Renal thromboembolism from a large pulmonary artery to a pulmonary vein fistula in an asymptomatic adolescent.

Pulmonary arteriovenous fistula is a rare vascular anomaly that can cause significant morbidity and mortality. The presence and significance of symptoms are dependent on the size of the right-to-left shunt. Thromboembolic events may result in cerebrovascular accidents or systemic vascular occlusions. We present a case of an adolescent without cardiorespiratory symptoms, who developed flank pain due to renal infarction, followed by a brief literature review.

Examination of hyperlucent foci and clinical outcomes in pediatric cardiac patients on extracorporeal membrane oxygenation.

BACKGROUND: A number of cardiac patients on venoarterial extracorporeal membrane oxygenation (VA-ECMO) at our institution were found to have hyperlucent foci evident on echocardiography. However, the clinical significance of these findings is not known. METHODS: To investigate this phenomenon further we undertook a retrospective analysis to determine the prevalence and clinical implications of hyperlucent foci in children supported with VA-ECMO. RESULTS: From January 2002 to September 2009 49 subjects were supported with VA-ECMO. Of these, 12 (24%) were found to have hyperlucent foci. Foci were multifocal in six subjects (50%) and the most common location was left ventricular wall. No association was noted between primary cardiac diagnoses, or for inciting reason for placement on ECMO (extracorporeal cardiopulmonary resuscitation vs. transition from cardiopulmonary bypass to ECMO). There was no association between the presence of the foci and death in less than 14 days following decannulation from ECMO, hospital survival 42% versus 43%, P = 0.1. Among subjects who survived at least 14 days after ECMO decannulation hyperlucent foci resolved in 5 of 7. CONCLUSIONS: Hyperlucent foci can be seen in children supported by VA-ECMO. They do not portend a poorer prognosis. Among hospital survivors resolution is the norm.

Development and impact of arrhythmias after the Norwood procedure: A report from the Pediatric Heart Network.

OBJECTIVES: The study objective was to determine the predictors of new-onset arrhythmia among infants with single-ventricle anomalies during the post-Norwood hospitalization and the association of those arrhythmias with postoperative outcomes (ventilator time and length of stay) and interstage mortality. METHODS: After excluding patients with preoperative arrhythmias, we used data from the Pediatric Heart Network Single Ventricle Reconstruction Trial to identify risk factors for tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, junctional ectopic tachycardia, and ventricular tachycardia) and atrioventricular block (second or third degree) among 544 eligible patients. We then determined the association of arrhythmia with outcomes during the post-Norwood hospitalization and interstage period, adjusting for identified risk factors and previously published factors. RESULTS: Tachyarrhythmias were noted in 20% of subjects, and atrioventricular block was noted in 4% of subjects. Potentially significant risk factors for tachyarrhythmia included the presence of modified Blalock-Taussig shunt (P = .08) and age at Norwood (P = .07, with risk decreasing each day at age 8-20 days); the only significant risk factor for atrioventricular block was undergoing a concomitant procedure at the time of the Norwood (P = .001), with the greatest risk being in those undergoing a tricuspid valve procedure. Both tachyarrhythmias and atrioventricular block were associated with longer ventilation time and length of stay (P < .001 for all analyses). Tachyarrhythmias were not associated with interstage mortality; atrioventricular block was associated with mortality among those without a pacemaker in the unadjusted analysis (hazard ratio, 2.3; P = .02), but not after adding covariates. CONCLUSIONS: Tachyarrhythmias are common after the Norwood procedure, but atrioventricular block may portend a greater risk for interstage mortality.

Novel electrocardiographic screening criterion for hypertrophic cardiomyopathy in children.

Electrocardiography is often advocated as a screening tool in children for hypertrophic cardiomyopathy (HC). We sought to establish an electrocardiographic screening tool to identify children with HC. We hypothesized that a pediatric-specific electrocardiographic criterion would perform better than the popular criteria used for screening children for left ventricular hypertrophy and HC. The earliest available electrocardiogram for children (n=108) with HC (ages 7 to 21 yrs) was reviewed. We sought to compare the diagnostic accuracy of 4 screening algorithms: (1) Sokolow-Lyon criterion (SV1+RV5/RV6>35 mm), (2) Cornell criterion (RaVL+SV3>28 mm in men, 20 mm in women), (3) total 12-lead voltage criterion (R wave to the nadir of Q/S wave>175 mm), and (4) pediatric-specific criterion (RaVL+SV2>23 mm). The same criteria were applied to a cohort of age-matched and gender-matched controls without cardiac disease. Statistically significant correlations were found between children with HC and positive screen using all 4 criteria. However, comparison of receiver operating characteristic demonstrated an area under the curve of 0.67 for Sokolow-Lyon criterion, 0.70 for Cornell criterion, 0.83 for total 12-lead criterion, and 0.82 for pediatric-specific criterion. Pediatric-specific criterion had superior sensitivity in gene-positive children and superior overall specificity than total 12-lead criterion. In conclusion, our study demonstrates that the pediatric-specific criterion employing leads RaVL+SV2 is more accurate in identifying children with HC in comparison with other popular screening criteria.