Obstruction of the reconstructed aortic arch, or the neoaortic arch, is now known to be an important factor increasing mortality after the Norwood operation for hypoplastic left heart syndrome. Transcatheter balloon angioplasty has been shown to provide effective relief of both native aortic coarctation and obstructions of the aortic arch occurring subsequent to therapeutic intervention. We sought to determine the outcomes of balloon angioplasty used as an initial treatment for obstruction of the neoaortic arch occurring after the Norwood operation. We gathered the characteristics of 58 patients with such obstruction from 8 institutions, noting procedural factors and outcomes of initial balloon dilation. Obstruction occurred at a median interval of 4 months, with a range from 1.5 months to 6.3 years, after a Norwood operation. Ventricular dysfunction was present before dilation in 13 patients. Mean peak to peak systolic pressure gradients were acutely reduced from 31±20 mm Hg to 6±9 mmHg (p<0.001), with outcome subjectively judged to be successful in 89%- Three patients with pre-existing ventricular dysfunction died within 48 hours of dilation. There were 10 additional deaths during the period of followup, with Kaplan Meier estimates of survival after intervention of 87% at 1 month, 77% at 12 months, and 72% after 15 months. In addition, 9 patients required re-intervention during the period of follow-up, with Kaplan Meier estimates of freedom from re-intervention after dilation of 87% at 6 months, 78% at 12 months and 74% after 18 months. Although transcatheter dilation of neoaortic arch obstructions after Norwood operation is successful, there is a high risk of re-intervention and ongoing mortality in this subgroup of patients. Close follow-up is recommended.

Knowledge of normal aortic dimensions is important in the management of children with aortic disease. So as to define such dimensions, we undertook a retrospective review of clinical data and aortic cineangiograms from 167 subjects without aortic disease having a mean age of 3.67 years, with a range from 0.01 to 14.95 years. Amongst the patients, 56 were without detectable cardiac lesions, 66 patients had mild pulmonary stenosis, 30 were seen with Kawasaki disease, and 15 with small interatrial defects within the oval fossa. Aorto-grams were available in all. No patient had any hemodynamic derangement that would have affected the aorta during intrauterine life or childhood. Systolic dimensions were measured in the ascending and descending aorta at the level of the carina, at the transverse aortic arch distal to the brachiocephalic, of the left common carotid artery at its origin, at the transverse aortic arch distal to the left common carotid artery, at the aortic isthmus, and of the aorta at the level of the diaphragm. A regression analysis model was used to establish the range of predicted normal values, with their confidence limits, standardizing the values to height as the biophysical parameter having the highest correlation to aortic dimensions. Normal ranges were established for all the levels of measurement. The data should prove useful in identifying abnormalities of the thoracic aorta during childhood, and when assessing the outcomes of interventions.

Occlusion of the atrial septal defects in the oval fossa by interventional catheterization has progressed, but still has limitations. Three-dimensional (3D) echocardiography can provide unique views unavailable by cross-sectional imaging. The objective of this study was to define the clinical application of 3D echocardiography in the assessment and monitoring of transcatheter occlusion of atrial septal defects. Three-dimensional echocardiography was attempted prior to occlusion of atrial septal defects in 41 patients (median age 8.6 years). Serial cross-sectional images were acquired by multiplane transoesophageal echocardiography and displayed by means of computer reconstruction. Dynamic 3D echocardiographic images of defects in the oval fossa were obtained in 40 of 41 patients (98%). Volume-rendering demonstrated the anterosuperior rim in 36 (90%) and the inferoposterior rim in 24 (60%), but failed to reveal small additional fenestrations in six. Sizes measured by 3D echocardiography were significantly larger than those provided by cross-sectional transoesophageal echocardiography (p =;0.007), but differed little from those obtained with balloon sizing (p =; 0.6). After occlusion, 3D echocardiography showed positions of all arms of the device in 20 of 24 cases. Location of any protruding arms, or residual defects, were also clearly revealed.Three-dimensional images obtained in 12 patients during deployment of the double-umbrella device were useful in monitoring its position (single-frame) and for explaining the mechanism of protrusion. Current 3D echocardiography provides clinically relevant information for selection of patients for closure of atrial septal defects by interventional catheterization and when monitoring during implantation. Information obtained by this technique can clarify the mechanism of deployment of the device and closure of the defect, therefore influencing outcomes.

Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.

Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.

To assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.

Communication of the coronary sinus with the left atrium is an unusual anomaly. Two cases of tricuspid atresia are described where systemic desaturation developed following a modified Fontan procedure. In both, investigation demonstrated a communication between the coronary sinus and the left atrium. Transcatheter closure of these communications was achieved with a ductal device in one case and coil embolization in the other. Following occlusion and elimination of the right-to-left shunt, both patients became fully saturated in room air.

Hypoplasia of the transverse aortic arch is commonly associated with aortic coarctation. Persistent or recurrent obstruction can occur at this level after successful repair of the native coarcted segment. The purpose of this report is to present a new technique to treat such lesions, namely with implantation of a balloon-expandable stent. This approach was used successfully in 4 children with such hypoplasia occurring after repair of coarctation. Implantation led to both anatomical and physiological relief of obstruction in all. The patients tolerated the procedure, and there were no major adverse events.