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

We reviewed clinical data, autopsy reports, and microscopic slides on 10 patients with sleep apnea/obesity hypoventilation syndrome (SA/OHS) to define the cardiopulmonary pathological features and establish clinicopathologic correlations. Ten obese (>136 kg) patients without SA/OHS were studied as controls. Patients with SA/OHS exhibited biventricular cardiac failure and pulmonary hypertension with a higher prevalence of moderate/severe pulmonary hemosiderosis (8 v 0 patients), alveolar hemorrhage (7 v 4 patients), capillary proliferation (4 v 0 patients), iron encrustation of elastica (1 v 0 patients) and medial hypertrophy of muscular pulmonary arteries (11.9 +/- 2.4 v 9.7 +/- 1.6%) (P < .05). In two patients capillary proliferation resembled capillary hemangiomatosis. Mean right ventricular thickness was higher in the SA/OHS group (0.71 +/- 0.17 v 0.42 +/- 0.1 cm) (P < .01). Four patients with SA/OHS and three controls had moderate/severe myocardial fibrosis. Biventricular cardiac failure caused death in seven patients with SA/OHS. Hypoxia is probably the most important cause of pulmonary hypertension, arterial muscularization, and right ventricular hypertrophy in SA/ OHS. Left ventricular failure in some SA/OHS patients may be the result of hypertensive cardiac disease. In others, the etiology of left ventricular failure was not determined morphologically, suggesting functional abnormalities related to obesity and/or apneic episodes.
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PMID:Cardiopulmonary pathology in patients with sleep apnea/obesity hypoventilation syndrome. 938 47

A 34-year-old man with an 18-year history of hypertrophic cardiomyopathy died of worsening right-sided heart failure. Central venous pressure was greatly increased to 25 cm H2O before death. Postmortem examination revealed features of severe congestive vasculopathy, including those of pulmonary capillary hemangiomatosis in the lungs. Marked proliferation of capillaries was seen chiefly in alveolar septa and extending into pulmonary veins and arteries, causing severe luminal occlusion with recanalization. Diffusely distributed intra-alveolar edema and hemorrhage with collections of hemosiderin-laden macrophages were also seen, which suggested that the pulmonary capillary hemangiomatosis was associated with longstanding chronic passive congestion of the lung. It is possible that severe pulmonary passive congestion may be one of the causes of development of idiopathic pulmonary capillary hemangiomatosis.
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PMID:Pulmonary capillary hemangiomatosis: a unique feature of congestive vasculopathy associated with hypertrophic cardiomyopathy. 944 26

An unusual autopsy case of systemic hemangiomatosis associated with honeycomb-like liver and splenic sarcomatoid changes is presented. The patient suffered from high-output ventricular heart failure secondary to hepatic arteriovenous shunting with hepatic failure, ending fatally due to disseminated intravascular coagulation and pulmonary bleeding. Postmortem examination revealed the characteristic distribution of vasoformative tumors presenting in the hematopoietic system including the liver, bone marrow, and spleen, with the liver appearing characteristically honeycomb-like. While these vasoformative tumors were mostly benign-appearing, partial shifts toward sarcomatoid change were observed in the splenic lesions.
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PMID:An autopsy case of systemic hemangiomatosis with honeycomb-like liver and focal splenic sarcomatoid changes. 984 Jul 6

A female neonate with diffuse hemangiomatosis and hepatic hemangiomas had cardiac insufficiency develop and had failure to thrive. Her disease was unresponsive to prednisone. She received two courses of cyclophosphamide 10 days apart and a final course 2.5 weeks later. Twelve days after the second course of cyclophosphamide, her liver was significantly smaller. She is now 6-years-old, well-developed, and has no signs of hepatomegaly, malnourishment, or heart failure. Cyclophosphamide appears to be a safe, effective, and rapid treatment of life-threatening hemangiomas of infancy.
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PMID:Successful treatment with cyclophosphamide of life-threatening diffuse hemangiomatosis involving the liver. 1113 22

With a cavernous hemangioma the vascular space does not form an anastomosis, and the blood flow is slow. In contrast, several atypical hemangiomas, including those with shunt formation, have been recently recognized. We report here two extreme cases of atypical hemangioma with severe clinical symptoms due to shunts. In the first case, hemangiomatosis of the entire liver with arteriovenous shunt was treated by embolization of the hepatic artery with as many as 82 steel coils to control high-output heart failure. Although the effect of the treatment was late, the patient is free from heart failure despite a remnant arteriovenous shunt 12 years after treatment. In the second case, an atypical hemangioma in the posterior segment with arterioportal and portosystemic shunt was treated by ligation of the right hepatic artery and the portal vein branch. The patient died of massive bleeding from a deep duodenal ulcer that penetrated the hepatic artery 6 years after treatment. The second case should have been curatively treated only by liver transplantation. It should be noted that there are atypical hemangiomas with shunt that are difficult to treat and cause fatal clinical symptoms and complications.
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PMID:Atypical liver hemangioma with shunt: long-term follow-up. 1265 11

Miliary neonatal hemangiomatosis is a rare, life-threatening condition associated with cutaneous and multiorgan involvement. We report two infants with this condition who had fulminant cardiac failure and cardiac septal hypertrophy. The first was a 5-day-old boy who presented with increasing numbers of cutaneous hemangiomata associated with worsening cardiac failure. Magnetic resonance imaging (MRI) showed extensive hepatic hemangioma. Despite treatment with systemic corticosteroids and subcutaneous interferon alfa-2b his disease progressed. Hepatic artery embolization was unsuccessful. The infant died of congestive cardiac failure at 6 weeks of age. Postmortem examination showed a massively enlarged cardiac interventricular septum and biventricular hypertrophy. The second patient was a 1-week-old girl who also had cutaneous hemangioma and cardiac decompensation. MRI showed extensive intrahepatic involvement. She was treated early with corticosteroids and interferon alpha, which resulted in involution of the cutaneous and hepatic lesions. Cardiac septal hypertrophy did not persist at late follow-up, and the association of miliary neonatal hemangiomatosis and cardiac septal hypertrophy has not yet been established.
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PMID:Miliary neonatal hemangiomatosis with fulminant heart failure and cardiac septal hypertrophy in two infants. 1528 93

We present 3-month-old male infant with diffuse neonatal hemangiomatosis. There were 63 cutaneous hemangiomas over the scalp, face, trunk, and extremities. Computed tomography scan revealed the presence of hemangiomas in the liver and kidneys; laryngobronchoscopy identified the presence of hemangioma in tracheobronchial tree. The child had symptoms of heart failure therefore digitals and diuretics were administrated. Thyroid functions were normal. Treatment with corticosteroids, in dose of 3 mg/kg/d intravenously, was initiated. As there was no significant clinical improvement, cyclophosphamide was administrated. He received 4 courses, 10 days apart. Each course consisted of 10 mg/kg/d of cyclophosphamide and 10 mg/kg/d of mesna for 4 consecutive days. After 4 cycles of cyclophosphamide, the liver was notably decreased in size and the cardiac failure was resolved. Magnetic resonance imaging of the abdomen revealed the marked decrease in size of the liver hemangioma. After 3 years of follow-up the child is well developed, fully recovered, without cardiologic or respiratory problems.
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PMID:Diffuse neonatal hemangiomatosis treatment with cyclophosphamide: a case report. 1982 52

We report a case of pulmonary capillary hemangiomatosis in a patient with aortic stenosis. An 86-year-old Japanese female with chronic heart failure due to aortic stenosis suddenly died during hemodialysis. At an autopsy, severe aortic stenosis and cardiomegaly with both left and right ventricular hypertrophy were noted. In the lung, a diffuse proliferation of capillaries in the thickened alveolar septum and collections of hemosiderin-laden macrophages in the alveolar space were observed. These indicated that long-standing passive congestion from aortic stenosis might result in the occurrence of pulmonary capillary hemangiomatosis.
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PMID:Pulmonary capillary hemangiomatosis in chronic cardiac failure due to aortic stenosis. 2000 7

Hemangioma is the most common vascular tumor of infancy; presentation is often as cutaneous infantile hemangioma (IH). Cutaneous hemangioma is a clinical diagnosis. Most IHs follow a benign course, with complete involution without treatment in the majority of cases. Visceral hemangioma often involves the liver and manifests as a life-threatening disorder. Hepatic hemangiomas may be associated with high output cardiac failure, coagulopathy, and hepatomegaly which generally develop between 1 and 16 weeks of age. Mortality has been reportedly high without treatment. We report a rare case of a male infant with neonatal hemangiomatosis with diffuse peritoneal involvement, which mimicked a malignant-looking tumor on imaging, and discuss therapeutic options and efficacy. Propranolol is efficacious for IH but generally not useful for other forms of vascular hemangiomas, tumors, and malformations. In our case of neonatal peritoneal hemangiomatosis, propranolol appears to have halted the growth and possibly expedite the involution of the hemangiomatosis without other treatments.
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PMID:Neonatal Abdominal Hemangiomatosis: Propranolol beyond Infantile Hemangioma. 2711 Apr 21


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