Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon disease with a severe prognosis. Initially considered as a constantly fatal disease, it can be cured since the advent in the late 80's of thromboendarterectomy. CTEPH occurs in approximately 0.1% of patients who survive acute pulmonary embolism. Such outcome is due to the failure of the normal thrombi resolution in the pulmonary circulation. This disease is observed in both sexes and occasionally as early as in the third decade. Many patients have no history of acute venous thromboembolism which is responsible for the frequent diagnosis delay. Therefore, it is of paramount importance to search for pulmonary vascular disease when patients complain solely of dyspnea on exertion. These patients should undergo echocardiography and, if necessary, right heart catheterization. Once the diagnosis of pulmonary hypertension is established the next step is to find the cause. Ventilation-perfusion scanning is probably the most sensitive non-invasive test to provide evidence that pulmonary hypertension is related to chronic thromboembolism. Angiography and helical computed tomography allow to confirm the diagnosis of CTEPH and to determine whether it is accessible or not to thromboendarterectomy. Most patients who undergo thromboendarterectomy improve clinically and in terms of gas exchange and pulmonary hemodynamics. When thrombi are inaccessible surgically, patients should be placed on the list for lung transplantation if they fulfill the criteria established for primary pulmonary hypertension. When all surgical procedures are contraindicated, anticoagulant and oxygen therapy remain the sole possibility of treatment.
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PMID:[Chronic thromboembolic pulmonary arterial hypertension]. 1090 51

We describe a case of long-term administration of nitric oxide (NO) in a 32-year-old man who was admitted with exertional dyspnea and anasarca. A diagnosis of primary pulmonary hypertension was made. An acute vasodilator trial with inhaled NO showed a 5% reduction of the mean pulmonary artery pressure. Long-term NO inhalation therapy was initiated. Twenty days later, the dyspnea improved, the anasarca resolved, and the PaO(2) level increased. After 12 months of NO therapy, the patient remained stable and no signs of toxicity or tachyphylaxis were observed. To our knowledge, this is the first report of 1 year of continuously inhaled NO in an adult patient with primary pulmonary hypertension. These findings suggest that prolonged NO therapy might be an effective alternative, at a lower cost, to the continuous IV infusion of epoprostenol.
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PMID:One-year continuous inhaled nitric oxide for primary pulmonary hypertension. 1124 87

Primary pulmonary hypertension is a rare disease of unknown etiology, whereas secondary pulmonary hypertension is a complication of many pulmonary, cardiac and extrathoracic conditions. Chronic obstructive pulmonary disease, left ventricular dysfunction and disorders associated with hypoxemia frequently result in pulmonary hypertension. Regardless of the etiology, unrelieved pulmonary hypertension can lead to right-sided heart failure. Signs and symptoms of pulmonary hypertension are often subtle and nonspecific. The diagnosis should be suspected in patients with increasing dyspnea on exertion and a known cause of pulmonary hypertension. Two-dimensional echocardiography with Doppler flow studies is the most useful imaging modality in patients with suspected pulmonary hypertension. If pulmonary hypertension is present, further evaluation may include assessment of oxygenation, pulmonary function testing, high-resolution computed tomography of the chest, ventilation-perfusion lung scanning and cardiac catheterization. Treatment with a continuous intravenous infusion of prostacyclin improves exercise capacity, quality of life, hemodynamics and long-term survival in patients with primary pulmonary hypertension. Management of secondary pulmonary hypertension includes correction of the underlying cause and reversal of hypoxemia. Lung transplantation remains an option for selected patients with pulmonary hypertension that does not respond to medical management.
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PMID:Diagnosis and treatment of pulmonary hypertension. 1135 91

In March 1995, a 54-year-old woman was admitted to a hospital because of exertional dyspnea and her condition was diagnosed as primary pulmonary hypertension. In September 1996, respiratory difficulty and hemosputum were woted. The chest computed tomographic scan showed diffuse micronodular opacities, and she was admitted to our hospital for examination of other pulmonary conditions giving rise to pulmonary hypertention. However no lung biopsy could be performed, because the pulmonary arterial pressure was too high. Although the patient received medication, she died of progressive right heart failure. The pathological diagnosis at autopsy was pulmonary capillary hemangiomatosis (PCH). Although PCH is a very rare disease that causes secondary pulmonary hypertension, it is important to distinguish it from primary pulmonary hypertension and pulmonary veno-occlusive disease.
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PMID:[A case of pulmonary capillary hemangiomatosis]. 1157 25

Primary pulmonary hypertension is a rare disease of the pulmonary vasculature manifested by dyspnea on exertion, syncope, and signs and symptoms of right heart failure. In the absence of adequate treatment, primary pulmonary hypertension has a grave prognosis, with a median survival of 2.8 years. Pulmonary arterial hypertension develops in association with known risk factors and predisposing clinical conditions, and shares many clinical, pathological and therapeutic characteristics with primary pulmonary hypertension. Therapeutic choices in pulmonary arterial hypertension depend on the etiology of the disease, severity of functional impairment and hemodynamic response following acute vasodilator administration during right heart catheterization. Agents currently approved for the specific treatment of pulmonary arterial hypertension are continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan. A small group of patients who demonstrate true acute vasoreactivity at right heart catheterization may be chronically treated with oral calcium channel blockers. In addition, most patients with pulmonary hypertension receive conventional treatment, represented by anticoagulants, diuretics, inotropic medication or oxygen supplementation. Treatment of pulmonary arterial hypertension has significantly altered the natural course of the disease, with pronounced symptomatic, functional and survival benefit. Current clinical research focuses on the discovery of new targets of therapy and the use of a combination treatment approach, which will offer hope and valuable insight into the pathogenetic basis of this devastating illness.
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PMID:Current medical treatment of pulmonary arterial hypertension. 1502 2

Case 1, a 28-year-old woman (third daughter of Case 2) delivered her first child in September 2000, but leg edema and dyspnea on exertion appeared the following January. At the time of our first examination of the patient in May 2001, a chest X-ray showed cardiomegaly and pulmonary artery enlargement. Echocardiography demonstrated enlargement of the right ventricle and small left ventricular dimensions, and an electrocardiogram revealed right ventricle hypertrophy. After perfusion-ventilation lung scintigraphy and cardiac catheterization, she was diagnosed as having primary pulmonary hypertension (PPH). Although she was discharged with prescriptions for a diuretic, warfarin and beraprost sodium, she died of a pulmonary hypertensive crisis twenty days after readmission. Case 2, a 60-year-old woman(mother of Case 1) developed the same symptoms as those in Case 1, in May 2001, but recovered after medication. PPH is a rare disease and only a few familial cases are reported. In this family, the eldest daughter of Case 2 had also died of pulmonary hypertension ten years ago, several months after her first delivery. In contrast to the daughters, the mother's symptoms developed gradually.
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PMID:Two cases of familial primary pulmonary hypertension. 1562 56

A 31-year-old woman in whom primary pulmonary hypertension had been diagnosed at age 18, was admitted because of dyspnea on exertion and for evaluation of pregnancy risk. The perfusion scanning was not diagnostic, but chest CT suggested stenosis of pulmonary arteries instead of primary pulmonary hypertension. Echocardiograms and cardiac catheterization revealed pulmonary hypertension of pulmonary arterial pressure 71/13 (mean 39) mmHg with no cardiac defects. Selective pulmonary angiograms showed multiple stenosis of pulmonary arteries with poststenotic dilatations. We diagnosed multiple peripheral pulmonary artery branch stenosis with the pulmonary hypertension because systemic vasculitis was excluded because there were no signs of inflammation or any systemic arterial invlolvement This case was thought to be congenital, considering her history of over 30 years. In reports from western countries, maternal rubella and familial factors were important etiologic factors of this disease. Few cases have been reported in Japan and all cases, including the present case, were unrelated to the specific etiological factors. We recommended her to use birth control and have been observing her very carefully since the progression of the pulmonary hypertension is the major determinant of her prognosis.
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PMID:[A case of the multiple peripheral pulmonary artery branch stenosis]. 1645 39

We report a case of severe pulmonary hypertension associated with sarcoidosis with a unique histopathological presentation. This 43-year old obese patient first presented eight years ago with complaint of dyspnea on exertion for four years and was diagnosed as primary pulmonary hypertension. Six years later, a skin biopsy performed on her left cheek to rule out squamous cell carcinoma revealed sarcoidosis. The patient was then put on steroid therapy and subsequently, the oxygen saturation improved transiently, although the mean pulmonary arterial pressure did not show improvement. The patient was then started on prostacyclin infusion and was hemodynamically stable, but the pulmonary artery pressures worsened. The patient died from complications subsequent to a diagnostic procedure. An autopsy limited to the lungs was performed and routinely prepared hematoxylin and eosin stained sections were examined. Immunohistochemical stains for CD31, factor VIII-related antigen and muscle-specific actin were performed on selected sections. The diagnosis of sarcoidosis was confirmed. However, pulmonary fibrosis was not seen. The granulomas surrounded medium- and small-sized pulmonary arteries, but did not destroy the vessel wall. Plexiform lesions indicating severe pulmonary hypertension were identified in pulmonary arteries, which were not involved by granulomas.
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PMID:Sarcoidois and pulmonary hypertension--a case report. 1672 92

Pulmonary hypertension (PH) is a serious disease with a poor prognosis. Pulmonary arterial hypertension (PAH) is distinguished from other forms of PHT. The leading symptom is exertional dyspnea. Patients with chronic severe PAH commonly die of right heart failure. PH has a complex heterogeneous aetiology, and precapillary forms are distinguished from postcapillary PH. A number of pathogenetically important signaling pathways such as the endothelin, nitric oxide and prostacyclin pathways have been identified, which constitute the target of the currently approved treatment options. Transthoracic echocardiography is the most important noninvasive diagnostic method. In addition, 6-min walking distance and measurement of serum markers, such as brain natriuretic peptide (BNP), are diagnostically useful and of prognostic significance.
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PMID:[Pulmonary hypertension: classification, pathophysiology and noninvasive diagnosis]. 1713 90


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