Adult Congenital Heart Disease Registry (QuERI)

This study is currently recruiting participants. (see Contacts and Locations)
Verified March 2015 by Actelion
Sponsor:
Collaborator:
Canadian Heart Research Centre
Information provided by (Responsible Party):
Actelion
ClinicalTrials.gov Identifier:
NCT01659411
First received: July 12, 2012
Last updated: March 9, 2015
Last verified: March 2015
  Purpose

Multi-center, observational, U.S.-based longitudinal program. Data will be collected prospectively for 3 years. Individual physician feedback will be provided on data collected with the purpose of improving the management of patients - quality enhancement research initiative (QuERI) process from adult patients enrolled with a history of repaired Congenital Heart Disease (CHD).


Condition Intervention
Pulmonary Arterial Hypertension
Congenital Heart Disease
Other: observational

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Official Title: Adult Congenital Heart Disease Quality Enhancement Research Initiative

Resource links provided by NLM:


Further study details as provided by Actelion:

Primary Outcome Measures:
  • To characterize the clinical course in a cohort of adult patients with repaired CHD at risk for developing PAH [ Time Frame: screening (visit 1) through end of study (3 years) ] [ Designated as safety issue: No ]
    outcome measure: clinical outcomes: Assessment of function status, medications, and laboratory results, as well as an evaluation of medical history, physical examination, ECG, and echocardiography, in adult congenital heart disease patients at risk for pulmonary hypertension.


Secondary Outcome Measures:
  • To characterize the clinical outcomes in a cohort of adult patients with repaired CHD at risk for developing PAH [ Time Frame: screening (visit 1) through end of study (3 years) ] [ Designated as safety issue: No ]
    outcome measure: clinical rate: To assess the rate of newly diagnosed pulmonary arterial hypertension in a cohort of adults with repaired congenital heart disease at risk for pulmonary arterial hypertension. To also compare clinical outcomes in patients who do and do not meet prespecified echocardiography criteria for suspected pulmonary arterial hypertension.


Estimated Enrollment: 800
Study Start Date: December 2011
Estimated Study Completion Date: June 2017
Estimated Primary Completion Date: December 2016 (Final data collection date for primary outcome measure)
Groups/Cohorts Assigned Interventions
Adult CHD Patients
observational
Other: observational
yearly clinical visits
Other Name: observational

Detailed Description:

Approximately 800 male and female adult patients with a history of repaired CHD will be recruited from approximately 100 cardiology practices and will be followed up every twelve months for the period of three years. Consecutive patients in each practice meeting inclusion and exclusion criteria should be considered for the study. Two groups of subjects will be enrolled based on identical exclusion criteria and inclusion criteria, with the exception only of inclusion criteria #3: cohort 1- those demonstrating historic high risk criteria and cohort 2 - those demonstrating current high risk criteria.

  Eligibility

Ages Eligible for Study:   18 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population

adult CHD patients meeting inclusion and exclusion criteria may be enrolled

Criteria

Inclusion Criteria: Cohort 1 (historic high risk) 1. Male and female adults (≥ 18 years of age) 2. Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD) 3. History of a large defect prior to closure as evidenced by any one of the following:

  • Size of: ASD > 2 cm; VSD > 1 cm; PDA > 0.6 cm
  • Shunt 2:1 or greater
  • Pre-operative PH (PAS > 40 mmHg) or documented shunt- related heart failure (radiographic evidence)
  • Pre-operative atrial fibrillation or flutter 4. High risk features (any one the following):
  • Age > 40 years
  • Later surgical repair:

    1. ≥ 2 years of age for PDA or VSD
    2. ≥ 1 year of age for AVC
    3. ≥ 10 years of age for ASD
  • sinus venosus defect
  • primum defect
  • WHO functional class > 1
  • atrial fibrillation or flutter 5. Echocardiographic evidence of high risk features. Any one of the following:
  • Degree of TR that is mild or greater
  • Right ventricular (RV) systolic dysfunction
  • Evidence of RV dilatation: Any one of the following:

    1. Evidence of RV dilation by general, qualitative assessment
    2. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve
    3. RV to LV ratio at end-diastole > 0.6 (RV and LV end diastolic dimensions measured from apical 4 chamber view, 1 cm apical of the respective valve annuli)
  • Any abnormality in the motion of the inter-ventricular septum 6. Ability and desire to execute the consent for follow up

Inclusion Criteria: Cohort 2 (current high risk)

  1. Male and female adults (≥ 18 years of age)
  2. Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD)
  3. Current (within the last 12 months) evidence of 1 or more of the 7 following criteria:

    • Desaturation on exercise (92% or less)
    • 6 MWD <380 m
    • PFT demonstrating DLC <70% predicted & FEV1>70% predicted
    • ECG demonstrating i) RAD and ii) RVH or RAE
    • Physical findings of edema accompanied by elevated JVP and +HJR
    • CXR evidence of enlarged main and/or hilar pulmonary arterial shadows in association with right ventricular enlargement
    • Elevated biomarks (BNP or NT-proBNP above upper limit of normal)
  4. High risk features (any one of the following:)

    • Age > 40 years
    • Later surgical repair:

1. ≥ 2 years of age for PDA or VSD 2. ≥ 1 year of age for AVC 3. ≥ 10 years of age for ASD

  • sinus venosus defect
  • primum defect
  • WHO functional class > 1
  • atrial fibrillation or flutter 5. Echocardiographic evidence of high risk features. Any one of the following:
  • Degree of TR that is mild or greater
  • Right ventricular (RV) systolic dysfunction
  • Evidence of RV dilatation: Any one of the following:

    1. Evidence of RV dilation by general, qualitative assessment
    2. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve
    3. RV to LV ratio at end-diastole > 0.6 (RV and LV end diastolic dimensions measured from apical 4 chamber view, 1 cm apical of the respective valve annuli)
  • Any abnormality in the motion of the inter-ventricular septum 6. Ability and desire to execute the consent for follow up

Exclusion Criteria:

  1. Poor mental function, drug or substance (e.g., alcohol) abuse, or unstable psychiatric illness, which, in the opinion of the investigator, may interfere with optimal participation in the study
  2. Diagnosis of PAH (defined as RHC demonstrating mPAP ≥ 25 mm Hg and PCWP ≤ 15 and PVR > 3 WU or PVR (indexed) > 4 WU or treatment with PAH specific therapy) after surgical repair and prior to visit 1.
  3. Prior inclusion in this registry.
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01659411

Contacts
Contact: Anatoly Langer, MD 416-999-6264 langera@chrc.net

  Show 59 Study Locations
Sponsors and Collaborators
Actelion
Canadian Heart Research Centre
Investigators
Study Director: Alain Romero, PharmD Actelion Pharmaceuticals U.S., Inc.
Study Chair: Michael Landzberg, MD Harvard Medical School / Boston Adult Congenital Heart
  More Information

No publications provided

Responsible Party: Actelion
ClinicalTrials.gov Identifier: NCT01659411     History of Changes
Other Study ID Numbers: AC-052-433, CHRC2011-ACHD001
Study First Received: July 12, 2012
Last Updated: March 9, 2015
Health Authority: United States: Food and Drug Administration
United States: Institutional Review Board

Keywords provided by Actelion:
Pulmonary Arterial Hypertension
Congenital Heart Disease

Additional relevant MeSH terms:
Heart Defects, Congenital
Heart Diseases
Hypertension
Cardiovascular Abnormalities
Cardiovascular Diseases
Congenital Abnormalities
Vascular Diseases

ClinicalTrials.gov processed this record on September 03, 2015