Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, peripheral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubulointerstitial nephritis, renal tubular acidosis, and mesangiolipidosis have been noted in AS. The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least four of the five major features of AS who developed secondary hypertension of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis and various other abnormalities of the main arteries in all five patients: aorta (3 cases), celiac artery (4 cases), superior mesenteric artery (1 case), subclavian artery (1 case). Our findings underscore the value of arterial blood pressure monitoring in patients with AS. If hypertension occurs, a renovascular origin should be sought. The diffuse vascular abnormalities which appeared to be a feature of AS in these patients should prompt larger studies of vascular abnormalities in AS.

This modification of the Lecompte operation using a segment of autograft allows an excellent early and late result, with no danger of compression of anteriorly placed pulmonary arteries, no significant right ventricular outflow obstruction, and normal appearance of the tubular autograft. In view of laboratory and clinical evidence, normal growth of the autograft can be anticipated. It allows an elective correction of transposition, ventricular septal defect, and left ventricular outflow tract obstruction without a previous Blalock-Taussig shunt (three patients) and correction at a young age (three patients younger than 1 year).

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A 9-year-old boy was admitted to our pediatric intensive care unit after multiple trauma. On the 17th day post trauma, he developed catheter-related sepsis with candidemia. After removal of the catheter and 6 days of unsuccessful intravenous antifungal therapy, conventional and transesophageal two-dimensional echocardiography was performed revealing a large right atrial thrombus. Surgical thrombectomy under cardiopulmonary bypass was performed and the patient recovered within a few days. Fungal right atrial thrombus is a rare, life-threatening complication of central venous catheterization. Two-dimensional echocardiography is a simple and effective diagnostic technique that should be performed when candidemia is detected. The proper therapeutic response depends on the findings of this examination. For a symptomatic patient with a large, mobile thrombus, we strongly recommend thrombectomy. Surgery not only allows removal of the mass and thus elimination of the mechanical complication but is also a key to management of infection.

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