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

Since January 1990, we have performed 29 isolated lung transplantations in 28 patients with end-stage lung disease (12 single, 16 bilateral). Recipient diagnoses were: cystic fibrosis (11), chronic obstructive pulmonary disease (6), pulmonary fibrosis (6), eosinophilic granulomatosis (1), postinfectious lung disease (1), adult respiratory distress syndrome (1), and primary pulmonary hypertension (2). There have been four deaths, two in patients with pulmonary fibrosis and two in patients with primary pulmonary hypertension. Four patients have undergone transplantation while on ventilatory support for respiratory failure (2 with cystic fibrosis, 1 having redo lung transplantation with cystic fibrosis, and 1 with adult respiratory distress syndrome); all of these have survived. Six patients required cardiopulmonary bypass, which was associated with increased transfusion requirement. All patients 2 months after discharge have returned to an active life-style, except for 2 patients who currently await retransplantation. Preoperative pulmonary rehabilitation has resulted in significant improvement in exercise performance in all patients. Immunosuppression consists of cyclosporine, azathioprine, and antilymphoblast globulin (University of Minnesota), withholding systemic steroids in the early postoperative period. We have employed bronchial omentopexy in all but four transplants; there has been one partial bronchial dehiscence, two instances of bronchomalacia requiring internal stenting, and one airway stenosis. Cytomegalovirus disease has been seen frequently (15 cases), but has responded well to treatment with ganciclovir. Other complication shave included one drug-related prolonged postoperative ventilation, thrombosis of a left lung after bilateral lung transplantation requiring retransplantation, five episodes of unilateral phrenic nerve palsy after bilateral lung transplantation (4 resolved), and the requirement of massive transfusion (greater than 10 units) in 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Isolated lung transplantation for end-stage lung disease: a viable therapy. 155 66

Single lung transplantation remains limited by a severe shortage of suitable donor lungs. Potential lung donors are often deemed unsuitable because accepted criteria (both lungs clear on the chest roentgenogram, arterial oxygen tension greater than 300 mm Hg with an inspired oxygen fraction of 1.0, a positive end-expiratory pressure of 5 cm H2O, and no purulent secretions) do not distinguish between unilateral and bilateral pulmonary disease. Many adequate single lung grafts may be discarded as a result of contralateral aspiration or pulmonary trauma. We have recently used intraoperative unilateral ventilation and perfusion to assess single lung function in potential donors with contralateral lung disease. In the 11-month period ending October 1, 1990, we performed 18 single lung transplants. In four of these cases (22%), the donor chest roentgenogram or bronchoscopic examination demonstrated significant unilateral lung injury. Donor arterial oxygen tension, (inspired oxygen fraction 1.0; positive end-expiratory pressure 5 cm H2O) was below the accepted level in each case (246 +/- 47 mm Hg, mean +/- standard deviation). Through the sternotomy used for multiple organ harvest, the pulmonary artery to the injured lung was clamped. A double-lumen endotracheal tube or endobronchial balloon occlusion catheter was used to permit ventilation of the uninjured lung alone. A second measurement of arterial oxygen tension (inspired oxygen fraction 1.0; positive end-expiratory pressure 5 cm H2O) revealed excellent unilateral lung function in all four cases (499.5 +/- 43 mm Hg; p less than 0.0004). These single lung grafts (three right, one left) were transplanted uneventfully into four recipients (three with pulmonary fibrosis and one with primary pulmonary hypertension). Lung function early after transplantation was adequate in all patients. Two patients were extubated within 24 hours. There were two late deaths, one caused by rejection and Aspergillus infection and the other caused by cytomegalovirus 6 months after transplantation. Two patients are alive and doing well. We conclude that assessment of unilateral lung function in potential lung donors is indicated in selected cases, may be quickly and easily performed, and may significantly increase the availability of single lung grafts.
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PMID:Unilateral donor lung dysfunction does not preclude successful contralateral single lung transplantation. 156 54

The techniques and indications for lung transplantation have evolved significantly in the past 6 years. We initially restricted single-lung transplantation to patients with pulmonary fibrosis and developed the double-lung transplantation procedure for patients with emphysema or cystic fibrosis. However, with the double-lung procedure, a 25% mortality rate resulted from ischemic complications at the tracheal anastomosis. The technique was altered to incorporate bilateral bronchial anastomosis, with a resulting reduction in airway complications. The double-lung transplantation procedure continued to have significant drawbacks, including intraoperative and postoperative hemorrhage, and cardiac complications due to prolonged cardiopulmonary bypass, ischemic cardiac arrest, and extensive manipulation of the heart. These problems recently have been addressed with a much simplified procedure incorporating a bilateral transverse thoracosternotomy, replacement of the right lung without cardiopulmonary bypass, and replacement of the left lung with or without a short period of partial bypass. This procedure has been successfully used for emphysema and for cystic fibrosis. The technique of single-lung transplantation also has been simplified and indications have been expanded to include selected patients with emphysema and with primary pulmonary hypertension. With single-lung transplantation, each of the lungs from a donor has been successfully used for a separate recipient on several occasions, improving the supply of available donor organs.
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PMID:The evolution of techniques and indications for lung transplantation. 220 16

Lung transplants have been attempted since 1963 but with little success. After fundamental work by the Toronto group with improved surgical techniques and after the introduction of cyclosporin that group was able to present convincing results. Of 16 unilaterally transplanted patients with pulmonary fibrosis in the final stage, 10 are still living, one of them for now 5 years. With bilateral lung transplantation the indication was extended to cover further pulmonary diseases in the final stage, such as emphysema, bronchiectases, eosinophilic granuloma, primary pulmonary hypertension and bronchiolitis obliterans. The unilateral lung transplantation performed by us failed after initially excellent functioning, on the 9th postoperative day because of a Pseudomonas infection that had been transferred with the donor organ.
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PMID:[Unilateral lung transplantation--an initial report of experiences]. 236 95

Twenty-three patients with pulmonary hypertension underwent right cardiac catheterization and measurement of their response to a short-term infusion of prostacyclin. Pulmonary vasodilation with a greater than 20 percent fall in PVR occurred in all five patients with primary pulmonary hypertension where the predominant lesions were plexogenic; in three out of five patients where the predominant lesions were thrombotic; in three out of five patients with pulmonary hypertension and obstruction of proximal vessels; in three out of five patients with COPD; in both patients with pulmonary fibrosis due to sarcoidosis; and in the one patient with pulmonary veno-occlusive disease, where the pulmonary vasodilation was offset by a fall in arterial oxygen content. Prostacyclin is a safe and effective drug to use for testing the response of the pulmonary circulation to a vasodilator in pulmonary hypertension due to different causes. Short-term testing for a vasodilator response, with a view to instituting long-term therapy, should not be restricted to those patients with primary pulmonary hypertension due to plexogenic pulmonary arteriopathy.
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PMID:Pulmonary vasodilation with prostacyclin in primary and secondary pulmonary hypertension. 250 32

We have achieved repeated success with unilateral lung transplantation for pulmonary fibrosis and have developed an en bloc, double-lung transplant procedure for patients with advanced lung disease of an obstructive or infective nature. Six such procedures have now been performed for end-stage emphysema, and all recipients are alive and well 5 to 15 months later. A seventh transplant for primary pulmonary hypertension was unsuccessful. All recipients were judged to have a life expectancy of 12 to 18 months on the basis of the degree of disability and the documented rate of disease progression. We feel the double-lung procedure is more appropriate than the combined heart-lung transplant for patients requiring replacement of both lungs when right heart function is adequate or deemed recoverable. With this procedure, the recipient is able to retain his or her own heart, avoiding the liabilities associated with cardiac transplantation. Furthermore, the donor heart is available for a separate recipient, and this sharing of the heart and lungs greatly increases the supply of transplantable lungs for patients with end-stage lung disease. Ischemia of the donor airway has been a source of complication, including the one death to date, but this appears to be a surmountable problem.
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PMID:Double-lung transplant for advanced chronic obstructive lung disease. 264 98

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

Lung transplantation nowadays has become a therapeutic modality in the treatment of patients with a variety of end-stage lung diseases. Between July 1991 and December 1992, twelve patients received an isolated lung transplant (eight single lungs and four double lungs) at the University Hospitals of Leuven. The indication for transplantation was emphysema in five patients, pulmonary fibrosis in three, cystic fibrosis in three and primary pulmonary hypertension in one. There were four early, in-hospital deaths (30%): two from sepsis and multi-organ failure, one from anoxia following a bronchial dehiscence and another patient exsanguinated following stent insertion for a partial bronchial dehiscence. Three more patients have died during follow-up: two from chronic respiratory failure secondary to the development of obliterative bronchiolitis (one at 8 months and one at 17 months), and one from a late bronchovascular fistula 4 months following transplantation. The overall actuarial one and two year-survival was 50.0% and 41.6% respectively. All patients discharged from hospital were oxygen free with an improved lung function and exercise capacity. We conclude that lung transplantation is a viable therapeutic option for selected patients with end-stage, irreversible lung disease. In our experience, the bronchial anastomosis remains an important keystone in the early success. Lung transplantation provides a good quality of life in patients free from infection and rejection. Nevertheless, chronic rejection resulting in obliterative bronchiolitis is a major problem in long-term survivors.
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PMID:Isolated lung transplantation; initial experience at the University Hospitals Leuven. Leuven Lung Transplant Group. 797 65

Ischemia of the donor airway remains a significant cause of morbidity after single-lung transplantation; serious manifestations may occur early (anastomotic dehiscence) or late (stricture). Direct, immediate revascularization of the donor bronchial arteries, using the recipient internal thoracic artery, was performed in 10 consecutive recipients of single-lung transplants for whom we procured the organs. Mean recipient age was 52.6 years (range, 43 to 59 years); 6 were male and 4 female. Recipient diagnoses were emphysema (6), obliterative bronchiolitis (2), pulmonary fibrosis (1), and primary pulmonary hypertension (1). Bronchial artery revascularization initially prolonged the ischemic time by only 15 to 20 minutes; this improved with experience. There was one early death and two late deaths in the series. Internal thoracic arteriography was performed 7 to 10 days postoperatively in all 9 surviving patients. There was excellent perfusion of the donor bronchial arteries in 7 of these 9 patients. Bronchoscopy was performed when clinically indicated. No patient had early or late airway healing complications at a median follow-up of 13 months (range, 6 to 16 months). We conclude that direct, immediate bronchial artery revascularization is feasible on a routine basis for single-lung transplantation, and airway healing has been excellent.
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PMID:Routine immediate direct bronchial artery revascularization for single-lung transplantation. 801 Jul 87

This paper reviews the effects of pulmonary artery hypertension on gas exchange by exploring three different issues, namely: 1) how does gas exchange behave in diseases characterized by increased vascular tone (primary pulmonary hypertension (PPH), chronic obstructive pulmonary disease (COPD) and interstitial pulmonary fibrosis (IPF)) or decreased vascular tone ("hepatopulmonary syndrome"); 2) how does exercise, as a non-pharmacological tool of increasing pulmonary blood flow, modify gas exchange in these diseases; and 3) how do several drugs that lower (vasodilators) or increase (almitrine) the active component of pulmonary hypertension interact with gas exchange. Available data show that: 1) in PPH a high pulmonary vascular tone enhances gas exchange and when it is lowered, either by oxygen or vasodilators, ventilation perfusion (VA/Q) distributions deteriorate; 2) in COPD a lowered (vasodilators) or augmented (almitrine) active vascular tone is almost invariably paralleled by a deterioration or enhancement of ventilation-perfusion matching, respectively; 3) in IPF an adequate active response of the pulmonary vasculature is essential to maintain gas exchange, both at rest and during exercise; and 4) in patients with liver cirrhosis a low pulmonary vascular tone induces an abnormal VA/Q distribution. In summary, these data show that any situation and/or therapeutic intervention that lowers the active vascular tone deteriorates VA/Q relationships and vice versa. The final effect of pulmonary vascular tone on arterial oxygen tension (PaO2) is less predictable. The reason for this uncertainty is that the actual PaO2 value depends on the interplay of the intra- and extrapulmonary factors that control gas exchange in humans, and not only on the degree of VA/Q mismatching.
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PMID:Effect of pulmonary hypertension on gas exchange. 828 57


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