Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

New approach to interstage care for palliated high-risk patients with congenital heart disease.

OBJECTIVE: Home surveillance monitoring might identify patients at risk for interstage death after stage 1 palliation for hypoplastic left heart syndrome. We sought to identify the effect that a high-risk program might have on interstage mortality and identification of residual/recurrent lesions after neonatal palliative operations. METHODS: Between January 2006 to January 2010, newborns after stage 1 palliation for hypoplastic left heart syndrome or shunt placement were invited to participate in our high-risk program. Patients enrolled in our high-risk program comprise the study group. Patients who had similar operations between January 2002 and December 2005 comprise the control group. Comparisons are made between the 2 groups with respect to interstage mortality and the frequency and timing of interstage admissions requiring medical, catheter, or surgical treatment. RESULTS: Seventy-two patients met the criteria for our high-risk program. Fifty-nine (82%) of 72 patients were enrolled. Among 19 patients with hypoplastic left heart syndrome in our high-risk program, outpatient interstage mortality was zero. Outpatient interstage mortality for the 36 control subjects with hypoplastic left heart syndrome was 6%. Among 40 patients with shunts in the study group, there was 1 outpatient interstage death compared with 4 (6%) deaths in 68 subjects in the control group. Significant residual/recurrent lesions were identified with similar frequency between the 2 groups. However, after shunt operations, these lesions were detected and treated at significantly younger mean ages for patients followed in the high-risk program (P < .005). CONCLUSIONS: Initiation of a high-risk program might decrease interstage mortality after high-risk neonatal palliative operations. Such an approach might contribute to earlier detection of significant residual/recurrent lesions amenable to therapy.

Urgent permanent pacemaker implantation in critically ill preterm infants.

The management of complete heart block in premature low birth-weight infants, particularly those with hydrops fetalis, is challenging. We report emergent implantation of permanent epicardial pacemakers in the first 48 hours of life in two premature infants (one with hydrops fetalis) with birth weights of 1,400 grams and 1,000 grams.

Radiofrequency ablation of pediatric AV nodal reentrant tachycardia during the ice age: a single center experience in the cryoablation era.

BACKGROUND: Radiofrequency catheter ablation of atrioventricular nodal reentrant tachycardia (AVNRT) has proven to be an effective therapy in the pediatric population. However, concerns of inadvertent permanent AV nodal block have resulted in many pediatric programs adopting cryoablation as their primary ablation approach for AVNRT. METHODS: A retrospective analysis of the results of pediatric radiofrequency catheter ablation at a single institution over the most recent 5 years (January 2004 through December 2008) was performed. Acute, intermediate, and long-term success, along with the incidence of AV block, were determined. RESULTS: There were 65 patients with a mean age of 12.1 + or - 5.2 years and weight of 46.5 + or - 17.3 kg who underwent radiofrequency catheter ablation for AVNRT. There was 100% acute success with no recurrences at a mean follow up of 32.5 months. Although two patients had a brief second-degree AV block, there was no permanent AV block of any degree. CONCLUSIONS: The safety and efficacy of radiofrequency catheter ablation for pediatric AVNRT demonstrated in this study support its continued application and should not be abandoned as a method of treatment.

Amiodarone for pediatric resuscitation: a word of caution.

Intravenous administration of amiodarone has recently been recommended for use during pediatric resuscitation of pediatric patients with ventricular fibrillation or pulseless ventricular tachycardia. We present two pediatric patients who received amiodarone for polymorphic ventricular tachycardia, although they were ultimately determined to have congenital long QT syndrome. Amiodarone is contraindicated in this setting and may have exacerbated the ventricular arrhythmia.

Design and operation of the National Survey of Children with Special Health Care Needs, 2005-2006.

OBJECTIVES: This report presents the development, plan, and operation of the National Survey of Children with Special Health Care Needs (CSHCN), a module of the State and Local Area Integrated Telephone Survey, conducted by the Centers for Disease Control and Prevention's National Center for Health Statistics. This survey was designed to produce national and state-specific prevalence estimates of CSHCN, describe the types of services that they need and use, and assess aspects of the system of care for CSHCN. Funding for this survey was provided by the Matemal and Child Health Bureau, Health Resources and Services Administration. METHODS: A random-digit-dial sample of households with children younger than 18 years of age was constructed for each of the 50 states and the District of Columbia. All children in each identified household were screened for special health care needs. If CSHCN were identified in the household, a detailed interview was conducted for one randomly selected child with special health care needs. Detailed interviews were also conducted for a separate national sample of children, to generate estimates for children without special health care needs, and permit comparisons with CSHCN on all study measures. The respondents were parents or guardians who knew about the children's health and health care. RESULTS: A total of 192,083 household screening interviews were completed from April 2005 to February 2007. This resulted in 40,840 completed special-needs interviews and 6,113 completed interviews for children in the comparison (referent) sample. The weighted overall response rates were 56.1% for special-needs and 50.3% for referent-sample interviews.

Echocardiographic considerations during deployment of the Helex Septal Occluder for closure of atrial septal defects.

The Helex Septal Occluder is a new device used to close atrial septal defects via interventional catheterization. In order to study the role of echocardiography during its use, and to describe the morphologic variants of defects suitable for closure with this occluder, we evaluated all patients undergoing intended closure of an atrial septal defect with the Helex occluder. A combination of transthoracic, transesophageal, three-dimensional, and intracardiac echocardiography were used before, during, and after the procedure to characterize anatomy, assess candidacy for closure, guide the device during its deployment, and evaluate results. Among the 60 candidates included in the study, 11 were excluded because of transesophageal echocardiographic and/or catheterization data obtained in the laboratory. Attempts at closure were successful in 46 patients, and unsuccessful in 3. We successfully treated four types of defects. These were defects positioned centrally within the oval fossa with appreciable rims along the entire circumference of the defect, defects with deficient or absent segments of the rim, defects with aneurysm of the primary atrial septum, and defects with multiple fenestrations. Follow-up transthoracic echocardiograms taken at a median of 7 months demonstrated no residual defects in 21, trivial residual defects in 17, and small residual defects in 8 patients. In 20 patients, three-dimensional reconstructions were used to characterize the morphology of the defect and the position of the device. Because transesophageal echocardiography was often limited by acoustic interference from the device, intracardiac echocardiography was utilized in 3 cases to overcome this limitation.