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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Left heart diseases, in particular mitral stenosis, are often associated with anatomic and functional alterations of the lung. According to the pulmonary structures involved they could be named chronic secondary intersticial and vascular lung diseases. Congenital heart diseases with pre- or post-tricuspid shunts are also often associated with anatomic and functional alterations of the lung. This condition also constitutes a chronic secondary vascular lung disease (atrial septal defect) or a chronic primary vascular lung disease ( ventricular septal defect, patent ductus arteriosus). Primary lung diseases (interstitial pulmonary fibrosis, pulmonary emphysema, recurrent pulmonary embolism) are often associated with right ventricular hypertrophy with or without dilation, a condition commonly named chronic cor pulmonale. On the whole the interrelationships between heart and lung diseases are as follows: a) anatomic and functional alterations of the lung due to left heart diseases are mediated through pulmonary venous hypertension; b) anatomic and functional alterations of the lung due to congenital heart diseases are mediated through the increased pulmonary blood flow with or without transmission of the systemic blood pressure to the pulmonary vasculature, and c) anatomic and functional alterations of the right ventricle due to primary or secondary lung diseases are mediated through arterial pulmonary hypertension. In summary, the interrelationships between heart and lung diseases are mainly mediated through the pulmonary venous or pulmonary arterial hypertension.
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PMID:Cardiac and pulmonary diseases. A pathophysiologic interelationship. 113 Sep 7

Patients with idiopathic pulmonary fibrosis (IPF) inevitably experience declines in functional status that are most frequently due to progressive pulmonary fibrosis. However, the cause of the clinical deterioration is often uncertain, and disease progression is difficult to distinguish from disease-associated complications or adverse effects of therapy. In studies of the clinical course of IPF, mortality is most frequently due to respiratory failure (38.7%); other causes of death include heart failure (14.4%), bronchogenic carcinoma (10.4%), ischemic heart disease (9.5%), infection (6.5%), and pulmonary embolism (3.4%). Other, usually nonfatal, disease-associated complications include pneumothorax, corticosteroid-induced metabolic side effects and myopathy, and therapy-related immunosuppression. In evaluating clinical deterioration in patients with IPF, disease-associated complications and adverse effects of therapy should be distinguished from progressive pulmonary fibrosis. The cause of clinical deterioration will alter the therapeutic intervention required and will influence patient prognosis and duration of survival. This article examines the causes of clinical deterioration in patients with IPF and the diagnostic procedures for assessing disease-associated complications and staging IPF progression.
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PMID:Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. 218 1

The collateral pulmonary blood flow was measured by the dye dilution method in four patients with chronic obstructive pulmonary disease, in 16 patients with diffuse pulmonary fibrosis, in 15 patients with pulmonary embolism, and in three patients with primary pulmonary hypertension. The authors found that collateral pulmonary circulation was small in the first two groups (0.3 % and 1.7% of the pulmonary blood flow, respectively), absent in primary pulmonary hypertension, and high (14.0% of pulmonary flow) in pulmonary embolism. The magnitude of the bronchopulmonary collateral circulation did not correlate with degree of precapillary pulmonary hypertension, left atrial pressure and left ventricular enddiastolic pressure. It seems that the bronchopulmonary collateral flow does not produce diastolic left ventricular overload in chronic pneumopathies.
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PMID:Left ventricular overload caused by collateral pulmonary circulation in chronic pneumopathies-myth or reality? 724 59

Obstetrician-gynecologists reviewed patient records of women delivering during January 1986-December 1992 to determine the maternal mortality rate and trends and the causes of maternal deaths in the maternity ward at the National University of Singapore. There were 26,173 deliveries and 9 maternal deaths (a maternal mortality rate of 22.9/100,000). The causes of maternal deaths were pulmonary embolism (underlying condition, systemic lupus erythematosus [SLE]), hemorrhage from multiple sites (thrombotic thrombocytopenia), acute exacerbation of SLE with interstitial pneumonitis, pulmonary fibrosis (systemic sclerosis), fulminant hepatitis (prior hepatitis and liver disease), and cerebral embolism (rheumatic heart disease with mitral valve replacement). There were also three incidental maternal deaths bringing the maternal mortality rate up to 34.4/1000. The incidental causes of death included septicemia from perforated peptic ulcer (uncontrolled thyrotoxicosis), multiple metastases from lung cancer, and suicide (family dispute over adoption of newborn). A cesarean section preceded 4 (44%) of the 9 maternal deaths. Two of these deaths were incidental maternal deaths. Cesarean section was related to two of the remaining six (33%) deaths. These findings show that traditional direct causes of maternal death (hemorrhage, sepsis, embolism, or hypertension) were not responsible for the maternal deaths at this tertiary facility. Instead, the women tended to have medical conditions that placed them at high risk of death regardless of pregnancy status.
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PMID:Maternal mortality: evolving trends. 781 Nov 98

Single-lung transplantation has been successfully performed in patients with pulmonary fibrosis and emphysema. In contrast, patients with end-stage pulmonary hypertension (either primary or secondary to Eisenmenger's syndrome) have conventionally been offered heart-lung transplantation. The rationale underlying this approach is that chronic pulmonary hypertension results in irreversible right ventricular dilatation and failure. Recovery of the right ventricle has previously been reported after thromboendarterectomy for chronic large-vessel pulmonary embolism, correction of atrial septal defect or mitral valve replacement. The evolution of right ventricular morphology and function after lung transplantation has not been previously described. This study examines the reversibility of right ventricle dysfunction following normalization of pulmonary artery pressure after single-lung transplantation in 4 patients with pulmonary hypertension. Cardiac function was assessed using electrocardiography, echocardiography and radionuclide angiography. Pulmonary hemodynamic measurements, including pulmonary artery pressure and pulmonary vascular resistance, decreased in all patients after single-lung transplantation. Electrocardiographic changes observed were leftward shift in the QRS axis, and a decrease in P-wave amplitude and in right ventricular force. Echocardiographic examination revealed decreased right atrial, right ventricular and tricuspid valve annular dimensions, normalization of septal motion, and decreased tricuspid regurgitation. Thus, improved pulmonary hemodynamics after single-lung transplantation for pulmonary vascular disease results in reversal of right heart dilatation and dysfunction, and improved myocardial performance. The extent of right ventricular dysfunction beyond which recovery is unlikely to occur has yet to be determined.
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PMID:Recovery of the right ventricle after single-lung transplantation in pulmonary hypertension. 814 Oct 91

Silicone fluid injection for mammary augmentation is a well-known illegal procedure. It has been associated with many complications, including local tissue granulomatous reaction, chronic infection, and sclerosis, but pulmonary involvement in human beings has been documented in only six cases. We describe three more such cases of pulmonary embolism. These three patients were all female, young, and previously healthy. They received the procedures only for cosmetic reasons. Unfortunately, one of them died and another lived with the sequelae of pulmonary fibrosis. The mortality rate of the nine total patients with pulmonary embolism induced by silicone fluid, including our three cases, is 33%. Because of this high mortality rate and long-term sequelae of pulmonary fibrosis, no silicone fluid injection should be given for cosmetic reasons, especially in mammary augmentation in which a large volume of silicone fluid is more likely to be used, and the early use of corticosteroid therapy may be helpful.
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PMID:Silicone fluid-induced pulmonary embolism. 848 47

The assessment of regional ventilation in human lungs is important for the diagnosis and evaluation of a variety of pulmonary disorders, including pulmonary emphysema, diffuse lung disease (e.g., sarcoidosis, and pulmonary fibrosis), lung cancer, and pulmonary embolism. This article introduces new MR imaging techniques of pulmonary ventilation and perfusion that will provide a framework for assessing regional pulmonary functions of the lung.
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PMID:Ventilation-perfusion MR imaging of the lung. 1038 68

Transthoracic echocardiography (TTE) is frequently performed in patients with suspected acute pulmonary embolism (APE) to search for right ventricular (RV) pressure overload. We prospectively assessed the diagnostic value of a new Doppler echocardiographic sign of APE based on the disturbed RV ejection pattern ("60/60 sign") and compared its diagnostic performances with that of the presence of RV pressure overload, as well as with "McConnell sign" based on RV regional wall motion abnormalities. We assessed 100 consecutive patients with clinical suspicion of APE, including those with previous cardiorespiratory diseases. After TTE, all of the patients underwent reference diagnostic tests for APE. The 60/60 sign required RV acceleration time of <or=60 ms in presence of tricuspid insufficiency pressure gradient <or=60 mm Hg. APE was ultimately confirmed in 67 of 100 patients. In 17 of 67 patients, 60/60 sign correctly suggested APE, whereas 2 false-positive results were noted: in pulmonary fibrosis and in acute respiratory distress syndrome following lung resection. McConnell sign was fully specific but was noted in only 13 of 67 patients. Sensitivity and specificity were 81% and 45% for "RV-pressure overload" signs, 25% and 94% for 60/60 sign, and 19% and 100% for McConnell sign. When combined, the 2 latter signs were 94% specific and 36% sensitive in diagnosing APE. Thus, RV overload at echocardiography is not specific for APE. The 60/60 and McConnell signs are insensitive, but are reliable and helpful in bedside diagnosis of APE when direct visualization of the pulmonary arteries is impossible. Combining these 2 signs may increase the sensitivity without compromising the specificity of echocardiographic diagnosis of APE.
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PMID:Disturbed right ventricular ejection pattern as a new Doppler echocardiographic sign of acute pulmonary embolism. 1220 11

This case illustrates the reopening of foramen ovale in a young patient with chronic pulmonary hypertension caused by bronchiectasis and chronic pulmonary fibrosis, which resulted in a prominent right-to-left shunt and severe hypoxia. Her clinically unsuspected right-to-left shunt was discovered during ventilation-perfusion scan, which was performed for the evaluation of pulmonary embolism. She had common variable immune deficiency, a primary immunodeficiency disease in which B-lymphocytes produce few or no antibodies. Most patients with this syndrome have an intrinsic defect in their B-lymphocytes that results in reduced immunoglobulin production. In these patients, recurrent respiratory tract infections are common and may result in chronic lung disease, fibrosis, particularly bronchiectasis (20-30%) and even cor pulmonale as happened in our patient [J. Clin. Immunol. 9 (1989) 22-33.].
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PMID:Lung scan in the diagnosis and management of patent foramen ovale pulmonary embolism, paradoxical embolism. 1221 71

Idiopathic pulmonary fibrosis (IPF) is a chronic fibrosing lung disease limited to the lungs and associated with the histologic appearance of usual interstitial pneumonia (UIP) on surgical lung biopsy. The estimated prevalence in the United States is between 35,000 and 55,000 cases,and evidence suggests that the prevalence is increasing for IPF. Risk factors associated with pulmonary fibrosis include smoking, environmental exposures, gastroesophageal reflux dis-ease, commonly prescribed drugs, diabetes mellitus, infectious agents, and genetic factors. The diagnosis requires a careful history and physical examination, characteristic physiological and radiological studies, and, in some cases, a surgical lung biopsy. The natural history of IPF is not known, but evidence supports the concept of a continuum of idiopathic interstitial pneumonias that may overlap in time. Most patients with IPF succumb to respiratory failure, cardiovascular disease, lung cancer, pulmonary embolism, infection, and other health problems. The median survival time for patients with IPF is less than 3 yr. Factors that predict poor outcome include older age, male gender, severe dyspnea, history of cigarette smoking, severe loss of lung function, appearance and severity of fibrosis on radiological studies, lack of response to therapy,and prominent fibroblastic foci on histopathologic evaluation. Conventional therapy (corticosteroids, azathioprine, cyclophosphamide) provides only marginal benefit. Lung transplantation should be considered for patients with IPF refractory to medical therapy. In light of the poor prognosis and lack of response to available anti-inflammatory therapy, alternative approaches to therapy are being pursued. Emerging strategies to treat patients with IPF include agents that inhibit epithelial injury or enhance repair, anti-cytokine approaches, agents that inhibit fibroblast proliferation or induce fibroblast apoptosis, and other novel approaches.
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PMID:Pulmonary fibrosis. 1613 Feb 30


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