Diagnosis of anomalous origin of the right subclavian artery from the right pulmonary artery in a patient with D-transposition of the great arteries utilizing transthoracic echocardiography.

Diagnosis of anomalous origin of the right subclavian artery (AORSA) from the right pulmonary artery (RPA) is usually made using CT, MRI, or invasive angiography. We report a patient diagnosed using transthoracic echocardiography (TTE). A newborn girl prenatally known to have d-TGA presented with cyanosis sparing the right hemithorax and arm. Oxygen saturations on the right hand were persistently higher than on the right ear and other extremities. Repeat TTE using a modified echocardiographic imaging plane allowed for full visualization of the entire subclavian artery course, revealing AORSA from RPA. We discuss further the approach to echocardiographic diagnosis and surgical implications.

Cardiac involvement in a pediatric patient with COVID-19: Looking beyond the nonspecific global cardiac injury.

We report a case of a 17-year-old healthy male presenting with multisystem hyperinflammatory shock temporally associated with COVID-19. Cardiac involvement was suspected based on evidence of significant cardiac injury (elevated cardiac biomarkers, electrocardiographic and echocardiographic abnormalities). Cardiac magnetic resonance imaging was performed demonstrating global biventricular systolic dysfunction, as well as a small area of T2 hyperintensity and mid-wall late gadolinium enhancement. This case discusses the varied cardiac involvement in pediatric patients with COVID-19 infection and highlights that cardiac injury is not just limited to hyperinflammatory syndrome related global dysfunction but a more focal myocarditis can also be seen.

Inferior Right Ventricular Wall Thickness by Echocardiogram: A Novel Method of Assessing Hypertrophy in Neonates and Infants.

An established echocardiographic (echo) standard for assessing the newborn right ventricle (RV) for hypertrophy has not been thoroughly developed. This is partially due to the RV's complex architecture, which makes quantification of RV mass by echo difficult. Here, we retrospectively evaluate the thickness of the inferior RV wall (iRVWT) by echo in neonates and infants with normal cardiopulmonary physiology. Inferior RVWT was defined at the medial portion of the inferior wall of the RV at the mid-ventricular level, collected from a subxiphoid, short axis view. iRVWT was indexed to body surface area (BSA) to the 0.5 power and normalized to iLVWT to explore the best normalization method. Ninety-eight neonates and 32 infants were included in the final analysis. Mean age for neonates and infants was 2 days and 59 days, respectively. Mean ± SD for neonate and infant end-diastole iRVWT was 2.17 ± 0.35 mm and 1.79 ± 0.28 mm, respectively. There was no residual relationship between the index iRVWT and BSA (r = 0.03, p = NS). In the infant cohort, the iRVWT was significantly lower and iLVWT was significantly higher compared to neonate, consistent with known physiologic changes of RV and LV mass. Thus, iRVWT may serve as a reliable and accurate proxy for RV mass and the parameter warrants further evaluation.

Impaired myocardial deformation and ventricular vascular coupling in obese adolescents with dysglycemia.

BACKGROUND: It is unknown that dysglycemia in obese adolescents has effects on myocardial deformation that are more pronounced when compared to obesity alone. We hypothesized that obesity associated abnormal glucose tolerance (dysglycemia) would have adverse effects on two-dimensional speckle tracking echocardiography derived longitudinal, radial and circumferential strain (LS, RS, CS) compared to age and gender lean controls. We also examined if changes in deformation would be reflected in abnormal ventricular vascular coupling indices (VVI). METHODS: In a prospective cross-sectional design 39 obese adolescents (15.9 ± 1.7 years; 101.5 ± 39 kg; female - 58%) were compared to age and gender matched lean controls (15.7 ± 1.8 yrs, 60 ± 12.8 kg). Based on results from an oral glucose tolerance test (OGTT), obese adolescents were categorized as obese normoglycemic (ONG, n = 25) or obese dysglycemic (ODG, n = 14). Left ventricular (LV) global and average LS, CS, RS and strain rate were measured. LV ejection fraction and mass index were measured and VVI approximated as ratio of arterial elasticity (Ea) and end-systolic elastance (Ees). RESULTS: Adolescents with ODG had significantly (P = 0.005) impaired global LS (- 20.98% ± 2.8%) compared to controls (- 23.01% ± 2.3%). A similar (P = 0.0027) reduction was observed in average LS for adolescents with ODG (18.87% ± 2.5%) compared to controls (20.49% ± 2%). Global CS was also decreased (P = 0.03) in ODG (- 23.95%) compared to ONG (- 25.80). A similar trend was observed in average CS after multivariate regression for BMI and blood pressure. CS correlated with HbA1c in both groups (P = 0.05). VVI had a negative correlation with both LS (r = - 0.4, P = 0.025) and CS rate (r = - 0.36, P = 0.04). CONCLUSIONS: Myocardial strain and strain rate were significantly altered in obese adolescents. Unfavorable subclinical reductions in global and average CS were more pronounced in adolescents with dysglycemia compared to obese adolescents with normoglycemia and controls. These data indicate progressive worsening of subendocardial function across the spectrum of glucose tolerance. Strain rate was predictive of VVI in obese adolescents, suggesting strain rate may be a sensitive marker for cardiac remodeling in abnormal glucose homeostasis states.

Left Ventricular Mass Quantification by Two-Dimensional Echocardiography in a Pediatric Population: Correlation with Cardiac Magnetic Resonance Imaging.

Quantification of left ventricular (LV) mass by echocardiography has not been validated against the gold standard of cardiac magnetic resonance imaging (CMR) in the pediatric population. The purpose of this study was to compare LV mass by two-dimensional and conventional M-mode echocardiography versus CMR in children. Consecutive CMR studies were paired with echocardiograms and retrospectively analyzed in children age ≤ 16 years (3 days old to 16 years old). Studies performed > 3 months between modalities and single ventricle anatomy were excluded. Unindexed LV mass was calculated using M-mode, area-length (AL), and truncated ellipsoid (TE) methods via echocardiography, and compared to cine stack CMR images. There were 46 patients included in the study (both MRI and echocardiography). Good correlations were observed for LV mass measured by CMR and all echocardiographic methods: M-mode (R = 0.965), AL (R = 0.975), and TE (R = 0.975). There was a significant overestimation using TE echocardiography, by a mean of 10.5 g (95% confidence interval 5.7-15.2 g, p < 0.05). There was no significant over- or underestimation of LV mass observed by M-mode or AL echocardiographic measurements, with tight limits of agreement when compared to CMR (95% confidence interval - 5.2 to 4.4 g and - 1.5 to 6.7 g, respectively). Interobserver agreement was good for each of the echocardiographic measurements, but inferior with M-mode (ICC, 0.89) compared to two-dimensional methods (ICC, 0.97). Echocardiographic estimates of LV mass have good correlation with CMR in children. Performance comparison showed AL echocardiographic method provides the most accurate measurement of LV mass with the best reproducibility compared to other methods.

Two-dimensional XD-GRASP provides better image quality than conventional 2D cardiac cine MRI for patients who cannot suspend respiration.

OBJECTIVES: Residual respiratory motion degrades image quality in conventional cardiac cine MRI (CCMRI). We evaluated whether a free-breathing (FB) radial imaging CCMRI sequence with compressed sensing reconstruction [extradimensional (e.g. cardiac and respiratory phases) golden-angle radial sparse parallel, or XD-GRASP] could provide better image quality than a conventional Cartesian breath-held (BH) sequence in an unselected population of patients undergoing clinical CCMRI. MATERIALS AND METHODS: One hundred one patients who underwent BH and FB imaging in a midventricular short-axis plane at a matching location were included. Visual and quantitative image analysis was performed by two blinded experienced readers, using a five-point qualitative scale to score overall image quality and visual signal-to-noise ratio (SNR) grade, with measures of noise and sharpness. End-diastolic and end-systolic left ventricular areas were also measured and compared for both BH and FB images. RESULTS: Image quality was generally better with the BH cines (overall quality grade for BH vs FB images 4 vs 2.9, p < 0.001; noise 0.06 vs 0.08 p < 0.001; SNR grade 4.1 vs 3, p < 0.001), except for sharpness (p = 0.48). There were no significant differences between BH and FB images regarding end-diastolic or end-systolic areas (p = 0.35 and p = 0.12). Eighteen of the 101 patients had poor BH image quality (grade 1 or 2). In this subgroup, the quality of the FB images was better (p = 0.0032), as was the SNR grade (p = 0.003), but there were no significant differences regarding noise and sharpness (p = 0.45 and p = 0.47). CONCLUSION: Although FB XD-GRASP CCMRI was visually inferior to conventional BH CCMRI in general, it provided improved image quality in the subgroup of patients with respiratory-motion-induced artifacts on BH images.

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.

Surgical planning for a complex double-outlet right ventricle using 3D printing.

Rapid prototyping may be beneficial in properly selected cases of complex congenital heart disease, providing detailed anatomical understanding that helps to guide potential surgical and cardiac catheterization interventions. We present a case of double-outlet right ventricle, where the decision to obtain a three-dimensional printed model helped for better understanding of the anatomy, with the additional advantage of surgical simulation in planning the surgical approach and type of surgical repair.

Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions?

Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

Altering management decisions with gained anatomical insight from a 3D printed model of a complex ventricular septal defect.

Rapid prototyping is quickly gaining utility in various complex forms of CHD. In properly selected cases, these printed models provide detailed anatomical understanding that help guide potential surgical and cardiac catheterisation interventions. We present a case of a tunnel-like ventricular septal defect referred for surgical repair, where the decision to obtain a three-dimensional printed model helped in better understanding of the anatomy, leading to delaying, and hopefully avoiding altogether, surgical repair.

Understanding the spectrum of sinus venosus interatrial communications.

BACKGROUND: It is still thought by some that a common wall is to be found in the normal heart between the attachments of the caval and pulmonary veins, with absence of this wall underscoring the presence of sinus venosus defects. Recent findings using episcopic microscopy in developing mice have shown the deficiencies of this notion. Understanding that the superior rim of the oval fossa is a fold, rather than a true septum, which can be distorted in the presence of partially anomalous pulmonary venous drainage, has provided an alternative explanation for the morphogenesis of sinus venosus defects. METHODS: We reviewed our experience with patients suspected of having a sinus venosus defect from August, 2011, through October, 2015, analysing the findings in light of the current hypotheses used to explain the development of the defects, along with correlations made by inspection of autopsy specimens. RESULTS: We evaluated findings from 16 patients, with a mean age of 7.7 years, ranging from 2.7 to 15 years. Of the group, 13 were ultimately diagnosed with a superior sinus venosus defect, two with an inferior defect, and one with isolated anomalous pulmonary venous connection in the absence of an interatrial communication. Initially, two patients were thought to have oval fossa defects, one from each subtype, but were correctly diagnosed following cardiac magnetic resonance interrogation. Anomalous pulmonary venous connections were present in all cases. CONCLUSION: Appreciation of the changes occurring during normal cardiac development helps in understanding the anatomical substrate underscoring the spectrum of sinus venosus defects. The lesions are veno-venous connections due to partially anomalous pulmonary venous connections, producing interatrial communications outside the confines of the interatrial septum.

Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery.

Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left-sided brachiocephalic artery in the setting of a right-sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three-vessel tracheal view.

Prenatal Diagnosis of a Persistent Fifth Aortic Arch, Pulmonary-to-Systemic type: An Unusual Association with Evolving Aortic Coarctation.

Persistent fifth arch (PFA) is a rare anomaly that is often underdiagnosed and missed. Different PFA types that have been reported in the literature are systemic-to-systemic type (most common), systemic-to-pulmonary artery (PA), and PA-to-systemic types. The designations of systemic-to-PA or PA-to-systemic are based on if the PFA is a source of PA or systemic blood flow, respectively, in the setting of critical proximal obstruction (pulmonary atresia or aortic atresia). This case describes an unusual PFA, which connects the distal PA to distal ascending aorta; however, it is not associated with critical proximal obstruction, and while it appeared to be an incidental finding in early gestation, progressive serial distal obstruction of the left fourth arch was seen to develop. This case highlights that prenatal diagnosis of PFA is possible and that once a diagnosis is made, serial fetal echocardiograms should be performed to evaluate for evolving lesions.

Preliminary Assessment of Tricuspid Valve Annular Velocity Parameters by Cardiac Magnetic Resonance Imaging in Adults with a Volume-Overloaded Right Ventricle: Comparison of Unrepaired Atrial Septal Defect and Repaired Tetralogy of Fallot.

The aim is to compare tricuspid valve (TV) atrioventricular junction (AVJ) annular motion parameters in unrepaired atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) by cardiac magnetic resonance (CMR) imaging. We retrospectively reviewed CMR studies performed between November 2007 and November 2013 in patients 16-45 years of age with unrepaired ASD (with or without partial anomalous pulmonary venous return) and with repaired TOF, who had previous infundibulotomy, but have not undergone pulmonary valve replacement. Longitudinal motion of lateral TV in four-chamber view cine image was tracked through the cardiac cycle with custom software. Twenty TOF patients and 12 ASD patients were included, and values were compared with 80 controls. Right ventricular end-diastolic volume index and right ventricular end-systolic volume index were similar in the ASD and TOF groups and were significantly higher in both groups than in controls. Maximum displacement of the TV in systole, velocity at half-maximal displacement during systole, and velocity at half-maximal displacement during early diastole were all significantly lower in the TOF group than the ASD group [1.39 ± 0.47 vs. 2.21 ± 0.46 (cm, p < 0.01), 5.9 ± 2.1 vs. 10.1 ± 2.3 (cm/s, p < 0.01), and 7.7 ± 2.6 vs. 10.9 ± 3.1 (cm/s, p < 0.05)]. TOF patients have diminished early diastolic TV AVJ velocity compared to patients with an unrepaired ASD, despite similar RV volumes. This observation could suggest diastolic dysfunction or cardiac mechanics unique to the postoperative, volume-overloaded right ventricle in patients with repaired TOF.

Evaluation of left ventricular function and volume by two-dimensional echocardiography in a pediatric population: Correlation with cardiac magnetic resonance imaging.

Background: Echocardiographic quantification of left ventricular (LV) volume and ejection fraction (EF) is widely used in the pediatric population. However, there is no consensus on the most accurate method of quantifying ventricular volumes and systolic function. Purpose: The purpose of this study is to compare two commonly used echocardiographic methods for the evaluation of LV volume and quantification of EF, the five-sixth area-length (5/6 AL) and the modified biplane Simpson (BS), to cardiac magnetic resonance (CMR) imaging in children. Methods: CMR studies were paired with echocardiograms and retrospectively analyzed in children 18 years of age and younger. Studies performed more than 3 months between modalities, patients with congenital heart disease, and patients who had changes in medication regimen between corresponding CMR and echocardiograms were excluded. LV volumes and EF were calculated using the 5/6 AL and BS methods and compared to volumes and EF measured on corresponding CMR studies. Subgroup analyses were conducted based on LV function, pathology, and weight. Results: We retrospectively analyzed 53 CMR and corresponding echocardiogram studies (23 studies for myocarditis and 30 studies for cardiomyopathy) in 46 patients. LVEF derived by both echocardiographic methods showed a good correlation to CMR (5/6 AL r = 0.85 and BS r = 0.82). However, both echocardiographic methods overestimated LVEF and underestimated LV volumes when compared to CMR. Conclusion: Left ventricular volumes and EF, as measured by echocardiography, correlate well with CMR measurements. Echocardiography underestimates LV systolic and diastolic volumes and overestimates LVEF. While echocardiography is a good surrogate for estimating LVEF, CMR should be considered in patients for whom accurate measurements are needed for critical clinical decision-making.

Cardiovascular magnetic resonance as an alternate method for serial evaluation of proximal aorta: comparison with echocardiography.

Thoracic aortic disease is a known cause of aortic dilatation and poses significant risk of aortic dissection and rupture. Serial assessment of aortic root dimensions is traditionally performed using echocardiography, which is limited with older age and following surgery, due to poor acoustic windows. Although diastolic measurements are utilized as standard practice in decision making of adult aortopathy, systolic diameters are utilized in pediatric practice. Three-dimensional steady-state free precision (3D-SSFP) has shown promise as an alternate method for providing accurate and reproducible aortic measurements. The agreement between proximal aorta measurements by diastolic 3D-SSFP and echocardiography (both systole and diastole) was examined in 40 subjects. The maximum inner diameters at aortic annulus, root and sinotubular junction demonstrated excellent agreement between 3D-SSFP and echocardiography for all the 3 levels. The best agreement was observed for diastolic root dimensions with a mean difference of +0.01 cm, limits of agreement being -0.26 to +0.28 cm. Three D-SSFP can be used interchangeably with echocardiography in the serial assessment of the aortic root size. Careful attention to obtain an imaging plane utilizing 3D multiplanar reformatting is critical to maximize the agreement between the two imaging modalities.

Correlation of MRI premature ventricular contraction activation pattern in bigeminy with electrophysiology study-confirmed site of origin.

Although PVCs commonly lead to degraded cine cardiac MRI (CMR), patients with PVCs may have relatively sharp cine images of both normal and ectopic beats ("double beats") when the rhythm during CMR is ventricular bigeminy, and only one beat of the pair is detected for gating. MRI methods for directly imaging premature ventricular contractions (PVCs) are not yet widely available. Localization of PVC site of origin with images may be helpful in planning ablations. The contraction pattern of the PVCs in bigeminy provides a "natural experiment" for investigating the potential utility of PVC imaging for localization. The purpose of this study was to evaluate the correlation of the visually assessed site of the initial contraction of the ectopic beats with the site of origin found by electroanatomic mapping. Images from 7 of 86 consecutive patients who underwent CMR prior to PVC ablation were found to include clear cine images of bigeminy. The visually apparent site of origin of the ectopic contraction was determined by three experienced, blinded CMR readers and correlated with each other, and with PVC site of origin determined by 3D electroanatomic mapping during catheter ablation. Blinded ascertainment of visually apparent initial contraction pattern for PVC localization was within 2 wall segments of PVC origin by 3D electroanatomic mapping 76% of the time. Our data from patients with PVCs with clear images of the ectopic beats when in bigeminy provide proof-of-concept that CMR ectopic beat contraction patterns analysis may provide a novel method for localizing PVC origin prior to ablation procedures. Direct imaging of PVCs with use of newer cardiac imaging methods, even without the presence of bigeminy, may thus provide valuable data for procedural planning.

Preservation of Antegrade Pulmonary Blood Flow in Kawashima Procedure With Prior Right Ventricular Outflow Tract Stent.

Surgical management of single ventricle with interrupted inferior vena cava and azygos continuation typically requires a Kawashima procedure with subsequent completion of Fontan. However, this group is at risk of development of pulmonary arteriovenous malformations. Evidence suggests preservation of hepatic venous flow into the pulmonary circulation can potentially delay this development. We hereby describe a method of preserving antegrade pulmonary blood flow during the Kawashima procedure in the setting of prior right ventricular outflow tract stents.