Adult Congenital Heart Disease Registry (QuERI)

Adult Congenital Heart Disease Quality Enhancement Research Initiative

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).

Study Overview

• Status: Completed
• Conditions: Pulmonary Arterial Hypertension
• Intervention / Treatment: Other: 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.

• Study Type: Observational
• Enrollment (Actual): 217

Contacts and Locations

Study Locations

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

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Adult CHD patients meeting inclusion and exclusion criteria may be enrolled

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Adult CHD Patients; observational	Other: Observational; Yearly clinical visits

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
To characterize the clinical course in a cohort of adult patients with repaired CHD at risk for developing PAH	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.	screening (visit 1) through end of study (3 years)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
To characterize the clinical outcomes in a cohort of adult patients with repaired CHD at risk for developing PAH	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.	screening (visit 1) through end of study (3 years)

Collaborators and Investigators

• Sponsor: Actelion
• Collaborators: Canadian Heart Research Centre
• Investigators: Study Chair: Michael Landzberg, MD, Harvard Medical School / Boston Adult Congenital Heart

Study record dates

Study Major Dates

• Study Start (Actual): December 1, 2011
• Primary Completion (Actual): May 16, 2018
• Study Completion (Actual): May 16, 2018

Study Registration Dates

• First Submitted: July 12, 2012
• First Submitted That Met QC Criteria: August 3, 2012
• First Posted (Estimate): August 7, 2012

Study Record Updates

• Last Update Posted (Actual): January 23, 2019
• Last Update Submitted That Met QC Criteria: January 22, 2019
• Last Verified: January 1, 2019

More Information

Terms related to this study

• Keywords: Congenital Heart Disease; Pulmonary Arterial Hypertension
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Congenital Abnormalities; Hypertension, Pulmonary; Cardiovascular Abnormalities; Heart Diseases; Hypertension; Pulmonary Arterial Hypertension; Heart Defects, Congenital
• Other Study ID Numbers: AC-052-433; CHRC2011-ACHD001 (Other Identifier: Canadian Identifier)