Adult Congenital Heart Disease Registry (QuERI)

• ClinicalTrials.gov Identifier: NCT01659411
• Recruitment Status: Completed
• First Posted: August 7, 2012
• Last Update Posted: January 23, 2019
• Sponsor: Actelion
• Collaborator: Canadian Heart Research Centre
• Information provided by (Responsible Party): Actelion

Tracking Information

• First Submitted Date: July 12, 2012
• First Posted Date: August 7, 2012
• Last Update Posted Date: January 23, 2019
• Actual Study Start Date: December 1, 2011
• Actual Primary Completion Date: May 16, 2018 (Final data collection date for primary outcome measure)

Current Primary Outcome Measures (submitted: August 3, 2012)

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) ]

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.

• Original Primary Outcome Measures: Same as current

Current Secondary Outcome Measures (submitted: January 28, 2013)

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) ]

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.

Original Secondary Outcome Measures (submitted: August 3, 2012)

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) ]

outcome measure: clinical rate: To assess the rate of newly diagnosed pulmonary arterial hypertension in a cohort of adults with repaired congenital heart desease 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.

• Current Other Pre-specified Outcome Measures: Not Provided
• Original Other Pre-specified Outcome Measures: Not Provided

Descriptive Information

• Brief Title: Adult Congenital Heart Disease Registry (QuERI)
• Official Title: Adult Congenital Heart Disease Quality Enhancement Research Initiative
• 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.
• Study Type: Observational [Patient Registry]
• Study Design: Observational Model: Cohort; Time Perspective: Prospective
• Target Follow-Up Duration: 3 Years
• Biospecimen: Not Provided
• Sampling Method: Non-Probability Sample
• Study Population: Adult CHD patients meeting inclusion and exclusion criteria may be enrolled
• Condition: Pulmonary Arterial Hypertension

Intervention

Other: Observational

Yearly clinical visits

Study Groups/Cohorts

Adult CHD Patients

observational

Intervention: Other: Observational

• Publications *: Not Provided

Recruitment Information

• Recruitment Status: Completed
• Actual Enrollment (submitted: January 22, 2019): 217
• Original Estimated Enrollment (submitted: August 3, 2012): 800
• Actual Study Completion Date: May 16, 2018
• Actual Primary Completion Date: May 16, 2018 (Final data collection date for primary outcome measure)

Eligibility 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

Sex/Gender

• Sexes Eligible for Study: All

• Ages: 18 Years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Contacts: Contact information is only displayed when the study is recruiting subjects
• Listed Location Countries: United States

Administrative Information

• NCT Number: NCT01659411
• Other Study ID Numbers: AC-052-433; CHRC2011-ACHD001 ( Other Identifier: Canadian Identifier )
• Has Data Monitoring Committee: No
• U.S. FDA-regulated Product: Not Provided
• IPD Sharing Statement: Not Provided
• Current Responsible Party: Actelion
• Original Responsible Party: Same as current
• Current Study Sponsor: Actelion
• Original Study Sponsor: Same as current
• Collaborators: Canadian Heart Research Centre

Investigators

• Study Chair: Michael Landzberg, MD; Harvard Medical School / Boston Adult Congenital Heart

• PRS Account: Actelion
• Verification Date: January 2019