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

Since 1970 46 infants and children with Down's Syndrome were subjected to palliation of congenital heart disease, and 54 mongoloid children underwent correction of their cardiac defects. The most common cardiac malformation was endocardial cushion defect (72%). Palliation consisted in pulmonary artery banding (PAB) with or without division of a patent ductus arteriosus (PDA) in 16 infants, and sole division of a PDA in another 16 infants with large left to right shunts due to common canalis atrioventricularis (avcanal) or ventricular septal defect (VSD). An aorto-pulmonary anastomosis was performed for relief of severe hypoxia due to right ventricular outflow tract obstruction (RVOTO) in 14 patients. Operative mortality was 41% for PAB, 21% for aortopulmonary anastomosis, anastomosis, and 0% for division of a PDA. In the group of corrective cardiac surgery the operative mortality rate was 0% in ostium primum defects and 4% in VSD closure. Correction of complete av-canal and of malformations with RVOTO had a high mortality rate of 20 and 40%, respectively. Similar results were obtained in nonmongoloid children operated upon for the same cardiac defects. The complications and causes of death were due to the complexity of the cardiac malformations and had no relation to the Down's Syndrome itself. Mongoloid children do not pose additional medical problems to management of congenital heart disease. Their results do not differ from those obtained in nonmongoloid children.
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PMID:[Palliative and corrective cardiac surgery in Down's syndrome (author's transl)]. 6 15

The authors report two cases of infants with complex heart disease comprising transposition of the great vessels, and a major unilateral facial anomaly involving the ear and the mandible, conforming to the description of hemifacial microsomia. The facial anomalies seen are defined and placed in the classification of known syndromes. The incidence of heart disease associated with facial microsomia is about 20% and in 2/3 of cases it is a ventricular septal defect or a tetralogy of Fallot. An analysis of aetiological factors shows that syndromes of the first two branchial arches are usually sporadic and that they may be the result of intra-uterine disease. The mechanism of the facial lesions is probably vascular.
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PMID:[Hemifacial microsomia and cardiac malformations. Apropos of 2 cases]. 10 88

Sixty-nine children with Down syndrome (mongolism, trisomy 21), with atrial septal defect, patent ductus arteriosus, ventricular septal defect, or endocardial cushion defects, and 315 children with similar cardiac anomalies without this syndrome underwent cardiac catheterization during an 8-year period from 1964 to 1973. Only patients under 17 years of age were included in the study. Nine tenths of the children with Down syndrome but only one fourth of the control group had abnormally high pulmonary arterial pressures. For example, 9 of 11 children with defects of the atrial septum and Down syndrome had pulmonary hypertension; in contrast, only 5 of 55 control subjects with similar defects had pulmonary hypertension. The data suggest that children with congenital heart disease and Down syndrome have an unusually high pulmonary vascular resistance and a propensity for early development of severe damage to the pulmonary vascular bed.
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PMID:The pulmonary vascular bed in children with Down syndrome. 12 55

Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.
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PMID:Down syndrome with congenital heart malformation. 13 59

The Authors are examing two cases of A-V canal associated with T. of Fallot. The most important clinical aspects of this rare cardiopathy result in the following: -- the frequent coexistence with Down dyndrome; -- the ECG and VCG aspects of counterclockwise rotation of the initial vectors of QRS in the frontal plane; -- the simultaneous observation of the typical echocardiographical aspects of both kinds of cardiopathy. The injection of dye into both ventricles allows the precise diagnosis. The post-mortem study in both cases shows the morphology of A-V canal associated with the one of T. of Fallot. From the surgical point of view it is important to take into account the abnormal morphology and extension of VSD. The importance of the part that the conal cushion plays in the morphogenesis of the malformation is discussed.
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PMID:[Atrioventricular canal associated with Fallot's tetralogy. Anatomo-clinical study of 2 cases]. 15 62

Clinical studies have suggested that patients with Down syndrome have precocious development of pulmonary hypertension, even in the absence of congenital heart disease. To examine the pathologic basis of this impression, we studied 82 patients with Down syndrome autopsied at The Johns Hopkins Hospital from 1913 to present. The patients ranged in age from 26 hours to 25 years; 41 (50%) were female. Atrioventricular canal defect was present in 40 patients, nine of whom also had pulmonary stenosis. Eight had isolated ventricular septal defect and seven had other malformations. Histologic sections of the lung from each patient were studied and the degree of hypertensive pulmonary vascular disease graded. Age and sex-matched controls without cardiovascular manifestations, all other autopsied patients with atrioventricular canal defect not associated with Down syndrome, and age-matched patients with ventricular septal defect or other malformations were similarly studied for pulmonary vascular changes. Comparison of the 27 patients with Down syndrome and no cardiovascular malformations with normal controls showed no differences, with one notable exception: an 8-month-old child with Down syndrome had severe HPVD secondary to idiopathic pulmonary hypertension. HPVD in patients with the various cardiovascular malformations was similar for those groups with Down syndrome and those without, when HPVD was considered as a function of the patients' age and the type of malformation. We conclude that, in general, patients with Down syndrome have no predisposition to develop severe or precocious hypertensive pulmonary vascular disease.
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PMID:Hypertensive pulmonary vascular disease in Down syndrome. 15 39

The authors report the study of 259 cases of congenital heart disease observed at Abidjan between 1969-1976. The average age of the patients was 7-8 years ranging from the newborn to 41 years. The principal malformations encountered in their order of frequency were: ventricular septal defect 38.6 p. 100, atrial septal defect 13.8 p. 100, Fallot's tetralogy 8.8 p. 100, pulmonary stenosis 8.1 100, patent ductus arteriosus 7.7 p. 100, atrioventricular canal 7.7 p. 100, transposition of the great arteries 3.8 p. 100, coarctation of the aorta 2.3 p. 100. There were associated malformations in 31 cases. 42 patients were catheterised at Abidjan, 28 were operated, 12 at Abidjan. These statistics are compared to those already reported of congenital heart disease in tropical and western countries. There is little difference in the distribution of the various cardiac malformations. Two congenital malformations appear to be less frequent in the Tropics: coarctation of the aorta and aortic stenosis.
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PMID:[Congenital cardiopathies in a tropical environment. Study of 259 cases seen at Abidjan from 1969-1976]. 22 23

Lung biopsy specimens were taken from 39 infants younger than 12 months of age with congenital heart disease and severe pulmonary hypertension (Pp/Ps greater than or equal to 0.75) accompanied by respiratory distress. The pathological change in lung specimens and clinical courses were compared. These 39 infants underwent surgical treatment of patent ductus arteriosus (PDA), seven patients; ventricular septal defect (VSD), 13 patients; and complex heart anomaly, 19 patients. The common pathological findings of the lung specimens taken from these infants were lymphoid cellular infiltration and thickening of the alveolar septum, which we have called "septitis" in the present study. In most cases pulmonary vascular obstructive change was within Grade 2 of the Health-Edwards criteria. Septitis was classified into three categories: mild, moderate, and severe. Only three of the 19 infants with severe septitis survived postoperatively, whereas 10 of the 12 infants with moderate septitis and all eight with mild septitis could be successfully weaned. The cause of septitis remains unidentified. We have found the patient's age and pulmonary hypertension to be closely related to the grade of septitis in this study. Septitis plays a much more important role than pulmonary vascular obstructive change in the prognosis of pulmonary hypertensive heart disease in early infancy.
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PMID:Lung pathology in infants with severe pulmonary hypertension and cardiac disease. 43 Nov 7

The Hancock conduit that contains a porcine xenograft valve has been used in part of the cardiac repair of 22 patients with complex congenital heart disease. Five patients had a severe form of tetralogy of Fallot; six had pulmonary atresia; five had transposition of the great vessels, ventricular septal defect (VSD), and pulmonic stenosis; five had truncus arteriosus; and one had "corrected" transposition, VSD, and pulmonic stenosis. The hospital mortality was 2/22. This conduit has proved a satisfactory method to establish right ventricular-pulmonary artery continuity.
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PMID:Discontinuity between the heart and the pulmonary circulation. Its management with the use of a valved external conduit. 43 31

During the years 1963--73, 276 children with congenital heart disease were admitted to this hospital during their first month of life. Ventricular septal defect was the most common cardiac anomaly and this lesion, together with transposition of the great arteries, comprised 35% of all cardiovascular malformations. Extracardiac malformations were found in 86 patients. The cumulative survival rate for all patients was 66% in the first month of life and 33% in the first year. Forty-three patients were operated upon, but it is estimated from necropsy reports and available clinical data that another 74 patients, who died without operation, would have been suitable candidates for total corrective surgery.
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PMID:Congenital heart disease in the first month of life. 47 69


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