To demonstrate the use of transthoracic contrast echocardiography in the detection of pulmonary arteriovenous fistulas in patients with a previously constructed anastomosis between the superior caval vein and the right pulmonary artery (Glenn shunt), and to examine their prevalence in this special population, we evaluated prospectively all patients followed up in the Grown-Up Congenital Heart Unit subsequent to construction of a classical or bi-directional Glenn shunt. We studied 12 patients, aged from 21 to 38 (mean 28 ± 4.8) years who had had a previous cavopulmonary shunt in place for a period of 4 to 33 years (mean 24±9 years). All were examined with cross-sectional contrast echocardiography, 11 patients had cardiac catheterisation and angiography, and 6 patients had magnetic resonance imaging. Systemic arterial oxygen saturations at rest, and during exercise using the modified Bruce protocol, were measured in all patients. Contrast echocardiography showed evidence of pulmonary arteriovenous fistulas in 7 of the 12 patients, with appearance of echo contrast in the left atrium 1–8 seconds after peripheral venous injection in the arm. Simultaneous appearance of microbubbles in the right atrium revealed a residual communication between the superior caval vein and the right atrium in 2 patients, and presence of collaterals between the superior and inferior caval veins in one. Cardiac catheterisation and angiography showed obvious fistulas in 4 patients, and revealed suggestive findings in 2. In patients deemed to have pulmonary arteriovenous fistulas on contrast echocardiography, arterial oxygen saturations at rest (51–94%, mean 75±15.3%) and on exercise (23–91%, mean 53±24.2%) were significantly lower compared to patients judged to be without fistulas (p<0.005). Pulmonary hypertension in the contralateral lung was more common in patients with fistulas (mean left pulmonary arterial pressure 22–110 mm Hg, p=0.014). In patients with cavopulmonary anastomoses, pulmonary arteriovenous fistulas occur frequently in the long term (10–33, mean 25.7±8 years), and are associated with worsening systemic arterial desaturation. Contrast echocardiography should be included in the regular evaluation of these patients as a simple and sensitive technique for the detection of pulmonary arteriovenous fistulas, particularly with the devel opment of increasing cyanosis.

Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings

Background: Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy. Methods and Results: Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications. Conclusions: Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

Introduction: Postoperative thrombosis after a cavopulmonary connection has been widely described. Abnormalities in coagulation seem to occur early in the course of patients with functionally univentricular physiology, and may precede surgery. Endothelial abnormalities due to chronic hypoxia, and hyperviscosity, may contribute to this scene. The purpose of our study was to investigate if patients with a superior cavopulmonary connection have altered levels of endothelial and coagulative markers in the plasma. Methods: We compared findings in 10 patients, aged from 4 to 19 years, with 6 age-matched normal controls. We measured levels of von Willebrand factor antigen, thrombomodulin, tissue-type plasminogen activator, plasminogen activator inhibitor-1 and d-dimer in the plasma using enzyme-linked immunosorbent assay. Results: We found increased levels of von Willebrand factor antigen (p = 0.01), tissue-type plasminogen activator (p = 0.01), and decreased levels of thrombomodulin (p = 0.008) in the patients when compared to controls, while levels of plasminogen activator inhibitor-1 were not different. Values of d-dimer were within the reference range. Levels of tissue-type plasminogen activator had a positive correlation with von Willebrand factor antigen (r = 0.66, p = 0.008). Conclusions: Altered levels of endothelial markers in the plasma, in the presence of normal levels of d-dimer, suggest that endothelial dysfunction may precede the occurrence of intravascular coagulation and thrombosis in patients with functionally univentricular physiology. These observations may have therapeutical implications.

The use of a conduit of polytetrafluoroethylene placed between the right ventricle and the pulmonary arteries as source of pulmonary arterial supply during the first stage of palliation for the hypoplastic left heart syndrome has facilitated post-operative management and resulted in decreased mortality. We describe here the use of a cryopreserved saphenous vein inserted in reversed direction to create the connection between the right ventricle and the pulmonary arteries in a neonate with low birth weight undergoing the modified Norwood procedure.