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- W2954400209 abstract "Introduction: A pulmonary vascular resistance (PVR) criterion of >3 Wu was introduced into the haemodynamic definition of pulmonary arterial hypertension (PAH) in 2013. Lower levels of PVR can be abnormal, particularly in the younger population. There is limited data on the natural history and response to PAH therapy in patients with precapillary pulmonary hypertension with PVR <3 Wu. Methods: Using the PHSANZ registry, we analysed outcomes of all patients fulfilling the haemodynamic criteria: mPAP ≥25 mmHg, PAWP ≤15 mmHg and PVR <3 Wu. Patients with left-to-right cardiac shunts, chronic liver, lung, and left heart disease were excluded. Results: 82 patients (mean age 63 ± 11 years, 82% female) were included. Underlying diagnosis included idiopathic (n = 39), connective tissue disease (n = 42) and HIV infection (n = 1). At diagnosis, haemodynamics showed mPAP 28 ± 4 mmHg, PAWP 12 ± 2 mmHg and PVR 2.3 ± 0.5 Wu. Baseline exercise capacity revealed mean 6MWD of 341 ± 124 m with 77% in NYHA FC 3/4. All patients were commenced on initial monotherapy with endothelin receptor antagonists (n = 66) or phosphodiesterase type 5 inhibitors (n = 16). At first post-treatment evaluation (median 5 months; IQR 4-12), 6MWD increased by 46 m (IQR 7-96) and 35% demonstrated improvement in NYHA FC status. After a median follow-up of 65 months (IQR 32-101), 22% were deceased, with estimated 1-yr, 3-yr and 5-yr survivals of 98%, 88%, 84%, respectively. Conclusions: Our data suggest that selected patients with precapillary PH with PVR <3 Wu may benefit from PAH therapy. Further studies are needed to determine whether early treatment of these patients confers beneficial impact on exercise capacity and outcomes." @default.
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- W2954400209 date "2019-01-01" @default.
- W2954400209 modified "2023-09-27" @default.
- W2954400209 title "Early Treatment of Pulmonary Arterial Hypertension: Is a PVR > 3 Threshold too High?" @default.
- W2954400209 doi "https://doi.org/10.1016/j.hlc.2019.06.084" @default.
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