Pulmonary hypertension
Intravenous Epoprostenol in Inoperable Chronic Thromboembolic Pulmonary Hypertension

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Background

Pulmonary thromboendarterectomy may be a cure for patients with chronic thromboembolic pulmonary hypertension (CTEPH) with proximal obstruction. Conversely, short-term prognosis is poor for inoperable CTEPH patients with distal obstruction. Vascular remodeling lesions can be found in lungs of patients with CTEPH. Little is known about epoprostenol therapy in inoperable distal CTEPH patients.

Methods

We conducted a retrospective analysis of 27 consecutive patients with inoperable distal CTEPH, who were in New York Heart Association Functional Class (NYHA FC) III (n = 20) or IV (n = 7) and treated with long-term intravenous epoprostenol.

Results

Before epoprostenol initiation (baseline), 6-minute walk distance (6MWD) was 265 ± 117 m, right atrial pressure 13 ± 5 mm Hg, mean pulmonary artery pressure (mPAP) 56 ± 9 mm Hg, cardiac index (CI) 1.9 ± 0.3 liters/min/m2, total pulmonary resistance (TPR) 29.3 ± 7.0 U/m2 and mixed venous oxygen saturation 52 ± 8%. After 3 months of epoprostenol (n = 23; mean epoprostenol dose 16 ± 2 ng/kg/min), NYHA FC improved by one class in 11 of 23 patients, 6MWD increased by 66 m (p < 0.0001), and hemodynamics also improved (mPAP: 51 ± 8 mm Hg, p = 0.001; CI: 2.3 ± 0.4 liters/min/m2, p = 0.0003; TPR: 23.0 ± 5.0 U/m2, p < 0.0001). At last evaluation (n = 18; 20 ± 8 months), mean dose of epoprostenol was 30 ± 8 ng/kg/min, NYHA FC was improved in 9 of 18 patients and there was sustained improvement in 6MWD (+46 m, p = 0.03) and hemodynamic parameters. Survival at 1, 2 and 3 years was 73%, 59% and 41%, respectively.

Conclusions

Long-term treatment with intravenous epoprostenol improves exercise capacity and hemodynamic parameters in patients with inoperable distal CTEPH. Epoprostenol may be used as bridge therapy in CTEPH patients awaiting lung transplantation.

Section snippets

Patients

Twenty-seven consecutive patients with CTEPH with contraindications to pulmonary thromboendarterectomy or with sustained pulmonary hypertension after surgery, who subsequently received medical treatment with continuous epoprostenol infusion in our hospital from March 1996 to October 2003, were retrospectively evaluated. This study was approved by our local institutional review board. All patients were informed and gave their consent to receive epoprostenol therapy. Ventilation/perfusion lung

Patients’ Characteristics

Twenty-seven consecutive patients with CTEPH were treated with epoprostenol therapy. Among these patients, 23 were not suitable for PTE because of lesions inaccessible by surgery, and 4 presented with persistent, symptomatic and severe pulmonary hypertension about 14 months after PTE. There were 14 women and 13 men, with a mean age of 51 years. Six patients (22.2%) had received previous vasodilator treatments consisting of inhalation (n = 5) or subcutaneous delivery (n = 1) of a prostacyclin

Discussion

Our study shows for the first time the long-term clinical, functional and hemodynamic effects of epoprostenol therapy in patients with PH associated with chronic thromboembolic disease who are not suitable for surgery or with residual PH after thromboendarterectomy. We also found that long-term survival under epoprostenol treatment is associated with baseline distance for the 6MWT.

Previous studies have addressed the need for treatment of inoperable or residual CTEPH. Efficacy of inhaled

References (25)

  • W. Miller et al.

    Reperfusion edema after thromboendarterectomy: radiographic patterns of disease

    J Thorax Imag

    (1998)
  • P. Dartevelle et al.

    Angioscopic video-assisted pulmonary endarterectomy for post-embolic pulmonary hypertension

    Eur J Cardiothorac Surg

    (1999)
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