Israel Medical Association Journal

Background: Primary idiopathic pulmonary hypertension is a rapidly progressive disease with a median survival of less than 3 years. Recently its prognosis was shown to dramatically improve with the use of epoprostenol, an arachidonic acid metabolite produced by the vascular endothelium, which increases the cardiac output and decreases the pulmonary vascular resistance and pulmonary arterial pressure. This drug enhances the quality of life, increases survival and delays or eliminates the need for transplantation.

Objective: To review the experience of Israel hospitals with the use of epoprostenol.

Methods: The study group comprised 13 patients, 5 men and 8 women, with an age range of 3–53 years. All patients suffered from arterial pulmonary hypertension. Epoprostenol was administered through a central line in an increased dose during the first 3 months, after which the dose was adjusted according to the clinical syndrome and the hemodynamic parameters.

Results: After 3 months the mean dose was 10 ng/kg/min and the pulmonary artery pressure decreased from 7 to 38%. After one year, the PAP decreased at a slower rate. Two cases required transplantation, three patients died, and seven continued taking the drug (one of whom discontinued). Four episodes of septicemia were observed. Today 10 patients are alive and well and 7 continue to take epoprostenol.

Conclusion: We found that epoprostenol improves survival, quality of life and hemodynamic parameters, with minimum side effects.

Objectives: To suggest a simple outpatient technique for evaluating the response of arterial oxygen saturation to exercise for use as a marker of disease severity.

Patients and methods: Ninety-six patients with various degrees of COPD¹ were divided into three groups: mild (forced expiratory volume in 1 sec >65%), moderate (FEV1² between 50 and 65%), and severe (FEV1 <50%). Using continuous oximeter recording we measured oxygen saturation during 15 steps of climbing, and quantified  oxygen desaturation by measuring the “desaturation area”, defined as the area under the curve of oxygen saturation from the beginning of exercise through the lowest desaturarion point and until after recovery to the baseline level of oxygen percent saturation. Desaturation was correlated to spirometry, lung gas volumes, blood gas analysis, and 6 min walking distance.

Results A good correlation was found between severity of COPD and baseline SaO2³, lowest SaO2, recovery time, and desaturation area.  A negative correlation was found between desaturation area and FEV1 (r=-0.65), FEV1/forced vital capacity (r=-0.58), residual volume to total lung capacity (r=0.52), and diffusing lung capacity for carbon monoxide (r=-0.52). In stepwise multiple regression analysis only FEV1 correlated significantly to desaturation area.  A good correlation was noted between 6 min walking distance and desaturation area with the 15 steps technique (r=0.56).

Conclusions: In patients with severe COPD, arterial hypoxemia during exercise can be assessed by simple 15 steps oximetry. This method can serve both as a marker for disease severity and to determine the need for oxygen supplementation.

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COPD = chronic obstructive pulmonary disease

FEV1 = forced expiratory volume in 1 sec

SaO2 = arterial oxygen saturation