Pulmonary hypertension (PH) is a rare disease of the pulmonary vasculature with diverse pathogenetic mechanisms. Vasoactive substances such as endothelin A receptor (ETA) antagonists and prostanoids have been used to improve haemodynamics and clinical outcome. We compared the hemodynamic response to BQ-123 (an ETA receptor antagonist) and prostacyclin or its analogue iloprost in ten patients (four men) with a mean age of 35.9±15.6 years. Seven patients had primary PH and three had PH owing to connective tissue disease. Patients underwent haemodynamic evaluation before and after administration of intra-atrial BQ-123 (200mmol/min for 60min), intravenous prostacyclin (3ng·kg-1·min-1 for 4h) or iloprost as an aerosol (100µg over 24h). Response to vasodilator administration was defined as >15% decrease in pulmonary vascular resistance index (PVRI). Of the ten patients, five showed a response to BQ-123 and eight responded to prostanoids. Four patients were responders and one patient was a non-responder to both agents. PVRI decreased by 16.6±13.4% with BQ-123, and 24.4±15.7% with prostanoids (not statistically significant). The aetiology of PH did not affect the response to either drug. In conclusion, response to ETA antagonist or prostanoid administration can be achieved in a large group of patients with severe PH, however few patients respond identically to both agents. These findings are consistent with a multifactorial mechanism involved in this disease.
Differential vasoactive response to endothelin receptor antagonists and prostacyclin in patients with severe pulmonary hypertension
Sotiria C. APOSTOLOPOULOU, Georgios KOURGIANNIDIS, Athanassios MANGINAS, Zenon S. KYRIAKIDES, David WEBB, Spyridon RAMMOS, Dimitrios T. KREMASTINOS, Dennis V. COKKINOS; Differential vasoactive response to endothelin receptor antagonists and prostacyclin in patients with severe pulmonary hypertension. Clin Sci (Lond) 1 September 2002; 103 (s2002): 298S–301S. doi: https://doi.org/10.1042/CS103S298S
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