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.

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.

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.

Associated occurrence of persistent truncus arteriosus and asplenia.

An infant with persistent truncus arteriosus associated with splenic agenesis and the asplenia syndrome is reported, including clinical, echocardiographic and autopsy findings. To the authors' knowledge this association has not been previously reported.

Ventricular fibrillation during transesophageal atrial pacing in an infant with Wolff-Parkinson-White syndrome.

A complication of transesophageal atrial pacing in an infant with Wolff-Parkinson-White syndrome (WPW) is reported. A newborn infant born with fetal hydrops had recurrent supraventricular tachycardia (SVT) that required repeated successful conversion by transesophageal atrial pacing. Because of secondary left ventricular dysfunction, digoxin was administered. During repeat transesophageal atrial pacing for recurrent SVT, ventricular fibrillation occurred. Although it is unclear which of several possible contributing factors was responsible for the ventricular fibrillation, recommendations are appropriate to minimize the risk in infants with WPW.

Late potentials and inducible ventricular tachycardia in surgically repaired congenital heart disease.

We compared signal-averaged electrocardiography with invasive electrophysiological study in patients after surgical repair of congenital heart disease to determine if potentially useful correlations exist between the two methods for assessment of risk for ventricular tachycardia. Thirty-one patients (age, 1-49 years; mean, 10.6 years) with congenital heart disease repaired with right ventriculotomy or postrepair right bundle branch block (77% postoperative tetralogy of Fallot) who had electrophysiological study were studied with signal-averaged electrocardiography. Patients were classified by electrophysiological study results as having no inducible ventricular tachycardia, nonsustained ventricular tachycardia, or sustained ventricular tachycardia. Signal-averaged electrocardiograms were examined for the duration of low-amplitude (less than or equal to 40 microV) QRS signal, duration of total QRS, and root-mean-square voltage of the terminal 40 msec of the QRS. Low-amplitude terminal root-mean-square voltage of 100 microV or less had 91% sensitivity and 70% specificity for ventricular tachycardia inducible by electrophysiological study. Similar sensitivity but less specificity were seen using the criterion of 20 msec or more total low-amplitude QRS signal (initial plus terminal) or using total QRS duration of 128 msec or more. There was a weaker association between terminal low-amplitude QRS signal of 15 msec or more and inducible ventricular tachycardia.(ABSTRACT TRUNCATED AT 250 WORDS)

Omphalocele associated with coronary sinus atrial septal defect: report of two cases.

Two children with omphalocele were found to have coronary sinus atrial septal defect as part of their congenital heart disease complex. This unusual atrial communication may elude preoperative detection unless specifically sought. Its occurrence in conjunction with omphalocele may represent more than a chance association.

Refractory congestive heart failure after ribavirin in infants with heart disease and respiratory syncytial virus.

Although treatment with ribavirin has been known to be associated with a decreased mortality in infants with congenital heart disease (CHD) who have respiratory syncytial virus (RSV), few data are available regarding morbidity. We reviewed records of 10 consecutively hospitalized infants with CHD during a recent RSV epidemic. Despite the presence of left-to-right shunt in each patient, symptoms of RSV were respiratory at presentation. After ribavirin, decreased respiratory symptoms were found in 8 infants but in 2 assisted ventilation were required 1 and 3 days after admission. Congestive heart failure worsened in 8 patients, 6 of whom had improved respiratory status after ribavirin. Of the 8 patients with worse CHF (pulmonary edema), 3 responded to medical management but 5 were refractory and 4 required surgical repair of CHD. One patient died of pulmonary hemorrhage. Medically refractory CHF may develop in infants with CHD who become infected with RSV and are treated with ribavirin. Further studies are needed to determine whether the pulmonary edema is caused by RSV, ribavirin, or combination of effects on pulmonary capillary function or some other unrecognized mechanism.

Signal-averaged electrocardiography of the terminal QRS in healthy young adults.

Interpretation of signal-averaged electrocardiograms (SAECG) in the young could be of value in detecting those at risk for episodic ventricular tachycardia, but suffers from a lack of data in normal young people. The purpose of this study is to determine normal values for QRS duration and the duration and amplitude of terminal potentials on the SAECG in young adults. Thirty-two normal medical students were examined. With high pass filtering at 25 Hz, normal total QRS duration (QRS) varied as a function of sex and body size whereas low amplitude signal duration (LAS) did not. Ninety-five percent confidence limits are: QRS (male) 85-117 msec, QRS (female) 76-102 msec, and LAS 6-35 msec. Root mean square voltage of the terminal QRS showed a broad scatter, however none was less than 20 microvolts. High pass filtering at 40 Hz did not change the QRS duration, but resulted in significantly longer LAS duration and diminished RMS voltage. Because of the longer QRS and shorter LAS previously reported in the presence of right bundle branch block, the normal values reported here should not be applied in the presence of intraventricular conduction delay following surgical repair of congenital heart disease. They will, however, provide a basis for interpretation of SAECG in young adults with normal QRS duration.

Transcatheter closure of patent ductus arteriosus in pediatric patients.

A "double disk" occlusion device (Rashkind PDA occluder system) has been designed to allow transcatheter closure of patent ductus arteriosus (PDA). During a 3-year period, we examined 37 infants and children (not premature neonates) with PDA as their only significant cardiovascular defect. Seventy-eight percent of these patients were considered potential candidates for the use of the PDA occlusion device, and it was successfully placed in all but 2 of 27 patients who underwent cardiac catheterization (PDA too small in one and too large in one). Only one of the remaining 25 patients had a significant residual shunt after placement of the device, which therefore was removed in the catheterization laboratory before surgical ligation of the PDA. There was no clinical evidence of a residual PDA in any patient. The duration of hospitalization was less than 24 hours for each of the last 20 procedures. Echocardiography demonstrated return to normal chamber dimensions in all patients, but a tiny, clinically undetectable residual leak around the device was found in three patients by Doppler evaluation. The transcatheter procedure was preferred over surgery by more than 90% of families and can be completed with a much shorter hospitalization than surgical ligation. We conclude that transcatheter closure of patent ductus arteriosus is feasible in the majority of children with PDA.

Juvenile polysaccharidosis with cardioskeletal myopathy.

Polysaccharidoses with ultrastructural features reminiscent of glycogenosis type IV, but without enzymatic correlation, have been observed in several adolescent and adult patients. Little is known of the clinical, pathologic, or biochemical nature of these disorders. We describe a patient with ultrastructural characteristics consistent with glycogenosis type IV, but with normal brancher enzyme activity in dermal fibroblasts and cardiac muscle. During life and at autopsy, electron microscopy revealed amylopectin-like polysaccharide deposits present in a wide variety of tissues. The polysaccharidosis of our patient and similar patients may be a variant of glycogenosis type IV with a yet to be defined enzymatic defect.