VHL and pulmonary arterial hypertension: Since the identification of the CP mutation, other polycythaemic individuals with different missense mutations in both VHL alleles have been shown to have pulmonary hypertension (Bond et al., 2011; Botros et al., 2017; Caravita et al., 2016; Chomette et al., 2022; Sarangi et al., 2014) and increased pulmonary vascular responses to hypoxia (Perrotta et al., 2020).