Table 1.

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

Indications			Supporting references

I. Asymptomatic patients with 2 or more of the following criteria:
	a. 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		^{10, 12}
	b. RV end-systolic volume index >80 ml/m²		^{11, 13}
	c. RV ejection fraction <47%		^{11, 15, 16}
	d. LV ejection fraction <55%		^{11, 15, 16}
	e. Large RVOT aneurysm		^{17, 18}
	f. QRS duration >160 ms		¹¹
	g. Sustained tachyarrhythmia related to right heart volume load		6
	h. Other hemodynamically significant abnormalities:
		○ RVOT obstruction with RV systolic pressure ≥0.7 systemic	¹⁹
		○ Severe branch pulmonary artery stenosis (<30% flow to affected lung) not amenable to transcatheter therapy
		○ ≥ moderate tricuspid regurgitation	¹⁹
		○ Left-to-right shunt from residual atrial or ventricular septal defects with pulmonary-to-systemic flow ratio ≥1.5	¹⁹
		○ Severe aortic regurgitation	¹⁹

II. Symptomatic patients fulfilling ≥1 of the quantitative criteria detailed above. Examples of symptoms and signs include:
	a. 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)
	b. Signs and symptoms of heart failure (e.g., dyspnea with mild effort or at rest not explained by extra-cardiac causes, peripheral edema)		¹⁹
	c. Syncope attributable to arrhythmia

III. Special considerations:
	a. Due to higher risk of adverse clinical outcomes in patients who underwent TOF repair at age ≥3 years, PVR may be considered if they fulfill ≥1 of the quantitative criteria in section I		¹⁶
	b. Women with severe PR and RV dilatation and/or dysfunction may be at risk for pregnancy-related complications. 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		²⁰