Sudden Cardiac Death in Systemic Right Ventricle

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Brief Title

Sudden Cardiac Death in Systemic Right Ventricle

Official Title

Sudden Cardiac Death in Congenital Heart Diseases With a Systemic Right Ventricle

Brief Summary

      In complete transposition of the great arteries (TGA) with previous atrial switch repair, and
      congenitally corrected transposition of the great arteries (ccTGA), the morphological right
      ventricle and its tricuspid valve continue to support the systemic circulation. This results
      in late complications including including sudden death.

      This retrospective multicentric study aims to evaluate the prevalence of SCD in a
      contemporary population of patients with a systemic RV and identify specific risk factors for
      SCD and hemodynamically significant ventricular arrhythmia This registry records
      demographics, clinical, imaging data, electrophysiological and laboratory of patients with a
      sRV and a transposition of the great arteries Primary end points are defined by sudden
      cardiac death, near-miss sudden death, as well as sustained VT requiring defibrillation.
    

Detailed Description

      1 Registry design

      The registry is a retrospective multicentric registry that involves the collection of
      demographics, clinical, imaging data, electrophysiological and laboratory of patients with a
      sRV and a transposition of the great arteries (TGA or ccTGA).

      Ethical or research committee approval should be obtained in each contributing center and all
      data will be collected retrospectively.

      A minimum of 900 patients are expected to be registered.

      Patient population Inclusion criteria: patients >16 years of age with a sRV and a
      transposition of the great arteries (TGA or ccTGA) with "two-ventricle circulation" under
      active follow-up between 2000 and 2018.

      Exclusion criteria: CHD with univentricular heart physiology

      2. Registry procedures

        1. Design

           Data on all adult patients (>16 years of age) with sRV under active follow-up between
           January 1, 2000 and September 30, 2018 will be recorded. Baseline data, as well data of
           the last follow-up recorded during this period will be used.

           Parameters are chosen for inclusion in data collection on the basis of a review of sRV
           literature on single predictors of outcome The patients' medical records will be
           reviewed to collect demographic information, medical and surgical details

           The chosen parameters of patient past medical history to record include:

             -  Type of TGA, TGA complexity, type of surgery, age at surgery, reoperation

             -  Cardio-vascular events such as atrial arrhythmia, heart failure, pacemaker
                implantation

             -  Performing electrophysiologic exploration and its results: normal or inducible
                sustained VT/VF, sinus node dysfunction, atrioventricular node dysfunction

             -  24H Holter ECG finding: sinus node disease, atrial arrhythmia, non-sustained VT,
                sustained VT

             -  Notion of complete pregnancy (> 20 weeks of gestation)

           Baseline data will be taken at the first assessment as an adult (> 16 years old). They
           include variables from clinical status, 12-lead ECG, transthoracic echocardiography, CMR
           (if available), cardiopulmonary exercise test, and laboratory tests (if available).
           Detailed data are:

             -  Clinical status: NYHA function class, syncope and palpitations.

             -  Cardiac treatment

             -  Electrocardiographic parameters, including rhythm assessment, heart rate, QRS
                duration, and QT interval manually collected

             -  Echocardiographic parameters with visual sRVEF, tricuspid regurgitation grade,
                presence of pulmonary stenosis and baffle stenosis or leak. Only transthoracic
                echocardiography performed by experienced operators will be considered.

             -  Measurements of oxygen consumption (VO2) during exercise test: peak oxygen
                consumption, VE/VCO2, peak blood pressure and peak heart rate.

             -  If available, cardiac magnetic resonance imaging parameters, such as sRV volumes,
                sRV mass, sRVEF and presence of late gadolinium enhancement lesions.

             -  If available, brain natriuretic peptide, with normal ranges provided by centers.

           Vital status at late follow-up during the study period (up to September 30, 2018) will
           be retrieved from national databases or patients' general practitioner records. Cardiac
           events such as heart failure, atrial arrhythmia, sustained ventricular tachycardia,
           death and cause of death will be reported.

        2. Preservation and confidentiality of registry records Data collected at each
           investigational site will be entered in a local database. In this database, patient will
           be identifiable only by the investigator of the site. The data will be anonymized via
           the use of a unique number. This one will be created by a secure process called the
           function of occultation of the nominative information (FOIN). It will be impossible to
           find the identity of a patient from this number (irreversibility of encryption). The
           data will be anonymously transmitted to the main investigational site (Inserm U970,
           Paris Cardiovascular Research Center, Hôpital Européen Georges Pompidou Paris, France).

      The data will be stored on a computer server dedicated only to the storage and processing of
      the project data. The computer used for the analyzes is in a locked room within the secure
      premises of the European Georges Pompidou Hospital ( INSERM U970, Cardiovascular Research
      Center of Paris), guarded 24/24 with access by name badge.

      Persons having direct access (for example, investigators, persons in charge of quality
      control, Clinical Research Assistants, and all persons involved in the study) take all
      necessary precautions to ensure the confidentiality of information relating to the persons
      who are suitable for it. All the co-investigators will have access to the data of the study
      to develop their working hypotheses within the premises of the INSERM U970, but no data can
      be transferred outside the dedicated and secure computer server.

      European George Pompidou Hospital as sponsor is subject to the rights and obligations as
      'data controller' set forth under the General Data Protection Regulation 2016/679 ("GDPR") in
      relation to the processing of personal data in accordance with the protocol. Participating
      site is subject to the rights and obligations as 'data processor' set forth under the GDPR in
      relation to the processing of personal data in accordance with the protocol. Sponsor and
      participating site agree that such data: (a) is to be used only for the purposes as described
      in the protocol; (b) will not be used for commercial purposes and (c) will not be transferred
      to a third party. Sponsor and participating site shall provide sufficient safeguards in
      respect of the administrative, technical and organizational measures for processing personal
      data and take all necessary measures to protect the confidentiality, privacy and prevention
      from accidental or unauthorized destruction, accidental loss, as well as from alteration,
      access and any other unauthorized processing of the data. Participating site shall promptly
      inform Sponsor of any security breach, reasonably assist Sponsor with the handling of (a)
      responses to any security breach and (b) any requests from data subjects under Chapter III of
      the GDPR. The Participating site shall obtain prior specific or general written consent from
      Sponsor before engaging a subprocessor of the Personal Data. Participating site remains data
      controller of the data contained in its patients' medical records for the purposes of
      providing medical care to its patients and for other academic research purposes initiated by
      participating site.

      3. Quality assurance:

        1. Verification of data:

           Source data will be verified by comparing the data to medical records and case report
           forms in the form of a random sample.

        2. Clinical events committee:

      To avoid or minimize bias, an independent clinical events committee at the main
      investigational site, consisting of independent physicians, assesses all primary endpoint
      clinical events.
    


Study Type

Observational


Primary Outcome

sudden cardiac death,

Secondary Outcome

 all causes of death

Condition

Sudden Cardiac Death

Intervention

imaging

Study Arms / Comparison Groups

 TGA
Description:  

Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information


Recruitment Status

Diagnostic Test

Estimated Enrollment

1240

Start Date

September 1, 2018

Completion Date

September 1, 2019

Primary Completion Date

September 1, 2019

Eligibility Criteria

        Inclusion Criteria:

          -  patients >16 years of age

          -  sRV and a transposition of the great arteries (TGA or ccTGA) with "two-ventricle
             circulation"

        Exclusion Criteria: congenital heart diaese with univentricular heart physiology
      

Gender

All

Ages

16 Years - N/A

Accepts Healthy Volunteers

No

Contacts

Magalie Ladouceur, Dr, , 

Location Countries

France

Location Countries

France

Administrative Informations


NCT ID

NCT03833843

Organization ID

STARS


Responsible Party

Principal Investigator

Study Sponsor

European Georges Pompidou Hospital

Collaborators

 KU Leuven

Study Sponsor

Magalie Ladouceur, Dr, Principal Investigator, Hopital Europeen Georges Pompidou


Verification Date

May 2021