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

Cerebral arteriovenous malformations with neonatal manifestations are infrequent and virtually always fatal. Heart failure with an intracranial bruit is the most common presentation. Exceptionally, the aneurysm is a manifestation of Rendu-Osler-Weber syndrome which is inherited on an autosomal dominant basis. Development of cerebral arteriovenous malformations occurs very early as demonstrated by the discovery of two aneurysms with major repercussions on the cerebral parenchyma in a female with severe prematurity. Pregnant women with suspected Rendu-Osler-Weber syndrome should undergo ultrasound studies targeted at identifying untreatable cerebral lesions antenatally.
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PMID:[Cerebral arteriovenous malformations in a probable familial form of Rendu-Osler disease]. 161 47

A 54 year-old man, who had a hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease; O-W-R) accompanied by pulmonary arteriovenous fistulas (PAVFs) and congestive heart failure, developed seizure, right hemiparesis and dysphasia. A brain CT scan revealed a cystic lesion with perifocal edema in left frontoparietal lobe. A contrast enhanced CT scan showed a ring-like enhancement. Dynamic CT scans disclosed that the ring in the cortical side was enhanced more thickly than that in the ventricular side. Considering the severity of the cardio-pulmonary condition, and the deep location of the abscess, we performed an echo-guided aspiration and drainage of the abscess under local anesthesia. No bacteria were demonstrated in the culture of the contents of the abscess. After the surgery, the right hemiparesis and dysphasia were much improved and a CT scan showed the marked reduction of the abscess. However, around eight days after the surgery, the patient showed severe pleural effusion due to progressive heart failure and died on the 11th postoperative day. Autopsy disclosed a shrunken brain abscess, multiple cerebral infarction, multiple PAVFs and severe constrictive pericarditis which was regarded as the cause of death in the patient. In this report, we presented the therapeutic advantage of echo-guided surgery for the treatment of brain abscess in a high-risk patient. We also discussed the mechanism of the formation of brain abscess in patients of O-W-R disease by reviewing published cases.
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PMID:[A case of Osler-Weber-Rendu disease with brain abscess; the mechanism of the formation of brain abscess and its treatment in Osler-Weber-Rendu disease]. 194 83

We present two cases of hemorrhagic hereditary telangiectasia (HHT) or Osler-Weber-Rendu disease which started as heart failure. Both had multiple arteriovenous fistulas, one of them in a rare localization in this disease, a lower extremity. We conclude stressing the importance of thinking of HHT in the presence of obscure heart failure or arteriovenous fistulas and of looking for internal fistulas when faced by a HHT.
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PMID:High out-put congestive heart failure as first manifestation of Osler-Weber-Rendu disease. 648 18

Heart failure occurred in a 50-year-old woman as a result of calcified haemangiomatosis of the liver with a high shunt volume. In the subsequent years there were several bleedings from peptic ulcers. Ultrasonography revealed an increase in liver size and the previously diagnosed calcification. 14 years later the patient was again hospitalized because of increasing weakness and stress dyspnoea; the haemoglobin level was 5.5 g/dl. In addition to the florid gastric and duodenal ulcers, angiodysplasias were for the first time demonstrated in the stomach, duodenum and sigmoid colon, as were telangiectasias in the face and echo-dense round foci in the spleen. After transfusion of red cell concentrates and healing of the peptic ulcers under a 14-day treatment with omeprazole (20 mg two times daily by mouth) and amoxycillin (750 mg three times daily by mouth) the haemoglobin level increased at first (10 g/dl), then tarry stools were once again noted. The condition stabilized after laser coagulation of all accessible gastrointestinal angiodysplasias. The isolated calcified hepatic haemangiomatosis, diagnosed 14 years previously, is most likely a rare variant of hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease).
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PMID:[Liver hemangiomatosis in Osler's disease]. 833 48

Hereditary hemorrhagic teleangiectasia, or M. Osler (Osler-Weber-Rendu disease), is an autosomal dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal varices grade IV. The patient underwent liver transplantation and post-transplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, however, is recommended only for patients with normal liver function and carries a considerably risk of bile duct necrosis.
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PMID:Effective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation. 970 2

A 55-year-old Japanese housewife, who had Osler-Weber-Rendu disease, was admitted to our hospital because of frequent epistaxis and worsening exertional dyspnea. The computed tomography and hepatic arteriography revealed large hepatic arteriovenous malformation, which was considered to be the leading cause of her high output heart failure. Two series of hepatic arterial coil embolization procedures were performed to reduce hepatic shunt flow. They temporarily improved her cardiac condition, but gradually induced progressive hepatic failure due to intrahepatic cholangitis. Hepatic dysfunction restricted her quality of life and lead to a fatal clinical course one year after the second coil embolization.
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PMID:Peripheral arterial coil embolization for hepatic arteriovenous malformation in Osler-Weber-Rendu disease; useful for controlling high output heart failure, but harmful to the liver. 1093 46

Hereditary hemorrhagic telangiectasia, commonly known as Osler-Weber-Rendu disease, is a systemic autosomal dominant inherited disorder, that occurs in Caucasian populations. We report the case of a 56-year-old housewife who was admitted to the gastrointestinal and cardiovascular ward because she had suffered from recurrent gastrointestinal bleeding and heart failure from 1994 to 1997. Panendoscopy showed vascular ectasia scattered over the tongue, larynx, esophagus, and posterior wall of the gastric body. Colonoscopy showed clusters of telangiectasia over the cecum and ascending colon. Arteriovenous malformations (AVMs) were found in the liver and lungs on computerized tomography. Recurrent gastrointestinal bleeding was controlled by estrogen treatment during the follow-up period. In July 1997, the patient was readmitted to our cardiovascular section due to aggravated dyspnea, orthopnea and bilateral lower leg edema. Cardiac catheterization showed a large fistula from the left pulmonary artery to the left atrium and left ventricle, pulmonary arterial pressure of 37/13 mmHg and cardiac output of 9.61/minute. Other studies excluded the possibility of sepsis, and high-output cardiac failure was suspected. The patient was discharged in a stable condition and scheduled for AVM embolization management. Unfortunately, she died of a suspected heart attack at home two weeks following discharge.
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PMID:Recurrent gastrointestinal bleeding and high output cardiac failure caused by hereditary hemorrhagic telangiectasia. 1082 Sep 15

A 62-year-old female with Osler's disease was admitted to our hospital because of fever and cardiac failure. The patient had undergone a mitral valve replacement (MVR) using a Carpentier-Edwards prosthetic valve 14 years earlier. A bacterial examination of arterial blood identified Streptococcus mitis. No arteriovenous malformations were detected in visceral organs. The patient underwent MVR using the same prosthetic xenograft after conservative treatment and management of repetitive epistaxis and decayed teeth. Intra- and postoperative bleeding were typical of a mitral valve reoperation. This is the first reported experience, to the best of our knowledge, of a second MVR in a patient with Osler's disease.
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PMID:A second mitral valve replacement in a patient with hereditary hemorrhagic telangiectasia (Osler's disease). 1555 31

Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.
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PMID:The biological "scrabble" of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery. 1568 49

Hereditary haemorrhagic telangiectasia is a genetic disease characterised by the presence of teleangiectases virtually involving every organ. Hepatic involvement is represented by a spectrum of vascular abnormalities, which evolve in a continuum from tiny teleangiectases to substantial vascular malformations, potentially with a progressively greater arteriovenous shunt. Liver involvement in hereditary haemorrhagic telangiectasia is almost always asymptomatic; on the other hand, hepatic vascular malformations can induce severe complications, depending on the predominant venous side of the arteriovenous fistulas-high-output cardiac failure in the case of hepatohepatic fistulas, and portal hypertension in the case of hepatoportal fistulas. Doppler sonography can detect and stage hepatic vascular malformations in subjects with hereditary haemorrhagic telangiectasia; according to Doppler sonographic grading, appropriate advice for follow-up and/or therapy can be given.
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PMID:Liver involvement in hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber disease. 1592 56


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