Adult Congenital Heart Disease Registry (QuERI)

Pulmonary Arterial Hypertension Clinical Trial

Official title:

Adult Congenital Heart Disease Quality Enhancement Research Initiative

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01659411
• Other study ID #: AC-052-433
• Secondary ID: CHRC2011-ACHD001
• Status: Completed
• Start date: December 1, 2011
• Est. completion date: May 16, 2018

Study information

• Verified date: January 2019
• Source: Actelion
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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).

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 information / eligibility

• Status: Completed
• Enrollment: 217
• Est. completion date: May 16, 2018
• Est. primary completion date: May 16, 2018
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Familial Primary Pulmonary Hypertension
• Heart Defects, Congenital
• Heart Diseases
• Hypertension
• Pulmonary Arterial Hypertension

Intervention

Other:
• Observational
Yearly clinical visits

Locations

Country	Name	City	State
United States	Capital District Pediatric Cardiology Associates	Albany	New York
United States	University of Michigan Adult Congenital Program	Ann Arbor	Michigan
United States	Asheville Cardiology Associates	Asheville	North Carolina
United States	University of Colorado Hospital	Aurora	Colorado
United States	University of Maryland	Baltimore	Maryland
United States	Center for Adults with Congenital Heart Disease	Boston	Massachusetts
United States	Children's Hospital Boston and Brigham and Women's Hospital	Boston	Massachusetts
United States	Bay Area Cardiology Assoc., P.A.	Brandon	Florida
United States	Montefiore Medical Center - Moses Division;The University Hospital for Albert Einstein College of Medicine	Bronx	New York
United States	Medical University of South Carolina	Charleston	South Carolina
United States	University of Virginia Health Systems	Charlottesville	Virginia
United States	Rush University Medical Center	Chicago	Illinois
United States	University of Chicago	Chicago	Illinois
United States	Cincinnati Adolescent and Adult Congenital Heart Disease Program, The Heart Institute at Cincinnati Childrens Hospital Medical Center	Cincinnati	Ohio
United States	The Cleveland Clinic Adult Congenital Heart Disease Center	Cleveland	Ohio
United States	Ohio State University Medical Center	Columbus	Ohio
United States	The Research Institute at Nationwide Children's Hospital	Columbus	Ohio
United States	Bassett Healthcare Network - Bassett Medical Center	Cooperstown	New York
United States	Central Bucks Specialists	Doylestown	Pennsylvania
United States	Duke University Medical Center	Durham	North Carolina
United States	University of Florida, Shands Hospital	Gainesville	Florida
United States	East Carolina Heart Institute of East Carolina University	Greenville	North Carolina
United States	Connecticut Children's Medical Center	Hartford	Connecticut
United States	The Pennsylvania State University College of Medicine and The Pennsylvania State Milton S. Herhsey Medical Center	Hershey	Pennsylvania
United States	Memorial Regional Hospital	Hollywood	Florida
United States	The Queen's Heart Physician Practice	Honolulu	Hawaii
United States	Texas Children's Hospital/Baylor College of Medicine	Houston	Texas
United States	Mid-America Heart Institute / St Luke's Hospital / Saint Luke's Cardiovascular Consultants	Kansas City	Missouri
United States	The University of Kansas Hospital	Kansas City	Kansas
United States	Childrens Heart Center Nevada	Las Vegas	Nevada
United States	The University of Arkansas for Medical Sciences - Arkansas Children's Hospital	Little Rock	Arkansas
United States	Ahmanson / UCLA Adult Congenital Heart Disease Center	Los Angeles	California
United States	University of Southern California ACHD Care Program	Los Angeles	California
United States	University of Louisville, Pediatric Cardiology	Louisville	Kentucky
United States	The Cardiovascular Group Central	Lynchburg	Virginia
United States	Methodist Lebonheur Healthcare, UT Lebonheur Pediatric Specialists	Memphis	Tennessee
United States	University of Minnesota Physicians Heart Fairview	Minneapolis	Minnesota
United States	West Virginia University Department of Pediatrics	Morgantown	West Virginia
United States	Vanderbilt University Medical Center	Nashville	Tennessee
United States	Yale School of Medicine - Adult Congenital Heart Disease Program	New Haven	Connecticut
United States	Cohen's Children's Medical Center of New York	New Hyde Park	New York
United States	Lenox Hill Heart and Vascular Institute-LIJ North Shore	New York	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	Children's Specialty Group PLLC, Children's Hospital of the King's Daughters	Norfolk	Virginia
United States	Advocate Medical Group	Oak Lawn	Illinois
United States	Berkeley Cardiovascular Medical Group	Oakland	California
United States	University of Nebraska Medical Center	Omaha	Nebraska
United States	Lucile Packard Children's Hospital	Palo Alto	California
United States	Hospital of University of Pennsylvania; Childrens Hospital of Philadelphia	Philadelphia	Pennsylvania
United States	Temple University Hospital	Philadelphia	Pennsylvania
United States	Phoenix Children's Hospital, Children's Heart Center	Phoenix	Arizona
United States	Oregon Health and Science Univ.	Portland	Oregon
United States	Pediatric Cardiology Associates	Portland	Maine
United States	Barnes-Jewish Hospital/Washington University School of Medicine	Saint Louis	Missouri
United States	University of California, San Francisco	San Francisco	California
United States	University of Washington	Seattle	Washington
United States	Pulmonary Health Physicians, PC	Syracuse	New York
United States	Children's National Medical Center and Washington Hospital Center	Washington	District of Columbia

Sponsors (2)

Lead Sponsor	Collaborator
Actelion	Canadian Heart Research Centre

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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	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)