Key result
CPET detects hereditary PAH onset and reveals partial exercise improvement after PAH-specific treatment.
Why the study?
CPET abnormalities in PAH patients are known but CPET is not routinely used as a screening method for disease onset or treatment response.
Does cardiopulmonary exercise testing (CPET) reveal onset of disease and response to treatment in hereditary pulmonary arterial hypertension?
Does cardiopulmonary exercise testing (CPET) reveal onset of disease and response to treatment in hereditary pulmonary arterial hypertension?
CPET can be a valuable tool for early detection of disease onset and monitoring therapeutic response in hereditary pulmonary arterial hypertension.
Case data support exploring CPET for early hereditary PAH detection and response monitoring; hypothesis-generating and should not yet change practice.
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.
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Trip et al. (2012) studied this question. Cardiopulmonary exercise testing detected the onset of hereditary pulmonary arterial hypertension and revealed partial improvement of exercise abnormalities following PAH-specific treatment.
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