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Congenital stenosis and/or hypoplasia of the pulmonary veins(s) is a rare cardiac malformation, whose treatment is difficult and not well defined, so conditioning an extremely poor prognosis. In fact, balloon angioplasty and surgery proved to be ineffective during a mid-term follow-up, thus not significantly modifying the natural history of this cardiovascular malformation. Although heart-lung transplantation is thought by someone to be the sole reliable treatment for congenital stenosis of the pulmonary vein(s), a recently available therapeutic option, the endovascular "stent" implantation during cardiac catheterization prompted to hope for a better outlook for these patients. However, this procedure is far from being an easy approach, due to technical problems that cause a high rate of failure and major complications. We report on two cases of pulmonary vein stenosis successfully treated by endoluminal "stent" implantation during open heart surgery, and suggest, and suggest that this safe and effective approach might be an alternative therapeutic option in the definitive or palliative treatment of this congenital cardiovascular malformation.

The case of a 34 month-old infant with cardiac murmur and echocardiographic signs of coronary artery fistula is presented. Cardiac catheterization was performed, revealing a right coronary artery-to-right ventricle fistula, and a significant left-to-right shunt. At 4.0 years of age she underwent a repeated catheterization, showing complete spontaneous closure of the fistula. Review of the literature shows that fistulas draining into the right ventricle have some chance to close spontaneously. Accordingly, clinical approach should therefore be considered.

Since the first clinical application of transcatheter closure technique for patent ductus arteriosus in children and infants in 1977 by Rashkind and Coll., this technique is routinely performed only in a few major Pediatric Cardiology Centers. We report our successful series which is the first of its kind both in Italy and in Southern Europe. The series included 11 children aged 1.6 to 10 years (mean age 4.5 years) all affected with patent ductus arteriosus (PDA). Subaortic stenosis (1) and ventricular septal defect with pulmonary stenosis (1) were associated anomalies. In all of the cases, after the standard percutaneous cardiac catheterization, the Mullins method was attempted to advance and deliver the Rashkind PDA double-disk occluder. Successful closure was accomplished in 10 while almost complete closure was achieved in the 11th. Normal Doppler flow pattern after the procedure confirmed the successful results. No complications occurred. Although our experience is limited, non-surgical PDA closure provided an excellent alternative to surgical procedure.

Before Mustard's operation 27 children, from 2 days to 11 months old, with simple or complex transposition of the great vessels, underwent an electrocardiogram at rest. After operation, all the children underwent a standard electrocardiogram and 24 hours continuous Holter monitoring. Successive electrocardiograms at rest were recorded in all patients, and eleven children were successfully examined by dynamic electrocardiogram. The electrocardiogram at rest showed a sinusal rhythm in 66.7 of the patients; during the follow-up, in 33.3% non sinusal rhythm was recorded. The continuous 24 hours dynamic electrocardiogram, immediately after surgery, revealed 25.9% of the patients with non-sinusal rhythm. After 7 months, the Holter monitoring showed, in one case, a severe sinus node disfunction. Our data and those of the literature confirm the importance to follow these patients during a long period of time, because it was found that, even bedately, can develop serious arrhythmias.

Seven patients with hemodynamic and angiocardiographic diagnosis of supravalvular aortic stenosis were studied by single plane echocardiography. 3 patients had the hypoplastic and 3 the "hourglass" type of the disease. 3 patients underwent surgical repair of the malformation, with postoperative echocardiographic control. There was no relationship between the lumen dimension variations as measured by echocardiography and the transtenotic pressure gradient. With respect to angiocardiography, echocardiography underestimates the severity of the disease. The method was always diagnostic, but the anatomic type of stenosis could not be determined. The severity of the disease can be only indirectly assessed by evaluating left ventricular hypertrophy induced by pressure overload.

The authors describe a case of aneurysm of the non-coronary sinus of Valsalva and aortic insufficiency caused by Salmonella Tiphi in a 7 year old child. The exceptional ethiology is discussed as well as the problems connected with surgical correction.

Two patients with Multiple Lentiginosis (ML) had hypertrophic cardiomyopathy with severe obstruction of the right and left ventricular outflow tracts. ML is a rare syndrome in which there is a typical skin pigmentation disorder together with various other abnormalities like slight mental retardation, sensorineural deafness, genital and skeletal anomalies and sometimes a characteristic facies. Obstruction of the right ventricular outflow tract is frequent in such patients. The association of ML and obstructive hypertrophic cardiomyopathy has been recently reported. A critical examination of the literature in addition to our clinical observations suggests that the biventricular obstructive hypertrophic cardiomyopathy may play a major role in the clinical feature of ML syndrome.

A case of double aortic arch with severe congenital mitral incompetence and mild pulmonary arteries stenosis is described. A possible embriogenetic explanation of the anomaly is discussed in the light of preferential foetal blood flows.