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.
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.
Study Type
OBSERVATIONAL
Enrollment
17
University Medical Center Groningen
Groningen, Netherlands
Ventricular remodeling on cardiac magnetic resonance
Absolute increase in RV ejection fraction
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Absolute increase in LV ejection fraction
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Absolute decrease in RV myocardial mass
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Absolute increase in LV myocardial mass
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Absolute decrease in RV end-diastolic volume
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Absolute increase in LV end-diastolic volume
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Restoration of septal displacement
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
Decrease in RV myocardial extracellulair volume assessed with T1-mapping
Time frame: Six months postoperative
Ventricular remodeling on cardiac magnetic resonance
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Decrease in LV end-systolic eccentricity index
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Increase in tricuspid annular plane systolic excursion (i.e. TAPSE)
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Increase in myocardial performance index (MPI) or Tei index
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Increase in tricuspid annular systolic motion velocity (i.e. RV s')
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Increase in RV (global/septal/free wall) longitudinal strain
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Decrease in RA size
Time frame: Six months postoperative
Functional remodeling patterns after LTX, assessed with echocardiography
Increase in LA size
Time frame: Six months postoperative
Change in heart failure biomarkers
Decrease in NT pro-BNP
Time frame: Six months postoperative
Electrocardiographic remodeling
Normalization of RV hypertrophy and strain
Time frame: Six months postoeprative
Electrocardiographic remodeling
Normalization of right axis deviation
Time frame: Six months postoeprative
Electrocardiographic remodeling
Normalization of right atrial enlargement
Time frame: Six months postoeprative