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1 Dept of Pulmonology, Academic Medical Centre, Amsterdam, the Netherlands. 2 Division of Pulmonary and Critical Care Medicine, 3 Division of Cardiothoracic Surgery, University of California San Diego, San Diego, CA, and 4 Division of Allergy, Pulmonology and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, USA
CORRESPONDENCE: P. Bresser, Academic Medical Centre, University of Amsterdam, Dept of Pulmonology, F5-162, PO Box 22700, 1100 DE Amsterdam, the Netherlands. Fax: 31 20691784. E-mail: p.bresser@amc.uva.nl
Keywords: chronic thromboembolic pulmonary hypertension, epoprostenol, pulmonary thromboendartectomy, right heart failure
Received: February 23, 2003
Accepted November 3, 2003
I.M. Robbins was supported by NIH grant RR 15534.
Pathophysiological findings in chronic thromboembolic pulmonary hypertension (CTEPH) have suggested that a secondary small vessel arteriopathy may contribute to the haemodynamic impairment observed in these patients. It was hypothesised that this element of the elevated vascular resistance may be responsive to continuous intravenous epoprostenol therapy.
Retrospectively, the clinical and haemodynamic responses to continuous intravenous epoprostenol were evaluated in nine CTEPH patients who subsequently underwent pulmonary thromboendarterectomy (PTE). Cardiopulmonary haemodynamics were determined prior to the initiation of epoprostenol, while on epoprostenol, prior to PTE, and after PTE.
Six patients, treated for 226 months prior to PTE, experienced either clinical stability or improvement that was associated with a mean reduction in pulmonary vascular resistance (PVR) of 28% (median 33%, range 046%). Three patients, treated for 39 months, experienced clinical deterioration during epoprostenol administration, with a significant increase in PVR in two patients. Subsequent PTE resulted in a highly significant improvement of cardiac index, mean pulmonary artery pressure and total pulmonary resistance.
To conclude, selected patients with chronic thromboembolic pulmonary hypertension may benefit clinically and haemodynamically from continuous intravenous epoprostenol treatment prior to pulmonary thromboendarterectomy. Factors predictive of a beneficial response, and whether this intervention influences either morbidity or mortality associated with pulmonary thromboendarterectomy, remain to be established.
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