Lifetime cardiac reinterventions following the Fontan procedure.

Patients with single ventricle physiology face significant morbidity and mortality following the Fontan procedure resulting in the need for additional cardiac reinterventions. Online patient education resources provide limited information on the reinterventions performed in single ventricle patients following the Fontan procedure. We sought to determine cardiac surgical and percutaneous reintervention rates and factors affecting reinterventions following the Fontan procedure. Databases from a single tertiary care center were retrospectively reviewed for all patients who underwent a Fontan procedure between 1978 and 2002. The number and type of cardiac surgical and percutaneous interventions following the Fontan procedure were determined, and relationships between need for reintervention and clinical variables were sought. A total of 91 patients (55 males) underwent the Fontan procedure at a median age of 5.50 years (IQR: 3.33-9.50 years). Median age at last follow-up, death, or transplant was 21.89 years (IQR: 10.87-25.51 years). Following the Fontan procedure, 60 (66%) patients required an additional 144 median sternotomies and 61 (67%) required 139 percutaneous cardiac interventions. Pacemaker system placement/replacement was the most common intervention following the Fontan procedure. The median time to first cardiac surgery following the Fontan was 1.96 years (IQR: 0.06-8.42 years) while the median time to the first percutaneous intervention was 7.63 years (IQR: 0.65-15.89 years). Families of single ventricle patients should be counseled on the likelihood of requiring additional cardiac interventions following the Fontan procedure.

Screening for aortic aneurysm after treatment of coarctation.

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.

Failure to impact prevalence of arterial ischemic stroke in pediatric cardiac patients over three decades.

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.

Multi-modality imaging of an unusual congenital left ventricular diverticulum.

The authors describe the case of a newborn presenting with a pulsatile abdominal mass that was subsequently diagnosed as a large congenital muscular left ventricular diverticulum. This case illustrates the role of multimodality imaging in the diagnosis and surgical planning of this rare congenital heart disease. The diverticulum was managed with surgical resection without the use of cardiopulmonary bypass. Unlike in previous case reports, this patient did not have any associated intracardiac defect.

Comprehensive analysis of antibody responses to streptococcal and tissue antigens in patients with acute rheumatic fever.

Acute rheumatic fever (ARF) is an autoimmune disease occurring in individuals following untreated group A streptococcal infection believed to be triggered by antibodies to bacterial components that cross-react with human tissues. We developed a multiplexed immunoassay for the simultaneous quantitation of antibodies to nine streptococcal-related antigens including streptolysin O (SLO), DNase B, collagen I and IV, fibronectin, myosin, group A carbohydrate, M6 protein and streptococcal C5a peptidase. Utilizing this method, we examined serum from 49 ARF, 58 pharyngitis patients and age- and sex-matched controls in samples collected at initial disease onset, and at 4 weeks, 6 months and 1 year after diagnosis. Antibody responses were significantly higher for SLO, DNase B, M6 protein, group A carbohydrate and the cross-reactive antigens collagen I and myosin in ARF compared with pharyngitis patients (P

Contractile dysfunction in hypertrophied hearts with deficient insulin receptor signaling: possible role of reduced capillary density.

Diabetics have worse outcomes than nondiabetics after a variety of cardiac insults. We tested the hypothesis that impaired insulin receptor signaling in myocytes worsens cardiac remodeling and function following injury, even in the absence of hyperglycemia. Mice with cardiomyocyte-restricted knock out of the insulin receptor (CIRKO) and wild type (WT) mice were treated with isoproterenol (ISO) for 2 or 5 days. Heart rates and cardiac mass increased comparably following ISO in WT and CIRKO mice. After 5 days, WT hearts were hyperdynamic by echocardiographic and left ventricular pressure measurements. However, CIRKO hearts had a blunted increase in contractility and relaxation following ISO. Interestingly, single myocytes isolated from both CIRKO ISO and WT ISO hearts had increased cellular shortening with prolonged time to peak shortening vs. respective shams. Thus, loss of myocytes or extramyocyte factors, rather than intrinsic dysfunction of surviving myocytes, caused the blunted inotropic response in ISO treated CIRKO hearts. Indeed, CIRKO ISO mice had increased troponin release after 2 days and greater interstitial and sub-endocardial fibrosis at 5 days than did ISO WT. Apoptosis assessed by TUNEL and caspase staining was increased in CIRKO ISO compared to WT ISO hearts; however, very few of the apoptotic nuclei were clearly in cardiac myocytes. After 5 days of ISO treatment, VEGF expression was increased in WT but not in CIRKO hearts. In keeping with this finding, capillary density was reduced in CIRKO ISO relative to WT ISO. Basal expression of hypoxia-inducible factor-1alpha was lower in CIRKO vs. WT hearts and may explain the blunted VEGF response. Thus, absence of insulin receptor signaling in the cardiac myocyte worsens catecholamine-mediated myocardial injury, at least in part, via mechanisms that tend to impair myocardial blood flow and increase ischemic injury.