Pulmonary hypertension

Impact of smoking history, DLCO, chest CT findings, and left heart disease risk factors on the classification of unexplained precapillary pulmonary hypertension (IPAH)

The present study aimed at a detailed characterisation of patients with IPAH. Data were analysed from two European pulmonary hypertension registries, COMPERA (launched in 2007) and ASPIRE (from 2001 onwards), to identify patients diagnosed with IPAH and a lung phenotype defined by a DLCO of less than 45% predicted and a smoking history. Patient characteristics, response to therapy, and survival of these patients were compared to patients with classical IPAH (defined by the absence of cardiopulmonary comorbidities and a DLCO of 45% or more predicted) and patients with pulmonary hypertension due to lung disease (group 3 pulmonary hypertension). A cohort of patients meeting diagnostic criteria for IPAH with a distinct, presumably smoking-related form of pulmonary hypertension accompanied by a low DLCO, resemble patients with pulmonary hypertension due to lung disease rather than classical IPAH. These observations have pathogenetic, diagnostic, and therapeutic implications, which require further exploration.

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis. Hoeper MM, Dwivedi K, Pausch C, Lewis RA, Olsson KM, et al. Lancet Respir Med. 2022 Oct;10(10):937-948.

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