Phoenix Children’s Hospital Research
Institute (PCRI) Website Clinical Trial Listing
** Please complete and return to Shy Walker at
swalker@phoenixchildrens.com
Study Title: Adult Congenital Heart Disease Registry (QuERI)
Study Purpose:
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).
Study Summary:
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.
Basic 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:
a. ≥ 2 years of age for PDA or VSD
b. ≥ 1 year of age for AVC
c. ≥ 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:
a. Evidence of RV dilation by general, qualitative assessment
b. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve
c. 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:
o
Desaturation on exercise (92% or less)
o
6 MWD <380 m
o
PFT demonstrating DLC <70% predicted & FEV1>70% predicted
o
ECG demonstrating i) RAD and ii) RVH or RAE
o
Physical findings of edema accompanied by elevated JVP and +HJR
o
CXR evidence of enlarged main and/or hilar pulmonary arterial shadows in association with right
ventricular enlargement
o
Elevated biomarks (BNP or NT-proBNP above upper limit of normal)
4. High risk features (any one of the following:)
o
Age > 40 years
o
Later surgical repair:
1. ≥ 2 years of age for PDA or VSD 2. ≥ 1 year of age for AVC 3. ≥ 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:
a. Evidence of RV dilation by general, qualitative assessment
b. RV end diastolic diameter > 3.2 cm on apical 4 chamber view at the tips of the tricuspid valve
c. 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.
Study Location(s):
Phoenix Children’s Hospital
Study Contact(s):
Erica Baimbridge, CCRC
ebaimbridge@phoenixchildrens.com