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This study of individuals with familial and sporadically occurring 46 XX or XY Turner phenotype documented a wide range of rightand left-sided cardiovascular abnormalities and a previously unreported eccentric hypertrophy of the left ventricle. A mother and five of her seven children had abnormal cardiovascular findings. Five had an abnormal(More)
Postoperative fever and pericardial-pleural reaction, designated postpericardiotomy syndrome (PPS), is a common complication of cardiac surgery involving entry into the pericardium. To determine whether the etiology of PPS is viral or immunologic, we undertook a prospective, triple-blind study of consecutive long-term survivors of intrapericardial surgery(More)
Sixty-two infants and children with the Wolff-Parkinson-White syndrome (WPW) were under long-term follow-up at The New York Hospital over a 20-year period. Twenty had associated congenital heart disease. In 29 infants and 6 children in hospitalfor paroxysmal supraventricular tachycardia, digitalis relieved the episodes in all but one instance and prevented(More)
Mitral valve prolapse (MVP) is known to be associated with thoracic skeletal anomalies. To determine the incidence and risk factors for mitral valve prolapse in the adolescent population with severe idiopathic scoliosis (IS), a prospective follow-up study on 139 adolescent patients with IS from the Pediatric Orthopedic Service was undertaken. Data collected(More)
To determine whether survival of patients with beta-thalassemia major has been prolonged by management that utilizes hypertransfusion and chelation with deferoxamine, we analyzed longevity by the Kaplan-Meier product-limit method. Group 1 patients (n = 71) followed between 1960 and 1976 with a low-transfusion regimen (pretransfusion hemoglobin level 7 to 8(More)
Two cases of pulmonary lymphangiectasia associated with Noonan's syndrome are described. Chest films showed diffuse pulmonary intersitial infiltrates from infancy. Lymphangiograms demonstrated obstructive changes and collateral formation in the retroperitoneal, mediastinal, pulmonary, and cervical lymphatics and extensive opacification of the pulmonary and(More)
Systematic, prospective data regarding phenotypic features, including echocardiographic findings, in pediatric patients with the Marfan syndrome are lacking. In addition, limited and conflicting information exists regarding the impact of pharmacologic therapy on aortic growth rate in children. Fifty-three children and adolescents with the Marfan syndrome(More)
His bundle electrograms and atrial pacing are reported in a 10 1/2-year-old boy and a 12-year-old girl, both of whom underwent Mustard repair for complete d-transposition of the great arteries. Each patient had progressive evidence of sick sinus syndrome culminating in syncope. Improvement occurred after insertion of a demand pacemaker.
GLYCOGEN-STORAGE DISEASE of the myocardium is a rare hereditary error of carbohydrate metabolism in which excessive quantities of the carbohydrate accumulate in the heart muscle and other tissues. In 1950 di Sant'Agnese et al.1 reviewed the literature and accepted only 14 cases that fulfilled their criteria for diagnosis of the disease. Since then 39 more(More)