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

At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10-136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly ( P<0.001) from 12.57+/-3.27 l/min pre-treatment to 8.36+/-2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE.
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PMID:Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations. 1531 41

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

The current treatment of splenic injury in children is conservative. The common concern with nonoperative management of splenic injury involves increased risk of posttraumatic splenic complications. Computed tomography angiography is a noninvasive and useful technique for the detection of the vascular abnormalities in conservatively managed splenic injuries. We present a case of posttraumatic splenic pseudoaneurysm and arteriovenous fistula (AVF) in a 12-year-old boy, which were prediagnosed by color Doppler ultrasound and consecutively shown by computed tomography angiography. The treatment of splenic AVF is indicated to avoid portal hypertension, high output cardiac failure, and related complications. To preserve the splenic immune function and to avoid the risk of surgery and splenectomy complications, splenic pseudoaneurysm and AVF were successfully occluded by transcatheter embolization.
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PMID:Posttraumatic splenic pseudoaneurysm and arteriovenous fistula: diagnosis by computed tomography angiography and treatment by transcatheter embolization. 1633 94

Pulmonary arterial hypertension is a serious and severe complication of liver cirrhosis and portal hypertension. We present a case of a 47 year old female who developed pulmonary hypertension and right ventricular heart failure symptoms 6 years from the diagnosis of liver biliary cirrhosis and portal hypertension.
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PMID:[Pulmonary arterial hypertension complicating portal hypertension and liver biliary cirrhosis--a case report]. 1650 74

In hereditary hemorrhagic telangiectasia (HHT), the liver demonstrates a large pattern of findings, including telangiectases, arteriovenous shuntings, focal liver lesions, and ischemic cholangitis, and provides severe complications such as portal hypertension and cardiac failure. Magnetic resonance imaging allows obtaining complete information (parenchyma, biliary tract, and vessels) of the liver in a 1-step examination. Acknowledgment of typical magnetic resonance imaging features of liver involvement in hereditary hemorrhagic telangiectasia case will result in more accurate diagnosis and can guide therapeutic options.
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PMID:Hereditary hemorrhagic telangiectases: magnetic resonance imaging features in liver involvement. 1677 14

Portopulmonary hypertension is characterized by a chronic liver disease associated with a mean pulmonary artery pressure >25 mmHg at rest, an increased pulmonary vascular resistance and a capillary pulmonary pressure <15 mmHg with portal hypertension. Schistosomiasis may be an aetiology of this syndrome, however, few cases have been reported. We describe the first cases of portopulmonary hypertension with schistosomiasis in Malagasy patients. There were 2 men aged of 18 and 20 from hyperendemic area of schistosomiasis in Madagascar Both had a history of repeated water contact. They presented a dyspnea associated with ascites and oedema. Clinical examination showed portal and pulmonary hypertension with right ventricular heart failure. Cardiac examination revealed a systolic murmur and splint of the second heart pulmonary Pulmonary hypertension was confirmed by cardiac ultrasonogaphy Serology of bilharzias was positive. Parasitological examination showed eggs of S. mansoni. The treatment based on salt-free diet, spironolactone and praziquantel led to a better evolution of symptoms (case 1). Symptoms of right heart failure remained for the second patient even though improvement was noted. In tropical countries, schistosomiasis may be one of the cause of portopulmonary hypertension and may appear in early age. Its treatment remains difficult as the drugs recommended are not affordable.
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PMID:[Portopulmonary hypertension due to schistosomiasis in two Malagasy patients]. 1740 90

We report here four cases of pediatric patients in whom the diagnosis of cystic fibrosis was made only after the histological examination of a liver specimen obtained by biopsy (three cases) or at autopsy (one case). There were two boys and two girls, aged 13 months to 7.5 years. None had a personal or familial history suggestive of cystic fibrosis. One patient, presenting with myocardial lesion and hepatomegaly, died of heart failure; at autopsy, the liver showed a typical aspect of focal biliary cirrhosis. In the three other cases, liver disease was the only manifestation of cystic fibrosis at the time of diagnosis. Liver biopsy examination showed focal biliary cirrhosis in one case and massive steatosis in two. In all four cases, the diagnosis was confirmed by the existence of known pathogenic mutations in the CFTR gene. The evolution was variable; one patient had progressive liver disease with severe portal hypertension after 7 years; another one had lung complications after 1 year. In conclusion, our experience recalls that the diagnosis of cystic fibrosis must be considered in children presenting with unexplained liver disease; its confirmation by molecular techniques makes it possible to set up an appropriate follow-up.
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PMID:Unexpected diagnosis of cystic fibrosis at liver biopsy: a report of four pediatric cases. 1755 56

The cardiac hemodynamics of patients awaiting liver transplantation is complex. Coronary atherosclerosis, a hyperdynamic circulatory state and cirrhotic cardiomyopathy are present to a variable degree in this population. In this contribution to the Symposium on Portal Hypertension, we expand on our published experience with coronary angiography and cardiac hemodynamics at the time of evaluation of candidacy for liver transplantation in a cohort of 161 patients. Although we confirmed the relation of systemic hemodynamics with the degree of liver failure, we noted a higher prevalence of high output heart failure, defined as an increased left ventricular end-diastolic pressure in the setting of an elevated cardiac output, most notably in patients classified as Child C. Most patients with high pulmonary artery pressure also exhibited evidence of elevated left ventricle filling pressures. A low systemic vascular resistance, a marker of arterial vasodilatation, was similar in the presence of atherosclerosis, a condition where impaired vasorelaxation occurs as a result of endothelial dysfunction. The high prevalence of coronary artery disease in this series supports the observations that atherosclerosis is a major issue in the current population with cirrhosis awaiting liver transplantation. A lower sensitivity of noninvasive screening tools for the detection of coronary atherosclerosis is likely the result of the interaction of the hyperdynamic circulation with the performance of these tests.
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PMID:Hemodynamic evaluation before liver transplantation: insights into the portal hypertensive syndrome. 1797 84

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by mucocutaneous and visceral telangiectasia. Hepatic involvement with vascular malformations may lead to portal hypertension, biliary ischemia, and high-output cardiac failure. Liver transplantation is indicated for life-threatening disease but carries significant risk from surgery and chronic immunosuppression. We report a case of a 47-year-old woman with HHT successfully treated with the vascular endothelial growth factor (VEGF) antibody bevacizumab. The patient was referred for consideration of liver transplantation because of hepatic HHT leading to high-output cardiac failure, diuretic resistant ascites, cholestasis, and malnutrition. As she was considered a high-risk candidate for transplantation, she underwent 6 courses of bevacizumab (5 mg/kg) over 12 weeks. A dramatic improvement in her clinical state was observed after 3 months with reversal of cholestasis, resolution of cardiac failure and ascites, and improvement in nutritional status with a 10% dry weight increase. Treatment induced a marked reduction in liver vascularity and halving of her liver volume from 4807 to 2269 mL over 6 months. This was associated with normalization of her cardiac output from 10.2 to 5.1 L/minute. Correspondingly, she ceased diuretic medications, returned to full-time work, and was delisted as a transplant candidate. She remains well 6 months after completing treatment. In conclusion, antagonism of VEGF receptors led to a dramatic regression of hepatic vascular malformations and reversal of high-output cardiac failure and complications of portal hypertension in this patient with HHT. Bevacizumab may potentially alleviate the need for liver transplantation in this group of patients.
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PMID:Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telangiectasia. 1897 80


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