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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Since January 1988, the Bordeaux group has performed 15 transplantations for lung disease: 9 heart-lung transplants, 1 heart + left lung, 1 double lung, 2 right lungs and 2 left lungs. The transplantations were performed for pulmonary emphysema (10 cases), pulmonary artery hypertension (2 cases), cystic fibrosis (1 case), pulmonary fibrosis (2 cases). Cardiopulmonary transplantation was not always performed because of associated heart failure but sometimes because of large intrahilar adenopathy or intractable bronchial infection. Pulmonary transplantation is recommended on the right side in cases of pulmonary fibrosis. One patient died postoperatively (ischaemia of the transplant). Four others died during the 2nd and 3rd months from poorly defined but probably infectious pulmonary syndromes. The tracheobronchial patency of the 10 survivors was 80% or 100% of the predicted value. The respiratory functional result was excellent in the short and intermediate term. Specific difficulties essentially consisted of pleural symphyses, hilar adenopathy, bronchial infection, steroid dependence of certain subjects, the difficulty of identifying the cause and treating lung opacities during the 2nd and 3rd months.
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PMID:[Lung and heart-lung transplantation in respiratory tract diseases. Evaluation and development of the indications based on our first 15 cases]. 258 94

Pulmonary hypertension is a relatively common complication of chronic lung disease in children that can cause diminished right ventricular performance (RVP) and, eventually, cor pulmonale and heart failure. Since oxygen may decrease pulmonary artery pressure in these patients, we questioned whether RVP would also improve concomitantly. We evaluated the effect of oxygen on RVP in two young hypoxemic patients by radionuclide angiography. A child with bronchopulmonary dysplasia and cor pulmonale who was not clinically in heart failure had acutely better RVP while breathing oxygen and a further improvement after continuous oxygen therapy for 1 year. In a young adult with cystic fibrosis who was suspected of being in heart failure RVP acutely improved when the FIO2 was increased. We conclude that oxygen may improve RVP in hypoxemic patients and speculate that the observation of such improvement may be valuable for the early detection of patients who can benefit from long-term oxygen therapy.
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PMID:Effect of oxygen on right ventricular performance evaluated by radionuclide angiography in two young patients with chronic lung disease. 303 45

Cardiac transplantation has emerged as a standard mode of clinical therapy for end-stage heart failure, while heart-lung transplantation is still an experimental rather than a standard mode of clinical therapy for end-stage heart and lung disease. Since clinical application of heart-lung transplantation began in March 1981, there has been continual improvement in donor care, surgery, post-operative management, and the diagnosis and treatment of lung and heart rejection. According to our 6-year clinical experience, both patients with Eisenmenger's physiology and patients with primary pulmonary hypertension are considered suitable candidates for heart-lung transplantation. In the future, our recipient population will include small children and patients with cystic fibrosis. Suitable candidates must have good liver and kidney function, and freedom from previous major pulmonary emboli, systemic diseases, and previous thoracic surgery. Patients must maintain their functional and emotional status to pass successfully the difficult postoperative period. Proper donor and recipient selection in connection with improved heart-lung preservation has resulted in excellent operative survival (100%) following the last ten operations, and only the lack of suitable donors can limit the number of heart-lung transplantations in the future.
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PMID:Recipient selection for heart-lung transplantation. 314 9

The clinical course of 13 cystic fibrosis patients with a total of 24 episodes of pneumothorax was analysed. The study is based on 488 (273/215) patients seen over 20 respectively 10 years at the University Children's Hospitals Frankfurt/Main and Essen. A pneumothorax was observed with a frequency of 2.7% mainly in adolescents of young adults with advanced pulmonary disease (mean age 17.4 years). It was not seen before the age of 10 years. Thus among 255 patients at risk above 10 years a pneumothorax occurred in 5.1%. Presenting symptoms were acute chest pain (n = 17), dyspnea (n = 17) and irritating cough (n = 8). In two patients pneumothorax was an incidental diagnosis. A tension pneumothorax was seen in 7 (= 30%; 3 initial, 4 recurrences of which 3 were ipsilateral). Out of 11 recurrences (n = 6, ipsi- and n = 5, contralateral) 4 occurred only once, one twice and in one patient five times. Two patients died as a consequence of the event (one initially due to tension pneumothorax, one due to heart failure). The therapeutic approach was conservative. Without specific treatment pneumothorax resolved in 12 cases. Ten patients were treated by chest tube drainage and only one patient by pleurodesis with a sclerosing agent. Though the therapeutic results were favorable in the patients presented, the authors suggest more aggressive treatment in view of the high ipsilateral recurrence rate. Detailed recommendations are given.
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PMID:[Spontaneous pneumothorax in cystic fibrosis]. 340 26

We describe the cases of two patients of 9 and 15 months of age who presented episodes of congestive heart failure at the age of 9 and 10 months respectively and who were diagnosed of cystic fibrosis. Death occurred during the first year after diagnosis due to heart failure. Necroscopic study showed pancreatic affectation and heart lesions consisting in areas of myocardial fibrosis and necrosis. We discuss the different hypotheses relating pancreatic affectation to myocardial fibrosis.
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PMID:[Myocardial fibrosis in 2 children with cystic fibrosis]. 343 62

Host factors play an important role in the dosing requirements of theophylline. Theophylline metabolism and clearance depend principally on liver cell function rather than on hepatic flow. The effects of acute hypoxemia require more study; however, patients with chronic obstructive pulmonary disease who have chronic hypoxemia appear to have some impairment of clearance. Clearance is variably and sometimes drastically reduced in patients with liver disease and heart failure, and is reduced by some viral infections. It is not impaired by renal failure. Current split-virus vaccine mixtures do not appear to affect clearance. Clearance is increased in patients with cystic fibrosis and hyperthyroidism. The depressed clearance seen in the severely ill patients who require intensive care improves with improvement in the patient's condition, but the individual factors involved have not been identified. An area requiring more study is the effect of pH on the apparent distribution volume for theophylline. In the presence of liver disease, heart failure, or serious illness, caution must be applied in theophylline dosing, with frequent monitoring of serum levels. Stable patients also warrant an initially conservative dose until serum levels are obtained to guide further dose adjustments.
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PMID:Effect of disease states on theophylline elimination. 353 54

Six clinically stable patients with cystic fibrosis (24 to 31 yr of age) and severe pulmonary impairment, right ventricular hypertrophy, and previous right-sided heart failure underwent cardiac catheterization to assess the hemodynamic effects of oxygen (fraction of inspired O2, 0.31, 0.50), phentolamine (5 mg intravenously), hydralazine (0.33 mg/kg intravenously), and nifedipine (20 mg sublingually). Measurements during dynamic exercise were also obtained before and after hydralazine therapy. Studies after 5 to 8 wk of continuous, orally administered hydralazine therapy were performed in 3 patients. The resting mean pulmonary artery pressure was 31 +/- 4 mmHg. At rest, only oxygen was a selective pulmonary vasodilator, decreasing pulmonary artery pressure and pulmonary vascular resistance in all patients. Systemic arterial pressure and resistance were not significantly changed. Phentolamine, hydralazine, and nifedipine did not alter pulmonary artery pressure or selectively affect the pulmonary vascular bed, reducing both calculated pulmonary and systemic vascular resistance, the latter to a similar or greater degree. Hydralazine and nifedipine significantly increased cardiac index and decreased systemic arterial pressure. Nifedipine mildly decreased systemic oxygenation. During exercise, the mean pulmonary artery pressure increased to 51 +/- 15 mmHg. Hydralazine increased systemic and mixed venous oxygenation both at rest and during exercise but did not alter the elevation in pulmonary artery pressure observed during exercise. After orally administered hydralazine therapy, oxygen delivery and cardiac index remained increased in 2 patients. These data support the use of oxygen but not of the other agents in patients with cystic fibrosis and chronic cor pulmonale unless the ability of hydralazine to increase oxygen delivery is determined to improve prognosis.
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PMID:Effect of vasodilators at rest and during exercise in young adults with cystic fibrosis and chronic cor pulmonale. 399 48

A boy suffering from cystic fibrosis (CF) complicated by generalized amyloidosis is reported. His condition was fairly good during the first 10 years of life, but after this time he had repeated pulmonary infections caused by Staphylococcus aureus. From the age of 14, he developed increasing hepato- and splenomegaly and a liver biopsy revealed massive amyloidosis. At the age of 16 he died of respiratory and cardiac failure. Post-mortem examination revealed wide-spread amyloidosis in addition to pulmonary and pancreatic findings characteristic of CF.
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PMID:Generalized amyloidosis in cystic fibrosis. 401 82

Cardiomyopathy in cystic fibrosis (CF) is an unusual heart disease, mainly characterized by a multifocal fibrosis of the left ventricle. The disorder chiefly occurs in the age group of 1-2 years and leads to fatal cardiac failure. The causal pathogenesis of the disease has not been discovered up to now. In two cases of CF-associated cardiomyopathy we found an oedema (mainly lymphoedema) of the myo- and epicardium and a lymph stasis in lymph vessels and lymph nodes of the heart. Based on a comparative study using animal models we speculate that a) CF may be complicated by a disorder of cardiac lymph circulation, and b) chronic cardiac lymphoedema of the heart in CF can cause focal myocardial damage with fibrosis.
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PMID:Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis. 621 64

Cardiac involvement in cystic fibrosis (CF) is characterized by acute cardiac failure in the infant with myocardial fibrosis found on pathological examination. In the older child with chronic pulmonary disease, cor pulmonale tends to predominate the clinical picture. We report the case of an adolescent with CF presenting with ventricular arrhythmia. Cardiac scan, repeated echocardiograms and Holter monitoring were suggestive of an infiltrative process. We propose this case to be similar to those of CF in infants with cardiac involvement.
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PMID:Cardiac involvement in cystic fibrosis revealed by a ventricular arrhythmia. 648 91


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