. Author manuscript; available in PMC: 2015 Jan 1.

Published in final edited form as: Circ Cardiovasc Imaging. 2014 Jan;7(1):190–197. doi: 10.1161/CIRCIMAGING.113.000553

Table 2.

Role of CMR in informing the decision for pulmonary valve replacement in patients with repaired tetralogy of Fallot. Criteria based on CMR are marked with (*)

Indications for pulmonary valve replacement in patients with repaired TOF or similar physiology with moderate or severe pulmonary regurgitation (regurgitation fraction ≥25%)
Asymptomatic patient with 2 or more of the following criteria: RV end-diastolic volume index >150 ml/m² or Z-score >4. In patients whose body surface area falls outside published normal data: RV/LV end-diastolic volume ratio >2 RV end-systolic volume index >80 ml/m² RV ejection fraction <47% LV ejection fraction <55% *Large RVOT aneurysm QRS duration >140 ms Sustained tachyarrhythmia related to right heart volume load Other hemodynamically significant abnormalities: RVOT obstruction with RV systolic pressure ≥2/3 systemic Severe branch pulmonary artery stenosis (<30% flow to affected lung) not amenable to transcatheter therapy ≥Moderate tricuspid regurgitation Left-to-right shunt from residual atrial or ventricular septal defects with pulmonary-to-systemic flow ratio ≥1.5 Severe aortic regurgitation Severe aortic dilatation (diameter >5 cm or progressive dilatation >0.5 cm/year)³⁷ Symptoms and signs attributable to severe RV volume load documented by CMR or alternative imaging modality, fulfilling ≥1 of the quantitative criteria detailed above. Examples of symptoms and signs include: Exercise intolerance not explained by extra-cardiac causes (e.g., lung disease, musculoskeletal anomalies, genetic anomalies, obesity), with documentation by exercise testing with metabolic cart (≤70% predicted peak VO₂ for age and gender not explained by chronotropic incompetence) Signs and symptoms of heart failure (e.g., dyspnea with mild effort or at rest not explained by extra-cardiac causes, peripheral edema) Syncope attributable to arrhythmia Special considerations Due to higher risk of adverse clinical outcomes in patients who underwent TOF repair at age ≥3 years,³⁹ PVR may be considered if fulfill ≥1 of the quantitative criteria in section I Women with severe PR and RV dilatation and/or dysfunction may be at risk for pregnancy-related complications.⁴⁰ Although no evidence is available to support benefit from pre-pregnancy PVR, the procedure may be considered if fulfilling ≥1 of the quantitative criteria in section I

Indications for pulmonary valve replacement in patients with repaired TOF or similar physiology with moderate or severe pulmonary regurgitation (regurgitation fraction ≥25%)

Asymptomatic patient with 2 or more of the following criteria:
1. *RV end-diastolic volume index >150 ml/m² or Z-score >4. In patients whose body surface area falls outside published normal data: RV/LV end-diastolic volume ratio >2
2. *RV end-systolic volume index >80 ml/m²
3. *RV ejection fraction <47%
4. *LV ejection fraction <55%
5. *Large RVOT aneurysm
6. QRS duration >140 ms
7. Sustained tachyarrhythmia related to right heart volume load
8. Other hemodynamically significant abnormalities:
  1. RVOT obstruction with RV systolic pressure ≥2/3 systemic
  2. Severe branch pulmonary artery stenosis (<30% flow to affected lung) not amenable to transcatheter therapy
  3. ≥Moderate tricuspid regurgitation
  4. Left-to-right shunt from residual atrial or ventricular septal defects with pulmonary-to-systemic flow ratio ≥1.5
  5. Severe aortic regurgitation
  6. Severe aortic dilatation (diameter >5 cm or progressive dilatation >0.5 cm/year)³⁷
Symptoms and signs attributable to severe RV volume load documented by CMR or alternative imaging modality, fulfilling ≥1 of the quantitative criteria detailed above. Examples of symptoms and signs include:
1. Exercise intolerance not explained by extra-cardiac causes (e.g., lung disease, musculoskeletal anomalies, genetic anomalies, obesity), with documentation by exercise testing with metabolic cart (≤70% predicted peak VO₂ for age and gender not explained by chronotropic incompetence)
2. Signs and symptoms of heart failure (e.g., dyspnea with mild effort or at rest not explained by extra-cardiac causes, peripheral edema)
3. Syncope attributable to arrhythmia
Special considerations
1. Due to higher risk of adverse clinical outcomes in patients who underwent TOF repair at age ≥3 years,³⁹ PVR may be considered if fulfill ≥1 of the quantitative criteria in section I
2. Women with severe PR and RV dilatation and/or dysfunction may be at risk for pregnancy-related complications.⁴⁰ Although no evidence is available to support benefit from pre-pregnancy PVR, the procedure may be considered if fulfilling ≥1 of the quantitative criteria in section I

Adapted from Geva T.²