Disease specific electrophysiological findings (e.g. QRS-duration, right bundle branch block). Laboratory evaluation: NT-pro-BNP eGFR Remaining serum will be stored.

Official Title
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Imaging of Ventricular Reversed Remodeling After Double Lung Transplantation in Patients With Pulmonary Arterial Hypertension

Conditions
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Pulmonary Arterial Hypertension

Participation Criteria
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Eligibility Criteria
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Inclusion criteria: Patients who are on the waiting list for double-LTX, in our institution, for pulmonary arterial hypertension. Eligible for CMR imaging No claustrophobia No pacemaker, ICD, etc. Informed consent Exclusion criteria: Inability to comply with primary endpoint measures. Body mass index ≥40 kg/m2. Pregnant patients will not be included, they may be included >3 months after pregnancy. Patients with age <18 years.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Pulmonary arterial hypertension<br>Patients who are on the waiting list for double-LTX for pulmonary arterial hypertension. | |
| Control group<br>Control patients without increased pulmonary artery pressure (i.e. RV peak pressure <35 mmHg measured with echocardiography) who are on the waiting list for double-LTX. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Ventricular remodeling on cardiac magnetic resonance | Absolute increase in RV ejection fraction | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Absolute increase in LV ejection fraction | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Absolute decrease in RV myocardial mass | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Absolute increase in LV myocardial mass | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Absolute decrease in RV end-diastolic volume | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Absolute increase in LV end-diastolic volume | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Restoration of septal displacement | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Decrease in RV myocardial extracellulair volume assessed with T1-mapping | Six months postoperative | 
| Ventricular remodeling on cardiac magnetic resonance | Decrease in LV end-systolic eccentricity index | Six months postoperative | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Functional remodeling patterns after LTX, assessed with echocardiography | Increase in tricuspid annular plane systolic excursion (i.e. TAPSE) | Six months postoperative | 
| Functional remodeling patterns after LTX, assessed with echocardiography | Increase in myocardial performance index (MPI) or Tei index | Six months postoperative | 
| Functional remodeling patterns after LTX, assessed with echocardiography | Increase in tricuspid annular systolic motion velocity (i.e. RV s') | Six months postoperative | 
| Functional remodeling patterns after LTX, assessed with echocardiography | Increase in RV (global/septal/free wall) longitudinal strain | Six months postoperative | 
| Functional remodeling patterns after LTX, assessed with echocardiography | Decrease in RA size | Six months postoperative | 
| Functional remodeling patterns after LTX, assessed with echocardiography | Increase in LA size | Six months postoperative | 
| Change in heart failure biomarkers | Decrease in NT pro-BNP | Six months postoperative | 
| Electrocardiographic remodeling | Normalization of RV hypertrophy and strain | Six months postoeprative | 
| Electrocardiographic remodeling | Normalization of right axis deviation | Six months postoeprative | 
| Electrocardiographic remodeling | Normalization of right atrial enlargement | Six months postoeprative | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary, Arterial, Hypertension, Lung, Transplantation, MRI