Systemic Right Ventricle Long-term Outcome

NCT06258083 | Completed

• 1 other identifier: 202100379
• Study Type: observational
• Target: 542
• Locations: 2 countries
• Sites: 9

Brief Summary

Patients with the transposition of great arteries (TGA) who undergo atrial switch operation and congenitally corrected TGA (ccTGA) patients have the right ventricle as their systemic ventricle. Function of the systemic right ventricle (SRV) could deteriorate which is associated with impaired prognosis. It is of paramount importance to understand the course and fate of these patients during a long-term follow-up to identify the determinants of adverse outcomes.

Conditions

Transposition of the Great Arteries; Systemic Right Ventricle

Keywords

Long-term outcome; Heart failure; Arrhythmias; Mortality

Outcome Measures

Primary Outcomes (14)

• All-cause mortality

  Death

  Through study completion, an average of 18 years

• Incident heart failure

  Initiation or increase in dosage (if previously prescribed for another cause, i.e. hypertension) of loop diuretic, ACE-inhibitor/ARB therapy, or evidence-based beta blocker therapy because of new heart failure signs and symptoms (shortness of breath, fatigue, reduced exercise tolerance) AND AT LEAST 1 OF THE FOLLOWING: • BNP ≥400 pg/mL OR the following NT-proBNP levels according to age: \< 50 years, ≥450 ng/L; 50-75 years, ≥900 ng/L; \>75 years, ≥1800 ng/L OR systolic dysfunction (Systemic ventricle EF \<50%) OR • BNP 100-400 pg/mL (or NT-proBNP levels below the thresholds according to age given above) AND structural or functional heart disease

  Through study completion, an average of 18 years

• Number of patients with heart failure hospitalization

  Rehospitalization, emergency ward visit, 24h observation stay AND Treatment with or increase in dosage if previously prescribed for another cause (i.e.hypertension) of loop diuretics or treatment with IV vasoactive agents.

  Through study completion, an average of 18 years

• Number of patients with arrhythmias

  Events that cause emergency ward visit or hospitalization.

  Through study completion, an average of 18 years

• Number of patients with thromboembolic events

  Cerebrovascular events including any of (Hemorrhagic or ischemic): 1, TIA (symptoms less than 24 hours) 2, CVA (symptoms more than 24 hours) and also pulmonary embolism.

  Through study completion, an average of 18 years

• Infective endocarditis

  Definitie Infective Endocarditis

  Through study completion, an average of 18 years

• Number of patients with tricuspid valve surgery

  Tricuspid valve surgery

  Through study completion, an average of 18 years

• Number of patients with ventricular Assist Device implantation

  Implantation of ventricular assist device

  Through study completion, an average of 18 years

• Number of patients with heart transplantation

  Heart transplantation

  Through study completion, an average of 18 years

• Number of patients with aortic aneurysm or dissection

  Aortic root more than 40 mm or aneurysm in other parts of aorta. Aortic dissection.

  Through study completion, an average of 18 years

• Number of patients with pulmonary artery hypertension

  Mean PAP of more than 25mmHg or systolic PAP of more than 40 mmHg.

  Through study completion, an average of 18 years

• Number of patients with baffle interventions

  Surgical or angiographic reintervention of baffles in patients with surgically corrected transposition of the great arteries (due to stenosis or leakage).

  Through study completion, an average of 18 years

• Number of patients with coronary artery interventions

  Due to ischemia. Angiographic or surgical intervention.

  Through study completion, an average of 18 years

• Number of patients with device implantation

  Including pacmakers, ICDs and CRTs.

  Through study completion, an average of 18 years

Secondary Outcomes (3)

• Number of patients with decreased exercise capacity

  At two time points. 1: Baseline 2. Through study completion, an average of 18 years

• Number of patients with right ventricular systolic dysfunction

  At two time points. 1: Baseline 2. Through study completion, an average of 18 years

• Number of patients with left ventricular systolic dysfunction

  At two time points. 1: Baseline 2. Through study completion, an average of 18 years

Study Arms (1)

Systemic right ventricle patients

Eligibility Criteria

• Age: 12 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients with systemic right ventricles who are followed up in different academic centers in the Netherlands and Belgium.

You may qualify if:

• Patients aged 12 years old and older
• Surgically corrected transposition of the great arteries (TGA) patients with a systemic right ventricle (SRV) (Mustard, Senning)
• Congenitally corrected TGA patients with a systemic right ventricle

You may not qualify if:

• \* None.

Sponsors & Collaborators

• University Medical Center Groningen: lead
• Hartekind: collaborator
• Hartstichting: collaborator

Study Sites (9)

Universitair Ziekenhuis Leuven

Leuven, Belgium

Location

Amsterdam University Medical Centre

Amsterdam, Netherlands

Location

Medisch Spectrum Twente Enschede

Enschede, Netherlands

Location

University Medical Center Groningen

Groningen, Netherlands

Location

Leiden University Medical Centre

Leiden, Netherlands

Location

Maastricht University Medical Centre

Maastricht, Netherlands

Location

Radboud University Medical Centre

Nijmegen, Netherlands

Location

Erasmus University Medical Centre

Rotterdam, Netherlands

Location

University Medical Centre Utrecht

Utrecht, Netherlands

Location

Related Publications (7)

• Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007 Jan 16;115(2):163-72. doi: 10.1161/CIRCULATIONAHA.106.627224. Epub 2007 Jan 8.

  PMID: 17210844 BACKGROUND

• Bull C, Yates R, Sarkar D, Deanfield J, de Leval M. Scientific, ethical, and logistical considerations in introducing a new operation: a retrospective cohort study from paediatric cardiac surgery. BMJ. 2000 Apr 29;320(7243):1168-73. doi: 10.1136/bmj.320.7243.1168.

  PMID: 10784538 BACKGROUND

• Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, Lung B, Kluin J, Lang IM, Meijboom F, Moons P, Mulder BJM, Oechslin E, Roos-Hesselink JW, Schwerzmann M, Sondergaard L, Zeppenfeld K; ESC Scientific Document Group. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554. No abstract available.

  PMID: 32860028 BACKGROUND

• Andrade L, Carazo M, Wu F, Kim Y, Wilson W. Mechanisms for heart failure in systemic right ventricle. Heart Fail Rev. 2020 Jul;25(4):599-607. doi: 10.1007/s10741-019-09902-1.

  PMID: 31853794 BACKGROUND

• Dennis M, Kotchetkova I, Cordina R, Celermajer DS. Long-Term Follow-up of Adults Following the Atrial Switch Operation for Transposition of the Great Arteries - A Contemporary Cohort. Heart Lung Circ. 2018 Aug;27(8):1011-1017. doi: 10.1016/j.hlc.2017.10.008. Epub 2017 Oct 31.

  PMID: 29525133 BACKGROUND

• Smood B, Kirklin JK, Pavnica J, Tresler M, Johnson WH Jr, Cleveland DC, Mauchley DC, Dabal RJ. Congenitally Corrected Transposition Presenting in the First Year of Life: Survival and Fate of the Systemic Right Ventricle. World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):42-49. doi: 10.1177/2150135118813125.

  PMID: 30760124 BACKGROUND

• Dobson R, Danton M, Nicola W, Hamish W. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Cardiovasc Surg. 2013 Jun;145(6):1493-501; discussion 1501-3. doi: 10.1016/j.jtcvs.2013.02.030. Epub 2013 Mar 13.

  PMID: 23490252 BACKGROUND

MeSH Terms

Conditions

Transposition of Great Vessels; Heart Failure; Arrhythmias, Cardiac

Condition Hierarchy (Ancestors)

Heart Defects, Congenital; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathologic Processes; Pathological Conditions, Signs and Symptoms

Study Officials

• Joost P van Melle, MD, PhD

  University Medical Center Groningen

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 14, 2023
• First Posted: February 14, 2024
• Study Start: August 26, 2022
• Primary Completion: August 24, 2023
• Study Completion: August 24, 2023
• Last Updated: February 14, 2024

Record last verified: 2024-02

Locations

BE (1); NL (8)