Abstract
BackgroundPulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality.MethodsPatients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N‑terminal pro-brain natriuretic peptide (NT-proBNP) level and 6‑min walking distance (6MWD)).ResultsIn total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22–92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure ( r = 0.23, p < 0.001). Male sex, higher age, shorter 6MWD and higher NT-proBNP level were independently associated with mortality, but PA diameter was not.ConclusionThe prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients.
Highlights
Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an elevated mean pulmonary arterial pressure (PAP) ≥25 mm Hg
Known predictors of survival were confirmed, but Pulmonary artery (PA) diameter at diagnosis was not associated with survival in pulmonary arterial hypertension (PAH) or chronic thrombo-embolic pulmonary hypertension (CTEPH) patients
Pulmonary artery diameter is not associated with prognosis in pulmonary arterial hypertension (PAH) and in chronic thrombo-embolic pulmonary hypertension
Summary
Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an elevated mean pulmonary arterial pressure (PAP) ≥25 mm Hg. PAH and chronic thrombo-embolic pulmonary hypertension (CTEPH) are considered to be rare diseases with an estimated incidence of 2.2–7.6 patients per one million adults [1,2,3]. Due to this low incidence, evaluation of survival and mortality is challenging [3, 4]. Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality
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