More information is needed to clarify whether stenting is superior to balloon angioplasty (BA) for unoperated coarctation of the aorta (CoA). From September 1997, 21 consecutive adolescents and adults (24 +/- 11 years) with discrete CoA underwent stenting (G1). The results were compared to those achieved by BA performed in historical group of 15 patients (18 +/- 10 years; P = 0.103; G2). After the procedure, systolic gradient reduction was higher (99% +/- 2% vs. 87% +/- 17%; P = 0.015), residual gradients lower (0.4 +/- 1.4 vs. 5.9 +/- 7.9 mm Hg; P = 0.019), gain at the CoA site higher (333% +/- 172% vs. 190% +/- 104%; P = 0.007), and CoA diameter larger (16.9 +/- 2.9 vs. 12.9 +/- 3.2 mm; P < 0.001) in G1. Aortic wall abnormalities were found in eight patients in G2 (53%) and in one in G1 (7%; P < 0.001). There was no major complication. Repeat catheterization (n = 33) and/or MRI (n = 2) was performed at a median follow-up of 1.0 year for G1 and 1.5 for G2 (P = 0.005). Gradient reduction persisted in both groups, although higher late gradients were seen in G2 (median of 0 mm Hg for G1 vs. 3 for G2; P = 0.014). CoA diameter showed no late loss in G1 and a late gain in G2 with a trend to being larger in G1 (16.7 +/- 2.9 vs. 14.6 +/- 3.9 mm; P = 0.075). Two patients required late stenting due to aneurysm formation or stent fracture in G1. Aortic wall abnormalities did not progress and one patient required redilation in G2. Blood pressure was similar in both groups at follow-up (126 +/- 12/81 +/- 11 for G1 vs. 120 +/- 15/80 +/- 10 mm Hg for G2; P = 0.149 and 0.975, respectively). Although satisfactory and similar clinical outcomes were observed with both techniques, stenting was a better means to relieve the stenosis and minimize the risk of developing immediate aortic wall abnormalities.

Percutaneous closure of perimembranous ventricular septal defects (VSDs) has been feasible, safe, and effective with the new Amplatzer membranous septal occluder. We report further experience with this device with emphasis on morphological aspects of the VSDs and technical issues. Ten patients (median age and weight, 14 years and 34.5 kg, respectively) with volume-overloaded left ventricles underwent closure under general anesthesia and transesophageal guidance (TEE). The VSD diameter was 7.1 +/- 4.0 mm by angiography and 7.8 +/- 3.7 mm by TEE. Three patients had defects associated with aneurysm-like formations (two with multiple exit holes), four had defects shrouded by extensive tricuspid valve tissue, two had defects with little or no tricuspid valve involvement, and one had a right aortic cusp prolapse with trivial aortic regurgitation. Implantation was successful in all patients, although in two the initial device had to be changed for a larger one. Kinkings in the delivery sheath, inability to position the sheath near the left ventricular apex, and device prolapse through the VSD prompted modifications in the standard technique of implantation. Device orientation was excellent except in one case. Nine patients had complete occlusion within 1-3 months. Device-related aortic or tricuspid insufficiency, arrhythmias, and embolization were not observed. Two patients had slight gradients across the left ventricular outflow tract, normalizing after 3 months. The Amplatzer membranous septal occluder was suitable to close a wide range of perimembranous VSD sizes and morphologies with good short-term outcomes. Longer follow-up is required.

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

Purpose -

Original ArticleAortic stenosis accounts for 5-6% of the congenital heart diseases and clearly predominates in the male sex 1 . The presence of commissural fusion with a variable thickening and a reduction in the mobility of the valvular leaflets are almost constant anatomic findings 1 . Association with a bicuspid valve is also frequent 1 . Longitudinal studies about the natural history of the disease suggest that the intervention is indicated in the presence of severe stenosis, which is defined by clinical, electrocardiographic, and hemodynamic findings 2 . Surgical valvulotomy has always been the classic therapeutic method considered the gold standard. With the evolution of interventional techniques from the mid 1980s onwards, balloon valvuloplasty started to be used in the initial treatment of aortic stenosis in different age groups with satisfactory short-and midterm results . We report the experience of the teams at the Instituto Dante Pazzanese and the Hospital do Coração with this type of approach. MethodsValvuloplasty was performed in conditions of severe stenosis, classically defined by the presence of a peak-topeak systolic gradient 30,31 detected in the catheterization room or derived from echocardiography 32,33 > 70 mmHg, or, when electrocardiographic alterations (of the T wave or ST segment) or clinical symptoms (dizziness and syncope with no other causes; significant chest pain) were present, a peak-to-peak systolic gradient > 50 mmHg. The procedure was also performed in neonates or young infants (between 1 and 6 months of age) with aortic stenosis and severe ventricular dysfunction, independent of the transvalvular gradient, because in that situation that gradient is underestimated due to the low systemic cardiac output. Valvuloplasty was contraindicated in the presence of moderate and severe aortic regurgitation, of other associated intracardiac lesions requiring immediate surgery, and of a hypoplastic left ventricle requiring palliative surgery, within a therapeutic Objective -To report short and midtem follow-up results of balloon aortic valvuloplasty to treat congenital aortic stenosis. Methods -