The reported prevalence of congenital heart defects (CHD) in the oculoauriculovertebral "spectrum" (OAVS) is 5-58%. This variability is mainly due to differences in the diagnostic criteria used, and to ascertainment bias introduced by selection of patients from different specialty databases. Two-thirds of the cardiac anomalies reported are either tetralogy of Fallot (TOF) or ventricular septal defect (VSD). We found a CHD frequency of 19% in a series of 32 patients identified either through a genetic or autopsy database. The cardiac lesions were much more varied and complex than previously reported and included asplenia syndrome, ventricular inversion associated with double outlet right ventricle, pulmonary atresia with VSD, double outlet right ventricle, and infradiaphragmatic total anomalous pulmonary venous connection. Pulmonary and renal abnormalities were more common in OAVS patients with CHD than in those where the heart was normal.
Cancer Hazards-Bonser clinician of the significance to him and his patent of what has been discovered in the laboratory. Means must be found to bridge this gap.
Background-Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. Observations-Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a closed ductus arteriosus within the first two hours of birth while on prostaglandin E, infusion.Conclusions-In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.
ABSTRACT. Objectives. To determine the prevalence of Kawasaki disease in older children and to evaluate its clinical presentation, time to diagnosis, and outcome in comparison with younger patients with the disease.Methodology. A retrospective analysis of all patients discharged with a diagnosis of Kawasaki disease at a pediatric tertiary care hospital over a 12-year period.Results. A total of 133 patients were included in this study; 7.5% were 9 years of age or older at the time of illness. Patients were grouped by age: infants included children age 1 to 8 years of age and children 9 years of age or older. Older children had a higher frequency of abnormal cardiovascular physical examination (50%) versus children (6%) and infants (10%). The older age group and the infants had a higher prevalence of coronary artery abnormalities and poor left ventricular function than did the 1-to 8-year-olds. Eighty percent of the older children had coronary arteries that were either dilated or aneurysmal, and 30% demonstrated left ventricular dysfunction on initial echocardiography. The number of days to diagnosis after meeting the diagnostic criteria was 5.8 ؎ 2.3 for infants, 5.2 ؎ 1.5 for older children, and 1.9 ؎ 0.3 for children. Older children had a complicated course of Kawasaki disease compared with younger patients.Conclusion. We found a higher prevalence of older children with Kawasaki disease at our center than has previously been reported. Older patients, as well as infants, had a higher rate of coronary artery abnormalities than did the children between 1 and 8 years of age. Older age at the time of illness or a delay in treatment may be important factors in determining cardiac involvement in Kawasaki disease. Pediatrics 1998;102(1). URL: http:// www.pediatrics.org/cgi/content/full/102/1/e7; Kawasaki disease, coronary arteries, intravenous immunoglobulin.ABBREVIATIONS. KD, Kawasaki disease; CVS, cardiovascular; ECG, electrocardiography; LV, left ventricular; WBC, white blood cells; ESR, erythrocyte sedimentation rate; IVIG, intravenous immunoglobulin; ASA, acetylsalicylic acid. S ince its first description in the Japanese literature in 1967, 1 much has been written about Kawasaki disease (KD). A systemic vasculitis, KD is characterized by fever, mucocutaneous manifestations, and musculoskeletal changes, and its most devastating effects are on the heart, particularly the coronary arteries. The etiology of KD remains unknown, and its presentation can be diverse.2,3 The illness usually affects infants and children younger than 3 years.1 KD is extremely uncommon in patients 9 years of age and older. 4 -6 The most recent epidemiologic survey from Japan found that Ͻ1% of cases occurred in children 9 years of age and older. 6 Our experience suggested that KD affecting older children was more common than reported previously. Our clinical impression was that most of the older patients were diagnosed late in the course of their illness and, consequently, had more morbidity associated with the illness. Of interest to us were...
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