Suzanne J. Vobecky

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Succinyl CoA: 3-oxoacid CoA transferase (SCOT; E.C.2.8.3.5) mediates the rate-determining step of ketolysis in extrahepatic tissues, the esterification of acetoacetate to CoA for use in energy production. Hereditary SCOT deficiency in humans causes episodes of severe ketoacidosis. We obtained human-heart SCOT cDNA clones spanning the entire 1,560-nt coding(More)
The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had(More)
In 36 patients who consulted for fecal incontinence or rectal pain, or both, there was grossly visible scarring of the rectum and biopsy revealed mucosal atrophy and fibrosis. A steal from the hemorrhoidal arteries to the iliac vessels was demonstrated in 3 subjects. Maximum tolerable volumes within a rectal balloon were smaller than in control subjects,(More)
Severe hyperammonemia (hyperNH3) in neonatal cardiac failure after cardiac surgery is rare. We report a case of a 2470-g female infant born at the week 37 of gestation with complex congenital heart disease (truncus arteriosus type III, interrupted aortic arch and tricuspid valve insufficiency) and hemodynamically non-significant intrahepatic arterio-venous(More)
BACKGROUND Isolated hemodynamically significant ventricular septal defects (VSD) were previously treated surgically. Since the introduction of percutaneous (PC) devices, the management of isolated VSD has evolved. In our center, Amplatzer devices have been implanted for selected isolated perimembranous VSD since 2002. METHODS The charts of all isolated PC(More)
In a patient complaining of constipation since birth, delayed transit time in the ascending colon was related to a congenital malformation of the hindgut, different from Hirschsprung's disease. This was associated with absence of the left lobe of the liver. There was no propagating electrical muscular activity in the distal bowel.
OBJECTIVES The purpose of this study was to characterize peripheral flow kinetics in response to progressive discontinuous maximal exercise in 10 patients who underwent repair of coarctation of the aorta and 11 age-matched healthy adolescents. BACKGROUND An impairment of leg blood flow has been suggested on the basis of exaggerated femoral muscle lactate(More)
Mucor indicus is a rare, emerging cause of zygomycosis with 7 cases previously reported since 1975. We report the first case of endovascular M. indicus infection in a pediatric recipient of the Incor (Berlin Heart AG, Germany) implantable left ventricular assist device, and review the literature to describe the broadening clinical spectrum of zygomycosis(More)
Hybrid procedures that combine surgical and interventional techniques are increasingly utilized in patients with congenital heart disease. We performed a novel hybrid intervention involving transcatheter ablation via a sternotomy approach and atriotomy incision. A recalcitrant accessory pathway was successfully ablated in a patient with hemodynamically(More)
OBJECTIVE To characterise cardiopulmonary baroreflex responses and examine the effects of a 45 minute cycling bout late after successful repair of coarctation of the aorta. SUBJECTS 10 young adults (mean (SEM) age 18.1 (2.6 years)) operated on for coarctation of the aorta 12.7 (3.5) years earlier, and 10 healthy controls. DESIGN Forearm blood flow(More)