Pulmonary Arterial HypertensionNCT01659411
Adult Congenital Heart Disease Registry (QuERI)
An observational study of Pulmonary Arterial Hypertension, run by Actelion.
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… Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Pulmonary Arterial Hypertension
- What is being tested?
- Observational
- How many people
- 217 participants
- Where
- No sites listed yet
- Study dates
- 1 Dec 2011to 16 May 2018
- How long will I take part?
- 3 Years
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Adult CHD patients meeting inclusion and exclusion criteria may be enrolled
Who may be able to join
- 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)
Show all 47Show fewer
- Pre-operative atrial fibrillation or flutter
- High risk features (any one the following):
- Age > 40 years
- Later surgical repair:
- ≥ 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:
- 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
- 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:
- ≥ 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:
- 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
Who cannot join
- 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
About this study
Official title: 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).
Read the 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
- Being tested
- Observational
- Keywords
- Congenital Heart Disease, Pulmonary Arterial Hypertension
- Medical subject headings
- Pulmonary Arterial Hypertension, Heart Defects, Congenital
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
ObservationalOther
Yearly clinical visits
| Study group | Role | What they receive |
|---|---|---|
| Adult CHD Patients | observational Receives: Other: Observational |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: screening (visit 1) through end of study (3 years)
Secondary outcomes (1)
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.
Measured: screening (visit 1) through end of study (3 years)
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted7 Aug 2012 (estimated)
- Study start1 Dec 2011
- Primary completion16 May 2018
- Study completion16 May 2018
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Pulmonary Arterial HypertensionStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 217 participants (actual)
- Target duration
- 3 Years
Sponsor and investigators
- Lead sponsor
- Actelion
- Sponsor type
- Industry (pharma, biotech, devices)
- Collaborators
- Canadian Heart Research Centre
- Responsible party
- Sponsor
- Investigators
- Michael Landzberg, MD, Study chair, Harvard Medical School / Boston Adult Congenital Heart
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT01659411
- Sponsor's protocol ID
- AC-052-433
- Other IDs
- CHRC2011-ACHD001
- Status last verified
- Jan 2025
- First submitted
- 12 Jul 2012
- Last updated
- 3 Feb 2025
- Results posted
- No
- Data monitoring committee
- No
Publications
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.
PubMed 38938704
Study at a glance
From the registry record, last updated by the sponsor on 3 Feb 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Adult Congenital Heart Disease Quality Enhancement Research Initiative
- ClinicalTrials.gov ID
- NCT01659411
- Acronym / sponsor ID
- AC-052-433
- Conditions
- Pulmonary Arterial Hypertension
- Keywords
- Congenital Heart Disease, Pulmonary Arterial Hypertension
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 217 participants (actual)
- Lead sponsor
- ActelionIndustry (pharma, biotech, devices)
- Collaborators
- Canadian Heart Research Centre
- Study start
- 1 Dec 2011 (actual)
- Primary completion
- 16 May 2018 (actual)
- Study completion
- 16 May 2018 (actual)
- First posted
- 7 Aug 2012 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 design
- Observational model
- Cohort
- Time perspective
- Prospective
- Target follow-up
- 3 Years
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Adult CHD Patients | – | Other: Observationalobservational |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Adult CHD patients meeting inclusion and exclusion criteria may be enrolled
Key inclusion criteria (47 listed)
- 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:
- High risk features (any one the following):
- Echocardiographic evidence of high risk features. Any one of the following:
Key exclusion criteria (3 listed)
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| ObservationalYearly clinical visits | Other | Adult CHD Patients |
Primary outcome
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.
1 secondary outcome
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)
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 3 Feb 2025.
- Derived from this study: PubMed 38938704Landzberg 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.
Related trials for Pulmonary Arterial Hypertension
Not yet recruitingPhase 2/3
An Open-Label Extension Study to Evaluate Inhaled Treprostinil SMI in Participants With Pulmonary Hypertension Associated With Chronic Obstructive Pulmonary Disease (PH-COPD)
Pulmonary Hypertension Associated With Chronic Obstructive Pulmonary Disease
Not yet recruitingPhase 2
Study of Treprostinil Administered Via Soft Mist Inhaler (SMI) in Participants With Pulmonary Hypertension Associated With Chronic Obstructive Pulmonary Disease (PH-COPD)
Pulmonary Hypertension Associated With Chronic Obstructive Pulmonary Disease
Active, not recruitingPhase 1
A Pilot Study of Microbiota Transplant With Antibiotic Preconditioning and Fiber Supplementation in Pulmonary Arterial Hypertension (GUT RESTORE-PAH)
Pulmonary Arterial Hypertension
RecruitingObservational study
Natural History Study of Biomarkers in Pulmonary Arterial Hypertension
Pulmonary Disease, Pulmonary Hypertension
Active, not recruitingPhase 1
Study of Intravenous ZMA001 in Healthy Subjects
Pulmonary Arterial Hypertension PAH
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Michael Landzberg, MD study chair
Harvard Medical School / Boston Adult Congenital Heart
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Arterial Hypertension, run by Actelion.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 47 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 3 Feb 2025. Status can change between updates, so confirm with the study contact before making plans.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
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)). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT01659411, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 3 Feb 2025.