2020 ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE Appropriate Use Criteria for Multimodality Imaging in Congenital Heart Disease

Distribution of Ratings

• 44% of scenarios rated as Appropriate
• 39% of scenarios rated as May Be Appropriate
• 17% of scenarios rated as Rarely Appropriate

Important Methodological Notes

• Each imaging modality for each indication was rated independently by a panel of 17 experts using a modified Delphi process
• AUC should supplement, not replace, clinical judgment and provider experience
• When a "Rarely Appropriate" test is used, clinicians should document the specific clinical circumstances justifying the decision
• Cost considerations are implicit in appropriateness ratings
• Local expertise, patient age, and clinical course may influence modality selection within and between categories

Key Indications:

• Initial diagnosis and anatomic assessment → Appropriate: TTE/TEE
• Routine surveillance (asymptomatic, unrepaired ASD, isolated PFO) → Appropriate: TTE every 3-5 years
• Prior to closure procedure → Appropriate: TEE or CCT for PFO/secundum ASD
• Post-device closure surveillance → Appropriate: TTE at 1, 6, 12 months, then annually
• Sinus venosus ASD → Appropriate: CMR or CCT for anatomy

Key Indications:

• Hemodynamically insignificant VSD (small, closed) → Appropriate: TTE at diagnosis, then as clinically indicated
• Moderate VSD without heart failure → Appropriate: TTE annually to assess LV size/function
• Large or complex VSD → Appropriate: CMR for anatomy and flow quantification
• Post-surgical repair surveillance → Appropriate: TTE post-op, then as clinically indicated

Key Indications:

• Initial diagnostic assessment → Appropriate: TTE
• Post-operative routine surveillance → Appropriate: TTE annually in early years
• Evaluate for pulmonary regurgitation severity → Appropriate: TTE; CMR if flow quantification needed
• Assess RV dilatation and dysfunction → Appropriate: CMR for RV volumes and function
• Evaluate for late arrhythmias or sudden death risk → May Be Appropriate: CMR, stress testing, EP studies as indicated

Key Indications (Post-Arterial Switch Operation):

• Initial post-operative assessment → Appropriate: TTE
• Routine follow-up surveillance → Appropriate: TTE annually
• Assess neo-aortic (LV) function and aortic regurgitation → Appropriate: TTE; CMR if detailed quantification needed
• Evaluate pulmonary artery stenosis or coronary origin anomaly → Appropriate: CCT
• Assess neo-pulmonary (RV) valve competence → May Be Appropriate: TTE; CMR for detailed assessment

Transthoracic Echocardiography (TTE) — Most Appropriate First-Line

• Initial diagnosis and baseline assessment of nearly all CHD lesions
• Serial surveillance of chamber sizes, ventricular function, and valve competence
• Assessment of simple lesions (small ASD, PDA, VSD)
• Routine follow-up when prior imaging adequate
• Frequency: Depends on lesion severity and stability (annually to every 3-5 years)

Transesophageal Echocardiography (TEE) — Selected Indications

• Suboptimal TTE windows (obesity, lung disease, post-operative change)
• Detailed assessment of ASD anatomy (secundum, sinus venosus, primum)
• PFO closure device selection and guidance
• Complex septal anatomy requiring high-resolution imaging
• Post-device closure assessment for leaks or other complications
• Requires sedation; use judiciously in pediatric population

Cardiovascular Magnetic Resonance (CMR) — Key for Complex Anatomy

• Comprehensive evaluation of complex anatomy when TTE suboptimal
• Quantification of shunt size and direction (Qp:Qs)
• Accurate ventricular volume and ejection fraction measurement (especially RV)
• Flow quantification across valves and conduits
• Myocardial tissue characterization (fibrosis, iron overload)
• Evaluation of great vessel anomalies (TAPVC, partial anomalous pulmonary venous connection)
• Limitation: Contraindicated with non-MRI-compatible devices/implants

Cardiovascular Computed Tomography (CCT) — Specific High-Quality Anatomy

• Three-dimensional reconstruction of complex cardiac anatomy
• Coronary artery anomalies and origin-from-opposite-sinus anomalies
• Aortic root and great vessel anatomy (coarctation, interrupted arch)
• Pulmonary artery stenosis and branch pulmonary artery anatomy
• Post-operative complications (conduit patency, stenosis)
• Limitation: Radiation dose; requires careful timing of contrast

Stress Testing — Risk Stratification in Select Lesions

• Exercise stress test: Assess exercise capacity, rhythm stability, BP response in tetralogy of Fallot, transposition post-arterial switch, moderate-severe valve disease
• Dobutamine/adenosine stress echo: Wall motion abnormalities if concerned for anomalous coronary or ischemia
• Use when sudden cardiac death risk being assessed or functional capacity in question

Examples: Secundum ASD <2 cm, small VSD, isolated PFO

Recommended Approach:

• Initial: TTE for baseline anatomy and function
• Follow-up: TTE every 3–5 years or per clinical indication
• CMR/CCT: May Be Appropriate for complete anatomic definition if considering closure
• Frequency can be extended if stable and completely characterized

Examples: Large ASD, moderate VSD, mild-moderate valve disease

Recommended Approach:

• Initial: TTE + CMR for comprehensive anatomy, shunt quantification, and baseline RV/LV assessment
• Follow-up: TTE annually to detect early dysfunction
• Repeat CMR: Appropriate every 2–5 years to monitor ventricular dilatation/dysfunction progression
• Plan for intervention if signs of LV or RV dysfunction emerge

Examples: Unrepaired TOF, TAPVC, heterotaxy, single ventricle physiology

Recommended Approach:

• Initial: TTE + CMR (or CCT) for detailed structural and functional assessment
• Follow-up: TTE annually; CMR every 1–3 years depending on clinical status
• Plan intervention based on severity and hemodynamic significance

Recommended Approach:

• Immediate post-op: TTE (typically before discharge)
• 1–3 months: TTE to assess early recovery and rule out complications
• 6 months: TTE to confirm stable result
• 12 months: TTE (comprehensive, baseline for long-term surveillance)
• CMR: May Be Appropriate if TTE suboptimal or complex anatomy

Recommended Approach:

• Lesion-specific frequency (typically TTE annually)
• CMR/CCT: Appropriate if detecting new dysfunction, valve disease, or conduit-related complications
• Interval between imaging can be lengthened (up to 3–5 years) if stable and fully characterized

Recommended Approach:

• ASD closure: TTE + TEE OR TTE + CCT (depending on anatomy)
• PFO closure: TEE is standard for guiding device selection and deployment
• VSD closure: TTE + TEE (or CMR if complex anatomy)
• Goal: Define anatomy precisely to ensure safe, effective closure

Conditional or Context-Dependent (39% of All Scenarios)

• CMR when TTE suboptimal due to image quality or post-operative changes
• TEE when TTE windows inadequate (obesity, lung disease, scar tissue)
• Annual CMR for surveillance in complex lesions if TTE already comprehensive
• Stress testing to assess functional capacity and exercise tolerance
• Dobutamine stress echo if anomalous coronary origin suspected
• Imaging frequency < annually in small, isolated, hemodynamically insignificant lesions
• Routine cardiac catheterization (without intervention) if noninvasive imaging adequate

Key Principle for Rarely Appropriate Ratings

When a "Rarely Appropriate" test is performed, clinicians should document the specific clinical circumstances and reasoning for the decision. This may include patient refusal of preferred modality, local expertise limitations, or unique clinical presentations not covered in typical scenarios.

Imaging intervals can be extended beyond recommended frequency when:

• Lesion is completely characterized by prior imaging
• Previous imaging has been stable (no change in chamber size, valve function, or hemodynamics)
• Patient is asymptomatic and clinically stable
• Lesion is hemodynamically insignificant or small/isolated
• High-quality prior imaging (CMR, CCT) provides comprehensive baseline

Imaging intervals should be shortened when:

• New symptoms develop (dyspnea, palpitations, chest pain)
• Prior imaging shows progression of ventricular dilatation or dysfunction
• Valve disease develops or worsens
• Arrhythmias detected on ECG or rhythm monitoring
• Post-operative complications suspected (conduit stenosis, residual shunt)
• Prior imaging quality was suboptimal

Pregnant Women with CHD

• TTE: Safe, first-line throughout pregnancy
• CMR: Appropriate in second/third trimester if needed; no contrast preferred
• CCT: Minimize radiation; use sparingly unless critical for management decisions
• Cardiac catheterization: Reserved for urgent hemodynamic questions or intervention

While this guideline addresses both pediatric and adult CHD patients, several principles differ:

• Pediatric: Growth of patient and residual shunts must be considered; may need frequent TTE to monitor for catch-up growth of defect
• Adult: Focus shifts to detecting long-term sequelae (chamber dysfunction, valve degeneration, arrhythmias)
• Imaging modalities are similar; frequency and specific indications may vary

The Appropriate Use Criteria implicitly consider cost in their ratings. Key principles:

• Use the least expensive, least invasive modality that adequately answers the clinical question
• TTE is cost-effective first-line for most indications
• CMR and CCT are more expensive; reserve for scenarios where added information significantly impacts management
• Avoid routine serial imaging if lesion is stable and fully characterized