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Biomedical subjects

R Dansky

Publications and source records attributed to R Dansky.

13 recordsLinked to original sources

Surgical correction of ventricular septal defects in black and white children--an 11-year study.

The clinical and ECG features, anatomical subtypes and outcome in 309 children (169 black; 140 white; 58% female) who underwent surgical closure of ventricular septal defects (VSDs) are presented. Clinical presentation was more severe in the black children; with recurrent pulmonary infections in 65% blacks compared with 34% whites. Congestive cardiac failure was noted in 60% blacks and in 37% whites. At cardiac catheterisation a left to right shunt greater than 2.5/1 was found in 45.5% of the black and in 39.3% of the white children. Severe pulmonary hypertension (greater than 80% of systemic systolic pressure) was evenly distributed in both groups. Of the 140 white children, 74.3% underwent surgery under the age of 2 years compared with 68.6% of the 169 blacks. A perimembranous VSD was found in 65% of patients in each group. Infundibular (muscular outlet) defects were observed in 28.6% of white and 30.8% of black children. Left axis deviation (LAD) on ECG was found in 11.9% of white and 9.2% of black patients; and 93% of the total of 27 cases had a perimembranous defect. Early mortality was 3.6% in white and 7.1% among the black children. Of the 17 fatal cases in the total group, 16 had severe pulmonary hypertension. It is concluded that: (i) operative results compared favourably with those reported elsewhere; (ii) the anatomical subtypes occurred with equal frequency in both ethnic groups; and (iii) this was also the case for LAD on ECG, which was most commonly associated with a perimembranous VSD.

Child↗

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta.

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.

Aortic Coarctation↗

Infective endocarditis in black South African children: report of 10 cases with some unusual features.

Ten black South African children with infective endocarditis seen over a 2-year period are reported. In five cases, including two neonates, the infection was nosocomial and in five cases it occurred in children with previously normal hearts. Of the bacteria isolated from nine cases, five were Staphylococcus aureus (all from nosocomial cases), one was Haemophilus influenzae and three were corynebacteria. The unusual aspects of this series are discussed, with an emphasis on preventing nosocomial cases and on making the diagnosis in children without underlying heart disease.

Black or African American↗

A disproportionately high incidence of symptomatic coarctation of the aorta in white infants in the Transvaal.

A retrospective study of Johannesburg Hospital records revealed that during a 4-year period (1978-1981) 49 infants who had been born in the Transvaal had presented with symptomatic coarctation of the aorta in the first year of life. The total number of live births for this period was 92,697. This incidence of 0.529 new cases per 1,000 live births, or 1/1,892 births, is three times higher than that observed in a careful study in the USA. The age at presentation and sex ratio were similar to other reported series. There was no definite seasonal incidence. Two of the patients had siblings with coarctation of the aorta. The exact reasons for the unusually high number of cases which occurred in the years 1979 and 1981 (1/1,405 and 1/1,241 live births respectively) could not be determined. It is suggested that it is probably due to a combination of genetic predisposition and as yet unidentified environmental factors.

Aortic Coarctation↗

Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases.

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.

Aorta↗

Asymmetric septal hypertrophy and hypothyroidism in children.

Any echocardiographic study of two children with hypothyroidism demonstrated the presence of asymmetric septal hypertrophy. One child died aged 11 months, and pronounced thickening of the interventricular septum was confirmed at necropsy. There was also hypertrophy of the left ventricular free wall. Histological examination showed only slight muscle fibre disarray, but there was striking vacuolation and hypertrophy of muscle fibres. In the second case, a child aged five years, the asymmetric septal hypertrophy disappeared after 18 months' treatment with L-thyroxine. Furthermore, other indices of myocardial function also returned to normal. The mechanism producing asymmetric septal hypertrophy in hypothyroidism is unknown. These are the youngest cases in which this association has been reported.

Cardiomegaly↗

Surgical correction of combined supravalvular and valvular aortic stenosis in homozygous familial hypercholesterolaemia.

Although premature coronary atherosclerosis is a well-recognized complication of homozygous familial hypercholesterolaemia, involvement of the aortic root and valve has not been recognized frequently during life. Two cases of supravalvular and valvular aortic stenosis due to familial hypercholesterolaemia are described. Both patients underwent successful surgical correction of these lesions. The importance of relieving left ventricular outflow tract obstruction by a technique of aortic root enhancement and oblique insertion of a prosthesis is stressed.

Aortic Valve↗

The I.C.U. treatment of acute laryngotracheobronchitis in a developing country.

The management of 50 children with acute laryngotracheobronchitis (LTB) in a general I.C.U. in a developing country is presented. The overall mortality was 16%, with no patient demising from LTB. It is stressed that LTB in developing countries is a very different disorder to that seen in Western societies, due to complicating factors such as bronchopneumonia, malnutrition and measles. Guidelines for therapy are proposed.

Bronchitis↗

Lomotil poisoning in children: two case reports.

Lomotil is a proprietary antidiarrhoeal agent in common use. Two cases of Lomotil poisoning, one of them fatal, are described. Symptoms are those of central nervous system depression and atropinism. Treatment consists of gastric emptying, administration of specific narcotic antagonists, cathartics and activated charcoal.

Atropine↗

Atrioventricular septal defect and type A postaxial polydactyly without other major associated anomalies: a specific association.

Four children are described, (three black and one white, two boys and two girls) with type A postaxial polydactyly. All four of them, in addition, had either a partial or complete atrioventricular septal defect (AVSD). None of these children had associated major malformations. Minor anomalies were observed (e.g., two patients with hypersegmentation of the sternal segments, one patient with undescended testes, one patient with hypoplastic lumbar vertebra, and one patient with a degree of craniofacial abnormality). Chromosome analysis was carried out for three of the four patients, and was normal in all of them. It is suggested that there is a specific association between type A postaxial polydactyly and the AVSD found in each of these patients. This picture does not conform to, but bears some resemblance to, the Ellis-van Creveld syndrome.

Child↗