Enhanced echocardiographic assessment of intracardiac flow in congenital heart disease.

BACKGROUND: 4D flow magnetic resonance imaging (4D flow MRI) can assess and measure the complex flow patterns of the right ventricle (RV) in congenital heart diseases, but its limited availability makes the broad application of intracardiac flow assessment challenging. Color Doppler imaging velocity reconstruction from conventional echocardiography is an emerging alternative, but its validity against 4D flow MRI needs to be established. OBJECTIVE: To compare intracardiac flow parameters measured by color Doppler velocity reconstruction (DoVeR) against parameters measured from 4D flow MRI. METHODS: We analyzed 20 subjects, including 7 normal RVs and 13 abnormal RVs (10 with repaired Tetralogy of Fallot, and 3 with atrial-level shunts). Intracardiac flow parameters such as relative pressure difference, vortex strength, total kinetic energy, and viscous energy loss were quantified using DoVeR and 4D flow MRI. The agreement between the two methods was determined by comparing the spatial fields and quantifying the cross-correlation and normalized difference between time-series measurements. RESULTS: The hemodynamic parameters obtained from DoVeR and 4D flow MRI showed similar flow characteristics and spatial distributions. The time evolutions of the parameters were also in good agreement between the two methods. The median correlation coefficient between the time-series of any parameter was between 0.87 and 0.92, and the median L2-norm deviation was between 10% to 14%. CONCLUSIONS: Our study shows that DoVeR is a reliable alternative to 4D flow MRI for quantifying intracardiac hemodynamic parameters in the RV.

Non-contrast free-breathing 2D CINE compressed SENSE T1-TFE cardiovascular MRI at 3T in sedated young children for assessment of congenital heart disease.

Cardiac MRI is a crucial tool for assessing congenital heart disease (CHD). However, its application remains challenging in young children when performed at 3T. The aim of this retrospective single center study was to compare a non-contrast free-breathing 2D CINE T1-weighted TFE-sequence with compressed sensing (FB 2D CINE CS T1-TFE) with 3D imaging for diagnostic accuracy of CHD, image quality, and vessel diameter measurements in sedated young children. FB 2D CINE CS T1-TFE was compared with a 3D non-contrast whole-heart sequence (3D WH) and 3D contrast-enhanced MR angiography (3D CE-MRA) at 3T in 37 CHD patients (20♂, 1.5±1.4 years). Two radiologists independently assessed image quality, type of CHD, and diagnostic confidence. Diameters and measures of contrast and sharpness of the aorta and pulmonary vessels were determined. A non-parametric multi-factorial approach was used to estimate diagnostic accuracy for the diagnosis of CHD. Linear mixed models were calculated to compare contrast and vessel sharpness. Krippendorff's alpha was determined to quantify vessel diameter agreement. FB 2D CINE CS T1-TFE was rated superior regarding image quality, diagnostic confidence, and diagnostic sensitivity for both intra- and extracardiac pathologies compared to 3D WH and 3D CE-MRA (all p<0.05). FB 2D CINE CS T1-TFE showed superior contrast and vessel sharpness (p<0.001) resulting in the highest proportion of measurable vessels (740/740; 100%), compared to 3D WH (530/620; 85.5%) and 3D CE-MRA (540/560; 96.4%). Regarding vessel diameter measurements, FB 2D CINE CS T1-TFE revealed the closest inter-reader agreement (Krippendorff's alpha: 0.94-0.96; 3D WH: 0.78-0.94; 3D CE-MRA: 0.76-0.93). FB 2D CINE CS T1-TFE demonstrates robustness at 3T and delivers high-quality diagnostic results to assess CHD in sedated young children. Its ability to function without contrast injection and respiratory compensation enhances ease of use and could encourage widespread adoption in clinical practice.

Individual Assessment of Perioperative Brain Growth Trajectories in Infants With Congenital Heart Disease: Correlation With Clinical and Surgical Risk Factors.

Background Infants with congenital heart disease (CHD) are at risk of neurodevelopmental impairments, which may be associated with impaired brain growth. We characterized how perioperative brain growth in infants with CHD deviates from typical trajectories and assessed the relationship between individualized perioperative brain growth and clinical risk factors. Methods and Results A total of 36 infants with CHD underwent preoperative and postoperative brain magnetic resonance imaging. Regional brain volumes were extracted. Normative volumetric development curves were generated using data from 219 healthy infants. Z-scores, representing the degree of positive or negative deviation from the normative mean for age and sex, were calculated for regional brain volumes from each infant with CHD before and after surgery. The degree of Z-score change was correlated with clinical risk factors. Perioperative growth was impaired across the brain, and it was associated with longer postoperative intensive care stay (false discovery rate P<0.05). Higher preoperative creatinine levels were associated with impaired brainstem, caudate nuclei, and right thalamus growth (all false discovery rate P=0.033). Older postnatal age at surgery was associated with impaired brainstem and right lentiform growth (both false discovery rate P=0.042). Longer cardiopulmonary bypass duration was associated with impaired brainstem and right caudate growth (false discovery rate P<0.027). Conclusions Infants with CHD can have impaired brain growth in the immediate postoperative period, the degree of which associates with postoperative intensive care duration. Brainstem growth appears particularly vulnerable to perioperative clinical course, whereas impaired deep gray matter growth was associated with multiple clinical risk factors, possibly reflecting vulnerability of these regions to short- and long-term hypoxic injury.

Association between the initiation of insurance coverage for fetal echocardiography and mortality from congenital heart disease in infants: An interrupted time series analysis.

BACKGROUND: The effectiveness of fetal echocardiography in reducing the mortality from congenital heart disease (CHD) is largely unknown. OBJECTIVES: This study aimed to evaluate whether the widespread use of fetal echocardiography owing to the initiation of insurance coverage in Japan was associated with a decreasing trend in the annual number of CHD-related deaths. METHODS: Data regarding the number of deaths from CHD in infants aged <12 months were extracted from Japanese demographic statistics (2000-2018). Segmented regression analysis was performed on the interrupted time series data by stratifying the sample into CHD subgroups based on ICD-10 classification and sex. RESULTS: After the initiation of insurance coverage for fetal echocardiography in 2010, a decrease was observed in the trends of annual deaths in patients with congenital malformations of aortic and mitral valves (ratio of trends before and after the initiation of insurance coverage for fetal echocardiography 0.96, 95% confidence interval 0.93, 0.99). In this group, the decrease persisted after adjusting for annual total infant deaths and cardiac surgery mortality and in the analysis of trends in the proportion of deaths in this group per total CHD deaths. However, a decrease in trends was not observed in other patient groups with CHD. In the sex-stratified analysis, a decrease was noted only in male patients with congenital malformations of aortic and mitral valves. CONCLUSIONS: The nationwide trend in annual CHD deaths decreased after the initiation of insurance coverage for fetal echocardiography only among patients with congenital malformation of aortic and mitral valves. These findings suggest that prenatal diagnosis with fetal echocardiography has led to improved mortality outcomes among these patients in Japan.

Free-breathing pseudo-golden-angle bSSFP cine cardiac MRI for biventricular functional assessment in congenital heart disease.

PURPOSE: To compare standard breath-hold (BH) cine imaging to a radial pseudo-golden-angle free-breathing (FB) technique in congenital heart disease (CHD). METHODS: In this prospective study, short-axis and 4-chamber BH and FB cardiac MRI sequences of 25 participants with CHD acquired at 1.5 Tesla, were quantitatively compared regarding ventricular volumes, function, interventricular septum thickness (IVSD), apparent signal to noise ratio (aSNR), and estimated contrast to noise ratio (eCNR). For qualitative comparison, three image quality criteria (contrast, endocardial edge definition, and artefacts) were rated on a 5-point Likert scale (5: excellent, 1: non-diagnostic). Paired t-Test was used for group comparisons, Bland-Altman analysis for agreement between techniques. Inter-reader agreement was compared using intraclass correlation coefficient. RESULTS: IVSD (BH 7.4 ± 2.1 mm vs FB 7.4 ± 1.9 mm, p =.71), biventricular ejection fraction (left ventricle [LV]: 56.4 ± 10.8% vs 56.1 ± 9.3%, p =.83; right ventricle [RV]: 49.5 ± 8.6% vs 49.7 ± 10.1%, p =.83), and biventricular end diastolic volume (LV: 176.3 ± 63.9 ml vs 173.9 ± 64.9 ml, p =.90; RV: 185.4 ± 63.8 ml vs 189.6 ± 66.6 ml, p =.34) were comparable. Mean measurement time for FB short-axis sequences was 8.1 ± 1.3 compared to 4.4 ± 1.3 min for BH (p <.001). Subjective image quality between sequences was deemed comparable, (4.6 ± 0.6 vs 4.5 ± 0.6, p =.26, for 4-chamber views) with a significant difference regarding short-axis views (4.9 ± 0.3 vs 4.5 ± 0.6, p =.008). aSNR was similar (BH 25.8 ± 11.2 vs FB 22.2 ± 9.5, p =.24), while eCNR was higher for BH (89.1 ± 36.1 vs 68.5 ± 32.1, p =.03). CONCLUSION: FB sequences yielded comparable results to BH regarding image quality, biventricular volumetry, and function, though measurement times were longer. The FB sequence described might be clinically valuable when BHs are insufficiently performed.

Discordance between 2D and 4D flow in the assessment of pulmonary regurgitation severity: a right ventricular remodeling follow-up study.

OBJECTIVES: Pulmonary regurgitation (PR) is common in adult congenital heart disease (ACHD). 2D phase contrast MRI is the reference method for the quantification of PR and helps in the decision of pulmonary valve replacement (PVR). 4D flow MRI can be an alternative method to estimate PR but more validation is still needed. Our purpose was to compare 2D and 4D flow in PR quantification using the degree of right ventricular remodeling after PVR as the reference standard. METHODS: In 30 adult patients with a pulmonary valve disease recruited between 2015 and 2018, PR was assessed using both 2D and 4D flow. Based on the clinical standard of care, 22 underwent PVR. The pre PVR estimate of PR was compared using the post-operative decrease in right ventricle end-diastolic volume on follow-up exam as reference. RESULTS: In the overall cohort, regurgitant volume (Rvol) and regurgitant fraction (RF) of PR measured by 2D and 4D flow were well correlated but with moderate agreement in the overall cohort (r = 0.90, mean diff. -14 ± 12.5 mL; and r = 0.72, mean diff. -15 ± 13%; all p < 0.0001). Correlations between Rvol estimates and right ventricle end-diastolic volume decrease after PVR was higher with 4D flow (r = 0.80, p < 0.0001) than with 2D flow (r = 0.72, p < 0.0001). CONCLUSIONS: In ACHD, PR quantification from 4D flow better predicts post-PVR right ventricle remodeling than that from 2D flow. Further studies are needed to evaluate the added value of this 4D flow quantification for guiding replacement decision. KEY POINTS: • Using 4D flow MRI allows a better quantification of pulmonary regurgitation in adult congenital heart disease than 2D flow when taking right ventricle remodeling after pulmonary valve replacement as a reference. • A plane positioned perpendicular to the ejected flow volume as allowed by 4D flow provides better results to estimate pulmonary regurgitation.

Functional assessment of atrial wall excursion and foramen ovale flap tracings in 3rd trimester as predictor of short-term hemodynamic stability in congenital heart defects fetuses.

OBJECTIVES: Right atrium and left atrium of the fetal heart play a fundamental role in fetal heart circulatory physiology. METHODS: Excursion of fetal atria walls and tracings of foramen ovale (FO) flap movement were analyzed by M-mode echocardiography by new FO index to determine fetal and neonatal outcome in the first week and month of life in various congenital heart defects. The study group was divided into 3 subgroups: group A: neonates stable after birth (all on I.V. prostaglandin infusion) for at least 7 days, group B: neonates unstable after birth (despite I.V. prostaglandin infusion) requiring catheterization procedure or early cardiac surgery <6th day of life and group C: fetuses with in utero demise. RESULTS: The average values of FO index: group A - 32, group B - 20, group C - 12.Schaffe test showed statistical difference of FO index between group A and B (p=0.029) and group A and C (p=0.001), but no difference between group B and C (p=0.24) The FO index of stability was determined by ROC curve analysis. Cut-off point distinguishing between postnatal stability and postnatal instability + in utero demise constituted FO index=25 (specificity 82%; sensitivity 90%). CONCLUSIONS: Prenatal features of atrial M-mode echocardiography shortly before birth might be helpful to predict the short term prognosis of fetal hemodynamic stability or instability shortly after birth regardless of the type of congenital heart defects (CHD).

Assessment of fetal heart aortic and pulmonary valve annulus area by three-dimensional ultrasonography: reference curves and applicability in congenital heart diseases.

OBJECTIVES: To determine reference curves for fetal aortic and pulmonary valve annulus area by three-dimensional ultrasonography using the spatio-temporal image correlation (STIC) in the rendering mode, and to ascertain its applicability in congenital heart disease (CHD). METHODS: We performed a retrospective cross-sectional study of 328 normal fetuses and 42 fetuses with CHD between 20 and 33 weeks 6 days of gestation. The outflow plane view of the great vessels was used to measure the areas of the valvar annuli, and the measurements were performed in systole. A linear regression model adjusted according to the determination coefficient (R2) was utilized to construct the reference intervals. The concordance correlation coefficient (CCC) was used to calculate the reproducibility of the mitral and tricuspid valve areas. RESULTS: The mean ± standard deviation (SD) of the aortic and pulmonary valve annulus areas ranged from 6.6 ± 1.2 to 32.9 ± 1.1 mm2 and 10.7 ± 1.3 to 40.3 ± 1.2 mm2, respectively. We observed a linear relationship and strong positive correlation between the area of the aortic and pulmonary valve annuli with r=0.97 and 0.96, respectively. Good intra (CCC=0.99) and interobserver agreement (CCC=0.98) was observed for the measurement of the aortic valve annulus area. A good intra (CCC=0.99) and interobserver (CCC=0.97) agreement was also observed for the measurement of the pulmonary valve annulus area. The mean ± SD of the difference of the areas of the aortic and pulmonary valve annuli between the normal fetuses and those with CHD were -1.801 ± 1.429 mm2 (p=0.208) and -1.033 ± 1.467 mm2 (p<0.0001), respectively. CONCLUSIONS: The reference curves for the areas of the aortic and pulmonary valve annuli of fetal hearts were determined, and showed good inter and intraobserver reproducibility. The constructed reference curves showed applicability in different types of CHD.

Impact of rural residence and low socioeconomic status on rate and timing of prenatal detection of major congenital heart disease in a jurisdiction of universal health coverage.

OBJECTIVE: Socioeconomic status (SES) and distance of residence from tertiary care may impact fetal detection of congenital heart disease (CHD), partly through reduced access to and quality of obstetric ultrasound screening. It is unknown whether SES and remoteness of residence (RoR) affect prenatal detection of CHD in jurisdictions with universal health coverage. We examined the impact of SES and RoR on the rate and timing of prenatal diagnosis of major CHD within the province of Alberta in Canada. METHODS: In this retrospective study, we identified all fetuses and infants diagnosed with major CHD in Alberta, from 2008 to 2018, that underwent cardiac surgical intervention within the first year after birth, died preoperatively, were stillborn or underwent termination. Using maternal residence postal code and geocoding, Chan SES index quintile, geographic distance from a tertiary-care fetal cardiology center and the Canadian Index of Remoteness (IoR) were calculated. Outcome measures included rates of prenatal diagnosis and diagnosis after 22 weeks' gestation. Risk ratios (RR) were calculated using log-binomial regression and stratified by rural (≥ 100 km from tertiary care) or metropolitan (< 100 km from tertiary care) residence, adjusting for year of birth and the obstetric ultrasound screening view in which CHD would most likely be detected (four-chamber view; outflow-tract view; three-vessel or three-vessels-and-trachea or non-standard view; septal view). RESULTS: Of 1405 fetuses/infants with major CHD, prenatal diagnosis occurred in 814 (57.9%). Residence ≥ 100 km from tertiary care (adjusted RR, 1.19; 95% CI, 1.05-1.34) and higher IoR (adjusted RR, 1.9; 95% CI, 1.1-3.3) were associated with missed prenatal diagnosis of major CHD. Similarly, residence ≥ 100 km from tertiary care (adjusted RR, 1.41; 95% CI, 1.22-1.62) and higher IoR (adjusted RR, 3.6; 95% CI, 2.2-8.2) were associated with prenatal diagnosis after 22 weeks. Although adjusted and unadjusted analyses showed no association between Chan SES index quintile and prenatal-diagnosis rate overall nor for residence in rural areas, in metropolitan regions, lower SES quintiles were associated with missed prenatal diagnosis (quintile 1: RR, 1.24; 95% CI, 1.02-1.50) and higher risk of diagnosis after 22 weeks' gestation (quintile 1: RR, 1.46; 95% CI, 1.10-1.93; quintile 2: RR, 1.66; 95% CI, 1.24-2.23). CONCLUSIONS: Despite universal healthcare, rural residence in Alberta is associated with lower rate of prenatal diagnosis of major CHD and higher risk of late prenatal diagnosis (≥ 22 weeks). Within metropolitan regions, lower SES impacts negatively prenatal-diagnosis rate and timing. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Serial Assessment of Cardiac Function and Pulmonary Hemodynamics in Infants With Down Syndrome.

BACKGROUND: There is a dearth of longitudinal data describing the evolution of cardiopulmonary hemodynamics in infants with Down syndrome (DS) beyond infancy. We hypothesized that babies with DS, independent of the presence of congenital heart disease (CHD), demonstrate biventricular systolic and diastolic impairment and sustained elevation of pulmonary pressures compared with controls over the first 2 years of age. METHODS: This was a prospective observational cohort study of 70 infants with DS (48 with CHD and 22 without CHD) and 60 controls carried out in 3 tertiary neonatal intensive care units in Dublin, Ireland. Infants with DS with and without CHD and non-DS controls underwent serial echocardiograms at birth, 6 months, 1 year, and 2 years of age to assess biventricular systolic and diastolic function using deformation analysis. Pulmonary vascular resistance was assessed using pulmonary artery acceleration time and left ventricular (LV) eccentricity index. RESULTS: Infants with DS exhibited smaller LV (birth: 27 ± 4 vs 31 ± 2 mm, P < .01; 2 years: 43 ± 5 vs 48 ± 4 mm, P < .01) and right ventricular (birth: 28 ± 3 vs 31 ± 2 mm, P < .01; 2 years: 40 ± 4 vs 44 ± 3 mm, P < .01) lengths and lower LV (birth: -19% ± 3% vs -22% ± 2%, P < .01; 2 years: -24% ± 2% vs -26% ± 2%, P < .01) and right ventricular (birth: -19% ± 4% vs -22% ± 3%, P < .01; 2 years: -29% ± 6% vs -33% ± 4%, P < .01) systolic strain over the 2-year period. Pulmonary artery acceleration time was lower in the DS group throughout the study period (birth: 44 ± 10 vs 62 ± 14 ms, P < .01; 2 years 71 ± 12 vs 83 ± 11 ms, P < .01). No differences were observed between DS infants with and without CHD (all P > .05). CONCLUSIONS: Infants with DS exhibit impaired maturational changes in myocardial function and pulmonary vascular resistance. Such novel findings provide valuable insights into the pathophysiology affecting cardiorespiratory morbidity in this population.

Comparison of Strain-Encoding and Feature-Tracking Derived Myocardial Deformation Assessment of Left Ventricular Function in a Pediatric and Adult Congenital Heart Disease Cohort.

BACKGROUND: Cardiac magnetic resonance (CMR) strain can be assessed with feature-tracking (FT), which utilizes a post-processing algorithm to quantify myocardial deformation on routine cine images, and strain-encoding magnetic resonance imaging (SENC), which uses parallel magnetization tags combined with out-of-plane phase-encoding gradients to quantify deformation. Assessing agreement is critical to determine whether results can be translated between methods. We compared SENC to FT in the assessment of left ventricle (LV) global longitudinal strain (GLS) and global circumferential strain (GCS) in a cohort of pediatric and adult congenital heart disease (ACHD) patients. METHODS: Pediatric subjects and ACHD patients underwent CMR on 1.5 T Siemens scanners, including balanced steady-state-free precession (bSSFP) cine imaging and SENC acquisitions in apical two and four chamber, left ventricular outflow tract, and short axis views. bSSFP cine imaging FT analysis was completed with Medis QStrain. Myocardial Solutions MyoStrain was used to analyze SENC. Correlation was assessed by Spearman's rank correlation coefficient. Agreement between techniques was assessed with concordance correlation coefficient (CCC) and Bland-Altman. RESULTS: The cohort included 134 patients, 75 with congenital heart disease (56%). The median age was 16.3 years (IQR 13.7, 19.5). Median LV ejection fraction was 57% (IQR 54.4, 61.6). SENC and FT were in poor agreement for GLS (Spearman's ρ = 0.58, p < 0.001; CCC 0.24) and GCS (Spearman's ρ = 0.29, p < 0.001; CCC 0.03). CONCLUSION: There was poor agreement between SENC and FT derived GLS and GCS in a cohort of pediatric and ACHD patients, suggesting that SENC and FT cannot be used interchangeably.

Operator experience impact on the evaluation of still images of a first trimester cardiac assessment protocol.

OBJECTIVE: Congenital heart disease (CHD) is the most common birth defect and represents the leading cause for mortality and morbidity in infants and young adults. Early fetal echocardiography is usually considered a highly specialized scan. The goal of this study is to evaluate the impact of operator's experience in assessing still images of the 4-chamber view and 3-vessels view and to evaluate the feasibility and the performance of a first trimester screening protocol for CHD. METHODS: An online questionnaire consisting of still images of the 4-camber view and 3-vessel view from 50 normal and abnormal cases was reviewed by an expert group made of seven obstetricians specialized in fetal medicine and a nonexpert group made of 13 obstetricians that are certified in ultrasound. After individually visualizing each image set made of the 4-chamber view and 3-vessel view, they had to conclude if the case was normal or abnormal and what images were abnormal. RESULTS: A total of 50 image sets of both normal and abnormal fetal hearts were examined by the 20 reviewers, resulting in 1000 evaluations. The expert group achieved a detection rate of 97.1% with a false positive rate of 5.7%. The nonexpert group achieved also a good detection rate of 91.3% but with a much higher false positive rate of 33.9%. The most frequently missed CHD involved the great arteries and had a normal 4-chamber view. In the majority of false positive cases the 3-vessel view was incorrectly interpreted as abnormal. CONCLUSIONS: A screening protocol for CHD, based on the 4-chamber view and 3-vessel view alone can offer a good detection rate for CHD with a small false positive rate, but only if it is implemented by highly specialized sonographers.

Accuracy of the Standardized Early Fetal Heart Assessment in Excluding Major Congenital Heart Defects in High-Risk Population: A Single-Center Experience.

OBJECTIVE: To evaluate the ability of standardized early fetal heart assessment (EFHA) that includes the 4-chamber view, outflow tract relationship (OTR), and transverse arches views (TAV) of the great arteries in excluding major congenital heart defects (CHDs) in high-risk populations. METHODS: This retrospective study included high-risk pregnancies for CHDs undergoing EFHA at 11-14 weeks of gestation. Risk factors for CHDs were diabetes mellitus, a family history of CHDs, a history of a CHD use of teratogenic medications, seizure disorder, assisted reproductive treatment-conceived pregnancy, presence of at least one of the ultrasound markers: enlarged nuchal translucency (>95th percentile), tricuspid regurgitation, and reversed a-wave in the ductus venosus, presence of an extracardiac or a suspected cardiac anomaly, a high-risk first trimester combined screen result, and multiple pregnancies. EFHA consisted of visualization of 4ChV, OTR, and TAV, with and without power Doppler ultrasound. The primary outcome was the negative predictive value of EFHA in excluding major CHDs. RESULTS: EFHA was performed on 1055 fetuses. Of these, 1024 were used for the final analysis. Of these, 94.9% (n = 923) were singleton. The most common indication for EFHA was maternal DM (n = 231, 22.6%). The sensitivity, specificity, false positive, false negative, and accuracy of EFHA were 93.2%, 99.9%, 1.4%, 0.4%, and 99.5% (P < .0001), respectively, in entire included cases. The performance of EFHA was 88%, 99.9%, 1.4%, 0.4%, and 99.5% (P < .0001) respectively when the terminated cases were excluded. CONCLUSION: Early fetal heart assessment is feasible for screening for major CHDs in high-risk populations. This approach may be expanded to the general obstetric population.

Embryonic echocardiography for assessment of congenital and functional cardiac defects.

Cardiac function and morphology by mouse fetal echocardiography can be assessed by scanning the uterus extracted from the abdominal cavity (trans-uterine ultrasound) or the womb (trans-abdominal ultrasound). Advantages of trans-abdominal ultrasound include (1) non-invasive longitudinal analysis at different stages, reducing animal use; and (2) maintenance of natural environment, diminishing perturbations on functional parameters, which are more frequent in trans-uterine conditions. Here we describe both approaches, explaining how to identify congenital cardiac defects and defining the correlation between echocardiography findings and histological analysis. For complete details on the use and execution of this protocol, please refer to (Menendez-Montes et al., 2016) and (Menendez-Montes et al., 2021).

Multi-institution Assessment of the Accuracy of Cardiac Computed Tomography in Preparation for Superior Cavopulmonary Connection.

BACKGROUND: Patients with single ventricle (SV) congenital heart disease (CHD) undergo several interventions in the first years of life. Advanced diagnostics are required for interstage assessment of anatomy, but are associated with significant diagnostic risk. We sought to evaluate image quality, risk, and accuracy of cardiac computed tomography (CCT) for evaluation of anatomy prior to superior cavopulmonary connection (SCPC) compared to surgical findings across 2 institutions. METHODS: A retrospective evaluation of image quality, risk, and accuracy of pre-SCPC CCT was performed at 2 institutions between January 1, 2010 and September 30, 2016. RESULTS: CCT was performed in 90 SV CHD patients with a median age of 4.03 months (interquartile range [IQR] 3.36, 5.33) prior to SCPC. Image quality was optimal (84%) or good (16%) in all patients, without significant discrepancy compared to surgical findings. 7 patients (8%) required interventional cardiac catheterization subsequent to CCT and before surgical intervention. 49% of scans were performed without sedation, 43% of scans were performed with mild to moderate sedation, and 8% of scans were performed with general anesthesia. The median total procedural dose-length product (DLP) was 18 (IQR 14, 26) mGy*cm, estimating an age adjusted radiation dose of 1.4 millisievert (mSv). One minor (1%) adverse event was reported within 24 h of the CCT. Surgical complications were unrelated to the presurgical findings. CONCLUSIONS: CCT for pre-SCPC evaluation is safe, with excellent accuracy for anatomy at the time of surgical intervention across 2 institutions. In select patients, noninvasive evaluation with CCT may be indicated.

Free-breathing high resolution modified Dixon steady-state angiography with compressed sensing for the assessment of the thoracic vasculature in pediatric patients with congenital heart disease.

BACKGROUND: Cardiovascular magnetic resonance angiography (CMRA) is a non-invasive imaging modality of choice in pediatric patients with congenital heart disease (CHD). This study was aimed to evaluate the diagnostic utility of a respiratory- and electrocardiogram-gated steady-state CMRA with modified Dixon (mDixon) fat suppression technique and compressed sensing in comparison to standard first-pass CMRA in pediatric patients with CHD at 3 T. METHODS: In this retrospective single center study, pediatric CHD patients who underwent CMR with first-pass CMRA followed by mDixon steady-state CMRA at 3 T were analyzed. Image quality using a Likert scale from 5 (excellent) to 1 (non-diagnostic) and quality of fat suppression were assessed in consensus by two readers. Blood-to-tissue contrast and quantitative measurements of the thoracic vasculature were assessed separately by two readers. CMRA images were reevaluated by two readers for additional findings, which could be identified only on either one of the CMRA types. Paired Student t test, Wilcoxon test, and intraclass correlation coefficients (ICCs) were used for statistical analysis. RESULTS: 32 patients with CHD (3.3 ± 1.7 years, 13 female) were included. Overall image quality of steady-state mDixon CMRA was higher compared to first-pass CMRA (4.5 ± 0.5 vs. 3.3 ± 0.5; P < 0.001). Blood-to-tissue contrast ratio of steady-state mDixon CMRA was comparable to first-pass CMRA (7.85 ± 4.75 vs. 6.35 ± 2.23; P = 0.133). Fat suppression of steady-state mDixon CMRA was perfect in 30/32 (94%) cases. Vessel diameters were greater in first-pass CMRA compared to steady-state mDixon CMRA with the greatest differences at the level of pulmonary arteries and veins (e.g., right pulmonary artery for reader 1: 10.4 ± 2.4 vs. 9.9 ± 2.3 mm, P < 0.001). Interobserver agreement was higher for steady-state mDixon CMRA for all measurements compared to first-pass CMRA (ICCs > 0.92). In 9/32 (28%) patients, 10 additional findings were identified on mDixon steady-state CMRA (e.g., partial anomalous venous return, abnormalities of coronary arteries, subclavian artery stenosis), which were not depicted using first-pass CMRA. CONCLUSIONS: Steady-state mDixon CMRA offers a robust fat suppression, a high image quality, and diagnostic utility for the assessment of the thoracic vasculature in pediatric CHD patients.

Usefulness of cardiac fusion imaging with computed tomography and Doppler echocardiography in the assessment of conduit stenosis in complex adult congenital heart disease.

BACKGROUND: Noninvasive assessment of stenotic lesions in patients with complex adult congenital heart disease (ACHD) is challenging due to its complex morphology. The simultaneous two-screen display of multidetector-computed tomography (MDCT) and real-time echogram (STDME) technology can display a virtual multi-planar reconstruction from MDCT corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). We investigated the usefulness of the STDME technology for stenosis severity assessment in complex ACHD patients. METHODS: Twenty-four complex ACHD patients with stenotic lesions were enrolled in this study. All patients underwent TTE and the STDME technology within a week after MDCT. Peak velocity and pressure gradient (PG) across the stenotic site were measured using continuous wave Doppler. Cardiac catheterization was performed in 17 patients. RESULTS: Nine out of the twenty-four patients had undergone repair with a conduit. Peak velocity and PG from the STDME technology were higher than those from TTE (peak velocity: 3.1 ± 1.1 vs. 2.8 ± 1.0 m/s; peak PG: 43 ± 28 vs. 34 ± 21 mmHg; both p < 0.01). Peak PG from the STDME technology showed significant correlations with those from catheterization in patients with a conduit (n=7) and those without a conduit (n=10) (r = 0.795 and 0.880, respectively; both p < 0.05), while peak PG from TTE was correlated with catheterization measurements only in patients without a conduit (r = 0.850, p < 0.05). CONCLUSIONS: The STDME technology enables more accurate assessment of conduit stenosis severity than does TTE in complex ACHD patients.

Free-breathing non-contrast flow-independent cardiovascular magnetic resonance angiography using cardiac gated, magnetization-prepared 3D Dixon method: assessment of thoracic vasculature in congenital heart disease.

BACKGROUND: To evaluate a non-contrast respiratory- and electrocardiogram-gated 3D cardiovascular magnetic resonance angiography (CMRA) based on magnetization-prepared Dixon method (relaxation-enhanced angiography without contrast and triggering, REACT) for the assessment of the thoracic vasculature in congenital heart disease (CHD) patients. METHODS: 70 patients with CHD (mean 28 years, range: 10-65 years) were retrospectively identified in this single-center study. REACT-CMRA was applied with respiratory- and cardiac-gating. Image quality (IQ) of REACT-CMRA was compared to standard non-gated multi-phase first-pass-CMRA and respiratory- and electrocardiogram-gated steady-state-CMRA. IQ of different vessels of interest (ascending aorta, left pulmonary artery, left superior pulmonary vein, right coronary ostium, coronary sinus) was independently assessed by two readers on a five-point Likert scale. Measurements of vessel diameters were performed in predefined anatomic landmarks (ascending aorta, left pulmonary artery, left superior pulmonary vein). Both readers assessed artifacts and vascular abnormalities. Friedman test, chi-squared test, and Bland-Altman method were used for statistical analysis. RESULTS: Overall IQ score of REACT-CMRA was higher compared to first-pass-CMRA (3.5 ± 0.4 vs. 2.7 ± 0.4, P < 0.001) and did not differ from steady-state-CMRA (3.5 ± 0.4 vs. 3.5 ± 0.6, P = 0.99). Non-diagnostic IQ of the defined vessels of interest was observed less frequently on REACT-CMRA (1.7 %) compared to steady-state- (4.3 %, P = 0.046) or first-pass-CMRA (20.9 %, P < 0.001). Close agreements in vessel diameter measurements were observed between REACT-CMRA and steady-state-CMRA (e.g. ascending aorta, bias: 0.38 ± 1.0 mm, 95 % limits of agreement (LOA): - 1.62-2.38 mm). REACT-CMRA showed high intra- (bias: 0.04 ± 1.0 mm, 95 % LOA: - 1.9-2.0 mm) and interobserver (bias: 0.20 ± 1.1 mm, 95 % LOA: - 2.0-2.4 mm) agreements regarding vessel diameter measurements. Fat-water separation artifacts were observed in 11/70 (16 %) patients on REACT-CMRA but did not limit diagnostic utility. Six vascular abnormalities were detected on REACT-CMRA that were not seen on standard contrast-enhanced CMRA. CONCLUSIONS: Non-contrast-enhanced cardiac-gated REACT-CMRA offers a high diagnostic quality for assessment of the thoracic vasculature in CHD patients.