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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with cyanotic congenital heart disease (CHD) and erythremia were seen for abrupt onset of focal neurologic deficits and/or seizure without signs of infection or increased intracranial pressure. Clinical features and initial computed tomography (CT) in both cases suggested stroke. Subsequent CT scans demonstrated cerebral abscess, proved at operation. Review of records of patients with CHD at the University of Rochester (NY) Medical Center from 1965 to 1981 disclosed 12 cases with brain abscess but only two cases with aseptic cerebral infarction. All but one patient with abscess were cyanotic. One third of patients with abscess had a clinical picture suggesting stroke. Clinical or radiologic features of half the cases indicated that cerebral infarction may have led to abscess formation. Diagnosis of brain abscess and immediate antibiotic therapy should be strongly considered in patients with cyanotic CHD who suffer a suspected cerebral infarction.
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PMID:Cyanotic congenital heart disease with suspected stroke. Should all patients receive antibiotics? 683 Apr 69

This report summarizes the spectrum of clinical and immunologic findings gathered prospectively in 13 patients with the DiGeorge syndrome. Our patients demonstrated marked variability in both the clinical manifestations and the degree of immunodeficiency, confirming the findings of earlier individual case reports and retrospective autopsy reviews. Ages at the time of presentation ranged from one day to 4 months. Congenital heart defects including truncus arteriosus, ventricular septal defect, interrupted aortic arch, and tetralogy of Fallot commonly brought these infants to medical attention within the first two weeks of life. Abnormal calcium homeostasis was found in all patients. Those patients presenting after the first month of life often had hypocalcemic seizures as the initial clinical manifestation. Parathyroid hormone levels and the number and location of parathyroid glands varied considerably. Immunologic evaluation revealed that total lymphocyte counts, percent T-cells, total T-cells, and T-lymphocyte function ranged from normal to severely depressed. The most consistent immunologic abnormality, found in 11 of the 13 patients, was a decrease in total T-cells. Sequential studies in five patients demonstrate that spontaneous resolution of immunodeficiency may occur in some, yet progressive loss of immune function may be observed in others. Complete immunologic evaluation and careful followup is mandatory in infants with persistent hypocalcemia and congenital heart disease who are suspected to have DiGeorge syndrome.
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PMID:Clinical and immunologic spectrum of the DiGeorge syndrome. 697 33

A retrospective case note survey of 67 surgically proven cases of intracranial abscess formation has been carried out. Males predominated, especially in the third decade. Only 15 per cent had a predisposing condition such as cyanotic heart disease though the majority had some evidence of a systemic infection or fever. A peripheral leucocytosis and elevated erythrocyte sedimentation rate (ESR) were also more frequently encountered than in a comparable group of cases of cerebral tumour. Despite these pointers to an infective process the correct diagnosis was commonly not considered on admission. Headache, vomiting, drowsiness and focal symptoms sometimes accompanied by focal or generalized seizures remain the classical features in the history, with papilloedema and focal signs commonly found in a drowsy ill-looking patient. Many individuals failed to show all these features however. The progressive nature of the clinical picture should itself be enough to prompt urgent referral for investigation, electro-encephalography, nuclear scanning and CT scanning all being reliable.
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PMID:The clinical presentation of intracranial abscesses. 715 24

Papillary muscle necrosis was found in 24 of 84 neonates without congenital heart disease who died and were autopsied during a 17-month study period. The lesion was most prevalent in infants greater than or equal to 3,000 gm birth weight (59%), but papillary muscle necrosis was also noted in 25% of the infants of medium birth weight (1,500 to 2,999 gm) and in 19% of the very low-birth-weight infants (less than 1,500 gm). Papillary muscle necrosis in the highest birth-weight group correlated with five-minute Apgar scores of 6 or less, meconium aspiration syndrome, seizures, congestive heart failure, increased cardiothoracic ratio (greater than or equal to 0.60), and ischemic changes on electrocardiogram. In the medium- and very low-birth-weight groups, however, patients with papillary muscle necrosis could seldom be differentiated clinically from their birth-weight peers without the lesion, even in retrospect.
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PMID:Papillary muscle necrosis in a neonatal autopsy population: incidence and associated clinical manifestations. 718 2

Electrophysiologic and histopathologic correlation has been carried out in a patient with scleroderma heart disease, affected by syncopal seizures, who died of recorded ventricular fibrillation. The electrophysiological investigation disclosed dysfunction of sinoatrial conduction, revealed by sinoatrial blocks and by an abnormal return cycle pattern after premature atrial beats. Atrial effective and functional refractory periods were increased and an unusual 'pseudo-Wenckebach' phenomenon between artificial stimulus and atrium was observed during atrial pacing. Intra-AV nodal conduction time was at normal upper limits and Wenckebach-type AV block was obtained on pacing the atrium at 100 beats/min. HV conduction was moderately prolonged in the presence of left anterior hemiblock. The histopathologic substrates of these electrophysiologic disturbances were fibrosis of the sinus node, disrupted internodal pathways and atrio-AV nodal connections, and left bundle branch atrophy. As far as fatal tachyarrhythmia is concerned, myofibrillar degeneration may have contributed to its pathogenesis. It is suggested that both lesions of the ordinary myocardium and specialized conduction system account for the electrical instability of sclerodermic patients.
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PMID:Clinicopathologic assessment of arrhythmias in a case of scleroderma heart disease with sudden death. 723 56

In the present study the pathology and treatment of occlusion of cerebral arteries in children were investigated in an attempt to find out an approach to the surgical treatment of cerebral arterial occlusive disease of childhood. We had a total of 55 children with cerebrovascular disorders seen at our Institute during the past 11 years. In this series there were 19 cases of cerebral arterial occlusive disease. The causes in 15 cases of cerebral arterial occlusive disease except for moyamoya disease were heart disease in 7 cases, trauma in 2 cases and unknown in 6 cases. The cerebral arterial occlusive disease had its onset at the age of less than 6 years in 12 of 15 cases. The disease began with hemiplegia of sudden onset. The disease onset was also attended frequently by a convulsive seizure, which distinguishes the condition from that in adults. In 10 of all 15 lesions the site of occlusion was in the distribution of the middle cerebral artery. One case in which there was occlusion of the basilar artery occurring in association with trauma was described in detail. In one instance the treatment consisted of STA-MCA anastomosis. Paroxysmal black out attacks, which had been of frequent occurrence in addition to hemiplegia were relieved postoperatively. In our opinion, STA-MCA anastomosis for cerebral arterial occlusive disease in children should be considered to be indicated only when 1) cerebral angiographic evidence of occlusion or stenosis of a trunk of cerebral artery is still present after the acute stage or 2) no extensive low density area is demonstrable on CT scan. Surgery is generally less indicated in those instances in which the occlusion is due to embolism. However since abscess may arise from such an arterial lesion, surgery should be considered, or at least its feasibility be evaluated, whenever 1) the underlying cardiac pathology well permits surgical intervention and 2) half a year has passed since an initial attack.
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PMID:[Cerebral arterial occlusive disease in children -- clinical aspects and surgical treatment (author's transl)]. 727 22

Congenital and acquired cerebrovascular diseases in the very young age group are reviewed and discussed. Whilst saccular aneurysms are rare arteriovenous malformations and cavernous hemangiomas represent the most frequent types of all congenital anomalies. The aneurysm of the great vein of Galen manifests in the newborn period and mostly is mistaken for congenital heart disease. If the infant survives this angioma causes hydrocephalus. A particular type of phakomatosis combines intracerebral pure venous malformation with homolateral port-wine nevus on the front, often causing epileptic seizures. Spontaneous intracerebral hemorrhage occurs in children with small cavernous hemangiomas. Acquired arterial lesions may develop during fetal life by embolism, causing porencephaly or unilateral brain atrophy. The "Moyamoya" syndrome represents a frequent multi-arterial lesion causing characteristic ischemic episodes. Etiology is still obscure. We do not even know if the disease is acquired or congenital.
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PMID:Cerebrovascular diseases in the first three years of life. 743 79

The cranial computerized axial tomography (CAT) findings in groups of patients with epilepsy, migraine, hypertension, and other general medical disorders have been reviewed to assess the frequency and patterns of focal and diffuse brain damage. In addition to demonstrating focal lesions in a proportion of patients with seizures and in patients presenting with a stroke, the CAT scan showed a premature degree of cerebral atrophy in an appreciable proportion of patients with long-standing epilepsy, hypertension and diabetes, and in some patients with migraine, valvular and ischaemic, heart disease, chronic obstructive airways disease, and chronic renal failure. The value of CAT as a means of screening for brain damage in groups of individuals at risk is discussed.
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PMID:Computerized axial tomography in the detection of brain damage. 2. Epilepsy, migraine, and general medical disorders. 746 20

We studied serum prolactin (PRL) in 28 newborn infants with acute encephalopathy. Six patients had electrographically confirmed seizures. Twenty-two patients comprised the nonictal group. In the seizure group, PRL was determined at the first onset of the seizure (baseline) and at 15 and 30 min postictal. In the nonseizure group, PRL was determined at the end of the EEG and 15 min later. EEGs were visually analyzed for the presence of seizures and background abnormality (normal or mildly, moderately, or markedly abnormal). Etiologic diagnoses included congenital heart disease (12), hypoxic-ischemic encephalopathy (4), sepsis (4), respiratory distress syndrome (5) meconium aspiration (1), and metabolic disease (2). Serum PRL was significantly higher (p < 0.05) at baseline and 15 min postictally in the patients with seizures than in the nonictal group. However, PRL levels 15 and 30 min postictally were not statistically different from baseline values. Baseline PRL correlated significantly (p < 0.001) with EEG background abnormality in both groups; therefore, patients with the most abnormal EEG backgrounds had higher levels of PRL than those with a relatively normal EEG background. We conclude that newborns with EEG-confirmed seizures, particularly if seizures are not associated with clinical signs, have high baseline serum PRL levels that do not increase significantly in the immediate postictal period. Serum PRL levels correlate with the severity of the brain insult as evaluated by EEG background. Further studies are needed to enhance our understanding of the dynamics of PRL secretion in newborns with seizures and acute encephalopathy.
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PMID:Serum prolactin in neonates with seizures. 755 85

Thirty patients with brain abscesses who were treated at the Department of Neurosurgery, Tan Tock Seng Hospital, Singapore, during the period July 1989 to July 1993, were reviewed. There were seven females and 23 males with mean follow up duration of 19 months. The predisposing factors identified were otogenic infections in 10 cases (33%), paranasal sinus infection in 2 cases (7%) and congenital heart disease in 6 cases (20%). All cases underwent aspiration or excision of the abscess. Repeat operations were carried out if the abscess reaccumulated. A total of 49 aspirations and 16 excisions were performed. Twenty-five patients (84%) made a good recovery, three patients (10%) were moderately disabled, one patient (3%) was severely disabled and one patient (3%) died. There was no postoperative haemorrhage or wound infections. Two patients (7%) developed hydrocephalus and three (10%) had seizures postoperatively.
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PMID:Brain abscesses: review of 30 cases treated with surgery. 757 99


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