Hypoplastic Circumflex Aorta With Anomalous Left Pulmonary Artery: Uncrossing Operation in a Neonate.

Circumflex aortic arch with coarctation and anomalous origin of the left pulmonary artery from the aorta are rare cardiovascular anomalies. These conditions can lead to early pulmonary hypertension and challenging management. Early diagnosis and surgical intervention are beneficial for optimal outcome. We present a case where both anomalies coexisted and were repaired with aortic uncrossing, arch augmentation, and reimplantation of the left pulmonary artery. To our knowledge, this is the first documented instance of these anomalies coexisting and being repaired in the neonatal period.

Comparison and Agreement between Cardiovascular Computed Tomography-Derived Mid-Diastolic and End-Diastolic Ventricular Volume in Patients with Congenital Heart Disease.

Prospective electrocardiogram (ECG)-triggered cardiovascular computed tomography (CCT) is primarily utilized for anatomical information in congenital heart disease (CHD) and has not been utilized for calculation of the end-diastolic volume (EDV); however, the mid-diastolic volume (MDV) may be measured. The objective of this study was to evaluate the feasibility and agreement between ventricular EDV and MDV. 31 retrospectively ECG-gated CCT were analyzed for the study of the 450 consecutive CCT. CCT images were processed using syngo.via with automatic contouring followed by manual adjustment of the endocardial borders of the left ventricles (LV) and right ventricles (RV) at end-diastolic and mid-diastolic phase (measured at 70% of cardiac cycle). The correlation and agreements between EDV and MDV were demonstrated using Spearman rank coefficient and intraclass correlation coefficient (ICC), respectively. Mean age ± SD was 28.8 ± 12.5 years, 19 were male (61.3%) and tetralogy of Fallot (TOF) was the most common diagnosis (58.1%), 35% (11/31) patients with a pacemaker, ICD or other such contraindication for a CMRI, 23% (7/31) with claustrophobia, and 6.5% (2/31) with developmental delay with refusal for sedation did not have a previous CMRI. The mean ± SD indexed LV EDV and LV MDV were 91.1 ± 24.5 and 84.8 ± 22.3 ml/m2, respectively. The mean ± SD indexed RV EDV and RV MDV were 136.8 ± 41 and 130.2 ± 41.5 ml/m2, respectively. EDV and MDV had a strong positive correlation and good agreement (ICC 0.92 for LV and 0.95 for RV). This agreement was preserved in a subset of patients (21) with dilated RV (indexed RV EDV z-score > 2). Intra-observer reliability (0.97 and 0.98 for LV and RV MDV, respectively) and inter-observer reliability (0.96 and 0.90 for LV and RV MDV, respectively) were excellent. In a select group of patients with CHD, measuring MDV by CCT is feasible and these values have good agreements with EDV. This may be used to derive functional data from prospectively ECG-triggered CCT studies. Further large-scale analysis is needed to determine accuracy and clinical correlation.

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner.

Truncus Arteriosus and Absent Ascending Aorta With Unusual Head and Neck Vessel Origins.

We describe a neonate with a unique variant of truncus arteriosus with interrupted aortic arch, an absent ascending aorta, persistent right dorsal aorta, and an unusual brachiocephalic artery pattern in which all head and neck vessels were supplied from the ductal arch-descending aorta continuum. (Level of Difficulty: Beginner.).

Correlation of transthoracic echocardiography-derived pulmonary to systemic flow ratio with hemodynamically estimated left to right shunt in atrial septal defects.

Background: Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods: Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed (n = 109), those with technically limited images for Qp/Qs calculation (n = 11) and those with time interval between TTE and cath >60 days were excluded (n = 14). Qp/Qs was calculated from stored clips by previously described methods and correlated with those obtained by oximetry. Patients were subdivided into two age groups <21 (Group 1) and ≥22 years (Group 2). TTE and cath methods for Qp/Qs estimation were compared using paired t-test, Pearson's correlation coefficient, and Bland-Altman plots. Results: Eighty-four subjects met inclusion criteria (age range 3-78 years). Group 1 n = 35; median age 10 years; Group 2 n = 49; median age 49 years. Transthoracic echocardiogram was performed 19.5 ± 15 days prior to cath. Mean Qp/Qs derived by cath and TTE were 2.09 ± 0.9 versus 2.54 ± 1.2 (P < 0.0001). Overall correlation was poor between the methods (r 2 = 0.32, P < 0.0001) and continued to be poor for Groups 1 and 2 (r 2= 0.24, P = 0.003 and r 2= 0.40, P < 0.0001 respectively). Bland-Altman plots demonstrated poor agreement between the predetermined limits of agreement (-0.5-1.5). Conclusion: Transthoracic echocardiography estimated Qp/Qs, although routinely utilized in clinical practice, has poor correlation and agreement with oximetry-derived Qp/Qs. The test performs poorly in all age groups in detecting a hemodynamically significant shunt and tends to overestimate the degree of left to right shunt.

Diagnostic Accuracy of Fetal Echocardiography in Congenital Heart Disease.

BACKGROUND: The accuracy of fetal echocardiography (FE) is not well defined, and reporting of diagnostic discrepancies (DDs) is not standardized. The authors applied a categorization scheme developed by the American College of Cardiology Quality Metric Working Group and applied it to FE. METHODS: A retrospective single-center study was conducted of prenatally diagnosed major structural congenital heart disease, defined as expected need for intervention within the first year of life. DDs between pre- and postnatal findings were identified and categorized. Minor DDs had no clinical impact, moderate DDs had impact without harm, and severe DDs resulted in adverse events. Multivariate regression analysis was used to determine factors associated with discrepancy. RESULTS: From December 2008 to September 2017, 17,096 fetal echocardiograms were obtained, among which 222 fetuses with a median gestational age at first FE of 24 weeks were included. There were 30 DDs (13.5%), of which the majority were false negatives (56.7%). Most were minor or moderate in severity, with one severe DD. The majority were possibly preventable (90%), with the most common contributing factor being technical limitations (43.3%). The most common anatomic segment involved was the ventricular septum (23%), primarily missed septal defects. Comparing cases with DDs versus those without, those with DDs were more likely to have high anatomic complexity (16.7% vs 3.6%, P = .01), maternal comorbidities (40.0% vs 22.1%, P = .03), and a younger maternal age (median, 27 vs 30 years, P = .02). They were also more likely to have later gestation at initial FE (median, 29.5 vs 24 weeks, P = .003), to have fewer total fetal echocardiograms (median, 2 vs 3, P = .002), and to have a fellow as the initial sonographer (36.7% vs 16.7%, P = .03). There were no significant differences in maternal race/ethnicity, fetal comorbidities, and interpreting physician experience between cases with DDs and those without. On multivariate analysis, variables associated with DD included high anatomic complexity, maternal comorbidities, and fellow as initial imager. A greater number of fetal echocardiograms was associated with reduced DD. CONCLUSIONS: FE had a DD rate of 13.5%, mostly minor and moderate in severity. Factors associated with DD included high anatomic complexity, maternal comorbidities, fellow as the initial sonographer, and fewer fetal echocardiograms. Strategies to reduce DD could include a regular secondary review and repeat FE, particularly when anatomic complexity is high.

Abnormalities in Left Ventricular Rotation Are Inherent in Young Children with Repaired Tetralogy of Fallot and Are Independent of Right Ventricular Dilation.

Left ventricular (LV) dysfunction is a risk factor for adverse outcomes in older children and adults with repaired Tetralogy of Fallot (rToF). Pulmonary regurgitation (PR), right ventricular (RV) dilation, and dysfunction have been shown to result in abnormal LV myocardial mechanics and dysfunction. The aim of our study was to evaluate LV rotational mechanics, especially apical rotation in young children with rToF with and without RV dilation. This is a retrospective, single center study in 28 asymptomatic young children with rToF (16 with RV dilation; 12 without RV dilation); 29 age-matched normal controls. RV and LV systolic and diastolic function was studied using conventional two-dimensional echocardiography (2DE) and speckle tracking echocardiography (STE). Rotational mechanics studied included basal and apical rotation (BR, AR), peak twist (calculated by difference between the apical and basal rotation), twist rate (TR), and untwist rate (UnTR). The mean age of the cohort was 4.7 years (± 2.3). Abnormal AR, BR, TR, and UnTR were noted in patients with rToF. The abnormalities were significant in magnitude as well as the direction of rotation; more pronounced in the absence of RV dilation. LV systolic and diastolic dysfunction as evidenced by abnormal AR and degree of untwist is inherent in rToF and not associated with RV dilation in rToF children. Abnormal BR may reflect a lack of maturation to adult type of rotational mechanics. Further longitudinal studies are required to study the progression of these abnormalities and their correlation with clinical outcomes.

Validation of Right Atrial Area as a Measure of Right Atrial Size and Normal Values of in Healthy Pediatric Population by Two-Dimensional Echocardiography.

Right atrial (RA) size is a prognostic indicator for heart failure and cardiovascular death in adults. Data regarding use of RA area (RAA) by two-dimensional echocardiography as a surrogate for RA size and allometric modeling to define appropriate indexing of the RAA are lacking. Our objective was to validate RAA as a reliable measure of RA size and to define normal reference values by transthoracic echocardiography (TTE) in a large population of healthy children and develop Z-scores using a validated allometric model for indexing RAA independent of age, sex, and body size. Agreement between RAA and volume by 2D, 3D TTE, and MRI was assessed. RAA not volume by 2D TTE is an excellent surrogate for RA size. RAA/BSA1 has an inverse correlation with BSA with a residual relationship to BSA (r = - 0.54, p < 0.0001). The allometric exponent (AE) derived for the entire cohort (0.85) also fails to eliminate the residual relationship. The entire cohort divided into two groups with a BSA cut-off of 1 m2 to provide the best-fit allometric model (r = 0). The AE by least square regression analysis for each group is 0.95 and 0.88 for BSA < 1 m2 and > 1 m2, respectively, and was validated against an independent sample. The mean indexed RAA ± SD for BSA ≤ 1 m2 and > 1 m2 is 9.7 ± 1.3 cm2 and 8.7 ± 1.3 cm2, respectively, and was used to derive Z-scores. RAA by 2D TTE is superior to 2D or 3D echocardiography-derived RA volume as a measure of RA size using CMR as the reference standard. RAA when indexed to BSA1, decreases as body size increases. The best-fit allometric modeling is used to create Z scores. RAA/BSA0.95 for BSA < 1 m2 and RAA/BSA0.88 for those with BSA > 1 m2 can be used to derive Z scores.