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

Recurrent apnoea and chronic hypoventilation have been implicated in the pathogenesis of the sudden infant death syndrome (SIDS) and markers of chronic hypoxaemia have been reported in such infants at post mortem examination. Markers of chronic hypoxaemia are common in cyanotic congenital heart disease. Glomerular enlargement in congenital heart disease is said to be related to hypoxaemia although the precise mechanism whereby this occurs is not clear. We have established a normal range of glomerular size for the postperinatal period and confirmed glomerular enlargement to be a common finding in children with congenital heart disease of similar age. In contrast glomerular size in SIDS is not different from controls. The results question the role of significant chronic hypoxaemia being involved in these deaths.
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PMID:Renal glomerular size in infants with congenital heart disease and in cases of sudden infant death syndrome. 373 35

A combined morphometric, ultrastructural, and biochemical study was done on carotid bodies (CBs) obtained at autopsy from 213 patients in a pediatric and young adult population. The objective was to determine whether this group had statistically significant differences in sudden infant death syndrome (SIDS, n = 38), cystic fibrosis (CF, n = 30) and cyanotic heart disease (CHD, n = 17), compared with an age-matched control population (n = 128). Average combined weights of CBs in CF and CHD were significantly greater than those of controls in most age intervals (Student t test, P less than 0.05), and computerized planimetry showed an increase in both total surface area and area of "functional" parenchyma. There was diminished chief cell argyrophilia in 72% of CF CBs, and in 8 cases studied ultrastructurally there was moderate to marked depletion of dense-core neurosecretory granules. Most CBs from patients with CHD showed intense cytoplasmic argyrophilia similar to that of controls. Quantitative analysis for tissue catecholamines showed that dopamine was present in greatest concentration in each group of patients but was significantly elevated in CHD. There were no significant differences in morphometry, ultrastructure, or catecholamine content of CBs from SIDS victims, compared with age-related controls. These data add further support to CBs having a chemoreceptor role in humans with compensatory hypertrophy and hyperplasia occurring in most patients with chronic hypoxia due to CF and CHD. There were no significant findings to indicate that CBs play a direct role in the etiopathogenesis of SIDS.
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PMID:Carotid body hyperplasia in cystic fibrosis and cyanotic heart disease. A combined morphometric, ultrastructural, and biochemical study. 399 43

In Down's syndrome, incidence of 1 0/00-2 0/00 have been reported in chromosomal surveys of consecutive liveborn infants and in population studies. Much attention has been focused on the influence of the decline in mean maternal age on the incidence of Down's syndrome. Decline in incidence and unchanged incidences have been reported. For mothers over 35 years old, a rise in incidence has been found in recent years in some societies. Environmental factors or seasonal fluctation might cause this trend. Data from antenatal diagnosis show a 30% higher incidence of Down's syndrome for age groups over 35 than population studies do. Late abortions of trisomic fetuses, a high perinatal mortality, and a small rise in incidence for higher maternal ages in recent years may account for this fact. With the growing tendency toward younger maternal age at childbirth, paternal factors also have to be considered. Between 10% and 30% paternal failures have been found by nondisjunction studies applying chromosomal variants. The rate of paternal failures may reflect environmental influences and young maternal age distribution. Recently, an increase in trisomy 21 children of mothers in the age group 30-39 who are pill users has been observed. The positive sex ratio of male Down's syndrome patients has been reversed in patients born to mothers using hormonal contraception. The mortality rate, in Down's syndrome is still high in early childhood, especially perinatally and in the 1st year of life. For the late 1940s in Denmark, newborns with Down's syndrome had a mortality rate of 53% in the 1st year of life. In the late 1960s and early 1970s, the rate had fallen to 22%. The main causes of death were congenital heart disease in connection with infections, especially pneumonia. Cot death might be more common in Down's syndrome. After the age of 1 year, mortality is reduced considerably and more and more cases will survive early childhood and live to rather old ages.
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PMID:Epidemiology of trisomy 21: population, peri- and antenatal data. 621 37

Knowledge regarding the etiology and optimal management of prolonged apnea and its relationship to SIDS is still limited. The majority of infants with prolonged apnea do not die of SIDS, although the risk of SIDS in this group is greater than in the general population. Many infants with prolonged apnea who are perceived by parents and physicians as having had a "life-threatening" event may be at risk for another. Appropriate assessment following this event includes a careful history and physical examination to determine cause and severity. Etiologies to be considered include infections, metabolic aberrations, seizure problems, cardiac arrhythmias or congenital heart disease, anatomic airway abnormalities, gastroesophageal reflux and impaired regulation of breathing. If a specific cause has been identified for the infant's apnea, appropriate treatment often will lead to resolution of the apnea problem. If a specific etiology has not been identified or if the risk of "life-threatening" prolonged apnea seems to persist, electronic cardiorespiratory monitoring may be considered. Appropriate treatment for asymptomatic infants who are at increased statistical risk of SIDS is controversial. Asymptomatic infants may be candidates for home monitoring, but as yet, there are no reliable tests to predict which infants are at risk for prolonged apnea. Monitoring at home must be prescribed by the physician and should be continued until judged no longer appropriate by the attending physician. Skilled caregivers are crucial to the continuous observation and management of these patients in the hospital and at home. Therefore parents should be taught monitor use and also CPR.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Evaluation and management of infantile apnea. 670 8

Recent studies have implicated an abnormality in carotid body structure and function in the pathogenesis of sudden infant death syndrome (SIDS). In the present investigation, the light and electron microscopic findings in carotid bodies from ten victims of SIDS were compared with those in six control infants and five infants dying of congenital heart disease. The cross-sectional area of carotid body chemoreceptor cells and the frequency, distribution, and size of neurosecretory granules were assessed morphometrically. The area of carotid body occupied by chemoreceptor cells (the functional area) was comparable in SIDS victims, control infants, and infants with congenital heart disease. By electron microscopy, the carotid body chief cells from all groups contained numerous electron-dense neurosecretory granules. Distribution, frequency, and size of neurosecretory granules in SIDS victims and control infants did not differ significantly. Morphology of carotid bodies from SIDS victims was found to be normal. The presence of neurosecretory granules in chemoreceptor cells of SIDS victims suggests that the cellular mechanism of neurotransmitter synthesis and storage is not altered.
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PMID:Ultrastructure of carotid bodies in sudden infant death syndrome. 671 21

In a three-year period, 3.8% (43) of 1,123 infants discharged from one neonatal intensive care unit (NICU) subsequently died, the vast majority before 1 year of age. Sudden infant death syndrome was responsible for 28% of the deaths, congenital heart disease for 25% of the deaths, chronic lung disease for 16%, and trauma, infections, and chromosomal disorders each accounted for 5% of the deaths. Postdischarge death rates in the three-year study period remained stable, while infant mortality in the NICU decreased from 26.5% to 16.3%. This study suggests that decreasing mortality among infants treated in NICUs is not necessarily reflected in improved postdischarge death rates and that the vast majority of deaths in these infants are currently not preventable.
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PMID:Mortality in infants discharged from a neonatal intensive care unit. 684 59

Sixteen patients with serologically proven Q-fever infection are reviewed. Fifteen had significantly raised antibody titres to both phase I and phase II Coxiella burneti antigens, indicating persistent or chronic infection. One patient, a premature infant who died, had raised phase II titres only, but is included together with the mother who had chronic Q-fever and was the presumed source of infection. Chronic Q-fever infection has previously been regarded as virtually synonymous with Q-fever endocarditis, but only seven of the patients in this survey had evidence of valvular endocarditis. In those who did, the infection had arisen on prosthetic valves or those affected by rheumatic or syphilitic heart disease. One patient had inexorably progressive destruction of an infected congenitally bicuspid aortic valve. Eight patients had infections associated with extra-valvular sites and several of these associations have not been previously described. These include extreme prematurity with perinatal death, possibly following transplacental infection, the sudden infant death syndrome (SIDS), multiple lower limb emboli from endocarditis of an abdominal aortic dacron graft, and colonization of ventricular endocardium following left ventricular myotomy/sub-aortic diaphragm resection. The current concept that chronic Q-fever is invariably associated with endocarditis is therefore untenable and the indications for phase I antibody screening should be extended to include patients other than those under investigation for 'culture-negative' endocarditis, for example those with unusual osteomyelitis of vertebrae.
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PMID:Chronic or fatal Q-fever infection: a review of 16 patients seen in North-East Scotland (1967-80). 687 20

Twenty-four hour recordings of respiratory wave form and ECG were made on low-birth-weight and/or premature infants within one week of discharge from eight neonatal intensive care units. Eight infants (0.7%) had episodes of apnea greater than 30 seconds in duration, all of which were accompanied by bradycardia less than 100 beats per minute; 25 infants (2.3%) had a total of 36 apneic episodes between 20 and 30 seconds in duration, 29 of which were accompanied by bradycardia less than or equal to 100 beats per minute; and 19 infants (1.7%) had episodes of bradycardia less than or equal to 50 beats per minute without prolonged apnea (as shown by a lack of breathing movement). Five infants had ventricular premature beats (including one with ventricular tachycardia). Eleven infants had supraventricular premature beats (including two with supraventricular tachycardia and one with preexcitation). Four infants had both supraventricular and ventricular premature beats. Two infants had preexcitation. Eleven infants who underwent 24-hour recordings died. Five infants were victims of sudden infant death syndrome. One infant death was sudden and unexpected and was attributed to bronchopneumonia. Two deaths were associated with congenital heart disease and three were associated with major cerebral disorders. None of the six babies who died suddenly and unexpectedly had apnea greater than or equal to 20 seconds, bradycardia less than or equal to 50 beats per minute, or cardiac arrhythmias on their 24-hour recordings.
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PMID:Prolonged apnea and cardiac arrhythmias in infants discharged from neonatal intensive care units: failure to predict an increased risk for sudden infant death syndrome. 714 36

The purpose of this report is to present the findings in a neonate with tetralogy of Fallot and a history of prolonged sleep-related apnea requiring resuscitation. At a baseline arterial oxygen pressure of 46 mm Hg, his preoperative responses to carbon dioxide during non-REM sleep (26.1 and 26.6 ml/kg/min/mm Hg of alveolar carbon dioxide tension [PACO2]) were identical to those reported in near-miss sudden infant death syndrome. Following a systemic-pulmonary arterial shunt, the arterial saturation reached 95 percent, and no further apnea occurred. The prompt normalization of the response to carbon dioxide following surgery (75.2 and 75.4 ml/kg/min/mm Hg of PACO2) indicates that, unlike infants with the sudden infant death syndrome, a low sensitivity to carbon dioxide in infants with cyanotic heart disease may be dependent on hypoxemia and, thus, reversible.
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PMID:Reversible central apnea in an infant with cyanotic heart disease. 735 86

Histiocytoid cardiomyopathy is a rare cardiac disorder of infancy and childhood that predominantly affects girls under the age of 2 years. The clinical picture is usually dominated by severe cardiac arrhythmias, and sudden death may occur. In such instances, sudden infant death syndrome (SIDS) is often considered. Grossly, the affected heart usually shows small, multifocal, subendocardial or myocardial yellow-tan nodular lesions or poorly defined plaques. Occasionally, the lesional tissue is not apparent. Histological findings include sharply demarcated groups and sheets of cells with abundant foamy or granular cytoplasm, justifying the use of the term "histiocytoid" cardiomyopathy. The abnormal cells appear to be transformed cardiac myocytes that possess some features of the conducting system fibers; therefore, Purkinje cells are now believed to be the origin of these distinctive lesions. We describe and discuss the light-microscopic, immunocytochemical, and ultrastructural findings in a previously healthy 12-month-old boy who died suddenly.
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PMID:Sudden death in an infant due to histiocytoid cardiomyopathy. A light-microscopic, ultrastructural, and immunohistochemical study. 777 90


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