Maurizio Marasini

Late intravenous gamma globulin treatment in infants and children with Kawasaki disease and coronary artery abnormalities

Abstract Kawasaki disease or mucocutaneous lymph node syndrome is a febrile illness affecting infants and children. The major complication leading to mortality and morbidity is the development of coronary artery aneurysms or ectasia, occurring in approximately 10 to 25% of the patients affected by this disease. 1–3 High-dose intravenous gamma globulin is effective in reducing the incidence of coronary artery abnormalities. 1,4 If this treatment is not administered within 10 days from the onset of the disease, its efficacy is questionable. 1,5 This study assesses the effect of a later gamma globulin treatment on the course of established coronary artery abnormalities.

Discrete subaortic stenosis: incidence, morphology and surgical impact of associated subaortic anomalies.

BACKGROUNDnThe association between discrete subaortic stenosis and other subaortic anomalies is a well known but rarely reported occurrence. The aim of this study is to define the incidence, morphology, and surgical impact of associated anomalies of the left ventricular outflow tract in children operated on for discrete subaortic stenosis.nnnMETHODSnBetween 1994 and 2000, 45 consecutive children were operated on for discrete subaortic stenosis. Patients were divided in two groups according to the obstructive lesion detected by echocardiography.nnnRESULTSnA localized shelf was found as an isolated lesion in 31 patients (group A), whereas additional subaortic anomalies were found in 14 cases (31%) and were multiple in 5 cases (group B). The anomalies included anomalous septal insertion of mitral valve (7 cases); accessory mitral valve tissue (2 cases); anomalous papillary muscle (2 cases); anomalous muscular band (8 cases); and muscularization of the anterior mitral valve leaflet (1 case). Cardiopulmonary bypass and aortic cross-clamping times were significantly shorter in group A. There were no operative deaths nor major complications or deaths during follow-up. A gradient of 15 mm Hg or more was found at follow-up in 5 cases whereas aortic regurgitation was estimated to be not clinically significant in all but 1 patient. Six cases of recurrent subaortic stenosis were found in our series, 3 of them with other subaortic anomalies.nnnCONCLUSIONSnThis study shows that discrete subaortic stenosis can often be associated with other subaortic abnormalities. Surgical treatment of these anomalies produces excellent early and mid-term relief of obstruction without any increase in mortality and morbidity.

Acquired pulmonary vein obstruction after open-heart surgery.

Acquired pulmonary vein obstruction is an extremely rare complication after open-heart surgery not including pulmonary vein procedures. We report on three cases of this unusual complication presenting peculiar angiographic findings in order to understand its possible etiology.

Giant aneurysm following coil occlusion of patent ductus arteriosus.

A case is described in which a giant aneurysm developed following successful PDA coil occlusion in an infant with Marfan syndrome. This rare and severe complication brings into question the need for careful evaluation of these children before and after transcatheter occlusion. Cathet. Cardiovasc. Intervent. 50:186–189, 2000.

Balloon dilatation of critically obstructed modified (polytetrafluoroethylene) Blalock-Taussig shunts

Abstract Balloon dilatation is increasingly used to treat a wide range of congenital and acquired vascular stenoses in infancy and childhood. 1–4 However, to our knowledge, there has been only 1 published report of a successful dilatation of a stenosed modified (polytetrafluoroethylene) Blalock-Taussig shunt. 5 In our report, we have reviewed the technique used and the results obtained in 8 patients with critically stenosed or completely occluded graft.

Aortic arch interruption: Two-dimensional echocardiographic recognition in utero

SummaryA case of aortic arch interruption detected in a 17-week-old fetus and confirmed after therapeutic abortion is reported. The potential usefulness of cross-sectional echocardiography in prenatal detection of aortic arch anomalies is discussed.

Early treatment of patent ductus arteriosus in premature infants with severe respiratory distress syndrome

SummaryIn 1983, a US National Collaborative Study (NCS) proposed criteria for the diagnosis of hemodynamically significant patent ductus arteriosus (PDA) in premature infants with respiratory distress syndrome (RDS), but the widespread use of pulsed Doppler cross-sectional echocardiography (PD-CSE) in neonatal intensive care units has made direct assessment of the ductus possible thus providing more timely therapy. We have compared the results in 30 premature infants with severe RDS, assessed according to the guidelines of the US NCS, with those in 51 infants whose PDA was diagnosed by PD-CSE. Together with a significant reduction in the age at treatment (7.8±3.9 vs 2.4±1.1 days), there was a reduced dependence on artificial ventilation (14.8±11.0 vs 7.8±2.7 days), a reduction in the number requiring surgical ligation of PDA (9 vs 2), a decreased incidence of bronchopulmonary-dysplasia (BPD) (40% vs 16%), and a reduction of unfavorable outcome of treatment (death or BPD) (76% vs 49%).

Evolution of left ventricular disease in the fetus : Case report

A fetal case is described that showed a rapid progression from the features of initial left ventricular fibroelastosis at 20 weeks of gestation to a more marked dilation at 22 weeks and finally to a hypoplastic left ventricle with aortic stenosis at 24 weeks of gestation. This case confirms the evolutive character of left ventricular disease during fetal life.

Unusual ultrasound finding of patent ductus arteriosus in a preterm infant

SummaryPatency of the ductus arteriosus (PDA) is a common finding in small premature infants. Recently pulsed-Doppler-cross sectional echocardiography (PD-CSE) has been successfully used in these patients. We report a case of a premature infant with an unusual PD-CSE pattern.