TABLE 1

Guidance for assessing operability in pulmonary arterial hypertension associated with congenital heart disease

Pulmonary vascular resistance index WU·m2Pulmonary vascular resistance WUCorrectability/favourable long-term outcome
<4<2.3Yes
4–82.3–4.6Individual patient evaluation in tertiary centres
>8>4.6No

WU: Wood Units. Special considerations include: age of patient, type of defect, comorbidities, resting or exercise-induced desaturation is a concern and PAH therapy (treat with intent-to-repair approach has not been proven).