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Query: UMLS:C0015672 (fatigue)
 51,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary pulmonary hypertension carries a poor prognosis, with a 5 year survival rate of less than 25%. However, a previous study of more than 100 patients with tissue-proved primary pulmonary hypertension suggested that antithrombotic therapy may have a beneficial effect on survival, especially in patients with the thromboembolic type of primary pulmonary hypertension. This report describes a 54 year old white man with primary pulmonary hypertension of the thromboembolic type (proved by right upper lobe lung biopsy) who, after long-term antithrombotic therapy, showed resolution of symptoms of dyspnea and fatigue, regression of electrocardiographic signs of right ventricular hypertrophy and regression of elevated pulmonary artery pressure. Base-line cardiac catheterization in January 1982 revealed elevated pulmonary artery pressure (104/37 mm Hg) and pulmonary vascular resistance (14.6 units/m2) that did not decrease with 100% oxygen or intravenous hydralazine (12 mg). The patient was treated with warfarin and dipyridamole, 100 mg four times daily. The most recent cardiac catheterization in January 1984 revealed a pulmonary artery pressure of 50/15 mm Hg and a pulmonary vascular resistance of 8.7 units/m2. It is believed that this is the first report of regression of the symptoms and signs of biopsy-proved primary pulmonary hypertension. In view of the lack of a response to vasodilators in 1982, it is suggested that antithrombotic therapy is partially responsible for the improvement of this patient.
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PMID:Regression in thromboembolic type of primary pulmonary hypertension during 2 1/2 years of antithrombotic therapy. 394 Dec 8

Clinical, echocardiographic and haemodynamic features in 14 patients (8 male and 6 females) with primary pulmonary hypertension, diagnosed by strict clinical and haemodynamic criteria are described. Age of the patients at diagnosis ranged from 11 years to 40 years with a mean of 23 years. The mean interval from onset of symptoms to diagnosis was 2.6 years. Common symptoms included dyspnoea on exertion (86%), fatigue (78%) and palpitation (78%). Raynaud's phenomenon was not encountered but one patient had signs and symptoms of left recurrent laryngeal nerve palsy. Pulmonary function studies showed mild restrictive ventilatory impairment (mean forced vital capacity 80% of predicted) with hypoxaemia and hypocapnea. The M-mode tracing of the pulmonary valve showed flat or negative E-F slope, a small or absent 'a' wave, and midsystolic notching in all the patients. Two-dimensional echocardiographic images showed a thickened right ventricular wall in 12(86%) patients; a normal to small left ventricular end-diastolic internal dimension in all the patients and right ventricular and right atrial enlargement in 78% of patients. Significant tricuspid regurgitation and pulmonary regurgitation was documented by Doppler interogation in 87% and 62% of patients examined respectively. Haemodynamic findings consisted of a marked rise of pulmonary artery pressure and pulmonary vascular resistance, low cardiac index and normal pulmonary artery wedge pressure. At cardiac catheterisation the mean right atrial pressure (mean +/- SD) was 9.8 +/- 4.8 mmHg; mean pulmonary artery pressure, 63.2 +/- 14.3 mmHg; cardiac index, 2.2 +/- 0.9 L/min. m2; and pulmonary vascular resistance index, 22.3 +/- 10.6 mmHg/L/min. m2; in these patients. No, death nor any sustained morbid events occurred during the diagnostic evaluation of the patients.
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PMID:Primary pulmonary hypertension: clinical, echocardiographic and haemodynamic features in 14 patients. 803 Dec 85

A Haitian woman with the human immunodeficiency virus (HIV) presented with dyspnea, cough, fatigue and lower abdominal pain of recent onset. Clinical, radiologic and hemodynamic investigations demonstrated pulmonary hypertension. The patient died a few days later. The pathological findings were compatible with primary pulmonary hypertension. This case is similar to others that have been reported and indicates a possible link between HIV infection and rapidly progressive primary pulmonary hypertension.
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PMID:Primary pulmonary hypertension associated with HIV infection. 842 54

Primary pulmonary hypertension (PPH), an often fatal disease, is characterized by elevated pulmonary artery pressures in the absence of a secondary cause. Endovascular occlusion in the smallest pulmonary arteries occurs by proliferation of cells and matrix, with thrombus and vasospasm. Diagnosis is often delayed because the initial symptoms of fatigue and dyspnea on exertion are nonspecific and definitive diagnosis requires invasive procedures. The average life expectancy after diagnosis is two to three years with death usually due to progressive right heart failure. The aetiology of the disease is unknown. Although most cases appear to be sporadic, approximately 6% of cases recorded in the NIH Primary Pulmonary Hypertension Registry are inherited in an autosomal dominant manner with reduced penetrance. Following a genome-wide search using a set of highly polymorphic short tandem repeat (STR) markers and 19 affected individuals from six families, initial evidence for linkage was obtained with two chromosome 2q markers. We subsequently genotyped patients and all available family members for 19 additional markers spanning approximately 40 centiMorgans (cM) on the long arm of chromosome 2. We obtained a maximum two-point lod score of 6.97 at theta = 0 with the marker D2S389; multipoint linkage analysis yielded a maximum lod score of 7.86 with the marker D2S311. Haplotype analysis established a minimum candidate interval of approximately 25 cM.
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PMID:Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32. 905 41

Prostacyclin (or epoprostenol), an arachidonic acid metabolite, is an effective treatment for patients with primary pulmonary hypertension. Interruption of chronic prostacyclin infusion can result in recurrent symptoms of dyspnea and fatigue. The etiology of this phenomenon is unknown. We hypothesized that sympathoadrenal activation could lead to increased vascular tone after abrupt termination of the infusion. To evaluate this effect, we monitored six chronically instrumented, awake sheep during and after infusion of prostacyclin. Prostacyclin decreased mean arterial pressure (MAP) by 14% and increased cardiac output by 33%. After the infusion ceased, MAP rebounded 23% above baseline, and cardiac output decreased by 28% from peak values within 10 min. We were unable to demonstrate an increase in norepinephrine levels after cessation of prostacyclin, nor did alpha-adrenergic blockade affect postinfusion hemodynamics. However, plasma renin activity increased >10-fold at peak infusion and remained elevated for up to 2 h after discontinuation of prostacyclin. Coinfusion of the angiotensin II-receptor antagonist L-158,809 resulted in complete abrogation of the postcessation rise in MAP. We conclude that renin-angiotensin system activation is primarily responsible for systemic hypertension occurring after abrupt cessation of prostacyclin infusion in sheep and that angiotensin II receptor blockade prevents this response. Our data do not support a role for sympathetic nervous system activation in the systemic pressor response after prostacyclin infusion.
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PMID:Angiotensin II mediates systemic rebound hypertension after cessation of prostacyclin infusion in sheep. 968 53

Primary pulmonary hypertension presents a challenge to practicing physicians, in both diagnosis and management. Exposure to anorexigens and complaints of dyspnea and fatigue should prompt careful physical examination and Doppler echocardiography to assess patients for pulmonary hypertension. The burden on office practitioners is heavy, considering how often fatigue and dyspnea are reported, but the key is recognizing when these findings are out of proportion to the patient's well appearance. The discovery of epoprostenol therapy has revolutionized the approach to primary pulmonary hypertension. It has markedly improved quality of life and extended survival in patients with the condition, and it has changed the physician's role from providing emotional support to dying patients to providing management of a chronic disease.
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PMID:How to manage primary pulmonary hypertension. Giving hope to patients with a life-threatening illness. 1008 33

Primary pulmonary hypertension (PPH) is a pulmonary vascular disease characterized by an elevation in mean pulmonary artery pressure and pulmonary vascular resistance. Recently, PPH gained national attention because of its association with appetite suppressants. PPH may also be associated with pregnancy, hypothyroidism, autoimmune disorders, human immunodeficiency virus infection, and the use of drugs such as oral contraceptives and cocaine. Patients with PPH may report dyspnea on exertion and fatigue. Early diagnosis is crucial. New therapeutic regimens have dramatically reduced mortality rates and improved quality of life by halting the progression of pulmonary vascular remodeling and averting right-sided heart failure. These therapies include high-dose calcium channel antagonists, anticoagulants, and continuous intravenous prostacyclin. Lung or heart-lung transplantation remains a viable therapeutic option for patients who are treated late in the disease process, who are not responsive to medical management, or who remain symptomatic and continue to deteriorate.
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PMID:Diagnosing and treating primary pulmonary hypertension. 1050 69

An obese 23-year-old man with sleep-disordered breathing and primary pulmonary hypertension (PPH) had been administered oral beraprost sodium, anticoagulant warfarin, and home oxygen therapy, at another hospital as treatment for the PPH, but he had not experienced any symptomatic improvement. The patient had a body mass index of 32.4kg/m2, and complained of fatigue, shortness of breath on exertion, excessive daytime sleepiness, and snoring. Arterial blood gas analysis showed a PaO2 and a PaCO2 of 70.9 and 31.2mmHg, respectively. A polysomnographic study revealed central sleep apnea with an apnea-hypopnea index (AHI) of 29.7episodes/h. The patient showed improvement of daytime sleepiness after starting nocturnal nasal bilevel positive airway pressure (BiPAP) therapy for the central sleep apnea, but his pulmonary hypertension, measured in the daytime, worsened. The patient died suddenly while walking to the bathroom in the morning 1 month after initiation of BiPAP therapy. It is necessary to consider the possibility of sudden death when nasal BiPAP therapy is given to a PPH patient with central sleep apnea.
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PMID:Primary pulmonary hypertension with central sleep apnea: sudden death after bilevel positive airway pressure therapy. 1098 61

Primary pulmonary hypertension (PPH) is a condition characterized by sustained elevation of pulmonary artery pressure (PAP) without demonstrable cause. The most common symptom at presentation is dyspnea. Other complaints include fatigue, chest pain, syncope, leg edema, and palpitations. Right heart catheterization is diagnostic, showing a mean PAP >25 mmHg at rest and >30 mmHg during exercise, with a normal pulmonary capillary wedge pressure. In the National Institutes of Health-PPH registry, the median survival period was 2.8 years. Treatment is aimed at lowering PAP, increasing cardiac output, and decreasing in situ thrombosis. Vasodilators have been used with some success in the treatment of PPH. They include prostacyclin, calcium-channel blockers, nitric oxide and adenosine. Anticoagulation has also been advised for the prevention of deep vein thrombosis, pulmonary embolism, and in situ thromboses of the lungs. New drug treatments under investigation include L-arginine, plasma endothelin-I, and bosentan. Use of oxygen, digoxin, and diuretics for symptomatic relief have also been recommended. Patients with severe PPH refractory to medical management should be considered for surgery.
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PMID:Primary pulmonary hypertension. 1172 93

Liver disease affects the lungs. The majority of patients exhibit mild to moderate arterial hypoxaemia essentially attributable to an alteration in ventilation/perfusion matching and limited by an increase in ventilation. A minority (some 10%) of patients exhibit a "hepatopulmonary syndrome" defined by severe hypoxaemia with arterial PO2 below 60 mm Hg, dyspnoea, cyanosis, digital clubbing, orthodeoxia, platypnoea and demonstrable pulmonary vascular dilatations causing a true pulmonary shunt and a diffusion/perfusion imbalance. The hepatopulmonary syndrome is incurable but resolves over time after liver transplantation. An even lower proportion of patients, approximately 1%, develop pulmonary hypertension. Clinically this "portopulmonary hypertension" resembles primary pulmonary hypertension, with dyspnoea and fatigue as the main symptoms, histopathology and response to prostacyclin therapy. Portopulmonary hypertension is irreversible. Liver transplantation mortality in patients with portopulmonary hypertension ranges from 50 to 100%. The common cause of the hepatopulmonary syndrome and portopulmonary hypertension is portal hypertension and portosystemic shunting, indicating that vasoactive and angiogenetic factors originating from the liver normally control the pulmonary circulation.
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PMID:Hepatopulmonary syndrome and portopulmonary hypertension. 1271 85


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