Cases reported "Cardiomegaly"

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11/43. Pulmonary vascular changes in scleroderma.

    To determine the incidence and significance of pulmonary vascular changes in scleroderma, all necropsy reports of patients with scleroderma who died at the massachusetts General Hospital were analyzed and correlated with clinical data. The records of 30 such patients were available. Fourteen had moderate or marked abnormalities in the pulmonary arterial tree. Nine of these 14 patients had predominantly respiratory symptoms. The arterial changes consisted pathologically of intimal and medial hyperplasia affecting pulmonary arteries of all sizes. Of the eight patients with the most severe pathologic changes in the pulmonary arteries, five had slight or no interstitial fibrosis. Three of these five patients had rapidly progressive respiratory failure and severe pulmonary hypertension leading to death. Such cases form a distinct clinicopathologic entity of malignant pulmonary hypertension in scleroderma, comparable to the better recognized entity of malignant renal hypertension in scleroderma.
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ranking = 1
keywords = death
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12/43. Hypodontia, impacted permanent teeth, spinal defects, and cardiomegaly in a previously diagnosed case of the Yunis-Varon syndrome.

    The Yunis-Varon syndrome is a rare hereditary disorder with ectodermal and skeletal anomalies that include agenesis or hypoplasia of the clavicles, craniofacial disproportion with severe micrognathia, digital anomalies, prenatal and postnatal growth deficiency, and neonatal death. Only 12 cases have been reported in the literature. Although neonatal death is a significant feature of this syndrome, two case reports describe children, both males, who were 30 months and 3 years of age at the time of investigation. The 30-month-old child was reexamined at 11 years of age, and had further clinical and radiologic features that included hypodontia, impacted permanent teeth, spinal defects, cardiomegaly, bilateral hearing loss, and metatarsus adductus. Children who survive the neonatal period and continue to thrive with many of the features of the Yunis-Varon syndrome as well as the new features described in this article may not have a distinct yet related syndrome.
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keywords = death
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13/43. sleep apnea and Q-T interval prolongation--a particularly lethal combination.

    We have discovered a 20-day-old infant who possessed anatomic evidence of chronic hypoxemia with right ventricular hypertrophy and who died in hypoxic hypoxemia with a postmortem PO2 of 4 mm. Hg. Subsequently, and ECG was discovered which had been obtained at one day of age and showed Q-T interval prolongation along with T-wave alternation. We believe this case to be one of the first to substantiate the mechanism for SIDS as proposed by Schwartz, 26 with hypoxia acting synergistically with a prolonged Q-T interval causing sudden unexpected death in this infant--providing a link between cardiac and respiratory mechanisms of death.
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ranking = 2
keywords = death
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14/43. Prolongation of the Q-T interval in a victim of sudden infant death syndrome: cause or effect?

    A 3-week-old infant died of sudden infant death syndrome (crib death, cot death). Although prolongation of the Q-T interval was found on an electrocardiogram taken at birth, this child appeared to have died of terminal respiratory failure as judged by a postmortem arterial oxygen tension of 4 mm Hg. The heart showed right ventricular hypertrophy, a finding consistent with chronic hypoxemia. The prolongation of the Q-T interval may have contributed to the death of this child with hypoxia predisposing to a fatal episode of ventricular fibrillation.
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ranking = 8
keywords = death
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15/43. Hypertrophic cardiomyopathy associated with sudden death during marathon racing.

    An experienced marathon runner died suddenly during a competitive race. At necropsy, ventricular hypertrophy but no asymmetrical septal hypertrophy was found. Histological studies showed features of hypertrophic cardiomyopathy. The coronary arteries were normal. We propose that the runner died from myocardial ischaemia, precipitated by marathon running on a background of hypertrophic cardiomyopathy. Excess cardiac work, induced by marathon running in the presence of mild congenital cardiac defects, could have contributed to the development of the cardiomyopathy.
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ranking = 4
keywords = death
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16/43. cardiomegaly and heart failure in a patient with prolactin-secreting pituitary tumour.

    Unexplained cardiomegaly with cardiac failure was observed in a 42-year-old woman in whom a pituitary tumour had been treated by radiotherapy five years previously. She had been amenorrhoeic for 10 years. Thyroid and adrenal function was normal. Despite treatment with digitalis and diuretic, her cardiac disease progressed until she died suddenly at the age of 45. Hyperprolactinaemia was evident some weeks before death, her serum concentration of 68 ng/ml being well above both the reported normal range (2--20 ng/ml) and the concentrations in eight female controls being treated for severe cardiac failure (5--25 ng/ml). Although the association of these two disorders might merely represent coincidence, heart disease with similar features is common in acromegaly and does not correlate with plasma growth hormone concentration. Since prolactin is known to exert metabolic growth hormone-like effects in animals and in man, the possibility should be considered that prolactin hypersecretion might induce or maintain cardiac disease in some patients with pituitary tumours. A preliminary survey of 35 subjects with hyperprolactinaemia has shown five with raised blood pressure and four, two of whom were normotensive, with cardiomegaly on chest radiography.
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ranking = 1
keywords = death
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17/43. Fatal congenital heart glycogenosis caused by a recurrent activating R531Q mutation in the gamma 2-subunit of AMP-activated protein kinase (PRKAG2), not by phosphorylase kinase deficiency.

    Fatal congenital nonlysosomal cardiac glycogenosis has been attributed to a subtype of phosphorylase kinase deficiency, but the underlying genes and mutations have not been identified. Analyzing four sporadic, unrelated patients, we found no mutations either in the eight genes encoding phosphorylase kinase subunits or in the two genes encoding the muscle and brain isoforms of glycogen phosphorylase. However, in three of five patients, we identified identical heterozygous R531Q missense mutations of the PRKAG2 gene, which encodes the gamma 2-subunit of AMP-activated protein kinase, a key regulator of energy balance. Biochemical characterization of the recombinant R531Q mutant protein showed >100-fold reduction of binding affinities for the regulatory nucleotides AMP and ATP but an enhanced basal activity and increased phosphorylation of the alpha -subunit. Other PRKAG2 missense mutations were previously identified in patients with autosomal dominant hypertrophic cardiomyopathy with wolff-parkinson-white syndrome, characterized by juvenile-to-adult clinical onset, moderate cardiac glycogenosis, disturbed excitation conduction, risk of sudden cardiac death in midlife, and molecular perturbations that are similar to--but less severe than--those observed for the R531Q mutation. Thus, recurrent heterozygous R531Q missense mutations in PRKAG2 give rise to a massive nonlysosomal cardiac glycogenosis of fetal symptomatic onset and rapidly fatal course, constituting a genotypically and clinically distinct variant of hypertrophic cardiomyopathy with wolff-parkinson-white syndrome. R531Q and other PRKAG2 mutations enhance the basal activity and alpha -subunit phosphorylation of AMP-activated protein kinase, explaining the dominant nature of PRKAG2 disease mutations. Since not all cases displayed PRKAG2 mutations, fatal congenital nonlysosomal cardiac glycogenosis seems to be genetically heterogeneous. However, the existence of a heart-specific primary phosphorylase kinase deficiency is questionable, because no phosphorylase kinase mutations were found.
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ranking = 1
keywords = death
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18/43. Cardiac sarcoidosis: two cases with autopsy findings.

    Cardiac sarcoidosis is a disease of young adults. In most cases it presents with sudden death, arrhythmias, conduction disorders, heart failure or cardiomyopathy. The authors describe two cases of myocardial involvement by sarcoidosis that lead to death of the patients. Case one was a 26-year-old Indian man who was previously well and presented with sudden death. autopsy showed nodules of sarcoid granuloma involving the heart, lungs and lymph nodes. Case two was a 47-year-old Indian lady who complained of reduced effort tolerance. echocardiography showed that she had restrictive hypertrophic cardiomyopathy with heart failure. Seven months after initial presentation, she developed worsening of heart failure and died. autopsy revealed involvement of the heart, lungs and liver by sarcoidosis.
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ranking = 3
keywords = death
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19/43. True-true, unrelated: a case report.

    OBJECTIVES: Sudden cardiac deaths in previously healthy children are frequently due to undiagnosed cardiovascular diseases, either congenital or acquired. In an uncommon clinical entity known as commotio cordis, sudden death from cardiac arrest can occur in young athletes after a blunt blow to the chest, in the absence of preexisting cardiovascular disease. We present a case in which the clinician's high index of suspicion leads to the diagnosis of acute myocarditis in a patient whose sudden cardiac deterioration was initially attributed to the result of recent blunt chest trauma. methods: A case report and review of literature via medline (1996-2004) search using the key words "myocarditis," "commotio cordis," and "myocardial contusion." RESULTS: A 12-year-old boy was admitted with elevated cardiac enzymes and respiratory distress after being hit in the chest with a dodgeball. Shortly after admission, the patient developed refractory ventricular arrhythmia, which was thought to be the result of blunt chest trauma. Further evaluation with endomyocardial biopsy, however, demonstrated acute myocarditis as the true etiology, for which the patient received immunosuppressive treatment. Unfortunately, the patient eventually required cardiac transplantation because of progressive irreversible cardiac dysfunction due to myocarditis. CONCLUSIONS: Although acute myocarditis, commotio cordis, and myocardial contusion can all present with malignant ventricular arrhythmia, other clinical features and approaches to management of each disease are very different. This case illustrates the importance of having a broad differential diagnosis in mind when presented with a previously healthy child in sudden cardiogenic shock.
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ranking = 2
keywords = death
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20/43. Total anomalous pulmonary venous drainage and sudden death in infancy.

    The clinicopathologic features of four infants with undiagnosed total anomalous pulmonary venous return who died suddenly and unexpectedly are presented. Two infants were found dead in their beds and two collapsed following very non-specific episodes of mild tachypnoea and tachycardia, respectively. Pulmonary venous blood drained via a common channel to the left innominate vein, the superior vena cava, the inferior vena cava at the junction of the hepatic vein and the supradiaphragmatic portion of the inferior vena cava. All cases demonstrated right atrial and ventricular hypertrophy and in one case there was associated hypoplastic left heart syndrome. Although those mechanisms responsible for sudden infant death syndrome could not definitely be excluded in two cases, the presence of anomalous pulmonary drainage with significant right-sided cardiac cardiac hypertrophy in each patient strongly suggests a contributing role for the vascular malformation in the aetiology of sudden unexpected death. The importance of careful in situ dissection of the major vessels in all cases of paediatric sudden death is emphasised, particularly in the presence of other congenital cardiac anomalies.
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ranking = 7
keywords = death
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