Age- and sex-specific reference values of biventricular flow components and kinetic energy by 4D flow cardiovascular magnetic resonance in healthy subjects.

BACKGROUND: Advances in four-dimensional flow cardiovascular magnetic resonance (4D flow CMR) have allowed quantification of left ventricular (LV) and right ventricular (RV) blood flow. We aimed to (1) investigate age and sex differences of 4D flow CMR-derived LV and RV relative flow components and kinetic energy (KE) parameters indexed to end-diastolic volume (KEiEDV) in healthy subjects; and (2) assess the effects of age and sex on these parameters. METHODS: We performed 4D flow analysis in 163 healthy participants (42% female; mean age 43 ± 13 years) of a prospective registry study (NCT03217240) who were free of cardiovascular diseases. Relative flow components (direct flow, retained inflow, delayed ejection flow, residual volume) and multiple phasic KEiEDV (global, peak systolic, average systolic, average diastolic, peak E-wave, peak A-wave) for both LV and RV were analysed. RESULTS: Compared with men, women had lower median LV and RV residual volume, and LV peak and average systolic KEiEDV, and higher median values of RV direct flow, RV global KEiEDV, RV average diastolic KEiEDV, and RV peak E-wave KEiEDV. ANOVA analysis found there were no differences in flow components, peak and average systolic, average diastolic and global KEiEDV for both LV and RV across age groups. Peak A-wave KEiEDV increased significantly (r = 0.458 for LV and 0.341 for RV), whereas peak E-wave KEiEDV (r = - 0.355 for LV and - 0.318 for RV), and KEiEDV E/A ratio (r = - 0.475 for LV and - 0.504 for RV) decreased significantly, with age. CONCLUSION: These data using state-of-the-art 4D flow CMR show that biventricular flow components and kinetic energy parameters vary significantly by age and sex. Age and sex trends should be considered in the interpretation of quantitative measures of biventricular flow. Clinical trial registration  https://www. CLINICALTRIALS: gov . Unique identifier: NCT03217240.

Right ventricular energetic biomarkers from 4D Flow CMR are associated with exertional capacity in pulmonary arterial hypertension.

BACKGROUND: Cardiovascular magnetic resonance (CMR) offers comprehensive right ventricular (RV) evaluation in pulmonary arterial hypertension (PAH). Emerging four-dimensional (4D) flow CMR allows visualization and quantification of intracardiac flow components and calculation of phasic blood kinetic energy (KE) parameters but it is unknown whether these parameters are associated with cardiopulmonary exercise test (CPET)-assessed exercise capacity, which is a surrogate measure of survival in PAH. We compared 4D flow CMR parameters in PAH with healthy controls, and investigated the association of these parameters with RV remodelling, RV functional and CPET outcomes. METHODS: PAH patients and healthy controls from two centers were prospectively enrolled to undergo on-site cine and 4D flow CMR, and CPET within one week. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes (EDV). Phasic (peak systolic, average systolic, and peak E-wave) LV and RV blood flow KE indexed to EDV (KEIEDV) and ventricular LV and RV flow components (direct flow, retained inflow, delayed ejection flow, and residual volume) were calculated. Oxygen uptake (VO2), carbon dioxide production (VCO2) and minute ventilation (VE) were measured and recorded. RESULTS: 45 PAH patients (46 ± 11 years; 7 M) and 51 healthy subjects (46 ± 14 years; 17 M) with no significant differences in age and gender were analyzed. Compared with healthy controls, PAH had significantly lower median RV direct flow, RV delayed ejection flow, RV peak E-wave KEIEDV, peak VO2, and percentage (%) predicted peak VO2, while significantly higher median RV residual volume and VE/VCO2 slope. RV direct flow and RV residual volume were significantly associated with RV remodelling, function, peak VO2, % predicted peak VO2 and VE/VCO2 slope (all P < 0.01). Multiple linear regression analyses showed RV direct flow to be an independent marker of RV function, remodelling and exercise capacity. CONCLUSION: In this 4D flow CMR and CPET study, RV direct flow provided incremental value over RVEF for discriminating adverse RV remodelling, impaired exercise capacity, and PAH with intermediate and high risk based on risk score. These data suggest that CMR with 4D flow CMR can provide comprehensive assessment of PAH severity, and may be used to monitor disease progression and therapeutic response. TRIAL REGISTRATION NUMBER: https://www. CLINICALTRIALS: gov . Unique identifier: NCT03217240.

Ventricular flow analysis and its association with exertional capacity in repaired tetralogy of Fallot: 4D flow cardiovascular magnetic resonance study.

BACKGROUND: Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) allows quantification of biventricular blood flow by flow components and kinetic energy (KE) analyses. However, it remains unclear whether 4D flow parameters can predict cardiopulmonary exercise testing (CPET) as a clinical outcome in repaired tetralogy of Fallot (rTOF). Current study aimed to (1) compare 4D flow CMR parameters in rTOF with age- and gender-matched healthy controls, (2) investigate associations of 4D flow parameters with functional and volumetric right ventricular (RV) remodelling markers, and CPET outcome. METHODS: Sixty-three rTOF patients (14 paediatric, 49 adult; 30 ± 15 years; 29 M) and 63 age- and gender-matched healthy controls (14 paediatric, 49 adult; 31 ± 15 years) were prospectively recruited at four centers. All underwent cine and 4D flow CMR, and all adults performed standardized CPET same day or within one week of CMR. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes. Four flow components were analyzed: direct flow, retained inflow, delayed ejection flow and residual volume. Additionally, three phasic KE parameters normalized to end-diastolic volume (KEiEDV), were analyzed for both LV and RV: peak systolic, average systolic and peak E-wave. RESULTS: In comparisons of rTOF vs. healthy controls, median LV retained inflow (18% vs. 16%, P = 0.005) and median peak E-wave KEiEDV (34.9 µJ/ml vs. 29.2 µJ/ml, P = 0.006) were higher in rTOF; median RV direct flow was lower in rTOF (25% vs. 35%, P < 0.001); median RV delayed ejection flow (21% vs. 17%, P < 0.001) and residual volume (39% vs. 31%, P < 0.001) were both greater in rTOF. RV KEiEDV parameters were all higher in rTOF than healthy controls (all P < 0.001). On multivariate analysis, RV direct flow was an independent predictor of RV function and CPET outcome. RV direct flow and RV peak E-wave KEiEDV were independent predictors of RV remodelling index. CONCLUSIONS: In this multi-scanner multicenter 4D flow CMR study, reduced RV direct flow was independently associated with RV dysfunction, remodelling and, to a lesser extent, exercise intolerance in rTOF patients. This supports its utility as an imaging parameter for monitoring disease progression and therapeutic response in rTOF. Clinical Trial Registration https://www.clinicaltrials.gov . Unique identifier: NCT03217240.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction.

BACKGROUND: Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. METHODS: A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. RESULTS: Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14-57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3-4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3-100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. CONCLUSIONS: Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.

Real-time assessment of right and left ventricular volumes and function in children using high spatiotemporal resolution spiral bSSFP with compressed sensing.

BACKGROUND: Real-time cardiovascular magnetic resonance (CMR) assessment of ventricular volumes and function enables data acquisition during free-breathing. The requirement for high spatiotemporal resolution in children necessitates the use of highly accelerated imaging techniques. METHODS: A novel real-time balanced steady state free precession (bSSFP) spiral sequence reconstructed using Compressed Sensing (CS) was prospectively validated against the breath-hold clinical standard for assessment of ventricular volumes in 60 children with congenital heart disease. Qualitative image scoring, quantitative image quality, as well as evaluation of biventricular volumes was performed. Standard BH and real-time measures were compared using the paired t-test and agreement for volumetric measures were evaluated using Bland Altman analysis. RESULTS: Acquisition time for the entire short axis stack (~ 13 slices) using the spiral real-time technique was ~ 20 s, compared to ~ 348 s for the standard breath hold technique. Qualitative scores reflected more residual aliasing artefact (p < 0.001) and lower edge definition (p < 0.001) in spiral real-time images than standard breath hold images, with lower quantitative edge sharpness and estimates of image contrast (p < 0.001). There was a small but statistically significant (p < 0.05) overestimation of left ventricular (LV) end-systolic volume (1.0 ± 3.5 mL), and underestimation of LV end-diastolic volume (- 1.7 ± 4.6 mL), LV stroke volume (- 2.6 ± 4.8 mL) and LV ejection fraction (- 1.5 ± 3.0%) using the real-time technique. We also observed a small underestimation of right ventricular stroke volume (- 1.8 ± 4.9 mL) and ejection fraction (- 1.4 ± 3.7%) using the real-time imaging technique. No difference in inter-observer or intra-observer variability were observed between the BH and real-time sequences. CONCLUSIONS: Real-time bSSFP imaging using spiral trajectories combined with a compressed sensing reconstruction showed good agreement for quantification of biventricular metrics in children with heart disease, despite slightly lower image quality. This technique holds the potential for free breathing data acquisition, with significantly shorter scan times in children.

Idiopathic pulmonary arterial hypertension with coexisting CHD.

Sinus venosus atrial septal defect can result in an increase in pulmonary blood flow and vascular resistance, leading to pulmonary hypertension. Rarely, the degree of pulmonary hypertension is out of proportion to the degree of intra-cardiac shunting. This case outlines the differences between pulmonary hypertension secondary to CHD and idiopathic pulmonary hypertension, and illustrates the investigation and management strategy used in a patient with features of both.

The comparative role of echocardiography and MRI for identifying critical lesions in patients with single-ventricle physiology, before bidirectional cavopulmonary connection.

BACKGROUND: In the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection. METHODS: A total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation. RESULTS: Median age at bidirectional cavopulmonary connection was 160 days (interquartile range 121-284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12-2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04-2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06-2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5-37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7-295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3-121.6, p=0.00001)). CONCLUSION: For branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.

Physiological and phenotypic characteristics of late survivors of tetralogy of fallot repair who are free from pulmonary valve replacement.

BACKGROUND: Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR. METHODS AND RESULTS: Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8-5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4-57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m(2)) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak o2=-0.91±1.3; z e/ co2=0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mm Hg), pulmonary annulus diameters <0.5z, and unobstructed branch pulmonary arteries. CONCLUSIONS: An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z.

Long-term outcome after neonatal intervention for congenital critical aortic stenosis.

BACKGROUND: This study explored long-term outcome and functional status of patients born with critical aortic stenosis (CAS) following neonatal surgical or catheter interventions. METHODS: A 40-year retrospective review of all consecutive patients within a large, single-center referral unit who required neonatal (<30 days) intervention for CAS. Additional detailed evaluation of surviving patients >7 years age was performed, with clinical assessment, objective cardiopulmonary exercise testing and state-of-the-art characterization of myocardial function (advanced echocardiography and cardiac MRI). RESULTS: Between 1970 and 2010, ninety-six neonates underwent CAS intervention (mean age 9 ± 7.5 days). Early death occurred in 19 (19.8%) and late death in 10 patients. Overall survival at 10 and 30 years was 70.1% and 68.5%, freedom from reintervention was 41.8% and 32.9% respectively. Among the 25 long-term survivors available for detailed assessment (median age 15.7 ± 6.4 years), 55% exhibited impaired peak oxygen uptake. Mean left ventricle (LV) ejection fraction was 65 ± 11.2%, with a mean LV end-diastolic volume z-score of 0.02 ± 1.4. Mean LV outflow tract Vmax was 2.3 ± 1.02 m/s. CAS patients had reduced LV longitudinal and increased radial strain (p = 0.003, p < 0.001 respectively). Five patients had severe LV diastolic dysfunction associated with endocardial fibroelastosis (EFE) (p = 0.0014). CONCLUSION: Despite high early mortality rate, long-term survival of patients with CAS is reasonable at the expense of high reintervention rate. With successful intervention, there remained long-term clinical and subclinical LV myocardial impairment, of which EFE was one marker. Long-term follow-up of all CAS patients is crucial, involving detailed myocardial functional assessment to help elucidate physiology and optimise management.

A triad: cardiac rhabdomyosarcoma, stroke and tamponade.

The case report describes a 9-year-old boy who presented with an acute cerebrovascular accident and was found to have cardiac tamponade caused by cardiac rhabdomyosarcoma. Symptoms of rhabdomyosarcoma can be indolent and nonspecific, even with metastatic disease. Echocardiography and cardiac magnetic resonance imaging are explored as adjuncts to diagnosis. The radiologic features that helped with the diagnosis of this rare condition are described.

The Importance of Mitral Valve Prolapse Doming Volume in the Assessment of Left Ventricular Stroke Volume with Cardiac MRI.

There remains a debate whether the ventricular volume within prolapsing mitral valve (MV) leaflets should be included in the left ventricular (LV) end-systolic volume, and therefore factored in LV stroke volume (SV), in cardiac magnetic resonance (CMR) assessments. This study aims to compare LV volumes during end-systolic phases, with and without the inclusion of the volume of blood on the left atrial aspect of the atrioventricular groove but still within the MV prolapsing leaflets, against the reference LV SV by four-dimensional flow (4DF). A total of 15 patients with MV prolapse (MVP) were retrospectively enrolled in this study. We compared LV SV with (LV SVMVP) and without (LV SVstandard) MVP left ventricular doming volume, using 4D flow (LV SV4DF) as the reference value. Significant differences were observed when comparing LV SVstandard and LV SVMVP (p < 0.001), and between LV SVstandard and LV SV4DF (p = 0.02). The Intraclass Correlation Coefficient (ICC) test demonstrated good repeatability between LV SVMVP and LV SV4DF (ICC = 0.86, p < 0.001) but only moderate repeatability between LV SVstandard and LV SV4DF (ICC = 0.75, p < 0.01). Calculating LV SV by including the MVP left ventricular doming volume has a higher consistency with LV SV derived from the 4DF assessment. In conclusion, LV SV short-axis cine assessment incorporating MVP dooming volume can significantly improve the precision of LV SV assessment compared to the reference 4DF method. Hence, in cases with bi-leaflet MVP, we recommend factoring in MVP dooming into the left ventricular end-systolic volume to improve the accuracy and precision of quantifying mitral regurgitation.

A prospective audit of safety issues associated with general anesthesia for pediatric cardiac magnetic resonance imaging.

BACKGROUND/OBJECTIVES: Cardiac MRI (CMR) is increasingly used for surgical planning and serial monitoring of children with congenital heart disease (CHD). For small children, general anesthesia (GA) is required. We describe our experience of the safety of GA for pediatric CMR, using data collected prospectively over 3 years. METHODS: All consecutive infants undergoing GA for CMR at our institution, between November 2005 and May 2008, were included. Informed and written consent to participate in research investigation was acquired from the guardians of every patient prior to CMR. The cardiac anesthetist completed a standardized data collection form during each procedure. Information collected included demographics, diagnosis, surgical history, anesthetic management, significant incidents, and discharge circumstances. RESULTS: A total of 120 patients with varying cardiac physiology and a range of hemodynamics underwent GA for CMR during the study period. Gas induction was predominantly used, even in those with impaired ventricular function. The majority (71%) of procedures were undertaken without significant incident. Minor adverse incidents were recorded in 32 patients, mild hypotension being most frequent. One major adverse event occurred. A patient with hypoplastic left heart syndrome (HLHS) suffered hypotension then cardiac arrest in the scanner. This patient was successfully resuscitated. CONCLUSION: Although the majority of cases were safe and without incident, the complication rate in children with CHD receiving a GA for CMR is higher than in the general pediatric population. This reinforces the need for a senior, multidisciplinary team to be involved in the care of these children during imaging.

Cardiac magnetic resonance imaging predicts cardiac catheter findings for great artery stenosis in children with congenital cardiac disease.

OBJECTIVE: To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis. METHODS: We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008. RESULTS: Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4-149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years - with an interquartile range of 3.8-16.9 years - and weighed 34 kilograms - with an interquartile range of 15-56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate-severe great vessel stenosis - involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion. CONCLUSION: Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion - for example, unilateral pulmonary artery stenosis - and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.

Quantification of Pulmonary Artery Configuration after the Arterial Switch Operation: A Pilot Study.

BACKGROUND: The arterial switch operation (ASO) is the preferred treatment for d-transposition of the great arteries (TGA). Freedom from reintervention is mainly determined by the performance of the arterial outflow tracts, with variable incidence of pulmonary artery stenosis (PAS), possibly related to aspects of surgical technique. This pilot study attempts to describe pulmonary artery (PA) configuration through several measurements using three-dimensional data from cardiac magnetic resonance (CMR) imaging and assesses whether PA configuration is associated with PAS. METHODS: A retrospective, single-centre analysis of paediatric patients undergoing CMR after ASO. The geometry of the pulmonary arteries was compared between patients with and without PAS as judged by the CMR report. RESULTS: Among all patients (n = 612) after ASO, 45 patients underwent CMR at a median age of 10 years (3.5-13). Twenty-two (57.9%) had PAS, categorized as mild (n = 1), moderate (n = 19) or severe (n = 2). Eighteen had stenosis on PA branches. Four had MPA stenosis. Comparison between groups with and without PAS revealed no significant differences in neo-aortic to pulmonary angle, MPA to LPA/RPA angle, or bifurcation angle. There was a significant difference in cranial displacement, with more cranial displacement in the group without PAS. However, this group was older, 10.8 (7.3-14.3) years compared to those with PAS, 6.8 (1.5-12.1). CONCLUSIONS: The spectrum of PAS after ASO is heterogenous. This study shows the feasibility of measuring PA configuration in three planes on CMR. There is no correlation between PA configuration and PAS. Therefore, other mechanisms are probably responsible for the occurrence of PAS, rather than the configuration on itself. Further multicentric studies are warranted to confirm the suggested measuring method and assessing the associations with PAS, to eventually advise surgical methodology.

Kat-ARC accelerated 4D flow CMR: clinical validation for transvalvular flow and peak velocity assessment.

BACKGROUND: To validate the k-adaptive-t autocalibrating reconstruction for Cartesian sampling (kat-ARC), an exclusive sparse reconstruction technique for four-dimensional (4D) flow cardiac magnetic resonance (CMR) using conservation of mass principle applied to transvalvular flow. METHODS: This observational retrospective study (2020/21-075) was approved by the local ethics committee at the University of East Anglia. Consent was waived. Thirty-five patients who had a clinical CMR scan were included. CMR protocol included cine and 4D flow using Kat-ARC acceleration factor 6. No respiratory navigation was applied. For validation, the agreement between mitral net flow (MNF) and the aortic net flow (ANF) was investigated. Additionally, we checked the agreement between peak aortic valve velocity derived by 4D flow and that derived by continuous-wave Doppler echocardiography in 20 patients. RESULTS: The median age of our patient population was 63 years (interquartile range [IQR] 54-73), and 18/35 (51%) were male. Seventeen (49%) patients had mitral regurgitation, and seven (20%) patients had aortic regurgitation. Mean acquisition time was 8 ± 4 min. MNF and ANF were comparable: 60 mL (51-78) versus 63 mL (57-77), p = 0.310). There was an association between MNF and ANF (rho = 0.58, p < 0.001). Peak aortic valve velocity by Doppler and 4D flow were comparable (1.40 m/s, [1.30-1.75] versus 1.46 m/s [1.25-2.11], p = 0.602) and also correlated with each other (rho = 0.77, p < 0.001). CONCLUSIONS: Kat-ARC accelerated 4D flow CMR quantified transvalvular flow in accordance with the conservation of mass principle and is primed for clinical translation.

The role of cardiovascular magnetic resonance in pediatric congenital heart disease.

Cardiovascular magnetic resonance (CMR) has expanded its role in the diagnosis and management of congenital heart disease (CHD) and acquired heart disease in pediatric patients. Ongoing technological advancements in both data acquisition and data presentation have enabled CMR to be integrated into clinical practice with increasing understanding of the advantages and limitations of the technique by pediatric cardiologists and congenital heart surgeons. Importantly, the combination of exquisite 3D anatomy with physiological data enables CMR to provide a unique perspective for the management of many patients with CHD. Imaging small children with CHD is challenging, and in this article we will review the technical adjustments, imaging protocols and application of CMR in the pediatric population.

Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA).

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly. This study shows the role of cardiovascular magnetic resonance (CMR) in assessing young patients following surgical repair of ALCAPA. METHODS: 6 patients, aged 9-21 years, with repaired ALCAPA (2 Tackeuchi method, 4 direct re-implantation) underwent CMR because of clinical suspicion of myocardial ischemia. Imaging used short and long axis cine images (assess ventricular function), late-gadolinium enhancement (LGE) (detect segmental myocardial fibrosis), adenosine stress perfusion (detect reversible ischaemia) and 3D whole-heart imaging (visualize proximal coronary arteries). RESULTS: The left ventricular (LV) global systolic function was preserved in all patients (mean LV ejection fraction = 62.7% ± 4.23%). The LV volumes were within the normal ranges, (mean indexed LVEDV = 75.4 ± 3.5 ml/m², LVESV = 31.6 ± 9.4 ml/m²). In 1 patient, hypokinesia of the anterior segments was visualized. Five patients showed sub-endocardial LGE involving the basal, antero-lateral wall and the anterior papillary muscle. Three patients had areas of reversible ischemia. In these 3, 3D whole-heart MRA showed that the proximal course of the left coronary artery was occluded (confirmed with cardiac catheterisation). CONCLUSIONS: CMR is a good, non-invasive, radiation-free investigation in the post-surgical evaluation of ALCAPA. In referred patients we show that basal, antero-lateral sub-endocardial myocardial fibrosis is a characteristic finding. Furthermore, stress adenosine CMR perfusion, can identify reversible ischemia in this group, and was indicative of left coronary artery occlusion.

Prevalence of associated cardiovascular abnormalities in 500 patients with aortic coarctation referred for cardiovascular magnetic resonance imaging to a tertiary center.

Coarctation of the aorta (CoA) is a common congenital defect whose overall incidence is 5-8% of all congenital cardiac anomalies. Associated cardiac anomalies have been well described in previous studies examining specific subgroups of CoA patients, particularly infants and necropsy specimens. The majority of studies, conducted from the 1970s to 1980s, excluded older children, adolescents, and adults. Given the advent of improved surgical and interventional techniques, many CoA patients are surviving into adulthood. This study examined a population of 500 CoA patients in the authors' cardiovascular magnetic resonance imaging (MRI) database involving a population of CoA survivors 5-79 years of age. This was to give a new perspective on the prevalence of associated cardiovascular abnormalities including the bicuspid aortic valve, arch hypoplasia, intracardiac shunts, and subaortic stenosis. These associated abnormalities are less prevalent than in previous studies, reflecting a milder spectrum of CoA. Cardiovascular MRI with its multiplanar imaging capabilities and lack of ionizing radiation is safe and suitable for evaluation and follow-up assessment of CoA patients. Evaluation of CoA by MRI should not be confined to the arch, but should include the heart and mediastinal vessels to assess for the presence and severity of any expected or unexpected associated anomalies.

Lessons from inter-stage cardiac magnetic resonance imaging in predicting survival for patients with hypoplastic left heart syndrome.

UNLABELLED: This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6-4.6) months and 5.0 (4.5-5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57-0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14-44%) and 25% (11-50%), respectively. The median right ventricular ejection fraction was 54% (48-59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8-46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85-0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14-6.44, p = 0.02). CONCLUSIONS: In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.