Diagnostic accuracy and clinical relevance of brain natriuretic peptide assay in pediatric patients with congenital heart diseases.

Background: The clinical usefulness of B-type natriuretic peptide (BNP) assay in congenital heart diseases is still controversial. We evaluated the diagnostic accuracy and clinical relevance of this assay in different cardiovascular hemodynamic conditions in pediatric patients with congenital heart diseases.

Materials And Methods: BNP was measured in 173 healthy individuals and in 382 pediatric patients with congenital heart diseases.

Myocardial infarction and arterial thrombosis in identical newborn twins with homozygosity for the PAI-1 4 G/5 G polymorphism.

Myocardial infarction in the perinatal period, in the absence of congenital heart disease or coronary artery lesions, is rare. The most common etiology described are the thromboembolism and perinatal asphyxia. We report a case of monozygotic twins who developed, after birth, acute vascular events and both had PAI-1 4G/4G homozygosity.

Prothrombotic mutations as risk factors for cryptogenic ischemic cerebrovascular events in young subjects with patent foramen ovale.

Background And Purpose: Patent foramen ovale (PFO) has been identified as a potential risk factor for cerebrovascular ischemia. Procoagulant mutations may increase the risk and impact the choice of appropriate therapy for secondary prevention. We evaluated the prevalence of the 2 most common genetic risk factors for thromboembolism, factor V Leiden (G1691A) and prothrombin G20210A, in young PFO patients who were referred for percutaneous transcatheter closure of their PFO.

A multicentre approach for the management of adults with congenital heart disease.

Objective: At present, the level of care for adult congenital heart disease is not completely satisfactory in many European countries including Italy. The aim of this multicentre study was to evaluate the possibility of creating an active connection among different centres involved in the care of this patient population.

Methods: This study lasted two years, from December 2002 to December 2004.

Surgical treatment of arrhythmias related to congenital heart diseases.

Background: Supraventricular arrhythmias complicate operated and unoperated congenital heart disease, especially when atrial dilatation coexists.

Methods: We evaluated the results of intraoperative ablation in a group of 23 patients with chronic supraventricular tachyarrhythmias (mostly intraatrial reentry) that were unresponsive to conventional medical therapy. All procedures were performed consecutively between September 1999 and November 2001.

[Transcatheter treatment of patent ductus arteriosus].

Isolated patent ductus arteriosus accounts for 10 to 18% of all cardiovascular malformations. Transcatheter closure of the patent ductus arteriosus has now become established practice with a high success rate and a low incidence of complications. Many devices have been proposed during the last 20 years.

Pulmonary artery banding and ventricular septal defect enlargement in patients with univentricular atrioventricular connection and the aorta originating from an incomplete ventricle.

Background: In patients with univentricular atrioventricular connection and the aorta originating from an incomplete ventricle, subaortic stenosis is generally due to a restrictive ventricular septal defect (RVSD), that may be present at birth or develop after palliative procedures. In particular, a primary role in the genesis of the RVSD has been ascribed to pulmonary artery banding (PAB). The aim of this paper is to analyse the possible risk factors for the development of an RVSD, including PAB, and the results of one of the proposed procedures for treatment of this condition (RVSD enlargement).

Transcatheter closure of congenital and acquired muscular ventricular septal defects using the Amplatzer device.

Surgical closure of congenital or post-myocardial infarction (MI) muscular ventricular septal defect (MVSD) is associated with significant mortality and morbidity; therefore, both surgeons and cardiologists would welcome a safe non-surgical approach. The aim of this study is to report the combined experience of 2 cardiac centers in the transcatheter occlusion of both congenital and acquired MVSDs using the Amplatzer MVSD occluder device (AGA Medical Corporation, Golden Valley, Minnesota). Thirty-two patients underwent attempted transcatheter closure of an MVSD.

Molecular characterization of 22q11 deletion in a three-generation family with maternal transmission.

Haploinsufficiency of chromosome 22q11.2 is a well-established cause of both the DiGeorge anomaly and the velocardiofacial syndrome. This condition shows a continuous spectrum of phenotypic manifestations with a considerable inter- and intrafamilial variability.