Summary

for people ages 18 years and up (full criteria)
at UCLA UCSF
study started
estimated completion:

Description

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).

Official Title

Adult Congenital Heart Disease Quality Enhancement Research Initiative

Details

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.

Keywords

Pulmonary Arterial Hypertension Congenital Heart Disease Hypertension Heart Diseases Familial Primary Pulmonary Hypertension Heart Defects, Congenital Observational

Eligibility

You can join if…

Open to people ages 18 years and up

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:
  4. Size of: ASD > 2 cm; VSD > 1 cm; PDA > 0.6 cm
  5. Shunt 2:1 or greater
  6. Pre-operative PH (PAS > 40 mmHg) or documented shunt- related heart failure(radiographic evidence)
  7. Pre-operative atrial fibrillation or flutter
  8. High risk features (any one the following):
  9. Age > 40 years
  10. Later surgical repair:
  11. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 10 years of age for ASD
  12. Sinus venosus defect
  13. Primum defect
  14. WHO functional class > 1
  15. Atrial fibrillation or flutter
  16. Echocardiographic evidence of high risk features. Any one of the following:
  17. Degree of TR that is mild or greater
  18. Right ventricular (RV) systolic dysfunction
  19. Evidence of RV dilatation: Any one of the following:
  20. 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)
  21. Any abnormality in the motion of the inter-ventricular septum
  22. 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:
  4. Desaturation on exercise (92% or less)
  5. 6 MWD <380 m
  6. PFT demonstrating DLC <70% predicted & FEV1>70% predicted
  7. ECG demonstrating i) RAD and ii) RVH or RAE
  8. Physical findings of edema accompanied by elevated JVP and +HJR
  9. CXR evidence of enlarged main and/or hilar pulmonary arterial shadows in association with right ventricular enlargement
  10. Elevated biomarks (BNP or NT-proBNP above upper limit of normal)
  11. High risk features (any one of the following:)
  12. Age > 40 years
  13. Later surgical repair:
  14. ≥ 2 years of age for PDA or VSD ii. ≥ 1 year of age for AVC iii. ≥ 10 years of age for ASD
  15. Sinus venosus defect
  16. Primum defect
  17. WHO functional class > 1
  18. Atrial fibrillation or flutter
  19. Echocardiographic evidence of high risk features. Any one of the following:
  20. Degree of TR that is mild or greater
  21. Right ventricular (RV) systolic dysfunction
  22. Evidence of RV dilatation: Any one of the following:
  23. 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)
  24. Any abnormality in the motion of the inter-ventricular septum
  25. Ability and desire to execute the consent for follow up

You CAN'T join if...

  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

Locations

  • Ahmanson / UCLA Adult Congenital Heart Disease Center
    Los Angeles California 90095 United States
  • University of California, San Francisco
    San Francisco California 94143 United States

Details

Status
in progress, not accepting new patients
Start Date
Completion Date
(estimated)
Sponsor
Actelion
ID
NCT01659411
Study Type
Observational [Patient Registry]
Last Updated