Adult Congenital Heart Disease Registry (QuERI)
- Conditions
- Pulmonary Arterial Hypertension
- Interventions
- Other: Observational
- Registration Number
- NCT01659411
- Lead Sponsor
- Actelion
- Brief Summary
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).
- 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.
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- All
- Target Recruitment
- 217
Cohort 1 (historic high risk)
-
Male and female adults (≥ 18 years of age)
-
Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD)
-
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
-
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
-
-
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
-
-
Ability and desire to execute the consent for follow up
Inclusion Criteria: Cohort 2 (current high risk)
-
Male and female adults (≥ 18 years of age)
-
Patients with documented history (at least one year) of an isolated, repaired congenital heart defect such as ASD, VSD, PDA, AVC (AVSD)
-
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)
-
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
-
-
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
-
-
Ability and desire to execute the consent for follow up
- 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
- 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
- Prior inclusion in this registry
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description Adult CHD Patients Observational observational
- Primary Outcome Measures
Name Time Method To characterize the clinical course in a cohort of adult patients with repaired CHD at risk for developing PAH screening (visit 1) through end of study (3 years) 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
Name Time Method To characterize the clinical outcomes in a cohort of adult patients with repaired CHD at risk for developing PAH screening (visit 1) through end of study (3 years) 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.
Trial Locations
- Locations (59)
Temple University Hospital
🇺🇸Philadelphia, Pennsylvania, United States
University of Chicago
🇺🇸Chicago, Illinois, United States
Texas Children's Hospital/Baylor College of Medicine
🇺🇸Houston, Texas, United States
Barnes-Jewish Hospital/Washington University School of Medicine
🇺🇸Saint Louis, Missouri, United States
The Research Institute at Nationwide Children's Hospital
🇺🇸Columbus, Ohio, United States
Montefiore Medical Center - Moses Division;The University Hospital for Albert Einstein College of Medicine
🇺🇸Bronx, New York, United States
The Pennsylvania State University College of Medicine and The Pennsylvania State Milton S. Herhsey Medical Center
🇺🇸Hershey, Pennsylvania, United States
Berkeley Cardiovascular Medical Group
🇺🇸Oakland, California, United States
University of Southern California ACHD Care Program
🇺🇸Los Angeles, California, United States
Children's Hospital Boston and Brigham and Women's Hospital
🇺🇸Boston, Massachusetts, United States
Children's National Medical Center and Washington Hospital Center
🇺🇸Washington, District of Columbia, United States
Capital District Pediatric Cardiology Associates
🇺🇸Albany, New York, United States
Center for Adults with Congenital Heart Disease
🇺🇸Boston, Massachusetts, United States
The Cleveland Clinic Adult Congenital Heart Disease Center
🇺🇸Cleveland, Ohio, United States
Bay Area Cardiology Assoc., P.A.
🇺🇸Brandon, Florida, United States
Memorial Regional Hospital
🇺🇸Hollywood, Florida, United States
Advocate Medical Group
🇺🇸Oak Lawn, Illinois, United States
Rush University Medical Center
🇺🇸Chicago, Illinois, United States
University of Virginia Health Systems
🇺🇸Charlottesville, Virginia, United States
Children's Specialty Group PLLC, Children's Hospital of the King's Daughters
🇺🇸Norfolk, Virginia, United States
University of Maryland
🇺🇸Baltimore, Maryland, United States
Hospital of University of Pennsylvania; Childrens Hospital of Philadelphia
🇺🇸Philadelphia, Pennsylvania, United States
Cohen's Children's Medical Center of New York
🇺🇸New Hyde Park, New York, United States
Methodist Lebonheur Healthcare, UT Lebonheur Pediatric Specialists
🇺🇸Memphis, Tennessee, United States
The Cardiovascular Group Central
🇺🇸Lynchburg, Virginia, United States
The Queen's Heart Physician Practice
🇺🇸Honolulu, Hawaii, United States
The University of Kansas Hospital
🇺🇸Kansas City, Kansas, United States
University of California, San Francisco
🇺🇸San Francisco, California, United States
Duke University Medical Center
🇺🇸Durham, North Carolina, United States
Phoenix Children's Hospital, Children's Heart Center
🇺🇸Phoenix, Arizona, United States
University of Colorado Hospital
🇺🇸Aurora, Colorado, United States
Childrens Heart Center Nevada
🇺🇸Las Vegas, Nevada, United States
University of Minnesota Physicians Heart Fairview
🇺🇸Minneapolis, Minnesota, United States
Cincinnati Adolescent and Adult Congenital Heart Disease Program, The Heart Institute at Cincinnati Childrens Hospital Medical Center
🇺🇸Cincinnati, Ohio, United States
University of Nebraska Medical Center
🇺🇸Omaha, Nebraska, United States
Oregon Health and Science Univ.
🇺🇸Portland, Oregon, United States
Vanderbilt University Medical Center
🇺🇸Nashville, Tennessee, United States
Ohio State University Medical Center
🇺🇸Columbus, Ohio, United States
Central Bucks Specialists
🇺🇸Doylestown, Pennsylvania, United States
East Carolina Heart Institute of East Carolina University
🇺🇸Greenville, North Carolina, United States
Ahmanson / UCLA Adult Congenital Heart Disease Center
🇺🇸Los Angeles, California, United States
Bassett Healthcare Network - Bassett Medical Center
🇺🇸Cooperstown, New York, United States
Mount Sinai Pulmonary Hypertension Program
🇺🇸New York, New York, United States
Schneeweiss Adult Congenital Heart Center - Columbia University Medical Center
🇺🇸New York, New York, United States
Pulmonary Health Physicians, PC
🇺🇸Syracuse, New York, United States
Lenox Hill Heart and Vascular Institute-LIJ North Shore
🇺🇸New York, New York, United States
Asheville Cardiology Associates
🇺🇸Asheville, North Carolina, United States
Lucile Packard Children's Hospital
🇺🇸Palo Alto, California, United States
Yale School of Medicine - Adult Congenital Heart Disease Program
🇺🇸New Haven, Connecticut, United States
University of Louisville, Pediatric Cardiology
🇺🇸Louisville, Kentucky, United States
University of Michigan Adult Congenital Program
🇺🇸Ann Arbor, Michigan, United States
Medical University of South Carolina
🇺🇸Charleston, South Carolina, United States
Mid-America Heart Institute / St Luke's Hospital / Saint Luke's Cardiovascular Consultants
🇺🇸Kansas City, Missouri, United States
Connecticut Children's Medical Center
🇺🇸Hartford, Connecticut, United States
The University of Arkansas for Medical Sciences - Arkansas Children's Hospital
🇺🇸Little Rock, Arkansas, United States
Pediatric Cardiology Associates
🇺🇸Portland, Maine, United States
University of Washington
🇺🇸Seattle, Washington, United States
West Virginia University Department of Pediatrics
🇺🇸Morgantown, West Virginia, United States
University of Florida, Shands Hospital
🇺🇸Gainesville, Florida, United States