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Isolated coarctation of the aorta (CoA) occurs in 6-8 % of patients with congenital heart disease. After successful relief of obstruction, patients remain at risk for aortic aneurysm formation at the site of the repair. We sought to determine the diagnostic utility of echocardiography compared with advanced arch imaging (AAI) in diagnosing aortic aneurysms in pediatric patients after CoA repair. The Congenital Heart Databases from 1996 and 2009 were reviewed. All patients treated for CoA who had AAI defined by cardiac magnetic resonance imaging (MRI), computed tomography (CT), or catheterization were identified. Data collected included the following: type, timing, and number of interventions, presence and time to aneurysm diagnosis, and mortality. Patients were subdivided into surgical and catheterization groups for analysis. Seven hundred and fifty-nine patients underwent treatment for CoA during the study period. Three hundred and ninety-nine patients had at least one AAI. Aneurysms were diagnosed by AAI in 28 of 399 patients at a mean of 10 ± 8.4 years after treatment. Echocardiography reports were available for 380 of 399 patients with AAI. The sensitivity of echocardiography for detecting aneurysms was 24 %. The prevalence of aneurysms was significantly greater in the catheterization group (p < 0.05) compared with the surgery group. Aneurysm was also diagnosed earlier in the catheterization group compared with the surgery group (p = 0.02). Multivariate analysis showed a significantly increased risk of aneurysm diagnosis in patients in the catheterization subgroup and in patients requiring more than three procedures. Aortic aneurysms continue to be an important complication after CoA repair. Although serial echocardiograms are the test of choice for following-up most congenital cardiac lesions in pediatrics, our data show that echocardiography is inadequate for the detection of aneurysms after CoA repair. Because the time to aneurysm diagnosis was shorter and the risk greater in the catheterization group (particularly for patients requiring more than one procedure), surveillance with cardiac MRI or CT should begin earlier in these patients.

Introduction: Over the past three decades, significant advances in treatment have improved the mortality of children with cardiac disease. The effect of these advances on the prevalence of arterial ischemic stroke (AIS) is unknown. We describe AIS in children with cardiac disease in the modern era., Design: The prospectively enrolled Intermountain Pediatric Stroke Database (including Utah, Wyoming, Idaho, and Nevada) was queried for all patients less than 18 years old with new-onset AIS between January 1, 2003 and August 31, 2009. Medical records of patients with AIS and cardiac disease were reviewed for cardiac diagnosis, age at AIS, anticoagulant therapy, diuretics, hematocrit, bolus fluids, and ongoing morbidity. Data were analyzed using chi-square test and a mixed-effects Poisson regression growth curve model., Results: AIS incidence in our catchment area was 0.01% (10.7/100,000; N = 97). The incidence of AIS in patients with cardiac disease was higher compared with AIS in the total population (incidence 0.13% [132/100,000], odds ratio [OR] 16.1, 95% confidence interval [CI; 9.7--25.9], P < 0.001). Of the 97 patients with AIS, 24 had cardiac disease (25%). The most common cardiac diagnosis was single ventricle (SV; 8/24, 33%). The incidence of AIS in patients with SV cardiac disease was higher compared with those with other cardiac diagnoses (incidence 1.38% [1380/100,000], OR 15.3, 95% CI [5.7--38.2], P < 0.001). Modeling the prevalence estimates reported since 1978, the prevalence of cardiac disease in AIS patients has remained unchanged across time (prevalence increase per each additional year, 0.5%, 95% CI [--2.1%, 3.1%], P = 0.71)., Conclusion: Children with cardiac disease (particularly those with SV) have increased risk for AIS. The prevalence is unchanged from reports over previous decades. AIS occurred in SV patients despite compliance with current anticoagulation recommendations. Future efforts should focus on best practices to prevent AIS in cardiac patients., (© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.)