Adult Congenital Heart Disease Registry (QuERI)

This study is ongoing, but not recruiting participants.
Sponsor:
Collaborator:
Canadian Heart Research Centre
Information provided by (Responsible Party):
Actelion
ClinicalTrials.gov Identifier:
NCT01659411
First received: July 12, 2012
Last updated: March 1, 2016
Last verified: March 2016
  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 (CHD)
Other: Observational

Study Type: Observational [Patient Registry]
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Target Follow-Up Duration: 3 Years
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: August 2018
Estimated Primary Completion Date: June 2018 (Final data collection date for primary outcome measure)
Groups/Cohorts Assigned Interventions
Adult CHD Patients
observational
Other: Observational
Yearly clinical visits

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   (Adult, Senior)
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:

      i. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 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:

      i. Evidence of RV dilation by general, qualitative assessment ii. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve iii. 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:

      i. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 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:

      i. Evidence of RV dilation by general, qualitative assessment ii. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve iii. 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

  Hide Study Locations
Locations
United States, Arizona
Phoenix Children's Hospital, Children's Heart Center
Phoenix, Arizona, United States, 85016
United States, Arkansas
The University of Arkansas for Medical Sciences - Arkansas Children's Hospital
Little Rock, Arkansas, United States, 72202
United States, California
University of Southern California ACHD Care Program
Los Angeles, California, United States, 90033
Ahmanson / UCLA Adult Congenital Heart Disease Center
Los Angeles, California, United States, 90095
Berkeley Cardiovascular Medical Group
Oakland, California, United States, 94609
Lucile Packard Children's Hospital
Palo Alto, California, United States, 94304
University of California, San Francisco
San Francisco, California, United States, 94143
United States, Colorado
University of Colorado Hospital
Aurora, Colorado, United States, 80045
United States, Connecticut
Connecticut Children's Medical Center
Hartford, Connecticut, United States, 06106
Yale School of Medicine - Adult Congenital Heart Disease Program
New Haven, Connecticut, United States, 06510
United States, District of Columbia
Children's National Medical Center and Washington Hospital Center
Washington, District of Columbia, United States, 20010
United States, Florida
Bay Area Cardiology Assoc., P.A.
Brandon, Florida, United States, 33511
University of Florida, Shands Hospital
Gainsville, Florida, United States, 32610
Memorial Regional Hospital
Hollywood, Florida, United States, 33021
United States, Hawaii
The Queen's Heart Physician Practice
Honolulu, Hawaii, United States, 96813
United States, Illinois
Rush University Medical Center
Chicago, Illinois, United States, 60612
University of Chicago
Chicago, Illinois, United States, 60637
Advocate Medical Group
Oak Lawn, Illinois, United States, 60453
United States, Kansas
The University of Kansas Hospital
Kansas City, Kansas, United States, 66160
United States, Kentucky
University of Louisville, Pediatric Cardiology
Louisville, Kentucky, United States, 40202
United States, Maine
Pediatric Cardiology Associates
Portland, Maine, United States, 04102
United States, Maryland
University of Maryland
Baltimore, Maryland, United States, 21201
United States, Massachusetts
Center for Adults with Congenital Heart Disease
Boston, Massachusetts, United States, 02111
Children's Hospital Boston and Brigham and Women's Hospital
Boston, Massachusetts, United States, 02115
United States, Michigan
University of Michigan Adult Congenital Program
Ann Arbor, Michigan, United States, 48109
United States, Minnesota
University of Minnesota Physicians Heart Fairview
Minneapolis, Minnesota, United States, 55455
United States, Missouri
Mid-America Heart Institute / St Luke's Hospital / Saint Luke's Cardiovascular Consultants
Kansas City, Missouri, United States, 64111
Barnes-Jewish Hospital/Washington University School of Medicine
St. Louis, Missouri, United States, 63110
United States, Nebraska
University of Nebraska Medical Center
Omaha, Nebraska, United States, 68198
United States, Nevada
Childrens Heart Center Nevada
Las Vegas, Nevada, United States, 89109
United States, New York
Capital District Pediatric Cardiology Associates
Albany, New York, United States, 12208
Montefiore Medical Center - Moses Division;The University Hospital for Albert Einstein College of Medicine
Bronx, New York, United States, 10467
Bassett Healthcare Network - Bassett Medical Center
Cooperstown, New York, United States, 13326
Cohen's Children's Medical Center of New York
New Hyde Park, New York, United States, 11040
Mount Sinai Pulmonary Hypertension Program
New York, New York, United States, 10029
Schneeweiss Adult Congenital Heart Center - Columbia University Medical Center
New York, New York, United States, 10032
Lenox Hill Heart and Vascular Institute-LIJ North Shore
New York, New York, United States, 10075
Pulmonary Health Physicians, PC
Syracuse, New York, United States, 13210
United States, North Carolina
Asheville Cardiology Associates
Asheville, North Carolina, United States, 28803
Duke University Medical Center
Durham, North Carolina, United States, 27710
East Carolina Heart Institute of East Carolina University
Greenville, North Carolina, United States, 27834
United States, Ohio
Cincinnati Adolescent and Adult Congenital Heart Disease Program, The Heart Institute at Cincinnati Childrens Hospital Medical Center
Cincinnati, Ohio, United States, 45229
The Cleveland Clinic Adult Congenital Heart Disease Center
Cleveland, Ohio, United States, 44195
Ohio State University Medical Center
Columbus, Ohio, United States, 43210
The Research Institute at Nationwide Children's Hospital
Columbus, Ohio, United States, 43215
United States, Oregon
Oregon Health and Science Univ.
Portland, Oregon, United States, 97239-3098
United States, Pennsylvania
Central Bucks Specialists
Doylestown, Pennsylvania, United States, 18901
The Pennsylvania State University College of Medicine and The Pennsylvania State Milton S. Herhsey Medical Center
Hershey, Pennsylvania, United States, 17033
Hospital of University of Pennsylvania; Childrens Hospital of Philadelphia
Philadelphia, Pennsylvania, United States, 19104
Temple University Hospital
Philadelphia, Pennsylvania, United States, 19140
United States, South Carolina
Medical University of South Carolina
Charleston, South Carolina, United States, 29425
United States, Tennessee
Methodist Lebonheur Healthcare, UT Lebonheur Pediatric Specialists
Memphis, Tennessee, United States, 38103
Vanderbilt University Medical Center
Nashville, Tennessee, United States, 37232
United States, Texas
Texas Children's Hospital/Baylor College of Medicine
Houston, Texas, United States, 77005
United States, Virginia
University of Virginia Health Systems
Charlottesville, Virginia, United States, 22908
The Cardiovascular Group Central
Lynchburg, Virginia, United States, 24501
Children's Specialty Group PLLC, Children's Hospital of the King's Daughters
Norfolk, Virginia, United States, 23507
United States, Washington
University of Washington
Seattle, Washington, United States, 98105
United States, West Virginia
West Virginia University Department of Pediatrics
Morgantown, West Virginia, United States, 26506
Sponsors and Collaborators
Actelion
Canadian Heart Research Centre
Investigators
Study Chair: Michael Landzberg, MD Harvard Medical School / Boston Adult Congenital Heart
  More Information

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 1, 2016
Health Authority: United States: Food and Drug Administration
United States: Institutional Review Board

Keywords provided by Actelion:
Congenital Heart Disease
Pulmonary Arterial Hypertension

Additional relevant MeSH terms:
Hypertension
Heart Diseases
Familial Primary Pulmonary Hypertension
Heart Defects, Congenital
Vascular Diseases
Cardiovascular Diseases
Hypertension, Pulmonary
Lung Diseases
Respiratory Tract Diseases
Cardiovascular Abnormalities
Congenital Abnormalities

ClinicalTrials.gov processed this record on August 24, 2016