Cardiac Resynchronization Therapy in Congenital Heart Diseases With Systemic Right Ventricle

This study aims to analyze outcomes of patients with systemic right ventricle (SRV) implanted with Cardiac Resynchronization Therapy (CRT) systems and to compare the impact of CRT in SRV patients with other congenital heart diseases (CHD).

Study Overview

• Status: Completed
• Conditions: Congenital Heart Disease
• Intervention / Treatment: Device: cardiac resynchronization therapy

Detailed Description

Heart failure is henceforth the main cause of death in adult patients with congenital heart disease (CHD). The number of affected patients is expected to continue to grow exponentially due to the important increase and aging of this population.

In symptomatic patients despite optimal pharmacological therapy, Cardiac Resynchronization Therapy (CRT) offers a well-recognized therapeutic option in dilated and ischemic cardiomyopathies with severely impaired left ventricle ejection fraction and complete left bundle branch block. In patients with CHD, the level of evidence is much more limited. Underlying phenotypes are heterogeneous, including systemic right ventricles (SRVs), isolated sub-pulmonary right ventricle dysfunctions, single ventricles, and most of patients have a right bundle branch block. All these specificities question the extrapolation of data reported in patients with acquired cardiomyopathies. Despite the lack of evidence, CRT systems have been progressively used in CHD patients, also because alternative options are poor, mainly represented by cardiac transplantation, but grafts are rare and congenital patients frequently have contraindications.

A positive impact of CRT in CHD has first been suggested in small series where hemodynamic invasive parameters (e.g. dP/dt max) were improved and QRS narrowed by multisite pacing. A few subsequent studies then reported an improvement of ventricular functions and patient-reported functional subjective parameters (mainly NYHA classification). However, the number of patients included in most studies was limited and heterogeneous forms of CHD were often pooled when interpreting results. The studies evaluating CRT specifically in patients with SRV are very rare and demonstrated conflicting results. The largest study to date assessing specifically CRT in patients with SRV included 20 patients with congenitally corrected transposition of the great arteries (cc-TGA) and demonstrated an acute improvement in two-thirds of patients.

Although patients with SRV represent one of the main groups of CHD patients currently implanted with CRT, the paucity of specific data in this population has been highlighted as a major evidence gap and as a high-impact research question by the American Heart Association/American College of Cardiology guidelines committee.

• Study Type: Observational
• Enrollment (Actual): 85

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: ADULT; OLDER_ADULT; CHILD
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

All patients with congenital heart disease and CRT device. The group of patients with systemic right ventricle include patients with D-transposition of the great arteries (D-TGA) with atrial switch operation (Mustard or Senning surgery) and patients with congenitally-corrected transposition of the great arteries (cc-TGA) with SRV (excluding cc-TGA with systemic left ventricle post surgical repair, e.g., arterial switch procedure or Rastelli surgery).

Description

Inclusion Criteria:

• All patients with congenital heart disease and CRT device

Exclusion Criteria:

• Patient's refusal

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Proportion of patients with response to CRT	Proportion of patients with improvement of NYHA classification by at least one grade or improvement of systemic ventricular ejection fraction by at least 10%	6-month, 12-month, 24-month
Proportion of patients with response to CRT	Proportion of patients with improvement of NYHA classification by at least one grade or improvement of systemic ventricular ejection fraction by at least 10%	12-month
Proportion of patients with response to CRT	Proportion of patients with improvement of NYHA classification by at least one grade or improvement of systemic ventricular ejection fraction by at least 10%	24-month

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Overall mortality	All cause mortality	5 and 10 years
Overall mortality or heart transplantation	All cause mortality or heart transplantation	5 and 10 years
Complications associated with CRT		Acute (<30 days after procedure) and late (>30 days)

Collaborators and Investigators

• Sponsor: Paris Cardiovascular Research Center (Inserm U970)
• Collaborators: University Hospital, Montpellier; Groupe Hospitalier Pitie-Salpetriere; European Georges Pompidou Hospital; Hopital Louis Pradel; Clinique Pasteur Toulouse; Marie Lannelongue Hospital, Le Plessis Robinson, France
• Investigators: Principal Investigator: Victor Waldmann, MD, PhD, Georges Pompidou European Hospital, Paris, France

Study record dates

Study Major Dates

• Study Start (ACTUAL): January 1, 2004
• Primary Completion (ACTUAL): December 31, 2020
• Study Completion (ACTUAL): December 31, 2020

Study Registration Dates

• First Submitted: April 6, 2021
• First Submitted That Met QC Criteria: April 9, 2021
• First Posted (ACTUAL): April 13, 2021

Study Record Updates

• Last Update Posted (ACTUAL): April 13, 2021
• Last Update Submitted That Met QC Criteria: April 9, 2021
• Last Verified: April 1, 2021

More Information

Terms related to this study

• Keywords: cardiac resynchronization therapy; outcomes; congenital heart disease; systemic right ventricle
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital
• Other Study ID Numbers: 2219047

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes
• product manufactured in and exported from the U.S.: No