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. In three patients, a non-valved aortic conduit was used to form an extra-cardiac Fontan circulation and in one patient, non-valved pulmonary and aortic conduits were used to repair an infected aortic aneurysm. Three patients died following surgery. Survivors were followed up using echocardiography between 2 and 24 months post-surgery. Results demonstrate that, with the help of a national organ-sharing programme, the use of fresh homograft conduits is feasible in a paediatric patient population with reasonable waiting times.

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. This case demonstrates an unusual but potentially life-threatening long-term complication of this procedure.

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. She was referred to our clinic upon the development of congestive heart failure and dilated cardiomyopathy was diagnosed based on clinical findings, electrocardiogram, chest X-ray and echocardiography. She had no evidence of other major complications of IDDM such as retinopathy, nephropathy or neuropathy.

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. The nutritional status of each patient was determined by using anthropometric parameters and calorie and protein intake ratios. The patients were divided into malnourished and well-nourished groups (21 and 8 patients, respectively) according to their nutritional status. Serum IGF-I concentrations were measured in the two patient groups and the controls. The malnourished group had the lowest IGF-I levels (48.14 +/- 21.8 ng/ml, p<0.05). However, the well-nourished group's IGF-I levels were significantly lower than the control subjects' despite improved nutritional status (85.5 +/- 30.2 and 107 +/- 19.7 ng/ml, respectively, p<0.05). In addition, we found a positive correlation between serum IGF-I levels and oxygen saturation of the patients (r=0.402, p<0.05). These findings indicate that chronic hypoxemia has a direct or indirect effect to reduce serum IGF-I concentrations and this may be a cause of the increased growth failure in patients with cyanotic congenital heart disease.

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). The mean diastolic pressure gradient across the mitral valve measured during heart catheterization before and immediately after PBMV was 18.8 +/- 10.42 and 9.4 +/- 7.7 mmHg, respectively (p < 0.01). The patients were followed for a mean period of 36.6 +/- 8.5 months (range 12 to 72 months) after the procedure. During follow-up, post PBMV mean diastolic transmitral gradient measured by color Doppler echocardiography decreased from 19.3 +/- 11.16 to 7.43 +/- 7.3 mmHg (p < 0.01) and the mitral valve area increased from 1.09 +/- 0.7 to 3.1 +/- 0.9 cm2 (p < 0.002). Functional capacity showed improvement to NYHA class I in four patients, to class II-III in the patient with congenital mitral valve stenosis and Eisenmenger's syndrome with PDA and to class II in one patient with severe mitral valve calcification in whom restenosis occurred three years after PBMV. Percutaneous balloon mitral valvuloplasty PBMV can achieve very good short- and mid-term results in relieving symptomatic rheumatic mitral valve stenosis.

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. The platelet activation in CHD was investigated in support of a hypothesis that platelet activation may play a role in the hemostatic abnormalities reported in these patients. Platelet activation was determined by using flow cytometry with anti-CD62 monoclonal antibody (mAb), which has been shown to be a specific marker of platelet activation. Thirteen children with cyanotic CHD, 33 children with acyanotic CHD and 17 healthy children serving as controls were studied. Platelet activation was significantly higher in the cyanotic group and also in the acyanotic group compared with the healthy children (P = 0.0000 and P = 0.019, respectively). In the cyanotic group, platelet activation showed a direct correlation with arterial O2 saturation (SaO2) (P = 0.014). There was no correlation between platelet activation and erythrocyte related parameters in either group. Platelet activation occurs in CHD, particularly in patients with cyanotic CHD (even in patients with no evidence of clinical thrombosis) and it may play a role in the pathogenesis of thrombotic disorders seen in these patients.

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. A retrograde, eccentric small jet was found within the right ventricular outflow tract at the pulmonary artery valvular level allowing us to detect the entrance site of the fistula. The diagnosis was confirmed by cardiac catheterization and angiocardiography. Although our case was asymptomatic, the decision to perform cardiac surgery was made because of the aneurysmatic appearance of the left coronary artery. In our opinion, visualization of coronary arteries by two-dimensional echocardiography, together with additional information obtained from the Doppler examination, provides an excellent technique for the noninvasive diagnosis of coronary artery fistula.

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. The patient's findings were complementary to the previous observations reporting HCM in IDM. The presence of disproportionate septal hypertrophy in the echocardiography of an infant born to a diabetic mother is highly suggestive of HCM in IDM. In our opinion, further cardiac evaluation is not indicated unless other cardiac abnormalities are suspected.

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. The results of patients in the HIVMP group showed statistically significant changes in ESR and CTR when compared with the patients receiving OP, and the recovery was more rapid. HIVMP therapy can be considered as a new method of treatment for acute rheumatic carditis.

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. The patients were separated into two identical groups and were given ranitidine or a placebo. Both groups were followed up for abdominal pain and GI bleeding. In the group administered ranitidine the duration and severity of abdominal pain and gastrointestinal bleeding decreased significantly as compared to the group taking placebo (P < 0.05). No side effects of ranitidine were observed. As a result, it was concluded that ranitidine could be used to treat HSV with GI symptoms.

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 +/- 3.7 years) were included. Mean +/- SD values of the variables obtained before and 48 hours after angiography were the following: plasma creatinine: 0.6 +/- 0.10 and 0.6 +/- 0.16 mg/dl; endogenous creatinine clearance: 76.1 +/- 17.0 and 80.9 +/- 19.3 ml/min/1.73 m2; plasma osmolality: 279 +/- 23 and 298 +/- 39 mOsm/kg H2O; urine osmolality: 429 +/- 225 and 459 +/- 196 mOsm/kg H2O; fractional sodium excretion: 2.1 +/- 1.3% and 2.4 +/- 1.3%; plasma uric acid: 3.9 +/- 1.3 and 3.4 +/- 1.0 mg/dl; urinary AST/creatinine: 5.2 +/- 4.8 and 4.2 +/- 2.6 mU/mg; ALT/creatinine: 16.8 +/- 12.4 and 15.3 +/- 12.6 mU/mg; LDH/creatinine: 52.0 +/- 39.6 and 42.3 +/- 31.5 mU/mg; NAG/creatinine: 20.1 +/- 2.8 and 16.8 +/- 2.3 mU/mg, respectively. The changes in renal function parameters and urinary enzyme levels were insignificant statistically (p > 0.05). In conclusion, iopromid injection at maximum doses of 5 ml/kg does not result in injury to the tubular epithelium leading to increased urinary enzyme levels.

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. The chromosome karyotype of the patient was 45 X/46 XY. On the laparotomy there was an infantile uterus, fallopian tubes, and a streak gonad on the left. The follow-up of the patient included sex assignment, genital reconstruction and cardiovascular treatment.