Adult Congenital Heart Disease Registry (QuERI)

NCT ID: NCT01659411

Last Updated: 2025-02-03

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 217 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2011-12-01
• Study Completion Date: 2018-05-16

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.

Conditions

Pulmonary Arterial Hypertension

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Adult CHD Patients

observational

Observational

Intervention Type: OTHER

Yearly clinical visits

Interventions

Observational

Yearly clinical visits

Intervention Type: OTHER

Eligibility Criteria

Inclusion Criteria

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

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

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Canadian Heart Research Centre

OTHER

Sponsor Role: collaborator

Actelion

INDUSTRY

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Michael Landzberg, MD

Role: STUDY_CHAIR

Harvard Medical School / Boston Adult Congenital Heart

References

Landzberg MJ, Daniels CJ, Forfia P, McLaughlin VV, Bell Lynum KS, Selej M, Opotowsky AR. Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights. JACC Adv. 2023 Oct 31;2(9):100649. doi: 10.1016/j.jacadv.2023.100649. eCollection 2023 Nov.

Reference Type: DERIVED

PMID: 38938704 (View on PubMed)

Other Identifiers

CHRC2011-ACHD001

Identifier Type: OTHER

Identifier Source: secondary_id

AC-052-433

Identifier Type: -

Identifier Source: org_study_id