Table 2.
All candidate quality indicators and rating scores for round 1 and round 2 ratings by the expert panel separated by lesion. The table also includes categorization of indicators by process and structure within each lesion and the level of evidence of each indicator.
| Secundum Atrial Septal Defect (ASD) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicators | |||||
| Demonstration of shunting across ASD by echocardiogram (IC) | 7.0, 2.0 (2–9) | 9.0, 1.1 (4–9) | 7.0, 1.8 (2–9) | 8.0, 1.7 (2–9) | Recommended |
| Report of presence of absence of right ventricular enlargement in unrepaired ASD by echocardiogram (IC) | 7.0, 1.0 (4–9) | 9.0, .7 (7–9) | 8.0, .7 (6–9) | 9.0, .7 (7–9) | Recommended |
| Echocardiogram report of pulmonary artery pressure by tricuspid regurgitation velocity (IC) | 8.0, .6 (6–9) | 8.0, 1.3 (3–9) | 8.0, .3 (7–9) | 9.0, .7 (6–9) | Recommended |
| Pulmonary vein anatomy determined prior to intervention (IC) | 9.0, .4 (7–9) | 8.0, 1.2 (4–9) | 9.0, .4 (7–9) | 8.0, .8 (5–9) | Recommended |
| Patient had appropriate indication for cardiac catheterization (IC) | 5.0, 1.9 (2–9) | 8.0, 1.4 (4–9) | 8.5, 1.1 (4–9) | 8.5, 1.0 (6–9) | Recommended |
| Patient did not have appropriate indication for ASD closure (IIIB) | 7.0, 1.0 (6–9) | 8.0, .9 (6–9) | 8.0, .8 (6–9) | 8.0, 1.0 (6–9) | Recommended |
| Annual clinic surveillance with cardiologist with ACHD expertise for patients with ASD closure and pulmonary hypertension, arrhythmia, ventricular or valve dysfunction (IC) | 7.0, 1.1 (5–9) | 7.0, 2.1 (3–9) | 8.0, .7 (6–9) | 9.0, .2 (8–9) | Recommended |
| Appropriate counseling of SBE prophylaxis (IIaC) | 8.0, 1.1 (5–9) | 7.0, 1.0 (6–9) | 9.0, 1.3 (5–9) | 7.0, 1.0 (6–9) | Recommended |
| Cardioversion to attempt restoration of sinus rhythm if atrial fibrillation (IA) | 6.0, 1.4 (4–9) | 7.0, 1.2 (4–9) | 3.0, 1.0 (1–6) | 5.0, 2.3 (1–9) | Rejected |
| Anticoagulation if atrial fibrillation in patient with or without ASD closure (IA) | 8.0, 1.7 (3–9) | 9.0, .2 (8–9) | 7.0, 2.1 (1–8) | 8.0, 1.9 (1–9) | Rejected |
| Coarctation of Aorta (COA) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicators | |||||
| Minimum of annual return visit with cardiologist with ACHD expertise for patients with COA repair followed by ACHD expert. (IC) | 5.0,1.6 (3–9) | 9.0,.8 (7–9) | 9.0,.4 (7–9) | 9.0,.2 (8–9) | Recommended |
| Annual check of bilateral upper extremity blood pressures (BP) and either lower extremity BP (IC) | 8.0,.8 (5–9) | 8.0, .9 (5–9) | 8.0, .6 (7–9) | 8.0, .7 (6–9) | Recommended |
| Right upper extremity blood pressure measured at each visit (IB(1), IC(2)) | 8.0, .6 (7–9) | 9.0, .1 (8–9) | 9.0, .6 (7–9) | 9.0, .0 (9-9) | Recommended |
| Any transthoracic echocardiogram includes proximal descending aortic Doppler gradient (IB(1), IC(2)) | 7.0, 1.3 (2–9) | 8.0, 1.3 (5–9) | 7.0, .9 (5–9) | 9.0, .6 (6–9) | Recommended |
| MRI or CT evaluation of complete thoracic aorta at least every 5 years for patients with repaired COA (IC) | 8.0, 1.1 (5–9) | 9.0, .3 (8–9) | 9.0, .7 (7–9) | 9.0, .2 (8–9) | Recommended |
| Documentation of aortic valve morphology (IC) | 9.0, .4 (7–9) | 9.0, 1.2 (4–9) | 9.0, .6 (7–9) | 9.0, .2 (7–9) | Recommended |
| Measurement of the ascending aorta by echocardiogram, CT or MRI at least every 2 years for those with bicuspid aortic valve or enlarged ascending aorta (IB(1), IC(2)) | 7.0, 1.2 (3–9) | 8.0, .9 (6–9) | 8.0, .6 (7–9) | 9.0, .6 (7–9) | Recommended |
| Minimum of annual visit with cardiologist and at least every 3 year referral to a cardiologist with ACHD expertise for patients with repaired COA not followed by ACHD expert (IC) | 6.0, 1.7 (3–7) | 7.0, 1.1 (5–8) | 9.0, .4 (7–9) | 9.0, .4 (7–9) | Recommended |
| Structure Indicators | |||||
| Catheter-based intervention on the COA site should be performed by a CHD specialist with cardiac catheterization expertise (IC) | 9.0, 1.4 (3–9) | 9.0, 1.3 (3–9) | 9.0, .4 (7–9) | 9.0, .6 (6–9) | Recommended |
| Eisenmenger Syndrome (ES) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicators | |||||
| Minimum of annual return visit with cardiologist with ACHD expertise for patients with ES followed by ACHD expert (IC) | 8.0, .9 (6–9) | 8.0, .7 (7–9) | 8.0, .7 (7–9) | 9.0, .2 (8–9) | Recommended |
| Annual measurement of pulse oximetry (IC) | 8.0, .7 (7–9) | 9.0, .0 (9-9) | 9.0, .4 (7–9) | 9.0, .0 (9-9) | Recommended |
| Annual hemoglobin or hematocrit (IC) | 9.0, 1.1 (4–9) | 9.0, .0 (9-9) | 9.0, .6 (6–9) | 9.0, .0 (9-9) | Recommended |
| Annual iron panel (Iron, transferring saturation, total binding capacity, ferritin) (IC) | 7.0, 1.7 (3–9) | 9.0, .3 (7–9) | 7.0, 1.2 (5–9) | 9.0, .2 (7–9) | Recommended |
| Discussion about use of pulmonary vasodilators in ES patients WHO functional class III or worse at least every 24 months (IIaC) | 8.0, .9 (5–9) | 9.0, .8 (6–9) | 8.0, .9 (6–9) | 8.0, 1.2 (4–9) | Recommended |
| Annual creatinine as measure of renal function (IC) | 8.0, 1.1 (3–9) | 9.0, .1 (8–9) | 9.0, .6 (7–9) | 9.0, .2 (8–9) | Recommended |
| Annual assessment of functional capacity by either cardiopulmonary exercise test or 6 minute walk test (IC) | 6.0, 1.2 (3–9) | 9.0, .6 (6–9) | 8.0, .9 (6–9) | 9.0, .2 (8–9) | Recommended |
| Recommendation to avoid pregnancy addressed annually (IB) | 8.0, 1.1 (5–9) | 8.0, 1.1 (6–9) | 8.0, .8 (7–9) | 8.0, .7 (7–9) | Recommended |
| Annual recommendation for use of SBE prophylaxis (IB) | 9.0, .7 (6–9) | 9.0, .4 (6–9) | 9.0, .6 (6–9) | 9.0, .7 (6–9) | Recommended |
| Annual recommendation for influenza vaccine (IC) | 9.0, .7 (5–9) | 9.0, .7 (5–9) | 9.0, .5 (7–9) | 9.0, .3 (7–9) | Recommended |
| No phlebotomy in ES patients unless symptoms of hyperviscosity and erythrocytosis without anemia or iron deficiency (IC) | 8.0, .7 (5–9) | 7.0, 1.4 (4–9) | 8.0, .8 (6–9) | 7.0, 1.0 (6–9) | Recommended |
| Minimum of yearly scheduled visit or referral to cardiologist with ACHD expertise for patients with Eisenmenger Syndrome (ES) not followed by ACHD expert (IC) | 7.0, 1.1 (3–9) | 7.0, 1.1 (5–9) | 8.0, .8 (6–9) | 8.0, .7 (7–9) | Recommended |
| Annual measurement of uric acid (IC) | 6.0, 1.6 (3–9) | 9.0, .2 (8–9) | 5.0, 2.1 (1–9) | 9.0, 1.2 (2–9) | Rejected |
| Fontan (FON) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicator | |||||
| Minimum of yearly scheduled return visit with a cardiologist with ACHD expertise for patients with Fontan surgery followed by ACHD expert (IC) | 9.0, .7 (7–9) | 9.0, .3 (7–9) | 9.0, .7 (7–9) | 9.0, .1 (8–9) | Recommended |
| Oxygen saturation at rest at least annually (IIaC) | 8.0, 1.1 (6–9) | 9.0, .1 (8–9) | 9.0, .4 (7–9) | 9.0, .2 (7–9) | Recommended |
| Anticoagulation if atrial shunt, atrial thrombus, or atrial arrhythmia (IC) | 8.0, 1.4 (3–9) | 9.0, .8 (4–9) | 9.0, .3 (8–9) | 9.0, .5 (8–9) | Recommended |
| Pregnancy or contraception counseling by cardiologist with ACHD expertise annually (IC) | 7.0, 1.4 (3–9) | 7.0, 1.4 (5–9) | 9.0, .7 (7–9) | 9.0, .8 (7–9) | Recommended |
| Annual measurement of liver function (IIaC) | 7.0, .9 (5–9) | 9.0, .2 (7–9) | 8.0, .7 (7–9) | 9.0, .0 (9-9) | Recommended |
| Documentation of hepatitis C status (not graded)* | 9.0, 1.8 (2–9) | 8.0, 1.1 (4–9) | 9.0, .9 (3–9) | 9.0, .1 (8–9) | Recommended |
| Yearly visit with or referral to a cardiologist with ACHD expertise for patients with Fontan surgery not followed by ACHD expert (IC) | 8.0, .9 (5–9) | 7.0, 1.2 (4–9) | 9.0, .3 (7–9) | 9.0, .9 (6–9) | Recommended |
| Fontan patients with onset of atrial arrhythmia should have TEE or MRI to evaluate for thrombus (IC) | 5.0, 1.9 (2–9) | 8.0, .7 (7–9) | 3.0, 2.0 (1–8) | 8.0, 1.0 (3–9) | Rejected |
| Structure Indicator | |||||
| Annual and comprehensive echocardiogram (MRI or CT may substitute) read by cardiologist with CHD imaging expertise (IC) | 7.0, 1.2 (5–9) | 9.0, .6 (6–9) | 8.0, .8 (6–9) | 9.0, .2 (8–9) | Recommended |
| Diagnostic or interventional cardiac catheterization performed by CHD specialist with cardiac catheterization expertise (IC) | 9.0, .4 (7–9) | 8.0, 1.1 (6–9) | 9.0, .2 (8–9) | 9.0, .6 (7–9) | Recommended |
| D-Transposition of the Great Arteries (TGA) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicator | |||||
| Minimum of yearly scheduled return visit with cardiologist who has ACHD expertise for patients with TGA and atrial baffle repair followed by ACHD expert (IC) | 7.0, .6 (7–9) | 9.0, .3 (7–9) | 8.0, .7 (7–9) | 9.0, .0 (9-9) | Recommended |
| At least annual electrocardiogram (IB) | 9.0, 1.1 (5–9) | 9.0, .0 (9-9) | 9.0, .8 (7–9) | 9.0, .0 (9-9) | Recommended |
| Pacemaker for symptomatic bradyarrhythmia or sick sinus syndrome (IB) | 9.0, 1.3 (5–9) | 8.0, 1.0 (5–9) | 9.0, 1.0 (5–9) | 9.0, 1.1 (5–9) | Recommended |
| Pregnancy or contraception counseling by cardiologist with ACHD expertise annually (IC) | 7.0, 1.2 (3–9) | 8.0, 1.3 (5–9) | 9.0, .9 (7–9) | 8.0, .8 (6–9) | Recommended |
| Appropriate counseling regarding SBE prophylaxis (IIaB) | 9.0, 1.3 (4–9) | 9.0, 1.0 (5–9) | 9.0, 1.4 (4–9) | 9.0, .8 (7–9) | Recommended |
| Minimum of yearly scheduled visit with or referral to cardiologist with ACHD expertise for patients with TGA and atrial baffle repair not followed by ACHD expert (IC) | 7.0, 1.3 (5–9) | 7.0, 1.0 (5–9) | 9.0, .6 (7–9) | 7.0, .7 (6–9) | Recommended |
| Valve repair or replacement for moderate to severe systemic atrioventricular valve regurgitation with normal ejection fraction (IC) | 6.0, .6 (4–7) | 6.0, 1.2 (3–8) | 3.0, 1.2 (1–6) | 6.0, 1.4 (3–8) | Rejected |
| Structure Indicator | |||||
| Echocardiographic imaging in TGA with atrial baffle repair should be performed by CHD specialist with imaging expertise (IB) | 9.0, .9 (6–9) | 9.0, 1.2 (5–9) | 9.0, .6 (7–9) | 9.0, .7 (7–9) | Recommended |
| Diagnostic cardiac catheterization should be performed by CHD specialist with cardiac catheterization expertise (IC) | 9.0, .9 (6–9) | 8.0, 1.1 (5–9) | 9.0, .4 (7–9) | 9.0, .4 (7–9) | Recommended |
| Interventional cardiac catheterization in TGA with atrial baffle repair should be performed by CHD specialist with cardiac catheterization expertise (IC) | 8.0, 1.1 (5–9) | 8.0, 1.2 (5–9) | 9.0, .4 (7–9) | 9.0, .4 (7–9) | Recommended |
| Tetralogy of Fallot (TOF) | Score Round 1 Median, Mean Absolute Deviation (range) | Score Round 2 Median, Mean Absolute Deviation (range) | Recommended/Rejected | ||
|---|---|---|---|---|---|
| General Description (level of evidence) | Validity | Feasibility | Validity | Feasibility | |
| Process Indicator | |||||
| Annual return visit with cardiologist with ACHD expertise for patients with TOF repair followed by ACHD expert (IC) | 7.0, 1.2 (3–9) | 9.0, .3 (8–9) | 8.0, .3 (7–9) | 9.0, .1 (8–9) | Recommended |
| At least annual surveillance electrocardiogram (ECG) in patients with TOF repair (IC) | 8.0, 1.1 (5–9) | 9.0, .2 (8–9) | 9.0, .6 (7–9) | 9.0, .0 (9-9) | Recommended |
| Surveillance of width of QRS complex on 12-lead ECG in patients with TOF repair (IB) | 8.0, 1.0 (6–9) | 9.0, 1.1 (6–9) | 8.0, .7 (7–9) | 8.0, .9 (6–9) | Recommended |
| Appropriate endocarditis prophylaxis counseling in patients with repaired TOF (IIaB) | 7.0, 1.1 (5–9) | 9.0, .9 (6–9) | 9.0, .3 (7–9) | 9.0, .6 (7–9) | Recommended |
| Minimum of yearly scheduled visit with or referral to cardiologist with ACHD expertise for patients with repaired TOF and not followed by ACHD expert (IC) | 7.0, .9 (5–9) | 7.0, .8 (5–9) | 8.0, .6 (7–9) | 9.0, .7 (7–9) | Recommended |
| Internal cardiac defibrillator implanted in patients with documented sustained and/or resuscitated ventricular arrhythmia (IC) | 9.0, .6 (5–9) | 9.0, 1.3 (5–9) | 5.0, 2.3 (1–9) | 5.0, 2.3 (1–9) | Rejected |
| Transthoracic echocardiography at least every 12 months in patients with TOF repair (IB(1), IC(2)) | 7.0, 1.0 (3–9) | 9.0, .2 (8–9) | 5.0, 4.0 (1–9) | 5.0, 4.0 (1–9) | Rejected |
| Structure Indicator | |||||
| MRI assessment of right ventricular ejection fraction in adults with TOF repair at least every 5 years and interpreted by CHD specialist with imaging expertise (IC) | 7.0, 1.1 (3–8) | 7.0, 1.0 (6–9) | 8.0, .7 (7–9) | 9.0, .3 (7–9) | Recommended |
| Echocardiographic assessment of adults with TOF repair interpreted by CHD specialist with imaging expertise (IB(1), IC(2)) | 8.0, 1.1 (6–9) | 8.0, 1.0 (6–9) | 9.0, .4 (7–9) | 9.0, .4 (7–9) | Recommended |
| Diagnostic or interventional cardiac catheterization in TOF patients performed by CHD specialist with cardiac catheterization expertise (IC) | 7.0, 1.4 (3–9) | 9.0, 1.1 (6–9) | 9.0, .6 (7–9) | 9.0, .6 (7–9) | Recommended |
*
This indicator was recommended by the working group but is not covered in any of the sets of available guidelines
1. Warnes CA, Williams RG, Bashore TM et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Journal of the American College of Cardiology 2008;52:e1–121.
2. Silversides CK, Kiess M, Beauchesne L et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome. Canadian Journal of Cardiology 2010;26:e80–97.