Status:
COMPLETED
PHOspholamban RElated CArdiomyopathy STudy - Intervention
Lead Sponsor:
M.p. van den Berg, MD, PhD, professor in Cardiology
Collaborating Sponsors:
University Medical Center Groningen
The Interuniversity Cardiology Institute of the Netherlands
Conditions:
Phospholamban R14del Mutation-related Cardiomyopathy
Eligibility:
All Genders
30-65 years
Phase:
PHASE3
Brief Summary
Phospholamban (PLN) R14del mutation carriers may develop dilated cardiomyopathy (DCM) and/or arrhythmmogenic cardiomyopathy (ACM). Analogous to other inherited cardiomyopathies, the natural course of ...
Detailed Description
In the Netherlands ≈15% of idiopathic dilated cardiomyopathy (DCM) and ≈10% arrhythmogenic right ventricular cardiomyopathy (ARVC) patients carry a single (founder) mutation in the gene encoding Phosp...
Eligibility Criteria
Inclusion
- Phospholamban (PLN) R14del mutation carriers
- Age ≥30 and ≤ 65 years
- New York Heart Association functional class ≤ 1
- LV ejection fraction ≥.45 (measured with MRI)
Exclusion
- Palpitations necessitating treatment (at the discretion of the attending physician)
- A diagnosis of DCM (see appendix 1). Note: regional LV wall motions abnormalities are acceptable.
- A diagnosis of ARVC (according to the task force criteria, see appendix 2)
- Global or regional RV dysfunction and/or structural alterations (according to task force criterion 1, see appendix 2).
- Ventricular premature complexes \>1000 during 24hours Holter-monitoring
- Non-sustained ventricular tachycardia during Holter-monitoring or exercise-testing
- History of sustained ventricular tachycardia or ventricular fibrillation
- Hypertension requiring the use of antihypertensive drugs, or when this is anticipated within the coming 3 years
- Evidence of ischemic heart disease
- Treatment with cardioactive medication
- Hyperkaliemia (serum potassium \>5.0 mmol/l)
- Severe renal dysfunction (eGFR \<30 ml/min/1.73 m2)
- Severe hepatic impairment (Child-Pugh class C)
- Women who are currently pregnant or report a recent pregnancy (last 60 days) or plan on becoming pregnant.
- Concomitant use of CYP3A4-inhibitors (see appendix 5)
- Concomitant use of NSAIDs (see appendix 5)
- Concomitant use of potassium sparing-agents (see appendix 5)
- Known intolerance or contraindication to aldosterone antagonists
- Participation in another drug trial in which the last dose of drug was within the past 30 days.
- Contra-indications for MRI (claustrophobia, metal devices)
- Subjects unable or unwilling to provide written informed consent
Key Trial Info
Start Date :
May 1 2013
Trial Type :
INTERVENTIONAL
Allocation :
ACTUAL
End Date :
October 1 2021
Estimated Enrollment :
84 Patients enrolled
Trial Details
Trial ID
NCT01857856
Start Date
May 1 2013
End Date
October 1 2021
Last Update
October 18 2021
Active Locations (4)
Enter a location and click search to find clinical trials sorted by distance.
1
AMC
Amsterdam, North Holland, Netherlands, 1105 AZ
2
Antonius ziekenhuis Sneek
Sneek, Provincie Friesland, Netherlands, 8600BA
3
UMCG
Groningen, Netherlands, 9700RB
4
UMCU
Utrecht, Netherlands, 3584 CX