In patients with repaired coarctation, reduced vascular reactivity is associated with hypertension during daily life and with increased LV mass, both of which are important predictors for late morbidity and mortality.

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.

The 22q11.2 deletion syndrome (22q11DS) is one of the most recognizable causes of congenital heart defects (CHDs), but the frequency varies in non-selected populations. The purpose of this study was to determine the incidence and clinical features of patients with CHD and 22q11DS admitted to a pediatric cardiology intensive care unit in Brazil. In a prospective study, we evaluated a consecutive series of 207 patients with a CHD following a clinical protocol and cytogenetic analysis by high resolution karyotype and fluorescent in situ hybridization (FISH). 22q11DS was identified in four patients (2%), a frequency similar to studies that evaluated subjects with major CHDs in other countries. Despite this similarity, we believe that the low rate of prenatal identification of CHDs and the limited access of these patients to appropriate diagnosis and care, which occur in our region, could have had an influence on this frequency. It is possible that 22q11DS patients with a severe CHD could have died before having a chance to access a tertiary hospital, leading to an underestimate of its frequency.

Background: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however. Aim: To determine the normal values and the effect of childhood vasculitis on arterial distensibility in children and teenagers. Methods: Distensibility of the brachioradial arterial segment was studied using pulse wave velocity (PWV ∝1/'distensibility), in 13 children with polyarteritis nodosa at a median age of 11.8 (range 4.9-16) years. As a control group, 155 healthy schoolchildren (6-18 years, 81 boys) were studied. PWV was assessed using a photoplethysmographic technique; blood pressure was measured by an automatic sphygmomanometer (Dinamap). Data from patients were expressed as z scores adjusted for age and compared to a population mean of 0 by a single sample t test. Determinants of PWV in normal children were assessed by univariate and multivariate linear regression analyses. Results: Age, height, weight, and systolic blood pressure correlated individually with the brachioradial PWV. Multivariate analysis identified age as the only independent determinant. Ten of the patients were in clinical remission, while three had evidence of disease activity at the time of study. The PWV in the patient group as a whole was significantly greater than those in healthy children (mean z score +0.99). Raised C reactive protein concentration (>2 mg/dl) in the three patients with active disease was associated with a higher PWV when compared to those in remission (z score +2.78 v +0.45). The diastolic blood pressure of the patients was higher than those of the controls (z score +1.04) while the systolic pressure was similar (z score −0.36). Conclusions: PWV in the brachioradial arterial segment increases gradually during childhood independent of body weight, height, mass, and blood pressure. Increased PWV, and hence decreased distensibility, in this peripheral arterial segment occurs in polyarteritis nodosa and is amplified during acute inflammatory exacerbation.

Background-Patients with repaired coarctation are at increased risk of hypertension and cardiovascular disease despite successful repair. We studied the function of conduit arteries in upper and lower limbs of patients late after successful coarctation repair and its relation to age at surgery. Methods and Results-Flow-mediated dilatation (FMD) and the dilatation after sublingual nitroglycerin (NTG, 25 g) were measured by using high-resolution ultrasound in the brachial artery in 64 coarctation patients (44 males and 20 females, aged 19Ϯ10 years; median age at operation 4 months) and 45 control subjects (28 males and 17 females, aged 19Ϯ10 years) and in the posterior tibial artery in 37 patients and 22 control subjects. Arterial stiffness was determined by pulse-wave velocity (PWV) of the brachioradial and femoral-dorsalis pedis tracts. Patients, compared with control subjects, had lower brachial FMD (7.16Ϯ3.4% versus 8.62Ϯ2.3%, respectively; Pϭ0.02) and NTG (11.46Ϯ4.3% versus 13.21Ϯ4.6%, respectively; Pϭ0.046) and higher brachioradial PWV (9.17Ϯ3.1 versus 8.06Ϯ1.9 m/s, respectively; Pϭ0.05). In contrast, posterior tibial FMD, NTG, and lower limb PWV were comparable. Age (months) at the time of repair was related to brachioradial PWV (rϭ0.42, Pϭ0.002) but not to brachial FMD or NTG. Conclusions-Patients