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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cor pulmonale is right ventricular enlargement secondary to pulmonary hypertension. Although most often caused by parenchymal lung disease, derangements of the ventilatory drive, the respiratory pumping mechanism, or the pulmonary vascular bed may also result in right ventricular hypertrophy and dilatation. Arterial hypoxemia (and resultant polycythemia), hypercapnia, and respiratory acidosis all contribute to the increased afterload on the right ventricle. Diagnosis is often difficult, since pulmonary vascular disease, pulmonary hypertension, and cor pulmonale have few specific manifestations, especially early in their evolution. Treatment is primarily directed at the underlying pulmonary or ventilatory disorder, rather than at the right ventricular failure per se. Supplemental oxygen is essential to avoid hypoxia; corticosteroids, anticoagulants, vasodilators, and other specific therapies are used as indicated to treat the underlying pulmonary disorders. When medical therapies fail, lung or heart-lung transplantation has become a possibility for selected patients.
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PMID:Chronic cor pulmonale. Etiology and management. 239 36

Unilateral lung transplantation has become a successful method for the treatment of end-stage pulmonary disease, whereas double-lung transplantation has provided benefit to patients with nonfibrotic lung disease such as emphysema and cystic fibrosis. In the past 5 years, 16 single-lung and 13 double-lung transplantations have been performed by the Toronto Lung Transplant Group in patients with end-stage lung disease. Seven perioperative and two late deaths have been recorded so far. Since the introduction of heart-lung transplantation at Stanford in 1981 and at Pittsburgh in 1982 for the treatment of Eisenmenger's syndrome and terminal pulmonary vascular disease, more than 350 combined heart-lung transplantations have been carried out throughout the world. Presently, the 2-year actuarial survival is about 62%. The long-term results have not yet reached the same level of success as those of cardiac transplantation alone. Although several factors have played a role in this difference, a prominent cause has been the lack of a reliable and simple method for pulmonary protection against prolonged ischemia. Most of the techniques proposed against ischemia can be classified as normothermic or static hypothermic cardiopulmonary preservation. The use of the normothermic method has not always been successful. For this reason, interest has now been directed toward the potential for hypothermic preservation of the heart-lung bloc and the use of free-radical scavenger therapy in the reduction of reperfusion injury.
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PMID:Lung and heart-lung transplantation. 218 26

The balance between proteases and antiproteases in the lower respiratory tract is believed to play a role in the outcome of interstitial lung diseases. In this cross-sectional study, we measure several phagocyte derived enzymes, namely plasminogen activator, neutrophil elastase and an ill-defined protease active on the trialanine chromophore substrate succinyl-alanine3-nitroanilide (SLAPN) in bronchoalveolar lavage (BAL) fluid from 42 patients with pulmonary sarcoidosis and from 43 patients with collagen vascular disease (CVD), 22 without lung disease (group I) and 21 associated with parenchymal lung disease (group II). The results show: a) that sarcoidosis is associated with increased plasminogen activator activity and with the presence of enzymatic activity against SLAPN corresponding at least in part to a metalloprotease; b) that CVD in the absence of radiographic lung disease is associated with an increase of plasminogen activator activity and increased levels of alpha 1-antiprotease-neutrophil elastase complexes; c) that the majority of untreated CVD (group II) patients have detectable levels of neutrophil elastase activity. These data show that patients with pulmonary sarcoidosis and CVD have different enzymatic profiles in their lower respiratory tract as assessed by BAL. Thus, sarcoidosis (mostly lymphocytic) is associated with enhanced macrophage-derived proteolytic activity in BAL, while CVD patients both with and without lung disease have increased neutrophil counts and neutrophil elastase complexed to alpha 1-protease inhibitor and presumably inactive in BAL. Finally, only BAL from untreated CVD patients with interstitial lung disease contain neutrophil elastase activity. This latter activity could contribute to the lung lesions frequently observed in these disorders.
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PMID:Phagocyte enzymes in bronchoalveolar lavage from patients with pulmonary sarcoidosis and collagen vascular disorders. 218 5

We retrospectively prepared step sections of the transbronchial lung biopsy (TBLB) materials which revealed nondiagnostic findings in their original sections in patients with diffuse lung disease, and evaluated the significance of the examination of step sections in the diagnosis of diffuse lung disease. Of 131 cases with nondiagnostic TBLB findings, the preparation of step sections resulted in specific findings in 6 cases (malignancy 3 cases, tuberculosis 1 case, cryptococcosis 1 case and viral infection 1 case), and histopathological changes consistent with the clinical diagnosis in 25 cases. The step section preparation was especially useful for the detection of epithelioid granuloma and tumor tissue in patients with sarcoidosis and carcinoma, respectively, while its contribution to the diagnosis of collagen-vascular disease, hypersensitivity pneumonitis, atypical pneumonia and pneumoconiosis was relatively small. The step section preparation was also useful for the detection of bronchiolitis obliterans. In addition, step sections uncovered clinically unnoticed infection (purulent exudate in the alveolar space) in 6 cases, 3 of whom actually developed pneumonia thereafter. Thus, the preparation of step sections was considered to be useful clinically in 37 cases (28.2%). The preparation of step sections is recommended before a further diagnostic procedure is chosen, when TBLB performed in patients with diffuse lung disease reveals nondiagnostic findings.
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PMID:[Step section preparation of transbronchial lung biopsy material in diffuse lung disease]. 229 Feb 27

One hundred and fifty-nine combined heart and lung transplantations were performed on 152 patients at Harefield Hospital from 1980 to February 1988. The age of the recipients ranged from 10 weeks to 52 years. The transplantation was indicated for pulmonary vascular disease on 106 patients (69.7 per cent), and for parenchymal lung disease on 46 patients (30.3 per cent). Eisenmenger syndrome was the commonest disease that required the heart-lung transplantation. A combination of cyclosporin A and azathioprine was administered for immunosuppression therapy postoperatively, and oral steroids were not routinely used. The hospital mortality rate was 32.2 per cent, and 103 patients were discharged from the hospital. The first-year actuarial survival rate was 64 per cent, and the second-year was 61 per cent. Although pulmonary deterioration due to obliterative bronchiolitis was a serious late complication, most of the recipients enjoyed a good quality of life after transplantation. Heart and lung transplantation offers the patient a chance of marked improvement both in survival and in quality of life.
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PMID:Heart-lung transplantation--Harefield experience. 235 2

The lungs are frequently affected in systemic sclerosis, and pulmonary disease may significantly influence morbidity and mortality. The clinical, radiographic, and physiologic features of scleroderma lung disease are discussed in this article, as are new approaches to the detection and study of interstitial pneumonitis. The pathogenesis of pulmonary fibrosis is discussed in the context of data derived from bronchoalveolar lavage studies. Pulmonary vascular disease and less common pulmonary features of systemic sclerosis are also discussed.
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PMID:Lung involvement in systemic sclerosis. 240 8

Much of the understanding of hypertensive pulmonary vascular disease comes from studies of primary pulmonary hypertension. The three subtypes of primary pulmonary hypertension, plexogenic pulmonary arteriopathy, thromboembolic pulmonary hypertension and venoocclusive disease, have served as a basis to understand the mechanisms and to develop treatments of all forms of pulmonary hypertension. However, many inconsistencies regarding presumed pulmonary vasoconstriction and recurrent embolization remain. With newer data on the influence of the endothelium on vascular responsiveness and thrombosis, it appears that older concepts regarding the pathophysiology of pulmonary hypertension need to be revised. Recent studies have shown that plexogenic pulmonary arteriopathy is associated with abnormalities of endothelial structure and function that could result in impaired release of endothelial derived relaxing factors. Thromboembolic pulmonary arteriopathy, or more properly thrombotic pulmonary hypertension, appears to be the result of endothelial cell injury that creates a procoagulant environment in the pulmonary vascular bed with the development of widespread eccentric intimal proliferation and thrombosis in situ. It is possible that the effectiveness of vasodilator or anticoagulant therapy depends on the nature of the endothelial injury. Secondary pulmonary hypertension without endothelial injury, such as that which occurs with hypoxic lung disease or mitral stenosis, appears more satisfactorily treated when the primary cause is reversed.
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PMID:Pulmonary hypertension: a cellular basis for understanding the pathophysiology and treatment. 267 Oct 93

We have discussed the relationship between systemic illness, infection, and lung disease. As we have seen, patients with a wide variety of disease states, including advanced age, diabetes mellitus, alcoholism, collagen vascular disease, cancer, heart failure, and organ transplantation are potentially at increased risk for pneumonia because of disease-related impairments in host defenses. In addition, two virtually ubiquitous conditions in hospitalized patients, malnutrition and therapeutic interventions (especially with common medications), frequently add to the risk of airway invasion by bacterial pathogens. Systemic illness not only makes lung infection more common, but may adversely affect outcome and resolution, as well as determine the clinical presentation of pneumonia. In one particular population, the intubated and mechanically ventilated patient, the risk of infection is particularly high, and nosocomial pneumonia is a major cause of mortality. To the extent that the host response itself leads to the symptoms and signs of infection, systemically ill individuals may have subtle clinical features when serious bacterial invasion is present. Many components of the host defense system can become abnormal with serious illness, but a common mechanism that ties many systemic diseases to pneumonia is an alteration in airway epithelial cell receptivity for bacteria, namely, bacterial adherence, a process that mediates airway colonization, the first pathogenetic step on the road to pneumonia. The impetus for understanding how serious illness promotes lung infection is that once these mechanisms are identified, potential preventative strategies to minimize infection risk in the individual with systemic disease may be developed. The relationship among systemic illness, the lung, and infection also exists in a different direction: infection of a systemic nature (the septic syndrome) can lead to disease in the lung (ARDS). We have described the features of the septic syndrome and identified how it may lead to lung injury, usually by indirect means, through activation of inflammatory mediators that are carried to the lung via the vasculature. Although it is frequently impossible to predict which specific patient with systemic sepsis will develop acute lung injury, the current state of knowledge does permit us to identify high-risk individuals. Surprisingly, clinical assessment rather than biochemical testing is the best predictor of the development of acute lung injury. Patients with severe injury, profound shock and multiple systemic insults are most prone to acute lung injury in the presence of systemic sepsis.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Respiratory infections and acute lung injury in systemic illness. 268 63

Over the last eight years a completely new form of treatment has been introduced for end-stage pulmonary vascular disease and chronic lung disease, including cystic fibrosis. Lung transplantation and heart-lung transplantation have moved from an experimental and innovative stage to clinical treatments. The exclusion criteria for selecting potential recipients have become relaxed whilst preservation techniques have improved to allow ischaemic times of donor organs to be extended beyond 4 h. Monitoring of graft function with daily spirometry and use of transbronchial lung biopsy have enabled early diagnosis of lung rejection or infection and thus effective treatment. Lung transplantation and heart-lung transplantation, by their success, are providing new insight into lung disease and pulmonary physiology.
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PMID:Transplantation of the lungs. 269 87

Cor triatriatum presenting in adulthood is extremely rare. We describe a case of adult cor triatriatum in which the diagnosis was initially masked by the concomitant existence of COPD. Cardiac catheterization revealed only slightly elevated pulmonary wedge pressure despite severe pulmonary arterial hypertension. Both the primary lung disease and cor triatriatum greatly accentuated the pulmonary vascular disease which led to a reduction of pulmonary blood flow. Consequently, pulmonary venous obstruction was masked and was not reflected by measuring pulmonary wedge pressure. The diagnosis was made by two-dimensional echocardiography and left ventriculography.
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PMID:Cor triatriatum masked by coexisting COPD in an adult. 276 32


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