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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary familial telangiectasis is a vascular dysplasia characterized by a triple syndrome: hereditary character, telangiectasias and hemorrhages. Its association with systemic and/or pulmonary arterio-venous fistulae is frequent and can condition several complications: cerebral abscess, digestive hemorrhages, epistaxis, hemoptysis, hypoxemia, polyglobulia, and rarely the appearance of hemothorax. We describe a patient who was admitted in hospital with a hypovolemic shock secondary to a massive hemothorax; during emergency surgery, which was necessary to perform, a pulmonary A-V fistula was observed in the middle lobe. The studies afterwards performed demonstrated that it was a Rendu-Osler-Weber disease with duodenal, hepatic, and cerebral involvement.
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PMID:[Massive hemothorax: an exceptional complication of Rendu-Osler-Weber disease]. 178 May 31

This report describes our 17-year experience with intracardiac repair in 200 patients older than 14 years with tetralogy of Fallot. Congestive failure, hemoptysis, and cerebral abscess were present in 15, 10, and 3% of patients, respectively. Prior palliative shunts had been performed in 24.5% only. Polycythemia with a hematocrit greater than 60% was noted in 100 patients but was not considered an incremental risk factor (p greater than 0.05). A transannular gusset was utilized in 74% of patients in the last 5 years of the study. Hospital mortality was 1.3% in the last 5 years. Ninety-seven percent of survivors at follow-up are asymptomatic and leading an active life. Recatheterization data from 86 patients revealed excellent or good results in 88%. The incidence of residual ventricular defect was 1% overall, with a zero incidence in the last 12 years. On the basis of this review, we consider that easier and hemodynamically satisfactory repair has been achieved in the adolescent and older patient compared with the child. The ultimate longevity, however, must await the results of long-term functional and serial hemodynamic evaluation.
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PMID:The clinical profile and surgical treatment of tetralogy of Fallot in the adult: results of repair in 200 patients. 370 44

Pulmonary arteriovenous fistula (PAVF) is a rare vascular malformation of the lung that may lead to cyanosis, epistaxis, hemoptysis, and neurological deficits or cerebral abscess. The purpose of this study is to assess the effectiveness of percutaneous transcatheter embolization of PAVF in pediatric patients. Transcatheter embolization of PAVF using spring coils was performed in three patients (two males and one female) who presented between 1989 and 1999. The age at presentation ranged from 8 months to 3 years (mean 19.6 months). All patients had cyanosis and clubbing. Neurological, dermatological, or other cardiac manifestations were absent. The arterial oxygen saturations at presentation ranged from 60 to 72% (mean 64%). During eight procedures, multiple coils (total of 41 coils, average 14 coils per patient) were delivered to occlude the fistulas successfully. There was complete occlusion of the fistulas in all patients after the multiple interventions. The aortic saturations increased from a mean of 66% to a mean of 95%. Chest radiographs demonstrated dramatic regression of the pulmonary shadows in all three patients. There were no complications encountered during the procedures or during follow-up. Transcatheter coil embolization of PAVF is a safe and effective method of treatment in the pediatric age group. Patients may require multiple procedures to completely occlude the fistulas. Long-term follow-up is essential to ensure absence of recurrence due to recanalization.
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PMID:Transcatheter coil closure of pulmonary arteriovenous malformations in children. 1500 67

Eisenmenger syndrome is the most common consequence of congenital cyanotic heart disease seen in adults; survival to the fifth decade of life is rare. Death is very difficult to predict: it is related to sudden cardiac ventricular arrhythmia, massive hemoptysis and right heart failure. In this paper, a patient with ventricular septal defect and Eisenmenger reaction is described. The patient was relatively well until 48 years of age, when she underwent surgery because of a cerebral abscess without cerebral complications but with some deterioration of her cardiac function. After discharge, the patient was readmitted to the hospital because the electrocardiogram showed persistent ST inferior elevation. Echocardiography demonstrated poor contractility and inferior akinesia. Sudden ventricular tachycardia occurred and the patient became unconscious. She was successfully resuscitated and, following a period of ventilation, the hemodynamics stabilized and she was discharged 17 days later. She remained well two years later.
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PMID:Eisenmenger syndrome: a case of survival after ventricular tachycardia due to inferior myocardial infarction in a 48-year-old patient with congenital large ventricular septal defect. 1522 66

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malformations (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15-33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10-19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5-19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.
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PMID:Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). 1764 82

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is an autosomic dominant disorder, which is characterized by the development of multiple arteriovenous malformations in either the skin, mucous membranes, and/or visceral organs. Pulmonary arteriovenous malformations (PAVMs) may either rupture, and lead to life-threatening hemoptysis/hemothorax or be responsible for a right-to-left shunting leading to paradoxical embolism, causing stroke or cerebral abscess. PAVMs patients should systematically be screened as the spontaneous complication rate is high, by reaching almost 50%. Neurological complications rate is considerably higher in patients presenting with diffuse pulmonary involvement. PAVM diagnosis is mainly based upon transthoracic contrast echocardiography and CT scanner examination. The latter also allows the planification of treatments to adopt, which consists of percutaneous embolization, having replaced surgery in most of the cases. The anchor technique consists of percutaneous coil embolization of the afferent pulmonary arteries of the PAVM, by firstly placing a coil into a small afferent arterial branch closely upstream the PAVM. Enhanced contrast CT scanner is the key follow-up examination that depicts the PAVM enlargement, indicating the various mechanisms of PAVM reperfusion. When performed by experienced operators as the prime treatment, percutaneous embolization of PAVMs, is a safe, efficient and sustained therapy in the great majority of HHT patients.
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PMID:Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. 2376 87