Marshall B. Kreidberg

Cardiac and coronary arterial involvement in infants and children from New England with mucocutaneous lymph node syndrome (Kawasaki disease). Angiocardiographic-echocardiographic correlations.

Forty-four patients, aged 6 months to 11 years, meeting the clinical criteria for mucocutaneous lymph node syndrome (Kawasaki disease) were studied from March 1980 to March 1981. A protocol was designed for evaluation of cardiac involvement including electrocardiogram, chest X-ray film, cardiac enzyme determinations and M mode and two dimensional echocardiograms. Aspirin was given during the acute febrile period as an anti-inflammatory agent in a dosage of 100 mg/kg per day; followed by 30 mg/kg per day for 10 weeks or longer if platelet counts were elevated. Angiocardiography was performed in 38 patients. Twenty-one patients (48 percent) showed abnormal M mode echocardiographic findings (flat or greatly decreased ventricular septal motion, pericardial effusion or decreased left ventricular function) during the acute febrile stage. Coronary artery disease developed in seven patients, all of whom had abnormal M mode echocardiographic abnormalities in the acute stage. Five patients had coronary aneurysms and two patients had dilated coronary arteries. Two dimensional echocardiograms identified coronary arterial lesions accurately if present proximally but failed to detect aneurysms beyond 1.5 cm from the aortic root. The study shows that serial M mode and two dimensional echocardiograms provide sufficient information to rule out cardiac involvement in Kawasaki disease. Echocardiograms should be obtained every 3 to 4 days during the acute febrile stage because all patients with coronary artery disease detected with angiography had abnormal echocardiographic findings during that period.

Combined discrete subaortic stenosis and ventricular septal defect in infants and children.

Eight patients, aged 1 to 8 years, with discrete subaortic stenosis (DSS) and ventricular septal defect (VSD) were studied by 2-dimensional (2-D) and M-mode echocardiography. Initial cardiac catheterization and angiocardiography showed VSD and other associated cardiac lesions, including coarctation of the aorta and patent ductus arteriosus. None had evidence of DSS. Six patients underwent surgical repair of the associated lesions, but none required closure of the VSD. Ultimately, 6 patients had spontaneous closure of VSD, and 2 had a residual small VSD. Subsequent serial echocardiography showed evidence of subaortic membrane, prompting repeat cardiac catheterization, which confirmed moderate to severe peak systolic pressure gradients between the left ventricle and ascending aorta. Surgical resection of the membrane was performed in 5 patients. Thus, in patients with small or spontaneously closed VSDs, DSS may develop. Evaluation of the left ventricular outflow tract area is recommended in patients with small or closed VSD in whom a significant heart murmur or electrocardiographic abnormality remains.

Ineffectiveness of penicillin prophylaxisin cardiac catheterization

In a total of 452 cardiac catheterizations, penicillin prophylaxis was given to oneseries of patients and withheld from another. Statistical analysis of the bacteriologic findings in these patients showed that the incidence of bacteremia and fever was not lessened by penicillin prophylaxis. Febrile reactions appeared to be the result of pyrogens. In no instance did bacterial endocarditis develop. The fact that antibiotic prophylaxis did not lower the incidence of significant bacterial cultures or fever following cardiac catheterization supports the contention that its use is not warranted in this procedure.

Ellis-van Creveld Syndrome

SYDNEY S. GELLIS, M.D. Professor and Chairman, Department of Pediatrics, and Pediatrician-In-Chief, Tufts-New England Medical Center (Boston Floating Hospital for Infants and Children) Dr. Jankc~ski Today we are going to discuss the Ellis-van Creveld syndrome. Dr. Johnson will present the case. Dr. Johnson: Our patient is a four and onehalf year-old white girl who was the product of a 40 week normal pregnancy. The 27-year old gravida III/para III white mother had no serious illness. She did not require medication during this pregnancy. Labor was three hours

Paroxysmal auricular tachycardia associated with Wolff-Parkinson-White syndrome in a newborn infant.

Summary This is a case report of a 48-hour-old infant hospitalized because of cyanosis and rapid respiration. Besides the common complications of paroxysmal tachycardia and congestive failure, 9 there were electrocardiographic evidences of paroxysmal tachycardia and of the Wolff-Parkinson-White syndrome. The congestive failure and paroxysmal tachycardia responded clinically and electrocardiographically to digitalization, the rhythm and rate returned to normal, and the cardiac dilatation subsided, but evidence of the Wolff-Parkinson-White syndrome still persisted on electrocardiograms.

Clinical evaluation of corticotropin therapy in children.

Summary Twenty-eight children were treated with corticotropin. Favorable results were obtained in bronchial asthma, non-thrombocytopenic purpura, dermatitis venenata, rheumatic fever, Sydenhams chorea, rheumatoid arthritis, and the nephrotic state. The course of subacute lymphatic leucemia was only temporarily modified. Beneficial results were not obtained in glomerulonephritis, Wilsons disease, polymyositis, and thrombocytopenic purpura. The major untoward effects observed as a consequence of therapy were convulsions and hypertension. Corticotropin represents a valuable and relatively safe addition to the therapy of a number of serious diseases of children.

Systemic to pulmonary anastomosis in truncus arteriosus with reduced pulmonary blood flow

Abstract Persistent truncus arteriosus is not a common anomaly. Two percent of children with potentially correctable variants (types I, II and III) have reduced rather than increased pulmonary blood flow. It is possible to provide palliation for such infants with a systemic to pulmonary anastomosis, allowing them the chance for a second stage total correction at a later age. Such a palliative procedure is described in a 6 day old infant with cyanosis.

Anastomosis of the ascending aorta to the main pulmonary aorta

Abstract The difficulties encountered in the performance of the Blalock-Taussig 1 and Potts 2 anastomoses in certain children led to the development 3 and popularization in the United States 4,5 of intrapericardial anastomosis between the ascending aorta and the right pulmonary artery. Although this shunt is applicable to almost all patients, an occasional patient has an anatomic arrangement that does not allow this anastomosis. In such an instance it may be possible to create a shunt between the ascending aorta and the main pulmonary artery.