Vera Rose

Outcome of coronary artery aneurysms after kawasaki disease

From 1974 through 1991, a total of 583 children with Kawasaki disease were seen at the Hospital for Sick Children, in Toronto, of whom 80 (13.7%) had coronary artery involvement. There were 55 boys and 25 girls, whose mean age at onset was 2.9 +/- 2.5 years, followed for a mean period of 4.0 +/- 3.6 years. Giant aneurysms (maximum diameter > or = 8 mm) were found in 22 children, moderate-sized aneurysms (> or = 4 to < 8 mm) in 44, and dilation lesions (< 4 mm) in 14. Myocardial infarction occurred in 9 (1.5%), all of whom had giant aneurysms. The persistence rate for aneurysms was 72% at 1 year and 41% at 5 years of follow-up. In multivariate analysis, the regression of an aneurysm was significantly related to the severity of coronary artery lesions, initial treatment, and gender. Although > 80% of small or moderate-sized aneurysms regressed within 5 years, giant aneurysms did not regress during the follow-up period. In patients who received immune globulin therapy, coronary lesions tended to resolve more rapidly than in those treated with salicylate therapy alone, because 91% of the lesions in the former were small or moderate. These findings suggest that the severity of coronary artery involvement during the initial stages of Kawasaki disease influences the regression of these lesions, and that immune globulin treatment may improve outcome by reducing the incidence of severe lesions.

Kawasaki disease: Review of risk factors for coronary aneurysms

Between June 1, 1979, and May 31, 1984, at The Hospital for Sick Children in Toronto, Kawasaki disease was diagnosed in 163 patients (112 boys, 51 girls, P less than 0.001). Fifteen percent of the children had coronary artery aneurysms. Prior to diagnosis, 24% had been given low doses of aspirin, and 50% acetaminophen. Children with coronary aneurysms had significantly higher temperature during days 10 to 13 of the disease. The febrile phase of the disease was also significantly longer in these children. Coronary artery involvement occurred with equal frequency in boys and girls. There was no significantly greater incidence of coronary artery involvement in infants younger than 1 year of age than in older children. Duration of fever (greater than or equal to 14 days vs less than 14 days) was equally as predictive of the eventual occurrence of coronary aneurysms as the modified Asai score.

A Possible Increase in the Incidence of Congenital Heart Defects Among the Offspring of Affected Parents

The incidence of congenital heart disease in the children of 219 probands was determined. Each of these probands had one of four selected defects: atrial septal defect, coarctation of the aorta, aortic valve stenosis or complex dextrocardia. Of their children, 8.8% had substantial congenital cardiac defects. This is a much higher incidence than that reported in most comparable studies. The difference is highly significant statistically and therefore is likely to be genuine. The cause of the high recurrence is probably environmental.

Incidence of Bacterial Endocarditis in Ventricular Septal Defects

In summary, the population figures presented place the risk of bacterial endocarditis for cases of ventricular septal defect in the 5 to 14-year age group as 1 in 470 patient years, or 2.1 per 100 cases in 10 years. The estimated risks for the 5-year-old and 15-year-old patient up to the age of 70 years would be 13.6% and 11.5% respectively. In the city of Toronto, only four cases were seen in 10 years in the general population of over half a million, while in metropolitan Toronto with the population of 1.3 million, eight cases were seen in a similar period. If the clinical experiences of the various authors reported in the literature are pooled with that at The Hospital for Sick Children in Toronto, one would have 21 cases of bacterial endocarditis in 11,328 patient years. This would place the incidence as approximately 1 in 500 patient years.It is suggested that the surgery for ventricular septal defect may itself initiate bacterial endocarditis as noted in three of our cases.On the basis of the available data, there is insufficient evidence to designate the risk of future bacterial endocarditis as an adequate reason for surgical closure of the defect.

Ventricular septal defect Incidence, morbidity, and mortality in various age groups

The true incidence of isolated ventricular septal defect and its subdivisions is difficult to estimate accurately, since the data available in the past have been obtained in a variety of incomplete ways. Necropsy studies usually included the complicated as well as the isolated cases. The clinical diagnostic criteria were less accurate than those used currently. Some studies dealt only with adults; other reports omitted infants or took patients into the investigation series at different ages. It is reasonable to conclude that no final figures will ever be arrived at, since the factors affecting prevalence data are changing continuously and no two studies are identical or complete. However, it is worth while considering some of the information obtained in the past from certain specific points of view.

Ventricular septal defect: Clinical and hemodynamic changes in the first five years of life

Abstract Two hundred consecutive infants with isolated ventricular septal defects were assessed clinically and hemodynamically and then followed throughout the first 5 years of life. Those infants who had flow ratios less than 2 to 1 generally became asymptomatic because of diminution in size of the defect. Complete closure occurred spontaneously in 20 per cent. The majority of infants had flow ratios greater than 2:1 and in these the natural evolution varied. At least 15 per cent of these infants may die from their cardiac lesion in infancy. However, approximately 40 per cent of those who survive will have closed defects, and 20 per cent of those who show this reduction in size will have spontaneously closed defects by 5 years of age. Those children in whom the large defect persists remain symptomatic and fail to thrive. There is little or no change in clinical, radiologic, or electrocardiographic findings up to the time of surgery, but hemodynamic reassessment indicates that at least 20 per cent of such children may develop progressive pulmonary arteriolar disease and that such changes, when they are going to occur, will be evident hemodynamically in the second year of life. When all the children with flow ratios greater than 2 to 1 are considered, the mortality rate was 7 per cent; 9 per cent required palliative surgery in infancy; 6 per cent underwent spontaneous closure; and 10 per cent developed the Eisenmenger reaction.

Dilated cardiomyopathy with neutropenia, short stature, and abnormal carnitine metabolism.

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Catecholamine cardiomyopathy: An unusual presentation of pheochromocytoma in children

CARDIOMYOPATHY may be primary or secondary. Primary cardiomyopathy is unrelated to systemic disease or diseases in other organs, whereas secondary cardiomyopathy is associated with other disease complexes or etiologic agents? We report two patients with pheochromocytoma, a catecholamine-se creting tumor, who presented to The Hospital for Sick Children, Toronto, with cardiomyopathy.

The hemodynamics in ventricular septal defect in childhood

Abstract (1) Hemodynamic groups are defined, and the frequency in a “catheter laboratory population” of isolated ventricular septal defects is described. The cases of 247 patients 1 to 16 years old are analyzed and considered, together with 151 previously presented cases of patients under 1 year of age. (2) A natural history is suggested; the majority of septal defects are benign; some defects get smaller or close; a few patients develop progressive pulmonary vascular obstruction. (3) The pulmonary vascular obstruction may progress after surgical closure of the defect. (4) No “congenital Eisenmengers” were found. (5) Those who developed progressive pulmonary vascular obstruction had greatly increased pulmonary blood flow early. (6) It is recommended that a careful watch be kept on “high-flow” patients in the first few years of life, and that they be restudied early to pick out the developing Eisenmenger reaction, with a view to prompt operation.

Endocardial fibroelastosis: Family studies withspecial reference to counseling

Primary endocardial fibroelastosis of the left ventricle was studied in 119 families. Themale to female sex ratio was 0.63. The mean maternal age of 27.2 years (S.D. 5.6 years) and the mean paternal age of 31.1 years (S.D. 6.2 years) were normal. The mean birth rank was significantly higher for patients than for sibs. Nine families had multiple affected children, including 7 with 2 affected sibs and 2 with 3 affected sibs. The family data did not fit either the recessive or the multifactorial mode of inheritance. The sib incidence in the families of affected girls was 4.3 per cent and in the family of affected boys 2.9 per cent. After the birth of the first affected child the sibling incidence was 17.7 per cent (11/62), and after the second affected child, 33.3 per cent (2/6). The empiric risk figures for sib incidence found in this study may be useful in genetic counseling.