Fresh homografts obtained through a national organ-sharing programme for repair of congenital heart disease.

Homografts have been used in congenital cardiac surgery for over 30 years. We utilized the resources of a national organ-sharing programme to obtain fresh homografts and report their use in correcting cardiac pathologies in 20 children between March 2001 and May 2003. In 16 patients, a valved conduit was used to form a connection between the pulmonary ventricle and the pulmonary artery.

Pseudoaneurysm of the right ventricular outflow tract complicating balloon dilatation for tetralogy of fallot.

Balloon dilatation is one of the treatment options in symptomatic infants with tetralogy of Fallot and hypoplastic pulmonary annulus and pulmonary artery. A balloon dilatation was performed on a 28-day-old infant with tetralogy of Fallot with an appropriate balloon. The patient developed two pseudoaneurysms on the right ventricular outflow tract after the procedure which were diagnosed when the patient was admitted for total correction at 20 months of age.

Dilated cardiomyopathy as the first early complication in a 14 year-old girl with diabetes mellitus type 1.

Diabetic cardiomyopathy (DC) has been reported in type 2 diabetics with short duration of clinically overt diabetes. Impaired left ventricular function has been reported in young patients with diabetes mellitus type 1 (IDDM), but severe cardiomyopathy as the first early major complication of IDDM is very rare. We report a 14 year-old girl with a 5-year history of IDDM and very poor compliance with treatment and follow-up.

Chronic hypoxemia leads to reduced serum IGF-I levels in cyanotic congenital heart disease.

Cyanotic congenital heart disease in children commonly causes more pronounced growth retardation in comparison with acyanotic congenital heart disease. Chronic hypoxemia has been suggested as the cause of poor growth in these patients, but the relationship between serum IGF-I levels and chronic hypoxemia is unclear. Serum IGF-I concentrations, oxygen saturation and nutritional status were evaluated in 29 patients with cyanotic congenital heart disease, and serum IGF-I levels were compared with a group of 20 well-nourished, age-matched control children to assess the relationship between IGF-I levels and chronic hypoxemia.

Percutaneous transvenous balloon mitral valvuloplasty: mid-term results in adolescents.

Six patients with mitral valve stenosis underwent percutaneous balloon mitral valvuloplasty (PBMV) in our department between November 1992 and December 1997. Five patients had rheumatic mitral valve stenosis and one had congenital mitral valve stenosis and Eisenmenger's syndrome with patent ductus arteriosus (PDA). Functional status before PBMV was class IV in two patients, class III in two patients, and class II-III in two patients, as classified by the New York Heart Association (NYHA).

Platelet activation in congenital heart diseases.

The research presented here investigated platelet activation in cyanotic and acyanotic congenital heart diseases (CHD). Children with cyanotic CHD are prone to both thrombosis and hemorrhage. However, patients with acyanotic CHD may also have a mild bleeding disorder.

Non-invasive diagnosis and management of coronary arteriovenous fistula. A case report.

Coronary arteriovenous fistulas are rare anomalies resulting in abnormal communication between the coronary artery and any chamber of the heart. An asymptomatic patient was referred for evaluation of her murmur. Two-dimensional and color Doppler echocardiographic evaluation revealed an enlarged left main coronary artery.

Transient right sided hypertrophic cardiomyopathy in an infant born to a diabetic mother.

Hypertrophic cardiomyopathy (HCM) is a rare primary myocardial disease, characterized by hypertrophy of the left and/or right ventricle. Infants of diabetic mothers (IDM) are at risk for development of HCM, respiratory distress and persistent pulmonary hypertension. A case of severe right sided HCM in an infant born to a diabetic mother is presented.

Effects of high-dose intravenous methylprednisolone in children with acute rheumatic carditis.

In order to measure the effects of high-dose intravenous methylprednisolone (HIVMP) and compare its efficiency with that of oral prednisolone (OP), 18 patients with active rheumatic carditis were studied. Ten patients received OP, while eight patients were treated with HIVMP. Clinical and laboratory responses to treatment were followed by sleeping pulse rate, systolic blood pressure, erythrocyte sedimentation rate (ESR), cardiothoracic ratio (CTR), PR interval on electrocardiogram, spectral and color flow imaging and Doppler echocardiographic findings; mitral and aortic regurgitant jet flow area, left atrial area, proximal width of mitral regurgitant jet area and regurgitation fractions.

Ranitidine administration in Henoch-Schönlein vasculitis.

In this study, we discuss 12 patients with gastrointestinal (GI) bleeding who were diagnosed as having Henoch Schoenlein vasculitis (HSV) in Dr Behçet Uz Children's Hospital, Izmir, between January 1991 and January 1992. Seven male and five female patients were included in the study. Their ages ranged between 6-14 years.

Urinary enzyme changes in children undergoing cineangiographic evaluation using iopromid.

Nephropathy due to radiocontrast media presents with a wide spectrum of changes from reversible renal dysfunction to oliguria requiring dialysis. Nineteen patients (mean age 4.5 +/- 3.

A new and rare form of Williams' syndrome.

A 7 year old girl and her mother have typical phenotypic features of the Williams' (elfin facies) syndrome, and both have only mild mitral regurgitation with mitral valve prolapse (MVP). Mitral valve prolapse has been reported in only three cases but pure mitral insufficiency has not been reported alone. In this paper two cases of a new rare form of Williams' syndrome with MVP are presented and this rare disorder is discussed.

A case of mixed gonadal dysgenesis with 45 X/46 XY karyotype and aortic coarctation.

We report a case of mixed gonadal dysgenesis with 45 X/46 XY karyotype and aortic coarctation. An eight month-old patient was admitted to our endocrinology department for evaluation of ambiguous genitalia. On physical examination, a wide neck with a low posterior hairline, bifid scrotum, small phallus, testis in the right labioscrotal fold, single orifice and typical findings of aortic coarctation were present.