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.

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.

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.

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.

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.

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.

Assessment of transposition of the great arteries associated with multiple malformations using dual-source computed tomography.

PURPOSE: To determine the value of dual-source computed tomography (DSCT) in depicting the morphological characteristics and diagnosing the associated malformations for patients with transposition of the great arteries (TGA) before surgery. MATERIALS AND METHODS: Twenty-five patients with TGA who underwent DSCT and transthoracic echocardiography (TTE) examination were retrospectively reviewed. The morphological types of TGA, the spatial relationship between the pulmonary artery and the aorta, as well as coronary artery-associated abnormalities were assessed by DSCT. In contrast to TTE, the diagnostic accuracy of associated malformations on DSCT were analyzed and calculated with reference to surgical or digital subtraction angiography (DSA) findings. Effective doses (EDs) were also calculated. RESULTS: Among the 25 patients, 12 (48%) had ventricular septal defects and left ventricular outflow tract stenosis. Sixteen patients (16/25, 64%) had great arteries with an oblique spatial relationship on DSCT. In addition, we found seven patients (7/25, 28%) with coronary artery malformation, including five with an abnormal coronary origin and two with signs of a myocardial bridge. According to DSA or surgical findings, DSCT was superior to TTE in demonstrating extracardiac anomalies (sensitivity, anomalies of great vessels: 100% vs. 93.33%, other anomalies: 100% vs. 46.15%). The mean estimated ED for those aged <10 years was <2 mSv (1.59 ± 0.95 mSv). CONCLUSIONS: DSCT can achieve an overall assessment of patients with TGA, including any associated malformations as well as the identification of the spatial relationship of the great arteries. DSCT can therefore be considered as an alternative imaging modality for surgical decision making.

Longitudinal sequential biventricular assessment in adults with transposition of the great arteries and relationship with adverse outcomes.

BACKGROUND: In a cohort of congenitally corrected transposition of the great arteries (cc-TGA) and transposition of the great arteries after atrial switch procedure (d-TGA) the study objectives were: 1) to assess the change of quantitative systemic right ventricle (sRV) parameters over time and; 2) to examine the relationship of quantitative sRV parameters with adverse clinical outcomes. METHODS AND RESULTS: Single-center cohort study that included 49 (39%) cc-TGA and 76 (61%) d-TGA patients >18years who had at least one MUGA sRV assessment, 18/39 had more than one respectively. The primary clinical endpoint was all-cause mortality, heart transplantation and/or heart failure hospitalization. At a median clinical follow-up of 7years following the first MUGA, the primary endpoint occurred more often in cc-TGA versus d-TGA patients (18 (36.7%) vs. 9 (11.8%), p=0.03). Median time between the MUGA assessments was 5.8 (cc-TGA) and 4.9years (d-TGA). At last MUGA follow-up: 6 (33%) cc-TGA/14 (36%) d-TGA patients showed a significant decline in sRVEF (>5%); 6 (33%) cc-TGA/17 (44%) d-TGA patients had a significant increase in sRVEDVi; and 7 (39%) cc-TGA/19 (49%) PA-TGA patients had a significant increase in sRVESVi. Baseline sRV parameters were not associated with the primary end point or sRV changes over time. CONCLUSIONS: An important proportion of both patient cohorts demonstrated a significant change in sRV parameters over time and these are likely related to multiple factors that vary between individuals given population heterogeneity. The TGA patients have distinct clinical trajectories with increased adverse heart failure outcomes in the cc-TGA population and sRV parameters were not related to adverse heart failure events in either group.

Prenatal Echocardiographic Assessment of Foramen Ovale Appearance in Fetuses with D-Transposition of the Great Arteries and Impact on Neonatal Outcome.

INTRODUCTION: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth. PATIENTS AND METHODS: We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia. RESULTS: We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p < 0.0001, p = 0.002 and p = 0.0001, respectively). CONCLUSIONS: Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth.

Noninvasive Assessment of Vascular Function in Postoperative Cardiovascular Disease (Coarctation of the Aorta, Tetralogy of Fallot, and Transposition of the Great Arteries).

Using noninvasive techniques, we sought to assess arterial stiffness, impedance, hydraulic power, and efficiency in children with postoperative tetralogy of Fallot (TOF), coarctation of the aorta (COA), and transposition of the great arteries (TGAs). Results were compared with those of healthy peers. Fifty-five children with repaired congenital heart disease (24 TOFs, 20 COAs, and 11 TGAs) were compared with 55 age-matched control subjects (CTRL). Echocardiographic Doppler imaging and carotid artery applanation tonometry were preformed to measure aortic flow, dimensions, and calculate pulse wave velocity, vascular impedance and arterial stiffness indexes, hydraulic power (mean and total), and hydraulic efficiency (HE) which were calculated using standard fluid dynamics equations. All congenital heart disease subgroups had higher pulse wave velocity than CTRL. Only the COA group had higher characteristic impedance. Mean power was higher in TGA than in CTRL and TOF, and total power was higher in TGA than in CTRL and TOF. Hydraulic efficiency was higher in TOF than in COA and TGA. In conclusion, children with TOF, COA, and TGA have stiffer aortas than CTRL. These changes may be related to intrinsic aortic abnormalities, altered integrity of the aorta due to surgical repair, and/or acquired postsurgery. These patients may be at increased long-term cardiovascular risk, and long-term follow-up is important for monitoring and assessment of efforts to reduce risk.

Quantitative assessment of systolic right ventricular function using myocardial deformation in patients with a systemic right ventricle.

AIMS: Late systolic dysfunction of the systemic right ventricle (RV) in patients with transposition of the great arteries (TGA) is of major concern. Right ventricular global longitudinal strain (GLS) might be able to identify early dysfunction. METHODS AND RESULTS: Adults with TGA after Mustard operation (TGA-Mustard) or congenitally corrected-TGA (ccTGA) underwent echocardiography, electrocardiography, and NT-proBNP measurement. Using speckle-tracking echocardiography, we analysed longitudinal strain and strain rate, and compared findings in both patients groups, to healthy controls and with clinical parameters. We included 42 patients (mean age 37 ± 7 years, 69% male) with a systemic RV [32 TGA-Mustard (34 ± 4 years after corrective surgery) and 10 ccTGA], and 32 healthy controls (mean age 36 ± 11 years). Global longitudinal strain of the systemic RV was lower in patients than GLS of the systemic LV in controls (-14.2 ± 3.5 vs. -20.0 ± 3.0%, P < 0.001). Average LS of the RV lateral wall was lower in patients with TGA-Mustard (-15.5 ± 3.4%) than ccTGA (-18.3 ± 3.6%, P = 0.047). Right ventricular GLS tended to be lower in patients in NYHA class II than I, and correlated with NT-proBNP (r = 0.49, P = 0.001), RV fractional area change (r = -0.39, P = 0.019), RV apex-base-diameter (r = 0.37, P = 0.021), and QRS duration (r = 0.41, P = 0.014). CONCLUSION: Global longitudinal strain of the systemic RV in patients is lower than GLS of the systemic LV in healthy controls, especially in the apical segment, and tended to be lower in TGA-Mustard than ccTGA patients. Since RV GLS correlates with RV function, myocardial deformation is useful as a more quantitative tool to measure systemic RV function. Decreased GLS was associated with elevated NT-proBNP and tended to correlate with worsening NYHA class, which strengthens the potential prognostic value of GLS in patients with a systemic RV.

Long-term follow-up assessment after the arterial switch operation for correction of dextro-transposition of the great arteries by means of exercise myocardial perfusion-gated SPECT.

The arterial switch operation (ASO) is the preferred technique for correcting transposition of the great arteries, but translocation and reimplantation of the coronary arteries can produce myocardial ischemia. This report aims to describe the authors' experience with exercise single-photon emission computed tomography (SPECT) used to evaluate myocardial perfusion. Exercise-rest gated-myocardial perfusion SPECT was performed for 69 patients (49 boys; median age, 9 years; 5th percentile [6.4 years] to 95th percentile [15.6 years]), 64 of whom were asymptomatic 9.98 ± 3.20 years after ASO. During exercise testing, the patients reached 9.85 ± 3.05 metabolic equivalents (METs) and a median heart rate of 160 beats per minute (bpm), 5th percentile (106 bpm) to 95th percentile (196 bpm). Whereas 61 patients (88.41 %) had normal myocardial perfusion, 2 patients (2.9 %) had reversible defects, and 6 patients (8.7 %) had fixed defects. All the patients with perioperative ischemic complications (4/4, 100 %) had myocardial perfusion defects, whereas four patients (4/65, 6.15 %) without ischemic complications had abnormal perfusion (p = 0.0005). Age at the time of surgery did not differ significantly (p = 0.234) between the patients with perfusion defects and those with normal study results. No significant difference was observed between the patients who had an A coronary pattern (left coronary artery originating from the left sinus and the right coronary artery originating from the right sinus, n = 47) and those who had a non-A coronary pattern (n = 22) (p = 1). The high rate for normality of exercise myocardial perfusion in our study suggests that myocardial perfusion gated-SPECT should be reserved for patients who have experienced perioperative ischemic complications or those with symptoms, at least during the first 10 years after the surgery.

Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult.

We studied 11 adult patients with dextro-transposition and 5 adult patients with levo-transposition (corrected transposition) of the great arteries with real time two-dimensional (2DTTE) and live/real time three-dimensional transthoracic echocardiography (3DTTE). All patients with dextro-transposition underwent a Mustard or Senning procedure during infancy. Incremental findings provided by 3DTTE and not delineated by 2DTTE were (a) comprehensive examination of all three leaflets of the tricuspid valve including the detection and measurement of anatomic defects in the leaflets and the assessment of systolic noncoaptation and segmental prolapse; (b) en face viewing and measurement of vena contracta areas of the valvular regurgitation jets and the assessment of regurgitant volumes; (c) en face viewing of the intra-atrial baffle and localization and measurement of baffle defects as well as the measurement of vena contractas of the baffle leaks; (d) recognition of a bicuspid pulmonary valve; and (e) the quantitative assessment of left ventricular outflow tract obstruction. 3DTTE appears to be a useful noninvasive modality which could supplement 2DTTE in the comprehensive assessment of adult patients with transposition of the great arteries.

[Tissue Doppler imaging in the assessment of right ventricular function in congenital heart disease. Correlation with echo-MRI]. / Szöveti Doppler-echokardiográfia a jobbkamra-funkció megítélésére veleszületett szívhibákban. Echo-szív mágneses rezonanciás összehasonlító vizsgálatok.

UNLABELLED: The noninvasive evaluation of right ventricular function is difficult, especially in postoperative complex congenital heart disease due to the special right ventricular geometry. The right ventricular dysfunction is a key factor in morbidity and mortality and in the determination of the necessity of reoperation. PURPOSE: To report the author's experiences of 133 TDI investigations. Pts age: 14 days-20 yrs, mean 12,4 yrs. To assess right ventricular function TDI investigations were performed for "systemic right ventricular" function in transposition of great arteries after Senning operation (Group I.21 TDI in 17 pts) and after reconstruction of Tetralogy of Fallot (Group II, 21 pts). METHODS: RV tricuspid anulus systolic velocity (Sy) and early diastolic velocity (E') were measured from Doppler inflow the E wave peak velocity, and the E/E' ratio was calculated. The deviation of these TDI velocities from normal values were analyzed and the correlation with RV ejection fraction was measured by MRI. RESULTS: In Group I we found significant differences from normal population Sy 9.3 +/- 3.7 cm/s vs. 14.2 +/- 2.3, E' 12.8 +/- 4.7 cm/s vs. 16.7 +/- 2.8 cm/s, E/E' 7.2 +/- 4.6 vs. 3.7 +/- 1.4. Sy TDI velocity and E/E' ratio showed good correlation with RV EF measured by MRI ( p > 0.001). In Group II Sy velocity was decreased: 11.3 +/- 4.3 cm/s and showed also good correlation with MRI EF ( p > 0.001). CONCLUSION: TDI for RV function analysis is recommended in congenital heart disease and also proposed in addition to other methods. Due to experience with MRI, reliable data can be collected for the postoperative pts, to decide the correct time of redo operation.

Transposition of the great arteries in the fetus: assessment of the spatial relationships of the arterial trunks by four-dimensional echocardiography.

OBJECTIVE: Coronary arterial abnormalities can be one of the few negative prognostic indicators in transposition of the great arteries (TGA), and their occurrence is related to the type of spatial relationship of the great arteries. The main objective of this study was to assess whether the use of the reconstructed en-face view with color Doppler imaging of the four cardiac valves can demonstrate the different types of spatial relationship of the arterial trunks in fetuses with TGA, in order to derive the risk of coronary abnormalities. A secondary end-point was the evaluation of the type of coronary arterial branching pattern. METHODS: Twenty-three fetuses with a confirmed diagnosis of TGA underwent four-dimensional (4D) echocardiography at 19-33 gestational weeks. The en-face view of the four cardiac valves and color Doppler with high persistence were employed to assess the spatial relationships of the great arteries. In all cases, confirmation of the vessels' arrangement and coronary arterial distribution was obtained at neonatal echocardiography and/or surgery. RESULTS: The spatial relationships of the great vessels was identified correctly in 20/23 (87%) cases. The aorta was found to be located anterior to and to the right of the pulmonary trunk in 13/23 (56.5%) cases and just anterior to the pulmonary artery in 6/23 (26.1%) cases; in the remaining four (17.4%) cases, the two vessels were side by side. With respect to the association between the spatial relationship of the great arteries and the occurrence of an unusual pattern of coronary arterial branching, five of the TGA fetuses had abnormal coronary arterial distribution. CONCLUSIONS: Using 4D echocardiography with color Doppler, it is possible to define the spatial relationships of the great arteries in fetuses with TGA with a high degree of accuracy. This information can be used during counseling to predict the likelihood of abnormal coronary arterial distribution.