Echocardiographic yield in children when innocent murmur seems likely but doubts linger.

The objective of our study was to determine the incidence and nature of heart disease found among children with murmurs clinically ambiguous to an expert examiner. The study was a prospective, blinded evaluation of accuracy of the expert examination using echocardiography as the diagnostic standard. The setting of the study was a pediatric cardiology outpatient department. The study comprised 903 outpatients with heart murmur under 21 years old without prior echocardiography or pediatric cardiology consultation. The intervention was echocardiography as clinically indicated for evaluation of heart murmur of uncertain cause. The outcome measure were a comparison of clinical diagnoses with echocardiographic results. In this clinical population, the presence of heart disease correlated with young age at presentation and with the expert examiner's level of suspicion of heart disease. However, 16 of 187 cases in which specific pathology was unsuspected had disease, and 6 of these 16 have had catheter or surgical intervention. Fourteen of the 16 unsuspected had objective indications for echocardiography and the other 2 were examined to allay anxiety. When evaluating very young outpatients with innocent-sounding murmur or older outpatients with innocent-sounding murmur and disconcerting symptoms, signs, or laboratory results, pediatric cardiologists should have a low threshold for echocardiography. Older outpatients with innocent-sounding murmur seldom have heart disease when anxiety is the only indication for echocardiography.

Clinical and basic laboratory assessment of children for possible congenital heart disease.

Accurate and cost-effective detection of congenital heart disease is a priority for the primary care physician, but there are many diagnostic modalities and strategies available. Within the past year, investigators have reported factors that contribute to the failure to diagnose congenital heart disease before autopsy. Recent research also highlights the strengths and weaknesses of the clinical evaluation by the primary care physician. An examination by a pediatric cardiologist is a more specific discriminator between heart disease and innocent murmur and can, in many instances, eliminate the expense of relying on echocardiography for diagnosis. Current reports document substantial limitations of diagnostic utility of electrocardiography and chest roentgenography. Suspicions that echocardiography can be misleading when performed in laboratories without expertise in congenital heart disease are confirmed in recent organized comparisons of accuracy. High-quality, well-designed tele-echocardiography programs are shown to be effective solutions when maldistribution of pediatric cardiology services hampers diagnostic efficiency.

Effects of electrocardiography and chest radiography on the accuracy of preliminary diagnosis of common congenital cardiac defects.

The objective of this study was to compare the accuracy of the expert clinical examination for certain common cardiac defects with and without electrocardiogram (EKG) and chest radiogram (x-ray). The design of the study was a prospective, blinded comparison of diagnostic accuracy of the expert examination with and without EKG and x-ray, using echocardiography as the diagnostic standard. The setting of the study was the pediatric cardiology outpatient department. There were 749 outpatients with heart murmur under 21 years of age without prior echocardiography or pediatric cardiology consultation. The intervention was echocardiography as clinically indicated for evaluation of heart murmur of uncertain cause. Measurements were carried out using the incorporation of EKG and x-ray into multiple linear regression models to assess independent associations, if any, with the accuracy of clinical examination. Results were reported as the presence or absence of independent significant impact of availability of EKG and x-ray on examiner's diagnostic accuracy for innocent murmur, ventricular septal defect (VSD), pulmonary stenosis (PS), aortic valve disease, atrial septal defect (ASD), and patent ductus arteriosus. EKG enhanced detection of ASD and may have helped detect PS. X-ray enhanced detection of intermediate to large VSD. X-ray and EKG were otherwise without demonstrable independent advantage for defect-specific diagnosis. Routine use of one or both of these tests in the initial evaluation of heart murmur in the pediatric cardiology clinic should remain an option.

CT angiography of complications in pediatric patients treated with intravascular stents.

OBJECTIVE: Our goal was to determine whether CT angiography can reveal complications in pediatric patients and young adults treated with intravascular stents for obstructive vascular lesions. CONCLUSION: CT angiography can reveal complications in pediatric patients treated with intravascular stents for obstructive lesions. Potentially, CT angiography could replace the more invasive conventional angiography currently used for intravascular stent placement and follow-up examinations.

Pulmonary stenosis: defect-specific diagnostic accuracy of heart murmurs in children.

OBJECTIVE: To determine the accuracy of expert examination for pulmonary stenosis (PS) among children with murmur. STUDY DESIGN: Five hundred twenty-one consecutive, previously unevaluated, pediatric patients were enrolled. The investigators prospectively recorded their diagnosis and level of confidence, categorizing any PS suspected as mild or severe. After echocardiography, PS was categorized by severity with peak systolic flow velocity. Receiver operating characteristic curves described accuracy of clinical examination. RESULTS: Sixty-two patients had PS (mild, 29; moderate, 27; and severe, 6). Receiver operator characteristic curve areas were: total, 0.834 +/- 0.033; mild, 0.862 +/- 0.044; and moderate to severe, 0.809 +/- 0.046 (P =.20). Specific difficulties in discrimination of PS from small ventricular septal defect, aortic valve disease, atrial septal defect, and innocent murmur were identified. All cases of severe PS in which PS was suspected were thought possibly severe. CONCLUSIONS: Although expert clinical examination is highly accurate for distinguishing PS from non-PS cardiac murmurs in pediatric patients, it is imperfect.

Radiofrequency catheter ablation for paroxysmal supraventricular tachycardia in children and adolescents without structural heart disease. Pediatric EP Society, Radiofrequency Catheter Ablation Registry.

Since 1990, management options available for children with paroxysmal supraventricular tachycardia (PSVT) have included radiofrequency catheter ablation (RCA). To determine the efficacy and safety of the procedure and to maintain a database for long-term follow-up, the Pediatric Electrophysiology Society began a Pediatric RCA Registry on January 1, 1991, to which 46 centers have submitted data from 4,135 total children and adolescents (patient age 0.1 to 20.9 years) who underwent 4,651 RCAs (through September 15, 1996). Of the 88% with a structurally normal heart, PSVT mechanisms (n = 4,030) included 3,110 accessory pathways and 920 atrioventricular node reentry tachycardia (AVNRT) during 3,653 procedures for 3,277 patients. During the 7 years of the Registry, analysis of indications for the procedure has shown a gradual shift. During the first year of the Registry for this PSVT group, "medically refractory tachycardia" was listed as the indication for 44% and "patient choice" was listed as 33%, compared with 29% and 58%, respectively, for the years 1995 to 1996 (p <0.005). Registry results were: 90% immediate success for accessory pathways (95% for left lateral; 87% for septal; 86% for right free wall) and 96% for AVNRT; mean fluoroscopy time 47.6 +/- 40 SD minutes; procedure time 257 +/- 157 SD minutes; major complication rate at the time of the procedure 3.2%. Procedure-related deaths included 1 immediate and 3 at 2, 12 and 68 weeks after the procedure (2 were infants). Follow-up revealed 77% and 71% freedom from recurrence at 3 years for accessory pathways AVNRT, respectively, and rare (<1%) detection of additional complications. RCA has evolved into a standard management option for PSVT in children with a structurally normal heart. RCA for children and adolescents should be recommended after consideration of the procedural risk/benefit compared with that of other management options, the natural history, and individual tolerance/symptoms related to PSVT.

Children with heart murmurs: can ventricular septal defect be diagnosed reliably without an echocardiogram?

OBJECTIVES: This study was undertaken to determine the accuracy of expert examination for ventricular septal defect (VSD) among children with a heart murmur. BACKGROUND: Because the frequency and nature of errors that might be made by reliance solely on expert examination for diagnosis of VSD are speculative, the role of echocardiography in such diagnosis is controversial. METHODS: Two hundred eighty-seven consecutive previously unevaluated pediatric subjects were enrolled in the study. For each child, the pediatric cardiologists prospectively recorded a working diagnosis and their level of confidence in the diagnosis, categorizing any VSD diagnosed as small or moderate to large. After echocardiography, VSDs were subcategorized by location and requirement for treatment as minor, intermediate or major. Receiver-operating characteristic (ROC) curves described the accuracy of the clinical examination. RESULTS: Seventy-three subjects had a VSD (minor in 52, intermediate in 10 and major in 11). ROC areas (1.0 = perfect discrimination, 0.5 = indiscriminate) were minor VSD 0.92 +/- 0.02 and major/intermediate VSD 0.69 +/- 0.07 (p = 0.0016). Four of 52 minor VSDs were not identified at any level of suspicion; the clinical diagnoses were moderate to large VSD in two patients and atrial septal defect and unlimited differential diagnosis in one patient each. Fourteen of 235 patients without a minor VSD were believed with confidence to have a small VSD, but the final diagnosis was intermediate VSD in 4, innocent murmur in 3, major VSD in 2, pulmonary stenosis in 2 and subaortic membrane, atrial septal defect and mitral regurgitation in 1 patient each. CONCLUSIONS: Almost all minor VSDs are recognized without echocardiography; however, errors can occur even when an expert examiner is confident. Clinical recognition of an intermediate or major VSD is less accurate than clinical recognition of a minor VSD. Failure to distinguish VSDs of major or intermediate importance from minor VSDs is a weakness of the expert clinical examination.

Management of infants, children, and adolescents with paroxysmal supraventricular tachycardia.

Several acceptable options are available for the successful management of children either with an acute PSVT episode or with ongoing episodes. These options include the "no treatment" management approach. Although an example of an algorithm used in one center is provided for this Medical Progress article, other algorithms also are successfully practiced among pediatric cardiologists together with primary care pediatricians. Current and ongoing updated data related to the important factors of presenting symptoms, natural history, results of the treatment options, and the risk/ benefit ratios of the treatment options are essential when one is choosing the specific management approach.

The learning curve for radiofrequency ablation of tachyarrhythmias in pediatric patients. Participating members of the Pediatric Electrophysiology Society.

The results of radiofrequency ablation for treatment of supraventricular tachyarrhythmias have been reported to improve with increasing experience; however, the precise nature of the learning curve in children is unknown. From November 1990 to October 1993, 1,546 consecutive procedures from the Pediatric Radiofrequency Ablation Registry were categorized into deciles based on number of prior pediatric procedures at the submitting institution. Negative exponential models were tested for strength of relation between volume of prior experience and 4 measures of outcome: success rate, complication rate, fluoroscopy time, and procedure time. Negative exponential curves described the experience-outcome relations well (r = 0.81 to 0.97). Learning rates were most rapid for successful ablation of left free wall accessory pathways, and slowest for right free wall pathway ablation. These models suggest that, given enough experience, procedural success rates > 90% (regardless of pathway location) and fluoroscopy and procedure times averaging < 40 minutes and 250 minutes, respectively, can be achieved in pediatric patients.

Cost-effectiveness of echocardiography for evaluation of children with murmurs.

Echocardiography is widely accepted as an accurate diagnostic test to evaluate heart murmurs in children, however its costs, and the ubiquity of murmurs in children, discourage its universal application. The purpose of this article is to identify some clinical circumstances in which the cost of echocardiography is justified for the evaluation of heart murmurs in infants and children. Eight common clinical problems were selected in which a heart murmur is present and a diagnosis is called for. Effectiveness of echocardiography and less costly clinical diagnostic methods in these settings were compared. In some circumstances, echocardiography is worth the cost, because clinical evaluation is unacceptably insensitive to important disease (the premature infant with a murmur which might represent a patent ductus arteriosus, the infant with a dysmorphic syndrome and a murmur). In others, the expert clinical examination is highly accurate (the asymptomatic child with a heart murmur) and is preferred over the echocardiogram as the initial diagnostic approach on the grounds of cost. When the expert clinical examination suggests minor structural heart disease, a continuum of echocardiographic cost-effectiveness relative to the expert clinical examination exists between these extremes depending on the working diagnosis. A threshold has not yet been defined at any point on this continuum above which the public will demand the greater diagnostic detail available echocardiographically, and below which the public will refuse to accept its greater cost. Quantitative formal cost-effectiveness analysis of echocardiography for evaluation of heart murmur in infants and children is not yet feasible because the benefits of echocardiography are indirect, dependent upon the as yet unmeasured benefits of correct management of congenital heart defects. To go beyond simple comparison of efficacy of echocardiography with less costly methods, further work is required in outcomes research in congenital heart disease.

Origin of both coronary arteries from the pulmonary artery.

Origin of both coronary arteries from the pulmonary artery is generally a lethal condition from progressive ventricular failure. We report the clinical and surgical course of two infants, ages 3 and 6 months, with this anomaly. One patient had normal intracardiac anatomy with low pulmonary artery pressures (30/12 mm Hg). The second patient had a restrictive subpulmonic ventricular septal defect with a moderately elevated pulmonary artery pressure (50/13 mm Hg). Left ventricular ejection and shortening fractions were profoundly depressed in both patients. The common coronary trunk arose from the right anterior facing sinus in one patient and from the left posterior facing sinus in the other. Both patients underwent repair by direct coronary implantation to the aorta. Left ventricular function improved with shortening fractions near normal at a follow-up of 6 months for one patient and 1 year for the other. Early diagnosis and prompt repair is compatible with survival and return of normal shortening fraction.

Radiofrequency catheter ablation for tachyarrhythmias in children and adolescents. The Pediatric Electrophysiology Society.

BACKGROUND: Although radiofrequency catheter ablation has been used extensively to treat refractory supraventricular tachycardia in adults, few data are available on its safety and efficacy in children and adolescents. We reviewed registry data obtained from 24 centers to evaluate the indications, early results, complications, and short-term follow-up data in young patients who underwent this procedure. METHODS: Standardized data were submitted for 652 patients who underwent 725 procedures between January 1, 1991, and September 1, 1992. The mean length of follow-up was 13.5 months. RESULTS: The median age of the patients was 13.5 years, and 84 percent of them had structurally normal hearts. The initial success rates for ablation of atrioventricular accessory pathways (508 of 615 procedures) and atrioventricular-node reentry (63 of 76 procedures) were both 83 percent. Greater institutional experience in performing ablation in children and location of the accessory pathway in the left free wall correlated with greater likelihood of sustained success. Conversely, a right free-wall pathway, the presence of other heart disease, and higher body weight were all associated with a lesser chance of sustained success. Recurrences of arrhythmia accounted for 45 percent of the failures overall in the series. Atrial ectopic-focus tachycardia had the highest recurrence rate. The total complication rate was 4.8 percent (35 of 725 procedures), and the only variables that independently correlated with a higher complication rate were very low weight and less institutional experience. CONCLUSIONS: These early results suggest that radio-frequency catheter ablation has a good success rate and a low complication rate in pediatric patients, especially when it is carried out in experienced pediatric cardiology centers.

Chronic monoarticular arthritis and acute pericardial tamponade in a child with Crohn's disease.

We describe a 7-year-old child who developed right knee arthritis unresponsive to nonsteroidal anti-inflammatory drug therapy. Additional rheumatologic disorders including pleuropericardial effusion with tamponade supervened before the recognition of Crohn's disease involving the colon. Suppression of all sites of inflammation resulted from corticosteroid therapy. Aspects of diagnosis and treatment of these extraintestinal components of inflammatory bowel disease are discussed.

Definition of inseparably fused ventricular myocardium in thoracopagus: fetal echocardiographic utility and pathologic refinement.

Correlative echocardiographic and pathological findings in a thoracopagus with conjoined hearts are reported. One twin had tricuspid atresia with discordant atrioventricular connections and concordant ventriculoarterial connections. The morphologic right ventricle was hypoplastic and there was a large muscular ventricular septal defect. The other twin had hypoplasia of the mitral valve anulus and left ventricle with double-outlet right ventricle and pulmonary valve atresia. The tricuspid valve was severely insufficient in part because of a large orifice and redundant, elongated leaflets with abnormal chordal attachments. The left ventricles of these two twins shared a perforated common "free wall" with at least two large defects allowing mixing of the circulations at that level. Not all anatomic details were established conclusively by fetal echocardiography; however, sufficient diagnostic information was obtained to support a decision not to aggressively resuscitate these twins after elective cesarean delivery at 31 weeks' gestation.