Improved detection of coarctation of the aorta using speckle-tracking analysis of fetal heart on last examination prior to delivery.

OBJECTIVE: The false-positive rate for prenatal diagnosis of coarctation of the aorta (FP-CoA) commonly exceeds 50%, with an accurate detection rate of < 50%. This study was conducted to determine if the sensitivity for prenatal detection of true CoA and the FP-CoA rate could be improved by evaluating the fetal epicardial size and shape in the four-chamber view (4CV) and the endocardial right (RV) and left (LV) ventricular size, shape and contractility. METHODS: We analyzed retrospectively Digital Imaging and Communications in Medicine (DICOM) clips of the 4CV from the last examination prior to delivery in a series of 108 fetuses with CoA suspected prenatally by pediatric cardiologists using traditional diagnostic criteria. Postnatal evaluation distinguished those fetuses which subsequently required CoA surgery (true positives; true CoA) from those that were FP-CoA. Postnatal cardiac abnormalities were identified for each group. For the prenatal evaluation, we measured the 4CV end-diastolic epicardial area, circumference, length, width and global sphericity index. Speckle-tracking analysis was used to compute the endocardial RV and LV end-diastolic area, length, 24-segment sphericity index, 24-segment transverse width and the following functional parameters: fractional area change; global longitudinal, free-wall and septal-wall strain; basal-apical-length, basal free-wall and basal septal-wall fractional shortening; septal-wall annular plane systolic excursion; 24-segment transverse-width fractional shortening; and LV end-diastolic and end-systolic volumes, stroke volume, cardiac output and ejection fraction. In addition, the RV/LV end-diastolic area ratio was computed. Using a control group of 200 normal fetuses, the mean and SD for each of the above cardiac measurements was used to compute the Z-scores for each measurement in each of the 108 study fetuses. Logistic regression analysis was then performed on the Z-score values to identify variables that separated the true CoA group from the FP-CoA group. RESULTS: Of the 108 study fetuses, 54 were confirmed postnatally to have true CoA and 54 were FP-CoA. Right/left area disproportion > 90th centile was present in 80% (n = 43) of the true-CoA fetuses and 76% (n = 41) of the FP-CoA fetuses. Fetuses with true CoA had a significantly greater number of associated cardiac abnormalities (93%, n = 50) compared with the FP-CoA fetuses (61%, n = 33) (P < 0.001). The most common associated malformations were bicuspid aortic valve (true CoA, 46% (n = 25) vs FP-CoA, 22% (n = 12); P < 0.01), aortic arch hypoplasia (true CoA, 31% (n = 17) vs FP-CoA, 11% (n = 6); P < 0.01), ventricular septal defect (true CoA, 33% (n = 18) vs FP-CoA, 11% (n = 6); P < 0.05) and mitral valve abnormality (true CoA, 30% (n = 16) vs FP-CoA, 4% (n = 2); P < 0.01). Logistic regression analysis identified 28 variables that correctly identified 96% (52/54) of the fetuses with true CoA, with a false-positive rate of 4% (2/54) and a false-negative rate of 4% (2/54). These variables included the epicardial size in the 4CV, size and shape of RV and LV, and abnormal contractility of RV and LV. The area under the receiver-operating-characteristics curve was 0.98 (SE, 0.023; 95% CI, 0.84-1). There was no significant difference in the percent of fetuses with RV/LV area disproportion between those with CoA and those that were FP-CoA. CONCLUSIONS: Speckle-tracking analysis of multiple ventricular measurements may be helpful to refine the diagnosis in fetuses that are suspected to have CoA prenatally. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Quantitative evaluation of fetal right and left ventricular fractional area change using speckle-tracking technology.

OBJECTIVES: To measure, using speckle-tracking technology, the fractional area change (FAC) of the right and left ventricles in normal fetal hearts between 20 and 40 weeks of gestation. METHODS: The four-chamber view of the fetal heart was obtained in 200 normal fetuses between 20 and 40 weeks of gestation. FAC was computed from the ventricular areas (((end-diastolic area - end-systolic area)/end-diastolic area) × 100) for the right and left ventricles, and regressed against seven independent biometric and age variables. FAC was correlated with longitudinal fractional shortening (LFS) (((end-diastolic longitudinal length - end-systolic longitudinal length)/end-diastolic longitudinal length) × 100) obtained from the mid-ventricular basal-apical lengths of the right and left ventricular chambers and with transverse fractional shortening (TFS) (((end-diastolic transverse length - end-systolic transverse length)/end-diastolic transverse length) × 100) from three transverse positions (base, mid, apical) located within each ventricular chamber. To evaluate potential clinical utility, FAC, LFS and TFS results were examined in nine fetuses with a congenital heart defect (CHD). RESULTS: Regression analysis demonstrated significant associations between FAC and the independent biometric and age variables (R2  = 0.13-0.15). FAC was significantly correlated with LFS (R2  = 0.18-0.28) and TFS (R2  = 0.13-0.33). Examination of the fetuses with CHD revealed that six of the nine had abnormal FAC Z-score values for the index pathological ventricle. When abnormal LFS and TFS values were compared to the FAC in these fetuses, the FAC was either abnormally low or normal. CONCLUSIONS: This study reports results from measuring the FAC of the right and left ventricles, and demonstrates a correlation with LFS and TFS. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

24-segment sphericity index: a new technique to evaluate fetal cardiac diastolic shape.

OBJECTIVE: Because of parallel circulation in the fetus and the differential effect that various disease states may have on the shape of the right and left ventricles, this study was conducted to evaluate the sphericity index (SI) of 24 transverse segments distributed from the base to the apex of each of the ventricular chambers. METHODS: Two hundred control fetuses were examined between 20 and 40 weeks of gestation. The displacement of the ventricular endocardium during the cardiac cycle was computed using offline speckle-tracking software. From the ASCII output of the analysis, we analyzed 24 end-diastolic transverse segments, distributed from the base to the apex of each ventricle, as well as the end-diastolic mid-basal-apical length. The SI was computed for each of the 24 segments by dividing the mid-basal-apical length by the transverse length for each segment. Regression analysis was performed against biometric measurements and gestational age according to last menstrual period and ultrasound. Eight fetuses, in which the four-chamber view appeared subjectively to demonstrate chamber disproportion, were evaluated as examples to demonstrate the utility of this technology. RESULTS: The SI for each segment was independent of gestational age and fetal biometric measurements. The SI of the right ventricle was significantly (P < 0.001) lower than that of the left ventricle for segments 1-18, suggesting that the right ventricle was more globular in shape than was the left ventricle at the base, mid and a portion of the apical segments of the chamber. Fetuses with various cardiac structural abnormalities and abnormal fetal growth had abnormal SI values that reflected either a more globular or a more flattened ventricular chamber. CONCLUSION: Determination of SI for each of 24 segments of the fetal right and left ventricles provides a comprehensive method to examine the shape of the ventricular chambers. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Valve-sparing operation for balloon-induced aortic regurgitation in congenital aortic stenosis.

OBJECTIVE: Aortic regurgitation after balloon dilation of congenital aortic stenosis may be treated with valve repair as an alternative to replacement. METHODS: Charts and echocardiograms of all patients undergoing aortic valve operations after balloon dilation of congenital aortic stenosis at our institution between January 1988 and December 1999 were reviewed. RESULTS: Twenty-one patients underwent valvuloplasty for predominant aortic regurgitation 9 months to 15 years (mean, 6.1 years) after balloon dilation. The mean +/- SD age at the time of the operation was 11 +/- 7 years. Aortic regurgitation was caused by a combination of commissural avulsion (10), cusp dehiscence with retraction (9), cusp tear (5), central incompetence (2), perforated cusp (1), or cusp adhesion to the aortic wall (1). Repair techniques included commissural reconstruction with a pericardial patch (8), pericardial patch cusp augmentation (6), primary suture repair (6), raphae release and debridement (4), commissurotomy (4), commissural resuspension with sutures (3), and cusp release (1). There were no deaths. At a mean follow-up of 30.1 months (range, 9 months-8 years), all patients were asymptomatic, and the grade of aortic regurgitation had been significantly reduced (P <.001). Left ventricular end-diastolic dimension z scores and proximal regurgitant jet/aortic anulus diameter ratios were significantly reduced (P <.001) and remained so over time. Freedom from reoperation for late failure was 100%, and overall freedom from reintervention was 80% at 3 years. CONCLUSION: Aortic valve repair for balloon-induced aortic regurgitation is reproducible and durable at medium-term follow-up.

Heart size on chest x-ray as a predictor of cardiac enlargement by echocardiography in children.

To determine the usefulness of heart size on chest radiograph (CXR) in predicting cardiac enlargement (CE) in children, we prospectively evaluated 95 consecutive outpatients, who had both a CXR and echocardiography performed. Their median age was 5.0 years (2 days to 19.9 years). All patients underwent CXR assessment by a pediatric radiologist, with classification of cardiac silhouette as normal, borderline, or enlarged. Echocardiographic assessment of CE was performed by a pediatric echocardiographer. Sensitivity, specificity, and predictive values of the pediatric radiologist's interpretation of heart size on CXR were estimated. The presence of CE by echocardiography was used as the gold standard. Seventy-nine patients (83.2%) had no CE on CXR, and 16 patients (16.8%) had CE. Sensitivity of the CXR to identify CE was 58.8%, 95% confidence interval (CI) [32.9, 81.6], with a positive predictive value of 62.5% [35.4, 84.8]. Specificity was 92.3% [84.0, 97.1], with a negative predictive value of 91.1% [82.6, 96.4]. These data suggest that the assessment of CE on CXR to predict CE by echocardiography has a relatively high specificity and negative predictive value, but a low sensitivity and positive predictive value. The limitations of CXR as a diagnostic test should be understood by clinicians using the test when screening children for cardiac disease.

Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum.

Coronary artery pathology is a major determinant of treatment strategy and outcome in patients with pulmonary atresia and intact ventricular septum (PA/IVS). For this reason, infants with PA/IVS routinely undergo preoperative cardiac catheterization. The goal of this study was to identify echocardiographic predictors of coronary artery pathology in infants with PA/IVS. The initial preoperative echocardiograms of 30 consecutive infants with PA/IVS (median age at diagnosis 1 day) were reviewed for indexes predicting the degree of coronary pathology. The tricuspid valve (TV) annulus diameter Z- score was determined and evidence of abnormal flow in the coronary arteries by Doppler was evaluated. Coronary pathology was defined by angiography and graded as: 0 = no fistulae; 1 = fistulae/no right ventricular (RV)-dependent coronary arteries; 2 = fistulae with 1 RV-dependent coronary; 3 = fistulae with >/=2- vessel RV-dependent coronary arteries. Outcome was classified as: 2 ventricles, "1.5" ventricles, and 1 ventricle. By angiography, 30% of the patients had grade 0 coronary pathology, 30% had grade 1, 20% had grade 2, and 20% had grade 3. There was 1 death in a patient with grade 3 coronary pathology. Among the survivors (median age at follow-up 28. 6 months), biventricular circulation existed in 12 patients (41%), 7 patients (24%) were 1.5, and 10 (34%) were 1 ventricle. All patients with TV Z-score -2.5. The sensitivity, specificity, positive, and negative predictive values of TV Z-score

Management of hypercholesterolemia in childhood and adolescence.

There is strong evidence that the onset of atherosclerosis occurs in childhood. Identifying and treating children and adolescents at risk for hypercholesterolemia should lead to a decrease in adult atherosclerotic disease. Based on current information, and the National Cholesterol Education Program (NCEP) guidelines, screening in children and adolescents should be limited to those individuals with specific cardiac risk factors or those from families with a strong history of atherosclerotic disease. Treatment of identified patients should be initiated with dietary control. Subsequent use of cholesterol-lowering medication is best limited to those patients who fail at least 6 months of dietary control measures. Drug therapy includes the use of bile acid sequestrants, nicotinic acid and, more recently, 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitors. There has been limited experience with HMG CoA reductase inhibitors in children and adolescents. However, preliminary data suggests that they are both more effective and have less side effects than either bile acid sequestrants or niacin. Long-term cohort studies will be needed to determine whether screening and treating children and adolescents with hypercholesterolemia is truly of long-term benefit and, if so, which treatment strategies will be preferred.

Repeat balloon dilation of congenital valvar aortic stenosis: immediate results and midterm outcome.

While balloon dilation (BD) has become the initial treatment for congenital valvar aortic stenosis (CVAS) at many institutions, repeat BD for recurrent obstruction has been reported only in a few. Between January 1985 and December 1996, 298 patients (70 neonates) underwent BD, 34 of whom underwent a repeat BD without mortality. A greater proportion of neonates had a repeat BD (26% vs. 8%, P < 0.001). At repeat BD (1 day-7.5 years post initial BD), the mean peak-to-peak gradient was reduced from 67+/-24 to 36+/-16 mm Hg (P < 0.0001). Aortic regurgitation (AR) increased immediately in 26%, being moderate or more in 24%. During a mean follow-up of 5.2 years, there was one surgically related death. Of the 33 survivors, 6 had surgery for residual stenosis and/or AR. Among the remaining 27 patients, 96% were asymptomatic, the peak instantaneous aortic valve Doppler gradient was 50+/-15 mm Hg with AR absent in 8%, mild in 62%, and moderate or more in 31%. In conclusion, repeat BD is effective and without mortality. AR was at least moderate in 24% of patients immediately after a second BD. Repeat BD was more common in patients who underwent the initial BD as neonates.