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

We describe a case of orthotopic liver transplantation used as a therapeutic method to correct high output cardiac failure related to a liver arteriovenous fistula due to hereditary hemorrhagic telangiectasia. Detailed hemodynamic changes as they occurred during liver transplantation are described.
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PMID:Liver transplantation for hepatic arteriovenous malformation with high-output cardiac failure in hereditary hemorrhagic telangiectasia: hemodynamic study. 1096 Feb 10

A hundred and six clinically diagnosed cases of epidemic dropsy, admitted in June to August 1998 to the P-III unit of RML Hospital and the Department of Pediatrics, Safdarjang Hospital, were studied. All of them consumed mustard oil contaminated with Argemona mexicana, confirmed by ferric chloride and nitric acid tests. No specific sex predilection was seen. No child was affected below the age of 3 years. Pedal edema and reddish hyperpigmentation were the most consistent findings (100 per cent). Frank cardiac failure was seen in only 24 (22.64 per cent), yet persistent tachycardia was alarmingly high (104/106, i.e. 98.4 per cent). Notably ECG showed prolonged Q-T interval in 24 children (22.64 per cent), unrelated to serum Ca2+ level in patients with congestive cardiac failure (CCF). Color Doppler echocardiography showed biventricular dilatation in all the 24 patients with CCF. Wide pulse pressure was recorded in two patients only. Mortality occurred in only two patients (1.89 per cent). Eye involvement was a late finding. All those who survived (i.e. 104/106) recovered completely, except two patients who were left with sarcoid-like changes of skin telangiectasia.
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PMID:A two-centre collaborative study on clinico-epidemiological profile of a recent outbreak of epidemic dropsy in New Delhi (India) with special emphasis on its cardiac manifestations in pediatric patients. 1169 29

Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) is highly variable and may lead to severe clinical symptoms such as heart failure. This controlled, prospective study defined sonographic criteria for hepatic involvement in HHT. Color Doppler sonography and pulsed Doppler sonography were used to study 25 patients with HHT and liver involvement, 20 patients with HHT without liver involvement, 25 patients with cirrhosis, and 25 patients without liver disease. The diagnosis of hepatic manifestation was confirmed by computed tomography and/or angiography. Liver size, parenchymal changes of the liver, vessel diameters, and flow velocities of the portal vein and the hepatic artery were determined. Resistance index (RI) and pulsatility index (PI) were calculated. The diameter of the common hepatic artery was significantly dilated without overlap between HHT patients with liver involvement and the 3 control groups (mean 11.3 +/- 2.8 mm [HHT with liver involvement], 4.6 +/- 0.9 mm [HHT without liver involvement], 4.8 +/- 1.0 mm [cirrhosis], and 4.4 +/- 1.0 mm [healthy controls], P <.001). Doppler parameters of the proper hepatic artery differed significantly (all P <.001). In all patients with HHT and liver involvement, areas with intrahepatic hypervascularization caused by dilated intrahepatic arteries were observed in varying intensity. Cardiac output significantly correlated with the diameter of the common hepatic artery (r = 0.53, P =.007) and the portal vein (r = 0.42, P =.05). In conclusion, the diameter of the common hepatic artery (>7 mm) and intrahepatic hypervascularization are suitable sonographic diagnostic parameters of HHT with high sensitivity and specificity. Dilated diameters of the hepatic feeding vessels are indicators for systemic circulatory distress in these patients.
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PMID:Sonographic criteria for the diagnosis of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT). 1271 95

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

Liver involvement in hereditary hemorrhagic telangiectasia may lead to high-output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we review literature on this subject. Our two patients resumed normal cardiac function after OLT. This procedure appears to be a promising therapy with good long-term results despite dissection difficulties encountered due to the collateral arterial network reorganization.
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PMID:Liver transplantation for cardiac failure in patients with hereditary hemorrhagic telangiectasia. 1597 23

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

We describe six patients with an uncommon variant of infantile hemangioma that we have termed reticular, occurring in the extremity, which were associated with intractable ulceration, anogenito-urinary-sacral anomalies, and sometimes cardiac overload. The extreme end of the spectrum is exemplified by a male neonate who presented with a stained, enlarged, pulsatile lower extremity, and cardiac failure. He also had hepatic hemangiomas and ambiguous genitalia. Progressive soft tissue necrosis and bony destruction necessitated amputation. The histopathologic features differed from those of typical infantile hemangioma: infiltrative (not lobular) and involving fascia, muscle, and bone. The mid-spectrum is illustrated by five females with reticular infantile hemangioma of the lower limb, buttock, and perineum. Four of these infants had a ventral-caudal anomaly, including omphalocele, recto-vaginal fistula, solitary/duplex kidney, imperforate anus, and tethered cord; one infant also had hepatic hemangiomas. Deep ulcerations healed following corticosteroid therapy; one patient required skin graft for closure of a thigh wound. The minor end of the spectrum is exemplified a patchy lesion in the distal limb. The reticular variant of infantile hemangioma can be confused with other vascular anomalies in the limb, such as capillary malformation, cutis marmorata telangiectasia congenita, diffuse arteriovenous malformation (Parkes Weber syndrome) and capillary-lymphatico-venous malformation (Klippel-Trenaunay syndrome). The macular network-like appearance of the tumor and coexisting ventral-caudal structural anomalies is analogous to the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities association in the craniofacial region.
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PMID:Reticular infantile hemangioma of the limb can be associated with ventral-caudal anomalies, refractory ulceration, and cardiac overload. 1784 55

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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