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

In seven infants with DiGeorge syndrome the major clinical manifestation was cardiac failure in the 1st week of life. All had severe congenital heart disease: five had interruption of the aortic arch and associated lesions, one had a ventricular septal defect and a cervical aorta, and one had truncus arteriosus. All but one died by 2 weeks of age. Necropsy data lent support to the hypothesis of a relation between the cardiovascular anomalies and defective development of structures derived from the third and fourth pharyngeal pouches.
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PMID:DiGeorge syndrome presenting as severe congenital heart disease in the newborn. 60 63

We evaluated digestive tract function in 21 young infants with severe congenital heart disease. One group had congestive heart failure and ventricular septal defect or single ventricle; the other had cyanosis and transposition of the great arteries. Enteric protein loss was excessive in eight patients, and steatorrhea was found in five. These abnormalities were mild and not related to the type or severity of the cardiac lesion. Available evidence points to a need of these babies for calories in excess of normal requirements for weight. The present study suggests that in designing dietary regimens for these very sick patients, their potential for defective gastrointestinal function must be considered. Because no consistent pattern of abnormalities in apparent, each patient who fails to thrive may deserve gastroenterologic evaluation.
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PMID:Intestinal function in infants with severe congenital heart disease. 63 15

Since 1968 a flow-guided-catheter (Pulmocath) was used in 326 examinations of 217 children. This is an excellent method to control the course of congenital heart disease. The findings of children with pulmonary stenosis and with ventricular septal defect are discussed.
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PMID:[Use of the flow-guided catheter to check the course of congenital heart-disease in children (author's transl)]. 64 48

We have studied 1148 'isolated" cases of congenital heart disease, taken from a general series of 1255 cases. The recurrence risk ranged from 0.9% for relatives of male cases of VSD and for female cases of aortic stenosis to 10.5% for relatives of female cases of atrioventricularis communis; mean value 2.5%. Heritability ranged from 50% to 95%, mean 65%. Full concordance was more frequent in 1st-degree relatives of Fallot's tetralogy patients, while discordance was common in more distant relatives. We propose a hypothesis of several overlapping polygenic systems for the causation of CHD.
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PMID:The recurrence risk in congenital heart disease. 66 53

The oto-vertebral syndrome is a rare combination of malformations of the ears, spinal anomalies, and congenital heart disease. The syndrome appears to be caused by early embryonic exogenous damage during the 6th or 7th week of embryonic development. The case is reported of a 28-year-old man with bilateral aplasia of the external ear, bilateral aplasia of the ear canal, hypoplasia of the mandibula, severe thoracic scoliosis, and ventricular septal defect with pulmonary hypertension. He was admitted with dyspnea on exertion, syncope, and severe cyanosis. Cardiac catheterization revealed severe pulmonary hypertension with moderate right-to-left and slightly left-to-right shunt (Eisenmenger syndrome). Right and left ventricular function, as evaluated by angiocardiography, was slightly reduced. Because of the severe hemodynamic alterations, symptomatic therapy with digitalis, repeated venesection, and anticoagulation was initiated.
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PMID:[The oto-vertebral syndrome]. 70 8

Congenital heart lesions are found in 0.8% of all newborns; four-fifths of them are correctable. Two thirds of all surgical procedures are done in acyanotic malformations, mainly in septal defects, patent ductus arteriosus, coarctations of the aorta and valvular stenosis. Within the last twenty years corrective repair has been established with good results in these lesions. Therapeutic problems still remain in cases of late diagnosis and in some rare and complex anomalies like malformations of the aortic arch, of the coronary arteries, in some types of aortopulmonary window and atrioventricular canal. Surgery becomes urgent in babies suffering from severe heart disease because of progressive pulmonary hypertension, inadequate growth or severe heart failure (despite intensive medical treatment). For certain lesions corrective repair (patent ductus arteriosus, coarctation) is definitely indicated, for others (ventricular septal defect, atrioventricular canal) urgent palliation or early total repair is debatable. In most patients with uncomplicated lesions treated in time during childhood a low operative mortality below 1% was observed. A higher mortality rate was found (10 to 50%) in patients with pulmonary artery disease or associated pulmonary lesions. A significantly higher mortality could also be observed in the advanced age group (4.7%) and in infants below one year of age (11,5%), underlining the important point of the age at operations. Recurrence of lesions occured in 1% of our patients after closure of ventricular septal defects, reconstructions of mitral valve disease and early corrected coarctations. Nevertheless, most of these patients can expect a normal growth, life span and ability to work.
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PMID:[Surgery of congenital acyanotic cardiac malformations (author's transl)]. 72 Oct 31

In young children with congenital heart disease the pulmonary circulation is exposed to abnormal haemodynamic conditions before it is fully developed. In the newborn infant the persistence or development of pulmonary hypertension rapidly leads to structural change. The speed with which an increase in muscularity can develop has hitherto been underestimated. In most children dying in early infancy with congenital heart disease and pulmonary hypertension the presence of thick walled small arteries is due not to persistence of the high wall thickness of foetal life, but to a rapid postnatal response of the pulmonary circulation to pulmonary hypertension. In older patients with a ventricular septal defect, aged between 3 months and 4 years, the presence of pulmonary hypertension has been shown to interfere with the growth and development of the pulmonary circulation, judging this by reduction in size and multiplication of intra-acinar arteries and an increase in muscularity of both pre and intra-acinar arteries and veins. In these patients elevation of pulmonary vascular resistance was associated with failure of the intra-acinar pulmonary circulation to develop normally and not with obliterative pulmonary vascular disease. Recent studies indicate that growth and development of the peripheral pulmonary circulation can be quantitated in lung biopsies taken from infants and young children with congenital heart disease. It should therefore be possibe to correlate structure and function at a critical period of lung development, before the changes of obliterative pulmonary vascular disease are established.
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PMID:The pulmonary circulation in congenital heart disease. II. Pulmonary hypertension. 72 Oct 42

Five children with polysplenia syndrome are described. Cardiac catheterization or postmortem examination revealed the following cardiac anomalies: interruption of the inferior vena cava with azygos (or hemiazygos) continuation to the left superior vena cava and a single atrium or a large atrial septal defect in all five children; a ventricular septal defect in three; and a primitive ventricle in one case. Other anomalies found were: central liver in all five; a right-sided stomach in three; and multiple small spleens and bilateral left, bilobed lungs--found on autopsy--in two of the children. In four of the five patients, whose ECG was available, a negative P wave was present in leads II, III and AVF. This leftward and superiorly directed P wave axis should suggest a diagnosis of polysplenia syndrome in an infant with congenital heart disease. The cardiac anomalies are surgically correctable; therefore, early recognition of this syndrome is of practical importance.
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PMID:Polysplenia syndrome. A study of five new cases. 72 22

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.
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PMID:Sudden unexpected death in children with congenital heart disease. 80 25

Infants with bounding arterial pulses frequently show a visible or palpable arterial pulsation of the fontanel. This simple clinical sign is neither described in the textbooks nor mentioned in connection with congenital heart diseases (CHD). To investigate its significance 501 infants were examined 178(35,5%) showed an arterial pulse of the fontanel. Out of 421 with no sign of cardiovascular malformation or disease on physical examination, 111(26,4%) had a fontanal pulse (f.p.). Out of 80 infants with CHD, 67(83,8%) exhibited a fontanel pulse (f.p.), while only 13(16,2%) did not. All infants with CHD had an ECG and biplane chest x-rays taken. In some of them also cardiac catheterization and heart or vessel surgery were performed. 23(79,3%) of 29 infants with PDA and eight of twelve with a VSD had a f.p.. Since other cardiac diagnoses were encountered less frequently, their relation to the presence or absence of f.p. could not be evaluated. From the variety of diagnoses, however, it became obvious that not only lesions with "early aortic runoff" may be accompanied by a f.p. Other clinical features like blood pressure, hemoglobin content and body temperature were discussed as possible causes of f.p.. Only the first mentioned, a high systemic pressure, was frequently accompanied by f.p.. Since the percentage of infants with f.p. and CHD was relatively high (37,6%) this clinical sign is considered to be of some value in the early detection of heart disease in infancy.
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PMID:[Arterial fontanel pulsation]. 83 92


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