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1/15. Pericardial heart disease: a study of its causes, consequences, and morphologic features.

    This report reviews morphologic aspects of pericardial heart disease. A morphologic classification for this condition is presented. An ideal classification of pericardial heart disease obviously would take into account clinical, etiologic and morphologic features of this condition but a single classification combining these three components is lacking. Pericardial heart disease is relatively uncommon clinically, and when present at necropsy it usually had not been recognized during life. The term "pericarditis" is inaccurate because most pericardial diseases are noninflammatory in nature. Morphologically chronic pericardial heart disease may present clinically as an acute illness. Even when clinical symptoms are present, however, few patients develop evidence of cardiac dysfunction (constriction). When pericardial constriction occurs, it is the result of increased pericardial fluid or increased pericardial tissue or both. Increased fluid is treated by drainage; increased tissue is treated by excision. In most patients with chronic constrictive pericarditis the etiology is not apparent even after histologic examination of pericardia.
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ranking = 1
keywords = heart disease, heart
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2/15. Neoplastic disease and deletion 22q11.2: a multicentric study and report of two cases.

    Deletion 22q11.2 is a chromosomal abnormality detected in young patients with clinical manifestations of the DiGeorge/velocardiofacial syndrome. Conotruncal heart defects are also associated with del22q11.2. An association of these cardiac malformations with neoplasias has been observed. Our series includes two cases of malignancies, a hepatoblastoma and a renal-cell carcinoma, arising in children with complex cardiac malformations. The aim of the study was to determine if the deletion at 22q11.2 was present and could be responsible for both pathological processes. Del22q11.2 was identified in both cases. comparative genomic hybridization revealed terminal gains on chromosomes 1q and Xq and terminal loss on 1p in the hepatoblastoma, and gains in 1p, 12q, 16p, 20q, 22q, and whole chromosome 19 and loss of Xq in the renal-cell carcinoma. Our results confirm a common genetic basis for cardiac malformations, and del22q11.2 presents a risk factor for the development of pediatric tumours.
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ranking = 0.030157609074023
keywords = heart
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3/15. Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.

    INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital systemic angiodysplasia with multiple vascular malformations in the skin, gastrointestinal tract and, less often, in other internal organs and the brain. CASE REPORT: A 36-year-old man with past history of BRBNS was admitted to our hospital for progressive dyspnea and fatigue. Primary pulmonary hypertension (PPH) was diagnosed. He then developed acute abdominal pain and dyspnea, dying in a few hours due to sudden cardiac arrest. Postmortem examination demonstrated angiomatous lesions located in the skin, small bowel, heart, lungs, liver and thyroid. The lesions were slightly raised, soft and compressible and microscopically consisted of dilated vascular channels lined by a flattened endothelium. The vascular wall was formed by several layers of smooth muscle cells, intermixed with abundant aggregates of elastic lamellae and thin collagen fibers. Luminal thrombi were a frequent finding. In the small bowel, we identified the presence of an abnormally large artery directly opening into a thin-walled venous channel. The most striking finding in the lungs was the presence of thrombi of varying age in the lumen of segmental and elastic arteries, as well as muscular arteries and arterioles. Severe medial hypertrophy of muscular arteries and muscolarization of arterioles were also present. Intimal proliferative lesions and plexiform lesions were never observed. CONCLUSION: The pulmonary findings are consistent with recurrent thromboembolic events from shunts in the visceral lesions. To our knowledge, this is the first report of BRBNS with visceral arterovenous (AV) fistulae complicated by thromboembolic pulmonary hypertension (PH).
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ranking = 0.030157609074023
keywords = heart
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4/15. Creative novation behaviour therapy as a prophylactic treatment for cancer and coronary heart disease: Part I--Description of treatment.

    This paper describes a novel method of behaviour therapy applied to cancer-prone and coronary heart disease-prone patients in a prophylactic manner, to reduce the probability of their dying of cancer or coronary heart disease. The treatment can also be applied to patients already suffering from cancer in order to prolong their lives. The methods used are described in considerable detail, together with the rationale leading to their adoption. In Part II of this paper are the results of several studies showing that the methods are surprisingly successful in preventing death in cancer-prone and coronary heart disease-prone probands, and prolonging life in patients already suffering from terminal disease.
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ranking = 0.875
keywords = heart disease, heart
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5/15. 3-Deazaguanine: report of a phase I trial and drug-related cardiac toxicity.

    3-Deazaguanine (Dezaguanine), a purine antimetabolite, was evaluated in a phase I trial in 42 patients with advanced solid tumors. Dezaguanine was given as a weekly intravenous infusion for three consecutive weeks of a four-week cycle. The dose ranged from 30 to 2000 mg/m2; no consistent dose-limiting hematologic or gastrointestinal toxicity was observed. Some patients reported brief episodes of burning at the infusion site or transient facial flushing immediately following the administration of dezaguanine. Three patients experienced cardiac toxicity. Two patients, at doses of 1130 and 2000 mg/m2 respectively, developed congestive heart failure. In one case the heart failure was fatal; the second patient recovered within 8 weeks. The third patient had a progressive fall in left ventricular ejection fraction but did not develop clinical evidence of heart failure before his death from progressive cancer two months later. Postmortem cardiac pathology in the two patients who died early following therapy revealed nonspecific interstitial fibrosis without inflammatory cell infiltrates. The myocardium of the third patient, who died 20 months after receiving dezaguanine, was normal. Electron microscopic analysis of myocardium from the first patient did not show myofibrillar loss or mitochondrial disorganization characteristic of anthracycline cardiomyopathy. Due to the probable cardiotoxicity of dezaguanine in this study and the lack of objective antitumor response, further development of this agent has been discontinued.
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ranking = 0.09047282722207
keywords = heart
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6/15. Cardiac transplantation in patients with preexisting neoplastic diseases.

    Cardiac transplantation has traditionally been reserved for individuals with end-stage congestive heart failure (CHF) in whom there is no history of other life-threatening systemic disorders. In most transplant centers, patients with a history of malignancy and severe heart failure have not been considered acceptable candidates for cardiac transplantation. In the last 4 years at Stanford University Medical Center, 8 cardiac transplants have been performed in 7 patients with a history of neoplastic disease. Six of these patients had already received treatment for lymphoproliferative disorders and in 1 case, a patient underwent a transplant after treatment for adenocarcinoma of the colon. Six of the 7 patients were discharged from the hospital and in that group, the 1-year posttransplant survival rate was 71%. This was comparable to an overall 1-year survival rate of 80% for patients undergoing a cardiac transplant at our center during the same period of time. At follow-up averaging over 2 years, there has been 1 case of recurrent neoplasia. One patient with evidence of radiation-induced pulmonary damage died of respiratory failure 2 days after transplantation. One patient required retransplantation because of intractable rejection and subsequently died from infectious complications. Immunosuppressive therapy in these patients has not been associated with an increased risk for neoplastic recurrence or for the development of posttransplant lymphoproliferative disorders. The current study demonstrates that in a carefully selected group, previously treated neoplastic disease should not represent a contraindication to cardiac transplantation.
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ranking = 0.060315218148047
keywords = heart
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7/15. Acute leukemia of megakaryocyte lineage with tumor formation. An autopsy case of patient with Down's syndrome.

    In this report we present the autopsy findings of acute megakaryoblastic leukemia with tumor formation in a 2-year-old female infant with Down's syndrome. Chromosomal analysis of blast cells revealed constitutional anomaly of trisomy 21 and two other related types of abnormal clones. Flow cytometric examination revealed blast cells expressing Ia-like or hla-dr antigens. Postmortem examination showed extensive infiltration of leukemic cells in most of the examined organs, including the bone marrow with myelofibrosis. Tumor masses in the maxillary, frontal and femoral bones and the atria of the heart had undergone massive infiltration of atypical blast cells with an increase in the reticulin network. The final diagnosis was confirmed by ultrastructural cytochemistry of the platelet peroxidase reaction as well as by immunological staining utilizing anti-platelet glycoprotein IIb/IIIa, antiplatelet factor 4 and anti-beta-thromboglobulin antibodies for the blast cells. It seems likely that platelet-derived growth factor, secondary to an increase in the reticulin network, plays a major role in myelofibrosis of acute megakaryoblastic leukemia with tumor formation.
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ranking = 0.030157609074023
keywords = heart
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8/15. calcinosis in nonparathyroid malignant disease: an unusual case report and clinicopathologic review of 17 cases.

    An unusual finding of systemic calcinosis in a patient with a nonparathyroid malignant neoplasm stimulated us to do a sclinicopathologic review of similar cases at our institution in the past seven years. Of 3,268 autopsies performed from 1968 to 1975, a total of 17 cases of calcinosis were found, 11 with solid tumors and 6 with hematopoietic neoplasms. calcinosis was most prominent in the lung, kidney, heart, and stomach and was rarely discovered prior to death. Eighty-two percent of the patients had hypercalcemia and 53% had associated bony metastatic disease. Corticosteroid or phosphate treatment for the hypercalcemia may have contributed to the tissue deposition of calcium. Significant hepatic, renal, metabolic, and pulmonary dysfunctions were also associated with this disorder. Thirty-six percent of the patients had hypercalcemia without skeletal involvement; tumor-produced parathormone-like substances may be responsible for these calcium abnormalities. calcinosis was a significant complication of neoplastic disease in these patients and contributed to morbidity and mortality.
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ranking = 0.030157609074023
keywords = heart
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9/15. fluorouracil cardiotoxicity.

    Our of 140 patients treated with intravenous 5-fluorouracil, four developed ischaemic chest pain within 18 hours of either the second or third dose. In three of these patients the pain recurred after subsequent doses. Predose electrocardiograms in two cases were normal. None of the four patients had a history of ischaemic heart disease, although all had received left ventricular irradiation. Although cardiotoxicity is a rare complication of fluorouracil treatment, it merits wider recognition.
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ranking = 0.125
keywords = heart disease, heart
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10/15. The value of the hospital autopsy. A study of causes and modes of death estimated before and after autopsy.

    Among 312 consecutive deaths in a Danish Central Hospital autopsy was performed in the pathology department on 266 cases, i.e. 85%. Retrospectively, the underlying causes of death were estimated from the clinical information alone by an experienced clinician and subsequently compared with the autopsy report. The definite cause of death was determined jointly by the clinician and the pathologist. The clinician's diagnosis was thereby confirmed as incorrect in 18% of the cases if small differences in site and type of malignant tumours were not considered. This is less than in many other investigations, but it is stressed that this could partly be because formal errors in completing the death certificate were avoided. The main causes of death were ischaemic heart disease and neoplasia. Clinical diagnosis of malignant diseases was never found to be erroneous. There was a slight tendency to clinically overestimate ischaemic heart disease, but in general the different errors outweighed each other, so that the total number of different causes of death before and after autopsy was nearly the same. The original death certificate was investigated in 12 accidental cases. Hereby it was found that the mode of death was originally stated erroneously as natural in 7 cases, i.e. 4.5%. It is concluded that hospital autopsy is still needed for the control and correction of causes of death, and it is stressed that clinicians as well as pathologists should be more aware of cases with a trauma in the history to avoid errors in the mode of death. Such errors can imply legal as well as insurance problems.
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ranking = 0.25
keywords = heart disease, heart
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