beraprost and Dyspnea

The purpose of this study was to assess the efficacy and safety of beraprost sodium, an orally active prostacyclin analogue, in New York Heart Association (NYHA) functional class II and III patients with pulmonary arterial hypertension (PAH).. Pulmonary arterial hypertension is a life-threatening disease for which continuous intravenous infusion of prostacyclin has been proven effective. However, this treatment is associated with serious complications arising from the complex delivery system.. In this double-blind, placebo-controlled study, 130 patients with PAH were randomized to the maximal tolerated dose of beraprost (median dose 80 microg four times a day) or to placebo for 12 weeks. The primary end point was the change in exercise capacity assessed by the 6-min walk test. Secondary end points included changes in Borg dyspnea index, cardiopulmonary hemodynamics and NYHA functional class.. Patients treated with beraprost improved exercise capacity and symptoms. The difference between treatment groups in the mean change of 6-min walking distance at week 12 was 25.1 m (95% confidence interval [CI]: 1.8 to 48.3, p = 0.036). The difference in the mean change of Borg dyspnea index was -0.94 (95% CI: -1.63 to -0.24, p = 0.009). In the sub-group of patients with primary pulmonary hypertension, the difference in the mean change of 6-min walking distance was 46.1 m (95% CI: 3.0 to 89.3, p = 0.035). Cardiopulmonary hemodynamics and NYHA functional class had no statistically significant changes. Drug-related adverse events were common in the titration phase and decreased in the maintenance period.. Beraprost improves exercise capacity and symptoms in NYHA functional class II and III patients with PAH and, in particular, in those with primary pulmonary hypertension.

Topics: Administration, Oral; Adult; Double-Blind Method; Dyspnea; Epoprostenol; Exercise Tolerance; Female; Heart Failure; Hemodynamics; Humans; Hypertension, Pulmonary; Male; Middle Aged; Prospective Studies; Severity of Illness Index; Treatment Outcome; Vasodilator Agents

Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.

Topics: Aged; Aneurysm; Arterial Occlusive Diseases; Cardiac Catheterization; Constriction, Pathologic; Cough; Dyspnea; Epoprostenol; Female; Humans; Hypertension, Pulmonary; Imaging, Three-Dimensional; Oxygen Inhalation Therapy; Piperazines; Pulmonary Artery; Purines; Sildenafil Citrate; Sulfones; Tomography, X-Ray Computed; Ultrasonography; Vasodilator Agents; Warfarin

Portopulmonary hypertension is a complication of chronic liver disease, which has significant effects on survival and prognosis. Although the pathogenesis of pulmonary arterial hypertension has been well known, portopulmonary hypertension is often underestimated in patients with chronic liver disease. Every clinician who manages patients with chronic liver disease complaining of dyspnea should consider portopulmonary hypertension because this disorder requires special treatment. Herein, a 40-year-old woman with liver cirrhosis who complained of dyspnea on exercise is presented. She was diagnosed with portopulmonary hypertension by echocardiography and right-heart catheterization. Beraprost was used to reduce the pulmonary arterial pressure and improve the symptoms. Her symptoms were improved after 2 weeks, and improved symptoms and reduced pulmonary arterial pressure were sustained for 18 months.

Topics: Adult; Antihypertensive Agents; Blood Pressure; Cardiac Catheterization; Dyspnea; Echocardiography, Doppler; Epoprostenol; Female; Humans; Hypertension, Portal; Hypertension, Pulmonary; Liver Cirrhosis; Time Factors; Treatment Outcome; Vasodilator Agents

To study whether there is any difference in the clinical characteristics between the two patterns of perfusion lung scan of idiopathic pulmonary arterial hypertension (normal vs. diffuse, multiple ill-defined defects) and whether the perfusion lung scan patterns of these patients would predict the effect of long-term use of beraprost sodium.. We evaluated 27 patients who used beraprost sodium for over 3 months, and noted a diffuse patchy pattern in 13 cases and a normal pattern in the remaining 14 cases. We judged that beraprost sodium was effective when at least two of the following conditions were met: improvement in symptom of dyspnea, more than 10% decrease in peak velocity of tricuspid valve regurgitation by echocardiography (Vmax), or more than 10% increase in 6-min walking distance.. At baseline there was no difference between the two groups in dyspnea, hemodynamic parameters, and 6-min walking distance. After the use of beraprost sodium, the normal group showed improvement in dyspnea, 6-min walking distance, and Vmax. But the diffuse patchy group showed no improvement. The use of beraprost sodium in the normal group was effective in 10 out of 14 cases, but was effective in only two out of 13 cases in the diffuse patchy group.. Perfusion lung scan pattern in patients with idiopathic pulmonary arterial hypertension is a useful prognostic indicator of the response to beraprost sodium.

Topics: Adolescent; Adult; Blood Pressure; Cardiac Output; Dyspnea; Echocardiography, Doppler; Epoprostenol; Exercise Tolerance; Female; Humans; Hypertension, Pulmonary; Lung; Male; Perfusion; Prognosis; Pulmonary Artery; Radionuclide Imaging; Treatment Outcome; Vascular Resistance; Vasodilator Agents

A 60-year-old man with dermatomyositis was admitted to our hospital because of dyspnea and hypertension. He had high fever and convulsive seizures after admission. Laboratory examinations showed hemolytic anemia, thrombocytopenia, and renal failure. A clinical diagnosis of thrombotic thrombocytopenic purpura (TTP) was made. He failed to respond to plasma exchange therapy, pulse therapy with methylprednisolone, high-dose gamma-globulin therapy, and antiplatelet therapies with ticlopidine, dipyridamole and a prostacyclin analog of beraprost sodium. He died on his 17th day in hospital. Autopsy examination revealed widespread microthrombi in his kidneys, lungs, spleen, and intestine. Only seven cases of dermatomyositis or polymyositis complicated by TTP have been cited in the literature. TTP was fatal in 6 of these 7 cases. Early diagnosis and prompt treatment may improve the outcome of TTP patients with dermatomyositis. Dermatologists should keep in mind that TTP occasionally arises as a serious complication of dermatomyositis.

Topics: Anti-Inflammatory Agents; Dermatomyositis; Dipyridamole; Dyspnea; Epoprostenol; Fatal Outcome; Fever; gamma-Globulins; Humans; Hypertension; Immunization, Passive; Male; Methylprednisolone; Middle Aged; Plasma Exchange; Platelet Aggregation Inhibitors; Purpura, Thrombotic Thrombocytopenic; Seizures; Ticlopidine; Treatment Outcome