Imaging of Ventricular Reversed Remodeling after Double Lung Transplantation in Patients with Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a fatal disease characterized by right
ventricular (RV) dysfunction and failure. Right heart catheterization (RHC) is
the golden standard for PAH diagnosis. However, over the years, cardiac
magnetic resonance (CMR) has evolved in an excellent non-invasive tool for the
assessment of major functional parameters that are frequently associated with
increased pulmonary artery pressure. Today, CMR is complementary to RHC in the
follow-up of patients with PAH. Furthermore, CMR by itself is of potential
importance in evaluating PAH-targeted therapies.
Current therapies for PAH have improved long-term outcome, although mortality
remains high. For patients refractory to medical therapy, lung transplantation
(LTX) is often indicated as destination therapy. Several studies investigated
functional outcome after LTX. However, these studies are outdated, used
invasive measurements and lack CMR assessment. Exploration of biventricular
remodeling after double-LTX in patients with PAH, using CMR, could be of
potential importance in the timing of surgical intervention and may elucidate
RV and LV functional recovery after long standing pulmonary hypertension and
pressure unloading.

To study remodeling patterns of the left and right ventricle in patients with
pulmonary arterial hypertension who underwent lung transplantation, using
cardiac magnetic resonance imaging.

Explorative case study

The following assessments will be performed twice in each patient (i.e.
preoperative and six months postoperative):
- Past and present medical history: including NYHA-functional class.
- Physical examination: including length and weight.
- CMR (for LV and RV volumetric and mass analysis and extracellular matrix
composition [i.e. T1-mapping]).
- Transthoracic echocardiography (TTE, standard protocol for chamber
quantification; function of valves; pulmonary artery systolic pressure; if
applicable other disease-specific assessments).
- Resting ECG: for the assessment of disease specific electrophysiological
findings.
- Laboratory evaluation: NT-pro-BNP, eGFR (remaining serum will be stored).

In all patients, laboratory evaluation, echocardiography and ECG are part of
the routine follow-up before and after LTX. In order to store serum for
biomarker analyses in the future, three extra blood samples (10 ml each) will
be obtained during the already planned vena-puncture.

In patients with PAH, preoperative and postoperative CMR are part of the
standard investigations.

In the control patients (i.e. no pressure load of the RV), preoperative and
postoperative CMR are not part of the standard investigations.

All investigations will be scheduled in combination with already planned visits
to the clinic. Therefore, participation in this study will expose the patients
to a minimum amount of extra effort. Patients who participate in this study
will be exposed to known risks and side effects of gadolinium-based contrast
agent during CMR.