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Surgical vegetectomy may be indicated in patients with unresolving sepsis, heart failure, recurrent embolism, or the presence of large vegetations >10 mm in size. Percutaneous vegetectomy using a snare may be a reasonable option instead of open-heart surgery in selected patients. We describe the case of a patient with operated tetralogy of Fallot and infective endocarditis who underwent vegetectomy via a percutaneous approach.

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Pulmonary arteriovenous malformations (PAVM) consist of an abnormal connection between the pulmonary artery and vein bypassing the pulmonary capillary bed. Pulmonary arteriovenous fistulae are characterized by a right-to-left shunt of variable magnitude, and they may be accompanied by other congenital anomalies. We describe a case of pulmonary arteriovenous fistula with a secundum type atrial septal defect (ASD) in a 38-year-old woman, who underwent successful transcatheter closure of both the ASD and PAVM.

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Autoimmune hepatitis is one of the causes of chronic progressive liver disease in childhood. Here we report 14 cases with clinical findings, therapeutic management and prognosis, in order to define the course of the disease. Diagnosis of autoimmune hepatitis was done with the presence of at least one of these autoantibodies; antinuclear antibody, smooth muscle antibody, liver-kidney microsomal type 1 antibody, and perinuclear antineutrophilic cytoplasmic antibody. Patients were seen every 3 to 6 months. After doing a complete physical examination, biochemical parameters and autoantibodies determined at each visit. Mean age at diagnosis was 10.9 +/- 2.6 years (range, 7-15.5 years) and female to male ratio was 1:3. Thirteen patients had jaundice and all had high levels of ALT, AST and gammaglobulin. Hepatomegaly was found in 71.4% and splenomegaly in 64.3% of the patients. All patients were classified as type 1 autoimmune hepatitis. Liver biopsies revealed severe active hepatitis with mononuclear cell infiltration in portal areas, piecemeal necrosis. Drug therapy consisted of prednisone (2 mg/kg/day) per oral at the beginning, and addition of azathioprine (1.5 mg/kg/day) per oral at the 3rd-6th month with slow tapering of prednisone in 12 children. Both drugs were started together to two patients. Follow-up period was 30.7 +/- 15.6 months (range, 12-72 months). Sustained normalization of ALT could not be obtained with tapering doses of prednisone alone. Decrease in ALT levels did not correlate with disappearance of serum autoantibodies. None of the patients showed decompensation of liver disease. Azathioprine administration is necessary to decrease prednisone dose and to maintain a sustained normal transaminase values.

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Fetal echocardiography can be used to detect congenital heart disease prenatally with a high degree of accuracy, and complex heart malformations have also been clearly described in the fetus. However, it is difficult to diagnose correctly or to exclude definitely aortic coarctation by fetal echocardiography. A 23-year-old woman was referred for fetal echocardiographic examination at 21 weeks' gestation after discovery of hydrops fetalis (nonimmune) on an obstetric ultrasound examination. Aortic isthmus appeared hypoplastic with a diameter < or = 3rd percentile for gestational age. There was a narrowing within the descending aorta immediately distal to the origin of the ductus arteriosus. Color flow imaging demonstrated acceleration and turbulent flow and the peak pressure gradient was measured 83 mmHg by continuous wave Doppler in the same area. The pregnancy terminated in spontaneous abortion at 22 weeks' gestation. The fetus was stillborn. The autopsy findings confirmed the prenatal diagnosis. We conclude that together with the quantitative estimation of the aortic arch, color Doppler and continuous wave Doppler are helpful in diagnosis and estimation of the pressure gradient.

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A case of situs inversus totalis and transposition of the great arteries (TGA) was diagnosed prenatally at 25 weeks' gestation. Postnatal echocardiographic examination confirmed the antenatal findings. This case underscores the importance of recognizing situs abnormalities during obstetric and fetal echocardiographic examination, as they are often associated with cardiac anomalies. Accurate prenatal diagnosis of structural heart defects is extremely important in family counselling and in planning obstetric and postnatal treatment.

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