Earlier studies have shown that administration of beraprost sodium (BPS), an orally active prostacyclin analogue, improves hemodynamics in patients with primary pulmonary hypertension (PH), but it is not known whether BPS has beneficial effects in secondary precapillary PH. The present study investigated the hemodynamic and hormonal parameters of 18 patients with secondary precapillary PH (8 patients with chronic thromboembolic PH, 7 with collagen vascular disease, and 3 with residual PH after surgery for atrial septal defect). Hemodynamics were repeatedly measured by right heart catheterization. Treatment with BPS improved New York Heart Association (NYHA) functional class in 10 of the 18 patients and significantly decreased pulmonary vascular resistance by 17% (12.9+/-1.1 to 10.7+/-1.2 Wood units, p<0.01). Circulating brain natriuretic peptide and uric acid significantly decreased from 246+/-61 to 215+/-65 pg/ml and from 6.5+/-0.6 to 5.3+/-0.3 mg/dl, respectively. In summary, BPS therapy improved NYHA functional class, hemodynamics, and hormonal parameters in patients with secondary precapillary PH. Thus, oral administration of BPS may be a new therapeutic strategy for the treatment of secondary precapillary PH.
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http://dx.doi.org/10.1253/circj.67.375 | DOI Listing |
BMJ Case Rep
January 2025
Department of Respiratory Medicine, Alfred Health, Melbourne, Victoria, Australia
Excipient lung disease (ELD) is a rare cause of pulmonary hypertension that occurs due to the intravenous injection of crushed tablets. We present the case of a healthcare professional in her late 30s who presented with a fever in the setting of a bacteraemia. During her hospital admission, she established a pattern of transient hypoxia and hypotension, with resolution without targeted management or clear cause identified.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
June 2024
Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, London, UK.
Connective tissue disease associated pulmonary arterial hypertension (CTD-PAH) has benefited from the major treatment advances that have occurred within pulmonary hypertension over the past three decades. Inclusion of CTD-PAH cases in pivotal clinical trials led to regulatory approval and drug availability. This has improved outcomes but there are additional challenges for management.
View Article and Find Full Text PDFJAMA Cardiol
November 2024
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
Int J Cardiol
January 2025
Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905, USA.
Sci Rep
September 2024
Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstraße 1, 69126, Heidelberg, Germany.
Current guidelines recommend oxygen (O) supplementation in patients with pulmonary hypertension (PH), despite scarce data on long-term O therapy (LTOT). The aim of this prospective, randomized, controlled trial was to investigate the effect of LTOT in patients with precapillary PH on exercise capacity, clinical parameters and hemodynamics. Patients with precapillary PH under stable therapy and O desaturations at rest and/or during exercise were randomized to receive LTOT (≥ 16 h/day) or no O (control group) for 12 weeks.
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