Patients affected by pulmonary arterial hypertension (PAH) show a typical pattern of abnormalities on cardiopulmonary exercise testing (CPET). However, CPET is not routinely used as a screening method. We discuss a patient with hereditary PAH in whom CPET revealed onset of disease. Furthermore, we show that the abnormalities observed can improve in part by PAH-specific treatment.
Letter to the Editor
The case presented by Trip et al. wonderfully highlights the importance of cardiopulmonary exercise testing (CPET) for evaluating patients with familial pulmonary hypertension (PH). Nevertheless, it is crucial to understand certain salient features of CPET which can further strengthen the interpretation.