Right ventricle remodeling after pulmonary valve replacement and percutaneous pulmonary valve insertion, a functional and histopathological assessment.

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 40

Inclusion Criteria

Grown-up congenital heart disease patients with pulmonary insufficiency and/or stenosis and an indication for pulmonary valve replacement or percutaneous pulmonary valve insertion according to local GUCH team advise (based on current ESC GUCH guidelines). Also subjects who do not meet the indication criteria for valve replacement are eligible to participate in the study. They will form a conservative cohort.

Exclusion Criteria

- Individual is younger than 18 years of age.
- Individual has an estimated glomerular filtration rate (eGFR) of <30mL/min/1.73m2, using the MDRD calculation.
- Individual has any serious medical condition, which in the opinion of the investigator, may adversely affect the safety and/or effectiveness of the participant or the study (i.e., patients with clinically significant peripheral vascular disease, abdominal aortic aneurysm, bleeding disorders such as thrombocytopenia, haemophilia, significant anaemia).
- Individual is pregnant, nursing or planning to be pregnant.
- Individual has a known, unresolved history of drug use or alcohol dependency, lacks the ability to comprehend or follow instructions, or would be unlikely or unable to comply with study follow-up requirements.
- Individual is currently enrolled in an investigational drug or device trial.
- Individual with any contraindications for MRI:
a. The presence of implanted non-MRI-compatible cardiac pacemaker or implanted cardioverter defibrillator.
b. Implanted electronic devices like cochlear implants and nerve stimulators.
c. Patients who are unable to fit into the bore of the magnet.
d. Claustrophobia

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Cardiac magnetic resonance parameters including T1, T1 rho relaxation time and<br /><br>LGE (late gadolinium enhancement) for fibrosis.<br /><br>Histopathologiy: percentage of of fibrosis. </p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Echocardiograpic parameters including strain and strainrate.<br /><br>Cardiopulmonary excercise testing.<br /><br>Electrocardiograpy and Holter testing.<br /><br>Laboratory tests (BNP, ANP, hs-troponine, hs-CRP, cystatine C, PICP, TIMP-1).<br /><br>Quality of life questionnaires (SF-36 and TAAQOL).</p><br>