Epoprostenol therapy for primary pulmonary hypertension

Although a rare disease, PPH is deadly. Until recently, patients had little hope for remission of this disease. Originally viewed as a "bridge to transplantation," new research findings suggest that epoprostenol significantly improves PPH so that transplantation may not be necessary. Treatment with epoprostenol is difficult to manage, however, because it requires continuous central infusion. Nurses have a key role in ensuring that patients safely and effectively manage this therapy.     

Epoprostenol use for pulmonary arterial hypertension in the palliative care setting

Prostacyclin analogues such as epoprostenol (Flolan?) are commonly used in the treatment of pulmonary arterial hypertension (PAH). However, their complex administration and significant cost may limit the access that patients with PAH have to palliative and hospice care. We herein report our experience using epoprostenol in a dedicated palliative care unit and present our inpatient protocol for the drug's administration.     

Understanding primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is a severe and progressive disease. It is characterized by persistent elevation of pulmonary artery pressure without any known cause. The hemodynamics demonstrates high pulmonary artery pressure and resistance to blood flow through the lung circulation. PPH is a diagnosis of exclusion. The diagnosis is often delayed because the initial symptoms may be mild and the exam findings subtle. The mean age of survival is 2.8 years without treatment. With treatment patients can survive for more than 10 years. Current lifetime treatment includes anticoagulation, oxygen, digoxin, calcium channel blockers, vasodilators, and diuretics. Promising results have been obtained with prostacyclin and endothelin receptor antagonists. Lung transplantation can be considered for patients with advances PPH and those not responding to medical therapy.     

Prothrombotic mechanisms in primary pulmonary hypertension

Pulmonary hypertensive states are associated with an increased propensity for thrombosis. This prothrombotic state appears to be a result of pulmonary hypertension promoting endothelial dysfunction and altered hemodynamic status. In some patients with primary pulmonary hypertension, however, a primary prothrombotic state directly induces the pulmonary hypertensive state. This review focuses on the evidence for the association between prothrombotic states, especially increased platelet activation, and the development of pulmonary hypertension.     

Vasodilator therapy for primary pulmonary hypertension

Primary pulmonary hypertension is an uncommon but serious disease that often results in debilitating symptoms and early death. One approach to treatment has been to attempt reduction of pulmonary arterial pressure and right ventricular afterload by using vasodilator agents to decrease pulmonary arteriolar resistance. Use of a variety of vasodilators has yielded only limited and infrequent success. Although an occasional patient may respond to vasodilator therapy with improved pulmonary hemodynamics and symptomatic status, most patients do not. Reasons for lack of success include the presence of predominantly fixed vascular obstructive disease rather than active vasoconstriction, inexorable progression of disease, and adverse nonpulmonary vascular effects of vasodilators, such as systemic hypotension. Because of occasional beneficial responses, a trial of vasodilators is warranted in patients with primary pulmonary hypertension. Initiation of drug therapy should be undertaken during hemodynamic monitoring in order to allow assessment of response, identification of adverse effects, and comparison of different agents. No vasodilator is clearly superior in primary pulmonary hypertension.     

A case of primary pulmonary hypertension

Primary lung hypertension (PLH) is a rare disease of unknown etiology seen largely in young persons. PLH shows rapid progression with lethal outcome 3-5 years after registration of the first symptoms. Early diagnosis of the disease is difficult. A case described in the article illustrates real difficulties in PLH diagnosis and treatment. The patient several times lost consciousness for a short time at insignificant exercise. This phenomenon has not been described in the literature so far.     

Epidemiology of primary pulmonary hypertension in Japan]

Primary pulmonary hypertension(PPH) is a progressive disease of unknown etiology with poor prognosis. Recent epidemiological studies in Japan have shown that there are 230 patients (95% confidence interval: 200-260) with mean age of 41.8 +/- 18.5 years old, female to male ratio of 1.72: 1 and their median survival time was 69.0 months. At a later time, the clinically effective modalities such as lung transplantation and prostaglandin I2 for PPH were available in Japan. In 1999, the Ministry of Health and Welfare of Japan approved PPH as a objective disease of therapeutic study. Once patient is approved as PPH based on the personal clinical inquiry, Doctor's fee will be paid at the public government. This system will promote earlier and more accurate diagnosis in PPH patients and will clarify more reliable clinico-epidemiological figure of PPH in a whole territory of Japan.     

多普勒超声心动图在诊断原发性肺动脉高压的应用

目的探讨多普勒超声心动图对原发性肺动脉高压(PPH)诊断和鉴别诊断的价值.方法回顾性分析75例PPH患者的超声诊断,部分与心导管结果对照.结果 75例PPH患者中,超声诊断67例,诊断符合率达89.3%;其中有18例与心导管检查对比,两者对肺动脉压力的测定结果无明显差异(89.3±23.2) mmHg vs (80.8±25.6) mmHg, P＞0.05,两种方法相关性好(r=0.82, P＜0.001);早期PPH患者左心收缩功能可正常,晚期严重PPH患者则降低.结论超声心动图可无创、准确测定PPH患者肺动脉压力,重复性好;超声心动图在PPH诊断与鉴别诊断方面有一定的价值;超声心动图检测PPH患者左室收缩功能,可了解病情进展和评估疗效.     

Lifespan of patients with primary pulmonary hypertension

AIM: To design a mathematical express model of expected lifespan of patients with primary pulmonary hypertension (PPH). MATERIAL AND METHODS: A retrospective analysis of 418 case histories of 132 PPH patients (81 females, 51 males, age 16-57 years). Ninety one of them were hospitalized at least twice. The following characteristics of the patients were registered: a PPH form, age at onset, gender, morphological structure of the lung, date of the first visit to doctor. Relationship of these factors with lifespan was analysed and the express formula of expected lifespan was calculated using the multifactorial analysis of variance with standard computer statistical programs. RESULTS: Lifespan was studied for dependence on static factors free of therapy effects (gender, age, form of PPH). The presence of a factor named "duration of the disease development" (DDD) is suggested. A correlation was found between DDD and lifespan in PPH patients explaining survival by 84.5%. The formula of the prognosis was derived for patients with known morphological structure of the lung in whom the above dependence explains survival by 93.5%. CONCLUSION: Among effect modifiers, of significance for survival are age, PPH form, morphological structure of the lung. An artificial index DDD was used for deriving a formula for prognosis of survival for a newly hospitalized PPH patient.     

Differential diagnosis of primary vascular pulmonary hypertension

The paper is concerned with 18 patients suffering from primary vascular pulmonary hypertension. Of these, 7 patients had the thromboembolic pattern of primary vascular pulmonary hypertension and 11 primary pulmonary hypertension. All the patients underwent comprehensive examinations including routine clinical examinations, x-raying of the chest organs, ECG, ultrasound cardiography, measurement of the pressure in the pulmonary artery by invasive and non-invasive methods. The patients were also subjected to radionuclide studies using radionuclide scintigraphy of the lungs by means of 99mTc microspheres and radionuclide phlebography. Analysis of the clinical picture and of the findings obtained with the majority of instrumental methods including radionuclide scintigraphy did not reveal any appreciable differences between the patients with the thromboembolic pattern of primary vascular pulmonary hypertension and those suffering from primary pulmonary hypertension. Radionuclide phlebography has demonstrated that the patients with the thromboembolic pattern showed the changes in the phlebogram significantly more often than those with primary pulmonary hypertension. It is advisable that radionuclide phlebography should be included into the schedule for examining patients with primary vascular pulmonary hypertension.     

Echocardiographic features of primary pulmonary hypertension.

Primary pulmonary hypertension (PPH) is essentially a diagnosis of exclusion and usually is made late because of the nonspecific nature of the early signs and symptoms. Echocardiography is a key screening test in the diagnostic algorithm of patients with suspected PPH. The purpose of this study was to define the echocardiographic Doppler features in patients with PPH at the time of diagnosis. From 1992 to 1997, 51 patients were diagnosed with PPH at our institution. All underwent a standardized transthoracic echocardiographic examination, including a contrast study and transthoracic echocardiographic examination if indicated. Pulmonary artery systolic pressure was calculated from the tricuspid regurgitation jet. The majority of patients had pulmonary artery systolic pressure greater than 60 mm Hg (96%) associated with systolic flattening of the interventricular septum (90%), enlarged right atrium (92%) and ventricle (98%), and reduced right ventricular systolic function (76%). There was an increase in the interventricular septal thickness (>1.2 cm) in 21 (43%) of 49 patients, accompanied by a septal/posterior wall ratio greater than 1.3 in 11 (22%) of 49. Although a reduction in both left ventricular systolic and diastolic volumes was noted, global left ventricular systolic function was preserved in all patients. Mitral E/A ratio was less than 0.7 in 7 (22%) patients studied. Color Doppler revealed moderate to severe tricuspid regurgitation and pulmonic insufficiency in 41 (80%) of 51 and 16 (31%) of 51 of cases, respectively. Pericardial effusion (7 small and 1 moderate) and patent foramen ovale (n = 12) were also frequently detected. At the time of initial diagnosis, PPH is associated with secondary cardiac abnormalities in the majority of patients.