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

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.
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PMID:Successful treatment of Kasabach-Merritt syndrome with prednisone and epsilon-aminocaproic acid. 831 77

A 4-month-old infant with cardiac hemangioendothelioma presented with thrombocytopenia, and pericardial effusion, as well as signs and symptoms of heart failure. This is the first reported case of infantile cardiac hemangioma successfully treated with steroids.
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PMID:Infantile cardiac hemangioendothelioma. 173 71

The most common benign liver tumors are hemangiomas and hamartomas, both of mesenchymal origin. Mortality for patients with these tumors has traditionally been substantial despite benign histology. Between 1965 and 1989, 22 patients were treated for a benign liver tumor. This represents 42% of all primary neoplasms of the liver observed during this period. Incidental findings of liver tumors at autopsy were excluded from this series. There were 9 boys and 13 girls with a mean age at presentation of 2.3 years (range, birth to 14 years). Sixteen had hemangiomas and presented earlier in life (mean age, 4.4 months). In this subgroup, high-output cardiac failure was present in 58% of the newborns. Seven hemangiomas were resected, four were observed, three were treated with digitalis, diuretics, and steroids, and one received epsilon-aminocaproic acid. Nonhemangiomatous tumors included four hamartomas, one focal nodular hyperplasia, and one nodular transformation. All six were resected. There was one death early in the series. At a mean follow-up of 38 months, 21 of the 22 patients are cured or asymptomatic. In the past, mortality rates of close to 90% have prompted many investigators to advocate resection of every symptomatic hemangioma. With the availability of more sophisticated imaging techniques and refinements in the treatment of cardiac failure, surgery can be used more selectively. Hepatic resections, once considered heroic, can now be performed with minimal morbidity and virtually no mortality. The 96% survival in this series of benign liver tumors contrasts with high mortality rates reported in the literature and illustrates the spectacular improvements that have been made in the diagnosis and management of these once ill-reputed tumors.
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PMID:Benign liver tumors in children: a 25-year experience. 181 68

We report a neonate who presented within hours of birth with severe congenital cardiac failure, thrombocytopenia, and consumption coagulopathy, caused by a massive hemangioma of the left arm. Initial treatment with glucocorticoids, platelet and clotting factor replacements, and cardiovascular support failed to control these hemangioma effects and amputation was avoided only when axillary artery ligation and an intermittent pneumatic compression device, manufactured in this hospital, achieved control of this lesion and hastened its subsequent resolution. The treatment of such lesions is reviewed, emphasizing individualized treatment protocols, and stressing that such lesions and their effects, cannot be regarded as variants of a single disease entity when planning management.
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PMID:Giant hemangioma of the arm associated with cardiac failure and the Kasabach-Merritt syndrome in a neonate. 194 68

A 62-year-old man developed symptoms of heart failure. Echocardiography showed a dense mass arising from the free wall of the right ventricle and occupying about 75% of the right ventricular cavity. Selective coronary angiography demonstrated intense neovascularization of a cardiac neoplasm arising in the distribution of the right coronary artery. Under cardiopulmonary bypass the tumor was resected completely and the defect of the ventricular wall was repaired with Gore-tex (EPTFE) patch (area 7.5 X 5.0 cm). The size of the resected tumor was 10.0 X 9.5 X 3.4 cm and weighed 170 g. Histological examination of the tumor disclosed cavernous hemangioma. The post operative course was uneventful and the patient has remained asymptomatic after the surgery. To our knowledge, only 23 cases of intramyocardial or intracavital hemangioma were reported in the world literature and this may be the first report in Japan.
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PMID:[Intramyocardial hemangioma of the right ventricle with successful resection--report of a case and review of the literature]. 268 49

Two cases are presented in which sinusoidal heart-rate (SHR) patterns were recorded antenatally. In the first case the pattern was associated with severe maternal exertion and prolonged exposure to freezing temperatures. The pattern returned to normal within an hour of maternal recovery. The second case was associated with fetal death due to high-output cardiac failure associated with a large liver haemangioma. A hypothesis is proposed to link the aetiology for this heart-rate pattern which would explain these, and previously reported cases.
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PMID:The aetiology and clinical significance of the sinusoidal fetal heart-rate pattern; two case reports. 275 26

A case of malignant fibrosarcoma originated from pericardium was reported. A 31 year-old female who complained of general fatigue and back pain showed dilated cardiac shadow in chest X-ray. Cardiac blood pool scan with 99mTc-RBC revealed avascular mass in pericardial cavity which push the heart up and left side. It was suspected to be malignant, since the mass had increased 67Ga uptake. CT and MRI also demonstrated that the tissue characterization of the pericardial mass was irregular, and the mass compressed venous return. The large mass originated from pericardium caused the right sided cardiac failure. In 12 years ago, she had a history of operation which resected benign hemangioma in the same space (pericardium). We could suspect the malignant transformation between the two rare pericardial tumors; benign hemangioma and malignant fibrosarcoma.
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PMID:[Pericardial fibrosarcoma demonstrated by Ga-67 scintigraphy]. 279 5

The unusual computed tomographic appearances of a hepatic hemangioma in a neonate are reported. The liver was replaced by cystlike spaces that enhanced with contrast. These blood-filled spaces were responsible for the cardiac failure that improved following ligation of the hepatic artery.
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PMID:Computed tomographic appearances of massive neonatal hemangioma of the liver: a case report. 316 23

Hemangioendothelioma is seldom seen in adults. Its severe evolution is due, not so much to the exceptional transformation into hemangiosarcoma, but mainly to haemorrhagic complications by rupture or consumption coagulopathy and to severe cardiac insuffficiency secondary to arteriovenous shunts. The case reported here concerns a 64 year-old woman presenting pain in the left hypochondrium and splenomegaly. A splenectomy was performed and the histological findings were compatible with the diagnosis of hepato-splenoganglionic hemangioendothelioma. The evolution was unfavorable. The patient died a few months later in a picture of haemorrhagic syndrome and cardiac insufficiency. Histological findings on autopsy specimens indicated a cavernous hemangioma. The treatment of these diffuse hemangiomas is a difficult one. Hepatic artery ligation has been advocated in certain desperate situations. Nevertheless, because of a collateral circulation, recurrences are frequent.
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PMID:[Lymph node-hepatosplenic hemangioma in an adult with consumption coagulopathy and fatal cardiac insufficiency]. 343 34

The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma. The latter may present with intractable cardiac failure as a result of considerable shunting of blood. Acquired liver lesions often present in the newborn period or early infancy and this includes hepatitis and biliary atresia. The difficulties in the differentiation of the two lesions will be discussed together with the management of biliary atresia. As the child grows older, Reyes encephalopathy with microvesicular fat in the liver is not uncommon. The pathophysiology of Reyes encephalopathy as seen locally will be described. The choledochal cyst with direct (Caroli's disease) or indirect effect on the liver will be described. Problems of childhood portal hypertension as well as congenital hepatic fibrosis will be described. Hemosiderosis of the liver is chiefly seen in homozygous beta-thalassaemia patients who have been kept alive with repeated blood transfusions. Amoebic and pyogenic hepatitis, fatty liver due to protein malnutrition, biliary ascariasis, etc, which are common in tropical and subtropical countries are rarely seen now in Singapore children.
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PMID:Paediatric liver disorders in Singapore. 346 38


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