Role of frontotemporal fiber tract integrity in task-switching performance of healthy controls and patients with temporal lobe epilepsy.

The objective of this study is to investigate the relationships among frontotemporal fiber tract compromise and task-switching performance in healthy controls and patients with temporal lobe epilepsy (TLE). We performed diffusion tensor imaging (DTI) on 30 controls and 32 patients with TLE (15 left TLE). Fractional anisotropy (FA) was calculated for four fiber tracts [uncinate fasciculus (UncF), arcuate fasciculus (ArcF), dorsal cingulum (CING), and inferior fronto-occipital fasciculus (IFOF)]. Participants completed the Trail Making Test-B (TMT-B) and Verbal Fluency Category Switching (VFCS) test. Multivariate analyses of variances (MANOVAs) were performed to investigate group differences in fiber FA and set-shifting performances. Canonical correlations were used to examine the overall patterns of structural-cognitive relationships and were followed by within-group bivariate correlations. We found a significant canonical correlation between fiber FA and task-switching performance. In controls, TMT-B correlated with left IFOF, whereas VFCS correlated with FA of left ArcF and left UncF. These correlations were not significant in patients with TLE. We report significant correlations between frontotemporal fiber tract integrity and set-shifting performance in healthy controls that appear to be absent or attenuated in patients with TLE. These findings suggest a breakdown of typical structure-function relationships in TLE that may reflect aberrant developmental or degenerative processes.

The ABCD stop signal data: Response to Bissett et al.

This paper responds to a recent critique by Bissett et al. of the fMRI Stop task used in the Adolescent Brain Cognitive Development℠ Study (ABCD Study®). The critique focuses primarily on a task design feature related to race model assumptions (i.e., that the Go and Stop processes are fully independent). In response, we note that the race model is quite robust against violations of its assumptions. Most importantly, while Bissett raises conceptual concerns with the task we focus here on analyzes of the task data and conclude that the concerns appear to have minimal impact on the neuroimaging data (the validity of which do not rely on race model assumptions) and have far less of an impact on the performance data than the critique suggests. We note that Bissett did not apply any performance-based exclusions to the data they analyzed, a number of the trial coding errors they flagged were already identified and corrected in ABCD annual data releases, a number of their secondary concerns reflect sensible design decisions and, indeed, their own computational modeling of the ABCD Stop task suggests the problems they identify have just a modest impact on the rank ordering of individual differences in subject performance.

Baseline brain function in the preadolescents of the ABCD Study.

The Adolescent Brain Cognitive Development (ABCD) Study® is a 10-year longitudinal study of children recruited at ages 9 and 10. A battery of neuroimaging tasks are administered biennially to track neurodevelopment and identify individual differences in brain function. This study reports activation patterns from functional MRI (fMRI) tasks completed at baseline, which were designed to measure cognitive impulse control with a stop signal task (SST; N = 5,547), reward anticipation and receipt with a monetary incentive delay (MID) task (N = 6,657) and working memory and emotion reactivity with an emotional N-back (EN-back) task (N = 6,009). Further, we report the spatial reproducibility of activation patterns by assessing between-group vertex/voxelwise correlations of blood oxygen level-dependent (BOLD) activation. Analyses reveal robust brain activations that are consistent with the published literature, vary across fMRI tasks/contrasts and slightly correlate with individual behavioral performance on the tasks. These results establish the preadolescent brain function baseline, guide interpretation of cross-sectional analyses and will enable the investigation of longitudinal changes during adolescent development.

Six-month atrophy in MTL structures is associated with subsequent memory decline in elderly controls.

Neurodegeneration precedes the onset of dementias such as Alzheimer's by several years. Recent advances in volumetric imaging allow quantification of subtle neuroanatomical change over time periods as short as six months. This study investigates whether neuroanatomical change in medial temporal lobe subregions is associated with later memory decline in elderly controls. Using high-resolution, T1-weighted magnetic resonance images acquired at baseline and six-month follow-up, change in cortical thickness and subcortical volumes was measured in 142 healthy elderly subjects (aged 59-90 years) from the ADNI cohort. Regression analysis was used to identify whether change in fourteen subregions, selected a priori, was associated with declining performance on memory tests from baseline to two-year follow-up. Percent thickness change in the right fusiform and inferior temporal cortices and expansion of the right inferior lateral ventricle were found to be significant predictors of subsequent decline on memory-specific neuropsychological measures. These results demonstrate that six-month regional neurodegeneration can be quantified in the healthy elderly and might help identify those at risk for subsequent cognitive decline.

A calcium signaling cascade essential for myosin thick filament assembly in Xenopus myocytes.

Spontaneous calcium release from intracellular stores occurs during myofibrillogenesis, the process of sarcomeric protein assembly in striated muscle. Preventing these Ca2+ transients disrupts sarcomere formation, but the signal transduction cascade has not been identified. Here we report that specific blockade of Ca2+ release from the ryanodine receptor (RyR) activated Ca2+ store blocks transients and disrupts myosin thick filament (A band) assembly. Inhibition of an embryonic Ca2+/calmodulin-dependent myosin light chain kinase (MLCK) by blocking the ATP-binding site, by allosteric phosphorylation, or by intracellular delivery of a pseudosubstrate peptide, also disrupts sarcomeric organization. The results indicate that both RyRs and MLCK, which have well-described calcium signaling roles in mature muscle contraction, have essential developmental roles during construction of the contractile apparatus.

Polygenic risk for psychiatric disorders correlates with executive function in typical development.

Executive functions are a diverse and critical suite of cognitive abilities that are often disrupted in individuals with psychiatric disorders. Despite their moderate to high heritability, little is known about the molecular genetic factors that contribute to variability in executive functions and how these factors may be related to those that predispose to psychiatric disorders. We examined the relationship between polygenic risk scores built from large genome-wide association studies of psychiatric disorders and executive functioning in typically developing children. In our discovery sample (N = 417), consistent with previous reports on general cognitive abilities, polygenic risk for autism spectrum disorder was associated with better performance on the Dimensional Change Card Sort test from the NIH Cognition Toolbox, with the largest effect in the youngest children. Polygenic risk for major depressive disorder was associated with poorer performance on the Flanker test in the same sample. This second association replicated for performance on the Penn Conditional Exclusion Test in an independent cohort (N = 3681). Our results suggest that the molecular genetic factors contributing to variability in executive function during typical development are at least partially overlapping with those associated with psychiatric disorders, although larger studies and further replication are needed.

Functional assessment of the Fontan operation: combined M-mode, two-dimensional and Doppler echocardiographic studies.

Combined M-mode, two-dimensional and Doppler echocardiographic studies were used to assess the postoperative status of 33 patients who had undergone the modified Fontan procedure. Twenty-four patients had surgical repair with use of a simple direct right atrium to pulmonary artery anastomosis. The remaining patients had repair with use of a prosthesis or associated Glenn shunt. Twenty-seven patients were studied early in the postoperative period (2 months or less) and the remaining patients were studied up to 6 years postoperatively. A total of 36 examinations were performed. Of the 33 patients, 13 had tricuspid atresia, 12 had double inlet left ventricle with hypoplastic right ventricular outlet chamber and 8 had complex lesions with atrioventricular canal, double outlet right ventricle or a hypoplastic ventricle. Postoperative assessment by M-mode and two-dimensional echocardiography demonstrated normal or mildly reduced ventricular function (ejection fraction greater than 40%) in 22 patients. In 24 patients, a "normal" flow pattern was observed in the pulmonary artery by pulsed Doppler echocardiography, with predominant diastolic flow and accentuation by atrial systole somewhat similar to the venous flow pattern observed in the superior vena cava. "Abnormal" flow patterns (disorganized systolic flow, absence of atrial waves and little or no increase with inspiration) were observed in nine patients with reduced ventricular function or residual shunt. Continuous wave Doppler study also demonstrated mild dynamic subaortic obstruction in two patients. Combined pulsed and continuous wave studies showed atrioventricular valve insufficiency in 10 patients. Follow-up studies revealed a satisfactory clinical course in most patients. Three patients died approximately 4 to 8 months after their Fontan operation.

Safety and utility of endomyocardial biopsy in infants, children and adolescents: a review of 66 procedures in 53 patients.

The benefits and risks of endomyocardial biopsy in infants, children and adolescents were determined by reviewing the indications for and complications and results of 66 procedures in 53 patients aged 2 months to 20 years. One patient had a pneumothorax, and three had a right ventricular perforation. Ventricular tachycardia developed in four patients; it was treated with lidocaine in three and was self-limited in one. The procedure was unsuccessful in two patients. Among 25 patients with a prebiopsy diagnosis of idiopathic dilated cardiomyopathy, microscopic features were consistent with cardiomyopathy in 24 (96%) and were normal in 1. Of nine patients with clinically suspected myocarditis, only two (22%) had microscopic evidence of inflammation, and seven had chronic nonspecific features suggestive of dilated cardiomyopathy. Of eight patients with unexplained arrhythmias, six (75%) had microscopic findings compatible with dilated cardiomyopathy and two had myocarditis. Biopsy tissue samples from seven patients with nondilated forms of cardiomyopathy (four hypertrophic, three restrictive) were consistent with the clinical diagnosis in six and were inadequate in one. Cardiac biopsies were also performed in four patients with other disorders. Among the 51 patients with adequate biopsy specimens, microscopic features were considered diagnostic in 5, confirmatory in 44 and not helpful in 2 with normal tissue. The results indicate that endomyocardial biopsy is safe in infants, children and adolescents. It is useful for the evaluation of cardiomyopathy and specific secondary forms of myocardial disease. There seems to be little correlation, however, between clinical and tissue diagnoses of myocarditis.

Two-dimensional echocardiographic spectrum of univentricular atrioventricular connection.

The spectrum of anomalies in hearts having a univentricular atrioventricular (AV) connection was examined by two-dimensional echocardiography in 183 patients and the anatomic findings were compared with angiography. The mode of AV connection was found to be of three types: double inlet via two AV valves; single inlet via one AV valve with absence of the other (left or right AV valve atresia); and common inlet via a common AV valve. Identification of an accessory chamber by two-dimensional echocardiography was possible with 90% sensitivity, but it was limited compared with angiography in patients with severely hypoplastic anterior chambers and pulmonary valve atresia. All patients with subaortic outlet foramen obstruction were detected. Great artery position and the presence of obstruction to pulmonary flow were correctly predicted in all but one patient. Two-dimensional echocardiography was superior to angiography for the detection of AV valve abnormalities which were present in 27% and included abnormal chordae, hypoplasia or dysplasia of either valve. Two-dimensional echocardiography should play an essential role in the complete preoperative assessment of patients with univentricular AV connection.

Surgical repair of uncomplicated atrial septal defect without "routine" preoperative cardiac catheterization.

Whether all patients with atrial septal defect should undergo cardiac catheterization before surgical correction is controversial. Of 152 patients of all ages with surgically documented isolated atrial septal defect (ostium secundum, ostium primum and sinus venosus types) who underwent preoperative two-dimensional echocardiography between January 1978 and December 1983, there were 55 (36%) (group 1) who did not have preoperative cardiac catheterization. These 55 patients are compared with the 97 patients (64%) who did have preoperative catheterization studies (group 2). Group 1 patients were younger (mean age 22 versus 36 years) and did not have clinical evidence of other associated cardiac disorders. Forty-four (80%) of the group 1 patients had typical findings for atrial septal defect on cardiac examination, chest radiograph and electrocardiogram compared with 16 (16%) of group 2 patients (p less than 0.001). The most frequent reasons for cardiac catheterization in group 2 patients were documentation of diagnosis, usually because of atypical clinical findings, and exclusion of associated cardiac abnormalities. Contrast echocardiography, radionuclide shunt studies and Doppler echocardiography were used as additional confirmatory tests in 36 patients (65%). In 19 patients (35%), two-dimensional echocardiography was the only confirmatory test. There were no false positive two-dimensional echocardiographic studies. There were no operative deaths or significant perioperative complications in any of the patients. At a mean follow-up of 28 months (range 6 to 64), there was one late death (3 1/2 years postoperatively) in a 61 year old man with chronic congestive heart failure.(ABSTRACT TRUNCATED AT 250 WORDS)

Caveats of balloon dilation of conduits and conduit valves.

The results and complications of percutaneous balloon dilation involving 10 patients with a stenotic right ventricle to pulmonary artery prosthetic conduit and 1 patient with an obstructed right atrium to left pulmonary artery Dacron graft (modified Fontan) are reported. For the 10 patients (14.5 +/- 5 years) with a right ventricle to pulmonary artery conduit, the mean (+/- SD) predilation conduit valve gradient was 57 +/- 22 mm Hg, right ventricular pressure 104 +/- 21 mm Hg and right ventricle to pulmonary artery gradient 75 +/- 23 mm Hg; 2 of the patients had additional pulmonary artery stenosis requiring dilation. In one patient, the balloon could not be advanced across the conduit valve. In 9 of 10 patients in whom dilation was successfully performed, the conduit valve gradient decreased by 59 +/- 13%, right ventricle to pulmonary artery gradient by 43 +/- 22% and right ventricular pressure by 31 +/- 11%. After dilation, right ventricular pressure was less than 65% of systemic pressure in seven patients, although no pressure was less than 40%. In 8 of the 11 patients, surgery was avoided or postponed. Complications included loss of a balloon fragment after rupture during the unsuccessful dilation of the right atrium to left pulmonary artery graft and circumferential balloon rupture requiring catheter retrieval of the distal portion of the balloon from the femoral vein after successful dilation of the right ventricle to pulmonary artery conduit. Conduit valve dilation by balloon can reduce but rarely eliminate conduit obstruction, and balloon rupture may occur and can result in fragment loss or embolization.

Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment.

This report details the two-dimensional echocardiographic assessment of 41 patients with Ebstein's anomaly. The anatomic spectrum of Ebstein's anomaly is correlated with surgical and autopsy observations in 66% of these patients. Morphologic abnormalities of the tricuspid valve and right heart structures were uniquely assessed by noninvasive two-dimensional echocardiographic examination. Features of the tricuspid valve that best related to decreased functional capacity were: 1) absence of the septal leaflet, and 2) pronounced tethering, restriction of motion and displacement of the anterior leaflet of the tricuspid valve. This 4 1/2 year experience suggests that two-dimensional echocardiography can replace angiography as the procedure of choice for diagnostic and morphologic assessment of Ebstein's anomaly. Cardiac catheterization is necessary only when specific hemodynamic questions or clinical inconsistencies exist.

Early and late results of the modified Fontan procedure for double-inlet left ventricle: the Mayo Clinic experience.

Between May 1974 and March 1989, 155 patients with double-inlet left ventricle had the Fontan procedure performed at the Mayo Clinic. Age at operation ranged from nearly 2 to 41 years (median 10). The operative mortality rate from 1974 through 1980 (39 patients) was 21%, but from 1981 through 1989 (116 patients) it was reduced to 9%. The 17 late deaths were secondary to reoperation (n = 8), progressive myocardial failure (n = 5), sudden arrhythmia (n = 3) and bleeding varices (n = 1). Neither operative nor late mortality rate was significantly related to age at operation. At follow-up of 6 months to 11 years (mean 4.9 years) in 111 patients, 88% were in good or excellent condition and 12% were in fair or poor condition. The Fontan operation can be performed with a mortality risk of less than 10% in properly selected patients with double-inlet left ventricle. Late results are encouraging when contrasted with the clinical course of patients before this operative approach was utilized.

Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases.

Atrial septal aneurysms have been related (either by association or as potential causes) to systolic clicks, atrial arrhythmias, systemic and pulmonary embolism, atrioventricular valve prolapse and atrial septal defect. To study these associations and the incidence of atrial septal aneurysm, we reviewed 80 consecutive patients (female to male ratio 1.9:1, mean age 47 years, range 1 day to 89 years) who had been identified prospectively as having an atrial septal aneurysm. These were found in 36,200 two-dimensional echocardiographic studies (incidence: 0.22% overall; 0.29% in the last year of the study done between 1978 and 1984). Three types of fossa ovalis aneurysm and one type of aneurysm involving the entire atrial septum were observed; a fossa ovalis aneurysm with leftward projection and excursion of less than 5 mm or an aneurysm involving the entire atrial septum with rightward projection was not observed. Atrial septal aneurysm occurred more often as an isolated abnormality than in association with other cardiac malformations, although all patients with an aneurysm involving the entire atrial septum had complex congenital cardiac anomalies of the hypoplastic right heart type. The reported associations between atrial septal aneurysms and atrial septal defect, atrioventricular valve prolapse, midsystolic clicks, atrial arrhythmias and cerebral ischemic events were examined. A hypothesis based on interatrial pressure gradients is proposed to explain the different motions and configurational characteristics of fossa ovalis aneurysms observed in these patients. All patients in whom atrial septal aneurysm is demonstrated should undergo examination for atrial septal defect. Atrial septal aneurysm should be specifically looked for in patients who have these associations and who undergo two-dimensional echocardiography, especially if these abnormalities are unexplained.

Acute results of balloon angioplasty of native coarctation versus recurrent aortic obstruction are equivalent. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators.

OBJECTIVES: This study sought to compare the immediate results and risk factors for suboptimal outcomes of percutaneous balloon angioplasty for native versus recurrent aortic obstruction. BACKGROUND: Some cardiology centers have been reluctant to adopt balloon angioplasty for treatment of native aortic coarctation, while advocating balloon angioplasty over an operation for treatment of postsurgical or recurrent aortic obstruction. METHODS: Acute results were analyzed from 970 procedures (422 native and 548 recurrent lesions) performed between 1982 and 1995 in 907 patients from 25 centers. An acute suboptimal outcome was defined as one or more of the following: residual systolic pressure gradient > or = 20 mm Hg, residual proximal to distal systolic pressure ration > or = 1.33 or a major complication (death, aortic transmural tear, stroke). RESULTS: Bal loon angioplasty significantly (p = 0.0001) increased lesion diameter fo r both native (mean [+/= SD] 128 +/= 94%) and recurrent aortic obstruction (97 +/= 87%), with a significantly greater increase in the native group (p = 0.0001). A reduction in systolic pressure gradients was significant in both groups (p = 0.0001), but slightly higher (p = 0.01) for native (-74 +/- 24%) versus recurrent obstruction (-70 +/- 31%). Death associated with angioplasty was reported in 0.7% of patients with native and in 0.7% of patients with recurrent lesions (p = 1.00). An acute suboptimal outcome was noted with angioplasty in 19% of native and in 25% of recurrent lesions (p = 0.04). Significant independent risk factors included higher preangioplasty systolic gradient (odds ratio [OR] 1.39/10-mm Hg increment; 95% confidence interval [CI] 1.28 to 1.50, p = 0.0001), earlier study date (OR 0.92/1-year increment, 95% CI 1.02 to 1.26, p = 0.02) and recurrent obstruction (OR 1.39 vs. native lesions, 95% CI 1.00 to 1.94, p = 0.05). CONCLUSIONS: Acute results and complications of balloon angioplasty of native coarctation appear to be equivalent or slightly superior to those of recurrent aortic obstructions.

Remote telemedical interpretation of neonatal echocardiograms: impact on clinical management in a primary care setting.

OBJECTIVE: The purpose of this study was to evaluate the utility of telemedical echocardiographically assisted neonatal cardiovascular evaluation in a primary care setting. BACKGROUND: Neonates with congenital heart disease are frequently born far from pediatric subspecialty centers and can be clinically unstable at presentation. Recent advances in telecommunication technology have made it possible to transmit echocardiographic images over long distances. This technology may be beneficial to newborns with heart defects who are born in primary care centers. METHODS: A retrospective review of all telemedical echocardiograms obtained from neonates (aged 1 day to 30 days) was performed. A telemedical link was created using a T-1 transmission line and a standard voice telephone line between the Mayo Clinic, Rochester, Minnesota (pediatric cardiology site), and the Altru Clinic, Grand Forks, North Dakota (primary care site), which is a general pediatric practice 400 miles from Rochester. Neonates with possible cardiac disorders were identified by the general pediatricians, who then requested telemedical echocardiography. RESULTS: The 133 neonates had 161 T-1 echocardiograms. Median patient age was two days (range, one day to 29 days). One hundred thirty-two of 133 initial echocardiograms (99%) were obtained because of urgent indications. Transmitted images provided adequate diagnostic information in all patients. Seventy-nine neonates (59%) had a change in medical management or required cardiology follow-up. An immediate change in management occurred in 32 patients (24%), including seven in whom emergency transfer was either arranged or avoided. CONCLUSIONS: Telemedical echocardiography provides accurate diagnostic data in neonates. Rapid telediagnosis facilitates appropriate care of sick neonates with possible congenital heart disease in the primary care setting. Unnecessary long-distance transfers can be avoided with this technology.

Sensitivity of two-dimensional echocardiography in the direct visualization of atrial septal defect utilizing the subcostal approach: experience with 154 patients.

In the standard precordial echocardiographic imaging planes, there is frequent dropout of atrial septal echoes in the region of the fossa ovalis that can be minimized by use of the subcostal imaging approach. The diagnostic sensitivity of this approach was reviewed in 154 patients (mean age 31 years, range 2 months to 74 years) with documented atrial septal defect in whom a satisfactory image of the atrial septum could be obtained. Subcostal two-dimensional echocardiography successfully visualized 93 (89%) of the 105 ostium secundum atrial septal defects, all 32 (100%) ostium primum defects and 7 (44%) of the 16 sinus venosus defects. A defect was not visualized (false negative response) in 12 patients (11%) with an ostium secundum defect and in 9 patients (56%) with a sinus venosus defect. In three of the former and five of the latter, a two-dimensional echocardiographic contrast examination established the presence of the interatrial shunt. Twenty-four patients (16%) with clinical findings of uncomplicated atrial septal defect confirmed by two-dimensional echocardiography underwent surgical repair of the defect without preoperative cardiac catheterization. There were no perioperative complications. Two-dimensional echocardiographic examination of the atrial septum utilizing the subcostal approach is the preferred method for the confident, noninvasive diagnosis and categorization of atrial septal defects. Two-dimensional echocardiographic contrast and Doppler examinations complement the technique and enhance diagnostic accuracy.

Anatomy and function of the heart and intrathoracic vessels in congenital heart disease: evaluation with the Dynamic Spatial Reconstructor.

The Dynamic Spatial Reconstructor is a unique high speed volume imaging X-ray scanner based on computed tomographic principles. It has several potential advantages over conventional angiographic methods, including reduced invasion, reduced rate of false negative results and increased accuracy of measurements of structure and function. To evaluate the utility of the Dynamic Spatial Reconstructor in the investigation of congenital heart disease, scanning was performed in several pediatric patients with pulmonary valve atresia. Early results show that three-dimensional images of the cardiac chambers and intrathoracic vessels can be acquired and displayed with the system. All the information necessary for quantitative analysis of the pulmonary arteries can be obtained with a single scan involving injection of 0.6 to 2.0 cc/kg of contrast agent and radiation exposure of 0.54 to 0.95 rad/s over 5 to 8 seconds.

Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients.

Simultaneous continuous wave Doppler echocardiography and right-sided cardiac pressure measurements were performed during cardiac catheterization in 127 patients. Tricuspid regurgitation was detected by the Doppler method in 117 patients and was of adequate quality to analyze in 111 patients. Maximal systolic pressure gradient between the right ventricle and right atrium was 11 to 136 mm Hg (mean 53 +/- 29) and simultaneously measured Doppler gradient was 9 to 127 mm Hg (mean 49 +/- 26); for these two measurements, r = 0.96 and SEE = 7 mm Hg. Right ventricular systolic pressure was estimated by three methods from the Doppler gradient. These were 1) Doppler gradient + mean jugular venous pressure; 2) using a regression equation derived from the first 63 patients (Group 1); and 3) Doppler gradient + 10. These methods were tested on the remaining 48 patients with Doppler-analyzable tricuspid regurgitation (Group 2). The correlation between Doppler-estimated and catheter-measured right ventricular systolic pressure was similar using all three methods; however, the regression equation produced a significantly better estimate (p less than 0.05). Use of continuous wave Doppler blood flow velocity of tricuspid regurgitation permitted determination of the systolic pressure gradient across the tricuspid valve and the right ventricular systolic pressure. This noninvasive technique yielded information comparable with that obtained at catheterization. Approximately 80% of patients with increased and 57% with normal right ventricular pressure had analyzable Doppler tricuspid regurgitant velocities that could be used to accurately predict right ventricular systolic pressure.

Correlation of two-dimensional echocardiographic and autopsy findings in complete transposition of the great arteries.

Autopsy and antemortem two-dimensional echocardiographic findings were compared in 30 cases to assess the predictive accuracy of two-dimensional echocardiography in complete transposition of the great arteries, and thus its potential for replacing or altering an invasive examination. These cases represent consecutive autopsy cases of transposition between January 1978 and December 1982. Ages at echocardiographic study ranged from 1 day to 15.5 years (median 8.5 months). Transposition of the great arteries had been diagnosed correctly in every case, and all associated anomalies identified by two-dimensional echocardiography were confirmed at autopsy. Several additional anomalies not found by two-dimensional echocardiography were apparent at autopsy; however, most were minor and insignificant. The only potentially significant false negative findings were one ventricular septal defect, two small atrial septal defects, one mitral valve anomaly, one tricuspid valve anomaly, three instances of patent ductus arteriosus and four instances of persistent left superior vena cava. Although significant pulmonary stenosis was predicted correctly, the site of stenosis was not accurately defined in four cases. Two-dimensional echocardiography is a reliable means of diagnosing transposition of the great arteries. It can limit the need for angiocardiography in the neonate; and in older infants and children, it should be used to complement cardiac catheterization and angiocardiography. The latter may be indicated, especially before surgical repair, to better define small ventricular septal defects, site of pulmonary stenosis, atrioventricular valve insufficiency and patent ductus arteriosus.