Echo-Doppler and strain assessment of filling pressures in adults with congenitally corrected transposition and systemic right ventricles.

AIMS: Systolic dysfunction of the systemic right ventricle (sRV) is common in adults with transposition of the great arteries and sRV. In acquired disease, diastology analysis for assessment of filling pressures (FP) is paramount in patient care. METHODS AND RESULTS: Retrospective analysis of 47 adults with sRV without prior systemic tricuspid valve surgery undergoing catheterization and echocardiography within 7 days (median -2 [-1, -3]) from January 2000 to February 2021 at our institution. Median age was 48 (31, 55) years, and 16 (34.0%) patients were female. FPs were normal in 21 patients (44.7%). Left atrial size was enlarged in most patients (83.0%) with mean indexed value 58.3 ± 23.4 mL/m2. Tissue Doppler e' was not significantly different between those with high FPs vs. normal (medial 0.07 ± 0.03 vs. 0.08 ± 0.03 m/s, P = 0.63; lateral 0.08 ± 0.04 vs. 0.08 ± 0.04 m/s, P = 0.88). E velocity and subpulmonic mitral regurgitant velocity were higher in those with high FPs (0.9 ± 0.3 vs. 0.6 ± 0.2 m/s, P = 0.005; 3.8 ± 1.1 vs. 2.8 ± 0.9 m/s, P = 0.004). Left atrial reservoir strain, sRV global longitudinal strain, and subpulmonic left ventricular strain were worse in those with high FP (18.0 ± 7.6 vs. 27.9 ± 10.2%, P = 0.0009; -13.0 ± 4.4 vs. -17.9 ± 5.0%, P = 0.002; -16.8 ± 5.7 vs. -23.0 ± 3.8%, P = 0.001). CONCLUSION: Despite the complex anatomy, FPs can be assessed non-invasively in adults with sRV without prior systemic tricuspid valve surgery. The current guideline algorithm for diastolic dysfunction in acquired heart disease has limited applicability in this population. Given the limitations of Doppler in this heterogeneous population, strain analysis can be a helpful adjunct for estimation of FPs.

Longitudinal functional assessment of atrial M-mode in dextro- transposition of the great arteries fetus may help to predict the necessity for urgent neonatal Rashkind procedure.

Six fetal echocardiographic examinations were analyzed and presented with special emphasis on atrial M-mode in one case of fetal isolated dextro- transposition of the great arteries. The only significant changes were seen based on analysis of the new index of foramen ovale (FO) flap movement. Emergency Rashkind procedure on the first day of postnatal life was predicted based on fetal atrial M-mode.

Magnetic Resonance Imaging Assessment of Right Ventricular Deformation in Patients With Transposition of the Great Arteries.

PURPOSE: To detect changes of right ventricular (RV) myocardial deformation in patients with systemic RV (SRV) and transposition of the great arteries (TGA) as compared with individuals without structural heart disease. MATERIALS AND METHODS: We performed a retrospective analysis of cine steady-state free precession cardiac magnetic resonance imaging sequences acquired using a 1.5 T scanner in short-axis and long-axis views in 25 patients with SRV (16 with atrial switch repair for D-TGA and 9 with congenitally corrected TGA; age range 19 to 68, 13 males). The control group consisted of 25 individuals without structural heart disease (age range 10 to 73, 14 males). Besides routine RV magnetic resonance imaging volumetry, mean longitudinal RV strain was measured on a 4-chamber view, and mean circumferential RV strain on 3 short-axis images (basal, midventricular, and apical) based on feature tracking. The strain parameters were statistically compared between patients with SRV and the control group. RESULTS: Patients with SRV, compared with the control group, had significantly higher RV-indexed end-diastolic volume (122±40 vs. 70±9 mL/m 2 , P <0.001), lower RV ejection fraction (45±12% vs. 62±6%, P <0.001), and reduced mean longitudinal RV strain (-13.7±3.6% vs. -21.6±2.7%, P <0.001). There was no relevant difference between mean circumferential SRV strain in the basal and midventricular plane; however, in patients with SRV, mean circumferential strain was reduced at the apical level (-12.0±6.1% vs. -17.9±5.6%, P <0.001). CONCLUSIONS: SRV failure could be explained by reduced longitudinal SRV strain caused by the longitudinal orientation of RV myocardial fibers. In patients with SRV, circumferential RV strain is only reduced in apical segments.

Functional assessment of atrial M-mode in 3rd trimester in the context of postnatal balloon atrial septostomy in fetuses with simple dextro-transposition of the great arteries.

OBJECTIVE: This study aimed to identify new functional echocardiographic features of fetal foramen ovale (FO) to predict the need of urgent balloon atrial septostomy (BAS) in neonates with simple dextro-transposition of the great arteries (d-TGA). METHOD: Single-center prospective study of fetuses, who had prenatal echocardiographic examination <30 days before delivery and fetal diagnosis of simple d-TGA. The subjects were divided according to the need of BAS within the first 24 h after neonatal birth and before arterial switch operation. We analyzed following parameters: FO diameter, pulmonary venous maximal velocity, new FO flap tracings index analyzed by M-mode echocardiography. RESULTS: Out of 130 fetuses, 12 patients were included. Cutoff value of FO index provided maximum sensitivity (100%) and negative predictive value (100%) and cutoff value of FO index multiplied by FO diameter provided maximum specificity (100%) and positive predictive value (100%) in fetuses with fetal d-TGA in predicting need of urgent BAS. CONCLUSION: FO index could potentially be used to predict postnatal haemodynamic stability in fetuses with simple d-TGA. FO index multiplied by FO diameter could potentially be used to predict prenatally the need of urgent BAS following the birth.

Assessment of ventricular flow dynamics by 4D-flow MRI in patients following surgical repair of d-transposition of the great arteries.

OBJECTIVES: To use 4D-flow MRI to describe systemic and non-systemic ventricular flow organisation and energy loss in patients with repaired d-transposition of the great arteries (d-TGA) and normal subjects. METHODS: Pathline tracking of ventricular volumes was performed using 4D-flow MRI data from a 1.5-T GE Discovery MR450 scanner. D-TGA patients following arterial switch (n = 17, mean age 14 ± 5 years) and atrial switch (n = 15, 35 ± 6 years) procedures were examined and compared with subjects with normal cardiac anatomy and ventricular function (n = 12, 12 ± 3 years). Pathlines were classified by their passage through the ventricles as direct flow, retained inflow, delayed ejection flow, and residual volume and visually and quantitatively assessed. Additionally, viscous energy losses (ELv) were calculated. RESULTS: In normal subjects, the ventricular flow paths were well ordered following similar trajectories through the ventricles with very little mixing of flow components. The flow paths in all atrial and some arterial switch patients were more irregular with high mixing. Direct flow and delayed ejection flow were decreased in atrial switch patients' systemic ventricles with a corresponding increase in residual volume compared with normal subjects (p = 0.003 and p < 0.001 respectively) and arterial switch patients (p < 0.0001 and p < 0.001 respectively). In non-systemic ventricles, arterial switch patients had increased direct flow and decreased delayed ejection fractions compared to normal (p = 0.007 and p < 0.001 respectively) and atrial switch patients (p = 0.01 and p < 0.001 respectively). Regions of high levels of mixing of ventricular flow components showed elevated ELv. CONCLUSIONS: 4D-flow MRI pathline tracking reveals disordered ventricular flow patterns and associated ELv in d-TGA patients. KEY POINTS: • 4D-flow MRI can be used to assess intraventricular flow dynamics in d-TGA patients. • d-TGA arterial switch patients mostly show intraventricular flow dynamics representative of normal subjects, while atrial switch patients show increased flow disorder and different proportions of intraventricular flow volumes. • Flow disruption and disorder increase viscous energy losses.

The assessment of patients with situs inversus and corrected transposition of the great arteries.

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect. There are different subgroups according to the location of the heart in the thorax, apical position and situs. OBJECTIVES: The purpose of this study was to assess pediatric patients with situs inversus (SI) ccTGA (SI-ccTGA), a rare subgroup of this condition, in detail. METHODS: The records of patients with SI-ccTGA followed between January 1, 2010 and January 1, 2019 in our clinic were analyzed retrospectively. Demographic features, associated cardiac defects, arrhythmias and follow-up data were recorded. RESULTS: Twenty-one out of 120 ccTGA patients had SI. The median age was 30 months (4 days-18 years). There were hemodynamically significant associated lesions in 85.7% (n=18) of the patients. A large ventricular septal defect was found in 16 patients (76.2%), severe pulmonary stenosis in 11 (52.4%), pulmonary atresia in six (28.5%), and severe tricuspid regurgitation in two (9.5%). Eleven out of 21 patients had biventricular physiology and eight had single-ventricle physiology. Bidirectional cavopulmonary anastomosis followed by a hemi-Mustard-Rastelli operation were planned for the remaining two patients. Twelve out of 18 patients with associated defects (66.6%) were operated and surgery was planned for three more patients (16.6%). The remaining three patients were scheduled for clinical follow-up. Arrhythmias developed in two (9.5%) patients on follow-up; ablation was performed in one of them and pacemaker implantation followed by cardiac resynchronization therapy was performed in the other. Two patients died during follow-up, one after a central shunt operation and the other preoperatively due to pneumonia and sepsis. CONCLUSION: SI-ccTGA is not a mirror image of situs solitus ccTGA (SS-ccTGA) due to important anatomic and physiologic differences between them. SI-ccTGA patients have a lower risk of tricuspid valve regurgitation than SS-ccTGA patients. The timing of clinical presentation of these patients mainly depends on the type and severity of the associated lesions, as in all subtypes of ccTGA.

Assessment and management of heart failure in the systemic right ventricle.

Transposition of the great arteries (TGA) is a common congenital cardiac malformation, in which many patients survive into adulthood. From the 1960s through much of the 1980s, the majority of those with D-TGA were repaired with an atrial switch procedure, in which the right ventricle (RV) remains the systemic ventricle. Congenitally corrected TGA patients often remain with a systemic RV. In these patients, heart failure risks result from residual sequelae of childhood repair, which often include tricuspid regurgitation, RV failure, and atrial and ventricular arrhythmias. Imaging techniques, including echocardiography, cardiac magnetic resonance imaging, multi-detector computed tomography, and radionuclide ventriculography focus on evaluation of anatomy and function as both diagnostic and prognostic tools. Biomarkers are used for risk stratification. Structural and surgical interventions are targeted for palliation and prevention of further decompensation in conjunction with pharmacologic, ablative, and device-based therapies for acute and chronic management of heart failure.

Development and validation of a procedure-specific assessment tool for hands-on surgical training in congenital heart surgery.

BACKGROUND: Hands-on surgical simulation has been sought to address training limitations within congenital heart surgery (CHS). However, there is a need for objective assessment methods to measure surgeons' performance to justify its global adoption. This study aimed to validate a procedure-specific assessment tool for the simulation of the arterial switch operation on 3D-printed models and to evaluate the consistency of scoring among evaluators with different levels of experience in CHS. METHODS: Five "expert" and 5 "junior" surgeons performed the arterial switch procedure on 3D-printed models with transposition of the great arteries during 2 hands-on surgical training courses. Their performance was retrospectively assessed by 9 evaluators with varying experience in CHS (staff surgeons, resident surgeons, and non-MD raters). Assessments were done using 2 assessment tools: the Hands-On Surgical Training-Congenital Heart Surgery (HOST-CHS) assessment tool and the global rating scale (GRS). RESULTS: The HOST-CHS tool showed a higher interrater and intrarater reliability compared with the GRS. Total scores for expert surgeons were highly consistent across all evaluators. Non-MD raters' total scores for junior surgeons were slightly higher than those of residents and staff evaluators. All grades of evaluator were able to discriminate between junior and expert surgeons. CONCLUSIONS: This study demonstrates the development and validation of an objective, procedure-specific assessment tool for the arterial switch operation with consistency among evaluators with different experience. There is now a platform for quantifying and accurately evaluating performance, which will be highly beneficial in training and developing the next generation of congenital heart surgeons.

Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn.

To improve the understanding of the pathophysiology of transposition of the great arteries with intact ventricular septum (TGA/IVS) and the cardiac remodeling occurring from fetal to neonatal life, we performed a morphometric and functional echocardiographic assessment in fetuses and newborns. This was a prospective case-control study performed in a tertiary referral center, which included fetuses and newborns with a diagnosis of TGA/IVS between 2011 and 2018. Morphometry and systolic and diastolic function parameters were compared with age and body surface-matched healthy controls. Twenty-one TGA/IVS patients were included during the study period and morphometric and functional echocardiographic data were recorded. TGA/IVS patients showed morphometric and functional changes of increased overall volume and output, predominantly in the aortic component from fetus to newborn, probably due to compensatory mechanisms secondary to brain hypoxia.

Neopulmonary Outflow Tract Obstruction Assessment by 4D Flow MRI in Adults With Transposition of the Great Arteries After Arterial Switch Operation.

BACKGROUND: The main complication in adult patients with transposition of the great arteries (TGA) treated by an arterial switch operation (ASO) is neopulmonary outflow tract stenosis (NPOTS). However, pulmonary flow velocity measurements cannot always be performed with transthoracic echocardiography (TTE) due to complex anatomical features. 4D flow MRI allows detection, quantification, and location of the obstruction site along the NPOTS. PURPOSE AND HYPOTHESIS: To investigate the accuracy of 4D flow for the diagnosis of NPOTS in adults with TGA corrected by ASO. STUDY TYPE: Prospective. POPULATION: Thirty-three adult patients with TGA treated by ASO (19 men, mean age 25.5 years old). FIELD STRENGTH/SEQUENCE: Accelerated 4D flow research sequence at 3T. ASSESSMENT: Maximum NPOTS velocities on TTE and 4D flow MRI done the same day. STATISTICAL TESTS: Pearson correlation coefficient, paired t-test, and Bland-Altman analysis were used to investigate the relationship between TTE and MRI data. RESULTS: In 16 patients (48.5%), evaluation of NPOTS anatomy was not obtained by TTE, while it was always possible by 4D flow. Peak flow velocity (PV) measurements in Doppler and 4D flow were highly correlated (r = 0.78; P < 0.001). PV >350 cm.s-1 was detected in only one patient (3%) by TTE vs. five patients (15%) by 4D flow. Moreover, a high correlation was found between PV and the right ventricle (RV) mass index to body surface area when using 4D flow (r = 0.63; P < 0.001). The location of NPOTS was determined in all patients using 4D flow and concerned the main pulmonary artery in 42%. DATA CONCLUSION: Compared to TTE, 4D flow MRI provides better sensitivity to detect and locate NPOTS in patients with TGA treated by ASO. 4D flow PV measurements in NPOTS were well correlated with TTE PV and RV mass. LEVEL OF EVIDENCE: 1 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2020;51:1699-1705.

Echocardiography vs cardiac magnetic resonance imaging assessment of the systemic right ventricle for patients with d-transposition of the great arteries status post atrial switch.

OBJECTIVE: Patients with Dextro-transposition of the great arteries status post atrial switch (dTGA s/p atrial switch) are "at-risk" for systemic right ventricular (RV) dysfunction. Due to complex RV geometry, echocardiography (Echo) does not allow accurate determination of ejection fraction (EF), but cardiac magnetic resonance imaging (CMR) allows quantitative right ventricular assessment. Measures of ventricular deformation may be precursors to global ventricular dysfunction. The primary aim of this study was to characterize imaging and clinical findings for adult patients with dTGA s/p atrial switch. DESIGN: This was a retrospective cohort study of patients with dTGA s/p atrial switch operation (February 1966 to August 1988) with CMR performed at Children's Hospital of Wisconsin (from September 2005 to May 2015). Eligible patients had clinic visit, Echo, and exercise stress test within 1 year of CMR. RESULTS: This study enrolled twenty-seven patients (16 males, 11 females) with dTGA s/p atrial switch (18 with Mustard operation and 9 with Senning operation; median age 30 years; 74% New York Heart Association class 1 and 26% class 2). Seventy-four percentage had normal RV systolic function (RV EF >45% by CMR). No correlation was observed between Echo strain data and clinical status (EF, exercise endurance, VO2 max, or New York Heart Association class). Cardiac magnetic resonance imaging RV global circumferential strain GCS and RV EF had moderate negative correlation (r = -0.65, P < .001). Global circumferential strain was significantly different for those with RV EF above and below 45%, while global peak longitudinal strain (GLS) was not. Patients had reduced CMR myocardial strain values compared with healthy controls. CONCLUSIONS: Reduced RV CMR GCS (for those with RV EF <45%) suggests that CMR evaluation may enhance early detection of detrimental changes in the systemic RV myocardium.

Association Between Variation in Preoperative Care Before Arterial Switch Operation and Outcomes in Patients With Transposition of the Great Arteries.

BACKGROUND: The arterial switch operation (ASO) is the gold standard operative correction of neonates with transposition of the great arteries and intact ventricular septum, with excellent operative survival. The associations between patient and surgeon characteristics and outcomes are well understood, but the associations between variation in preoperative care and outcomes are less well studied. METHODS: A multicenter retrospective cohort study of infants undergoing neonatal ASO between January 2010 and September 2015 at hospitals contributing data to the Pediatric Health Information Systems database was performed. The association between preoperative care (timing of ASO, preoperative use of balloon atrial septostomy, prostaglandin infusion, mechanical ventilation, and vasoactive agents) and operative outcomes (mortality, length of stay, and cost) was studied with multivariable mixed-effects models. RESULTS: Over the study period, 2159 neonates at 40 hospitals were evaluated. Perioperative mortality was 2.8%. Between hospitals, the use of adjuvant therapies and timing of ASO varied broadly. At the subject level, older age at ASO was associated with higher mortality risk (age >6 days: odds ratio, 1.90; 95% CI, 1.11-3.26; P=0.02), cost, and length of stay. Receipt of a balloon atrial septostomy was associated with lower mortality risk (odds ratio, 0.32; 95% CI, 0.17-0.59; P<0.001), cost, and length of stay. Later hospital median age at ASO was associated with higher odds of mortality (odds ratio, 1.15 per day; 95% CI, 1.02-1.29; P=0.03), longer length of stay ( P<0.004), and higher cost ( P<0.001). Other hospital factors were not independently associated with the outcomes of interest. CONCLUSIONS: There was significant variation in preoperative care between hospitals. Some potentially modifiable aspects of perioperative care (timing of ASO and septostomy) were significantly associated with mortality, length of stay, and cost. Further research on the perioperative care of neonates is necessary to determine whether modifying practice on the basis of the observed associations translates into improved outcomes.

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease-Related Heart Failure.

Background This study assessed trends in heart failure ( HF) hospitalizations and health resource use in patients with adult congenital heart disease ( ACHD ). Methods and Results The Nationwide Inpatient Sample was used to compare ACHD with non- ACHD HF hospitalization and health resource trends. Health resource use was assessed using total hospital charges, hospital length of stay, and procedural burden. A total of 87 175±2676 ACHD -related HF hospitalizations occurred between 1998 and 2011. During this time, ACHD HF hospitalizations increased 91% (4620±438-8809±740, P<0.0001) versus a 21% increase in non- ACHD HF hospitalizations ( P=0.003). ACHD HF hospitalization was associated with longer length of stay ( ACHD HF versus non- ACHD HF, 7.2±0.09 versus 6.8±0.02 days; P<0.0001), greater procedural burden, and higher charges ($81 332±$1650 versus $52 050±$379; P<0.0001). ACHD HF hospitalization charges increased 258% during the study period ($26 533±$1816 in 1998 versus $94 887±$8310 in 2011; P=0.0002), more than double that for non- ACHD HF ( P=0.04). Patients with ACHD HF hospitalized in high-volume ACHD centers versus others were more likely to undergo invasive hemodynamic testing (30.2±0.6% versus 20.7±0.5%; P<0.0001) and to receive cardiac resynchronization/defibrillator devices (4.7±0.3% versus 1.8±0.2%; P<0.0001) and mechanical circulatory support (3.9±0.2% versus 2.4±0.2%; P<0.0001). Conclusions ACHD -related HF hospitalizations have increased dramatically in recent years and are associated with disproportionately higher costs, procedural burden, and health resource use.

Assessment of cardiac function in infants with transposition of the great arteries after surgery: comparison of two methods.

BACKGROUND: Assessment of cardiac function is crucial in pediatric patients undergoing cardiovascular surgery, monitoring cardiac output and changing hemodynamic conditions during surgery accordingly is important to improve post-surgical outcome. We aimed to measure cardiac index (CI) and maximal rate of the increase of left ventricular pressure dp/dt(max) with the pressure recording analytic method (PRAM, MostCare®) and compared it with transthoracic echocardiographic cardiac index estimation in infants with transposition of the great arteries (TGA) undergoing surgical correction. METHODS: We enrolled 74 infants with TGA consecutively into this study. CI and dp/dt(max) were measured with PRAM and echocardiography at 0, 4, 8, 12, 24 and 48 h postoperatively. Blood brain natriuretic peptide (BNP) and blood lactate (Lac) were measured at baseline and after operation. RESULTS: The median age at surgery was 13 days (range 1-25 days) with an average weight of 3.24 kg (range 2.31-4.17 kg). CI estimated by PRAM was 1.11 ± 0.12 L/min/m2 (range 0.69-1.36) and by Doppler echocardiography was 1.13 ± 0.13 L/min/m2 (range 0.76-1.40). dp/dt(max) estimated by PRAM was 1.31 ± 0.03 mmHg/s (range 1.23-1.43) and by Doppler echocardiography was 1.31 ± 0.04 L/min/m2 (range 1.25-1.47). CI (r = 0.817, P < 0.001) and dp/dt(max) (r = 0.794, P < 0.001) measured by two methods were highly correlated with a linear relation. Blood BNP and lactate increased to the highest level at 8-12 h post-operatively. CONCLUSIONS: In the early post-operative period, PRAM provides reliable estimates of cardiac index and dp/dt(max) value compared with echocardiographic measurements. PRAM through mostcare® is a reliable continuous monitoring method for peri-operative management in children with congenital heart disease.

Longitudinal strain by two-dimensional speckle tracking to assess ventricular function in adults with transposition of the great arteries: Can serial assessment be simplified?

INTRODUCTION: Transposition of the great arteries (TGA) is a rare form of congenital heart disease in which most patients reach adulthood. Right ventricular dysfunction is the most severe residual complication in long-term follow-up, both in patients treated by atrial switch and in those with congenitally corrected TGA. New echocardiographic tools such as longitudinal strain by two-dimensional (2D) speckle tracking may improve assessment of ventricular function in these patients. METHODS AND RESULTS: We performed a retrospective analysis of echocardiograms in adult patients with TGA (26 patients with dextro-TGA - 15 treated by atrial switch and six by arterial switch - and five with congenitally corrected TGA) and in a control group of 14 healthy individuals. Right ventricular strain was significantly worse (p<0.001), as was the corresponding annular plane systolic excursion (p=0.010) in atrial switch patients, in comparison to arterial switch patients, while no differences were found in left ventricular parameters. In the overall population, systemic right ventricular parameters were significantly less negative than pulmonary right ventricular parameters, and these were less negative than in controls. Left ventricular parameters were similar across groups, except for pulmonary left ventricular strain, which was worse than in controls (p=0.008) as well as pulmonary right ventricular strain. CONCLUSIONS: Assessment of ventricular function in patients with TGA by 2D speckle tracking longitudinal strain is easy and feasible and may be a useful tool for serial follow-up. Of particular note, we found that there is also some degree of ventricular dysfunction even after re-establishment of normal connections.

Can visual analogue scale be used in radiologic subjective image quality assessment?

BACKGROUND: Assessment of qualitative or subjective image quality in radiology is traditionally performed using a fixed-point scale even though reproducibility has proved challenging. OBJECTIVE: Image quality of 3-T coronary magnetic resonance (MR) angiography was evaluated using three scoring methods, hypothesizing that a continuous scoring scale like visual analogue scale would improve the assessment. MATERIALS AND METHODS: Adolescents corrected for transposition of the great arteries with arterial switch operation, ages 9-15 years (n=12), and healthy, age-matched controls (n=12), were examined with 3-D steady-state free precession magnetic resonance imaging. Image quality of the coronary artery origin was evaluated by using a fixed-point scale (1-4), visual analogue scale of 10 cm and a visual analogue scale with reference points (figurative visual analogue scale). Satisfactory image quality was set to a fixed-point scale 3=visual analogue scale/figurative visual analogue scale 6.6 cm. Statistical analysis was performed using Cohen kappa coefficient and agreement index. RESULTS: The mean interobserver scores for the fixed-point scale, visual analogue scale and figurative visual analogue scale were, respectively, in the left main stem 2.8, 5.7, 7.0; left anterior descending artery 2.8, 4.7, 6.6; circumflex artery 2.5, 4.5, 6.2, and right coronary artery 3.2, 6.3, 7.7. Scoring with a fixed-point scale gave an intraobserver κ of 0.52-0.77 while interobserver κ was lacking. For visual analogue scale and figurative visual analogue scale, intraobserver agreement indices were, respectively, 0.08-0.58 and 0.43-0.71 and interobserver agreement indices were up to 0.5 and 0.65, respectively. CONCLUSION: Qualitative image quality evaluation with coronary 3-D steady-state free precession MR angiography, using a visual analogue scale with reference points, had better reproducibility compared to a fixed-point scale and visual analogue scale. Image quality, being a continuum, may be better determined by this method.

Preoperative Coronary Anatomy Assessment with Echocardiography and Morbidity After Arterial Switch Operation of Transposition of the Great Arteries.

In transposition of the great arteries (TGA), certain coronary patterns have been associated with major adverse events early after the arterial switch operation (ASO). We sought to determine the impact of preoperative echocardiographic (ECHO) diagnosis on the intra- and postoperative morbidity. All patients with TGA born between June 2001 and June 2017 and who underwent ASO were reviewed. Data on presumed coronary anatomy (CA) preoperatively were obtained from the preoperative ECHO report. Intraoperative CA was categorized according to Yacoub classification. Major postoperative morbidity included at least one of the following: delayed sternal closure (DSC), prolonged (> 72 h) mechanical ventilation, reintubation, peritoneal dialysis (PD), ECMO, reoperation, and readmission within 30 days after surgery. 240 patients with median age of 5 days (range 1-614) and mean weight at surgery was 3.6 kg (1.8-8.4) were included. Preoperative ECHO assessment of CA was available in 228 patients. Intraoperatively, 181 patients (75%) were found to have type A, 25 patients had type B or C or intramural (B-C-IM; 10%), and 34 patients had type D or E (D-E; 14%). Patients with types B, C, and intramural coronary (B-C-IM) had increased risk for delayed sternum closure (9/25 vs. 20/181 in type A and 8/34 in type D-E; p = 0.04), peritoneal dialysis (4/25 vs. 8/181 and 1/34; p = 0.04), and ECMO (2/25 vs. 1/131 and 1/34; p = 0.02). Within the B-C-IM group, preoperative ECHO raised suspicion of type A in 13 patients (i.e., incorrect diagnosis, ID; 52%), whereas non-A CA was suspected in 12 patients (i.e., correct diagnosis, CD; 48%). With the exception of reoperation, which was seen only in the ID subgroup (4/12 vs. 0/10 in the CD subgroup; p = 0.04), the intraoperative (cardiopulmonary bypass time and cross-clamp time) and postoperative morbidity indices were comparable in both ID and CD subgroups (p > 0.1). Although there is a significant risk for early postoperative morbidity in TGA patients with single, interarterial, and intramural CA, there seems to be relatively limited influence of preoperative ECHO assessment of coronary anatomy on this morbidity burden.

Out-of-pocket medical expenses in severe CHD.

IntroductionFamilies of children born with CHD face added stress owing to uncertainty about the magnitude of the financial burden for medical costs they will face. This study seeks to assess the family responsibility for healthcare bills during the first 12 months of life for commercially insured children undergoing surgery for severe CHD. METHODS: The MarketScan ® database from Truven was used to identify commercially insured infants in 39 states from 2010 to 2012 with an ICD-9 diagnosis code for transposition of the great arteries, tetralogy of Fallot, or truncus arteriosus, as well as the corresponding procedure code for complete repair. Data extraction identified payment responsibilities of the patients' families in the form of co-payments, deductibles, and co-insurance during the 1st year of life. RESULTS: There were 481 infants identified who met the criteria. Average family responsibility for healthcare bills during the 1st year of life was $2928, with no difference between the three groups. The range of out-of-pocket costs was $50-$18,167. Initial hospitalisation and outpatient care accounted for the majority of these responsibilities. CONCLUSIONS: Families of commercially insured children with severe CHD requiring corrective surgery face an average of ~$3000 in out-of-pocket costs for healthcare bills during the first 12 months of their child's life, although the amount varied considerably. This information provides a framework to alleviate some of the uncertainty surrounding healthcare financial responsibilities, and further examination of the origination of these expenditures may be useful in informing future healthcare policy discussion.

Neonatal Brain Injury and Timing of Neurodevelopmental Assessment in Patients With Congenital Heart Disease.

BACKGROUND: Brain injury (BI) is reported in 60% of newborns with critical congenital heart disease as white matter injury (WMI) or stroke. Neurodevelopmental (ND) impairments are reported in these patients. The relationship between neonatal BI and ND outcome has not been established. OBJECTIVES: This study sought to determine the association between peri-operative BI and ND outcomes in infants with single ventricle physiology (SVP) and d-transposition of the great arteries (d-TGA). METHODS: Term newborns with d-TGA and SVP had pre-operative and post-operative brain magnetic resonance imaging and ND outcomes assessed at 12 and 30 months with the Bayley Scales of Infant Development-II. BI was categorized by the brain injury severity score and WMI was quantified by volumetric analysis. RESULTS: A total of 104 infants had follow-up at 12 months and 70 had follow-up at 30 months. At 12 months, only clinical variables were associated with ND outcome. At 30 months, subjects with moderate-to-severe WMI had significantly lower Psychomotor Development Index (PDI) scores (13 points lower) as compared with those with none or minimal WMI for d-TGA and SVP (p = 0.03 and p = 0.05, respectively) after adjusting for various factors. Quantitative WMI volume was likewise associated. Stroke was not associated with outcome. The Bland-Altman limits of agreement for PDI scores at 12 and 30 months were wide (-40.3 to 31.2) across the range of mean PDI values. CONCLUSIONS: Increasing burden of WMI is associated with worse motor outcomes at 30 months for infants with critical congenital heart disease, whereas no adverse association was seen between small strokes and outcome. These results support the utility of neonatal brain magnetic resonance imaging in this population to aid in predicting later outcomes and the importance of ND follow-up beyond 1 year of age.