Recent advances in multimodal imaging in tetralogy of fallot and double outlet right ventricle.

PURPOSE OF REVIEW: In the ever-evolving field of medical imaging, this review highlights significant advancements in preoperative and postoperative imaging for Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) over the past 18 months. RECENT FINDINGS: This review showcases innovations in echocardiography such as 3D speckle tracking echocardiography (3DSTE) for assessing right ventricle-pulmonary artery coupling (RVPAC) and Doppler velocity reconstruction (DoVeR) for intracardiac flow fields evaluation. Furthermore, advances in assessment of cardiovascular anatomy using computed tomography (CT) improve the integration of imaging in ablation procedures. Additionally, the inclusion of cardiac magnetic resonance (CMR) parameters as risk score predictors for morbidity, and mortality and for timing of pulmonary valve replacement (PVR) indicates its significance in clinical management. The utilization of 4D flow techniques for postoperative hemodynamic assessment promises new insights into pressure mapping. Lastly, emerging technologies such as 3D printing and 3D virtual reality are expected to improve image quality and surgical confidence in preoperative planning. SUMMARY: Developments in multimodality imaging in TOF and DORV are poised to shape the future of clinical practice in this field.

Magnetization Transfer BOOST Noncontrast Angiography Improves Pulmonary Vein Imaging in Adults With Congenital Heart Disease.

BACKGROUND: Cardiac MRI plays an important role in the diagnosis and follow-up of patients with congenital heart disease (CHD). Gadolinium-based contrast agents are often needed to overcome flow-related and off-resonance artifacts that can impair the quality of conventional noncontrast 3D imaging. As serial imaging is often required in CHD, the development of robust noncontrast 3D MRI techniques is desirable. PURPOSE: To assess the clinical utility of noncontrast enhanced magnetization transfer and inversion recovery prepared 3D free-breathing sequence (MTC-BOOST) compared to conventional 3D whole heart imaging in patients with CHD. STUDY TYPE: Prospective, image quality. POPULATION: A total of 27 adult patients (44% female, mean age 30.9 ± 14.8 years) with CHD. FIELD STRENGTH/SEQUENCE: A 1.5 T; free-breathing 3D MTC-BOOST sequence. ASSESSMENT: MTC-BOOST was compared to diaphragmatic navigator-gated, noncontrast T2 prepared 3D whole-heart imaging sequence (T2prep-3DWH) for comparison of vessel dimensions, lumen-to-myocardium contrast ratio (CR), and image quality (vessel wall sharpness and presence and type of artifacts) assessed by two experienced cardiologists on a 5-point scale. STATISTICAL TESTS: Mann-Whitney test, paired Wilcoxon signed-rank test, Bland-Altman plots. P < 0.05 was considered statistically significant. RESULTS: MTC-BOOST significantly improved image quality and CR of the right-sided pulmonary veins (PV): (CR: right upper PV 1.06 ± 0.50 vs. 0.58 ± 0.74; right lower PV 1.32 ± 0.38 vs. 0.81 ± 0.73) compared to conventional T2prep-3DWH imaging where the PVs were not visualized in some cases due to off-resonance effects. MTC-BOOST demonstrated resistance to degradation of luminal signal (assessed by CR) secondary to accelerated or turbulent flow conditions. T2prep-3DWH had higher image quality scores than MTC-BOOST for the aorta and coronary arteries; however, great vessel dimensions derived from MTC-BOOST showed excellent agreement with standard T2prep-3DWH imaging. DATA CONCLUSION: MTC-BOOST allows for improved contrast-free imaging of pulmonary veins and regions characterized by accelerated or turbulent blood flow compared to standard T2prep-3DWH imaging, with excellent agreement of great vessel dimensions. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.

Imaging and surgical management of congenital heart diseases.

Congenital heart disease affects approximately 1% of live births per year. In recent years, there has been a decrease in the morbidity and mortality of these cases due to advances in medical and surgical care. Imaging plays a key role in the management of these children, with chest radiography, echocardiography and chest ultrasound the first diagnostic tools, and cardiac computed tomography, catheterization and magnetic resonance imaging reserved to assess better the anatomy and physiology of the most complex cases. This article is a beginner's guide to the anatomy of the most frequent congenital heart diseases (atrial and ventricular septal defects, abnormal pulmonary venous connections, univentricular heart, tetralogy of Fallot, transposition of the great arteries and coarctation of the aorta), their surgical management, the most common postsurgical complications, deciding which imaging modality is needed, and when and how to image gently.

Three-dimensional printing, holograms, computational modelling, and artificial intelligence for adult congenital heart disease care: an exciting future.

Congenital heart disease (CHD) is often comprised of complex three-dimensional (3D) anatomy that must be well understood to assess the pathophysiological consequences and guide therapy. Thus, detailed cardiac imaging for early detection and planning of interventional and/or surgical treatment is paramount. Advanced technologies have revolutionized diagnostic and therapeutic practice in CHD, thus playing an increasing role in its management. Traditional reliance on standard imaging modalities including echocardiography, cardiac computed tomography (CT) and magnetic resonance imaging (MRI) has been augmented by the use of recent technologies such as 3D printing, virtual reality, augmented reality, computational modelling, and artificial intelligence because of insufficient information available with these standard imaging techniques. This has created potential opportunities of incorporating these technologies into routine clinical practice to achieve the best outcomes through delivery of personalized medicine. In this review, we provide an overview of these evolving technologies and a new approach enabling physicians to better understand their real-world application in adult CHD as a prelude to clinical workflow implementation.

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe.

BACKGROUND: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. METHODS: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. RESULTS: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8-12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died. CONCLUSIONS: Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.

Estimating central blood pressure from aortic flow: development and assessment of algorithms.

Central blood pressure (cBP) is a highly prognostic cardiovascular (CV) risk factor whose accurate, invasive assessment is costly and carries risks to patients. We developed and assessed novel algorithms for estimating cBP from noninvasive aortic hemodynamic data and a peripheral blood pressure measurement. These algorithms were created using three blood flow models: the two- and three-element Windkessel (0-D) models and a one-dimensional (1-D) model of the thoracic aorta. We tested new and existing methods for estimating CV parameters (left ventricular ejection time, outflow BP, arterial resistance and compliance, pulse wave velocity, and characteristic impedance) required for the cBP algorithms, using virtual (simulated) subjects (n = 19,646) for which reference CV parameters were known exactly. We then tested the cBP algorithms using virtual subjects (n = 4,064), for which reference cBP were available free of measurement error, and clinical datasets containing invasive (n = 10) and noninvasive (n = 171) reference cBP waves across a wide range of CV conditions. The 1-D algorithm outperformed the 0-D algorithms when the aortic vascular geometry was available, achieving central systolic blood pressure (cSBP) errors ≤ 2.1 ± 9.7 mmHg and root-mean-square errors (RMSEs) ≤ 6.4 ± 2.8 mmHg against invasive reference cBP waves (n = 10). When the aortic geometry was unavailable, the three-element 0-D algorithm achieved cSBP errors ≤ 6.0 ± 4.7 mmHg and RMSEs ≤ 5.9 ± 2.4 mmHg against noninvasive reference cBP waves (n = 171), outperforming the two-element 0-D algorithm. All CV parameters were estimated with mean percentage errors ≤ 8.2%, except for the aortic characteristic impedance (≤13.4%), which affected the three-element 0-D algorithm's performance. The freely available algorithms developed in this work enable fast and accurate calculation of the cBP wave and CV parameters in datasets containing noninvasive ultrasound or magnetic resonance imaging data.NEW & NOTEWORTHY First, our proposed methods for CV parameter estimation and a comprehensive set of methods from the literature were tested using in silico and clinical datasets. Second, optimized algorithms for estimating cBP from aortic flow were developed and tested for a wide range of cBP morphologies, including catheter cBP data. Third, a dataset of simulated cBP waves was created using a three-element Windkessel model. Fourth, the Windkessel model dataset and optimized algorithms are freely available.

MRI for Guided Right and Left Heart Cardiac Catheterization: A Prospective Study in Congenital Heart Disease.

BACKGROUND: Improvements in outcomes for patients with congenital heart disease (CHD) have increased the need for diagnostic and interventional procedures. Cumulative radiation risk is a growing concern. MRI-guided interventions are a promising ionizing radiation-free, alternative approach. PURPOSE: To assess the feasibility of MRI-guided catheterization in young patients with CHD using advanced visualization passive tracking techniques. STUDY TYPE: Prospective. POPULATION: A total of 30 patients with CHD referred for MRI-guided catheterization and pulmonary vascular resistance analysis (median age/weight: 4 years / 15 kg). FIELD STRENGTH/SEQUENCE: 1.5T; partially saturated (pSAT) real-time single-shot balanced steady-state free-precession (bSSFP) sequence. ASSESSMENT: Images were visualized by a single viewer on the scanner console (interactive mode) or using a commercially available advanced visualization platform (iSuite, Philips). Image quality for anatomy and catheter visualization was evaluated by three cardiologists with >5 years' experience in MRI-catheterization using a 1-5 scale (1, poor, 5, excellent). Catheter balloon signal-to-noise ratio (SNR), blood and myocardium SNR, catheter balloon/blood contrast-to-noise ratio (CNR), balloon/myocardium CNR, and blood/myocardium CNR were measured. Procedure findings, feasibility, and adverse events were recorded. A fraction of time in which the catheter was visible was compared between iSuite and the interactive mode. STATISTICAL TESTS: T-test for numerical variables. Wilcoxon signed rank test for categorical variables. RESULTS: Nine patients had right heart catheterization, 11 had both left and right heart catheterization, and 10 had single ventricle circulation. Nine patients underwent solely MRI-guided catheterization. The mean score for anatomical visualization and contrast between balloon tip and soft tissue was 3.9 ± 0.9 and 4.5 ± 0.7, respectively. iSuite provided a significant improvement in the time during which the balloon was visible in relation to interactive imaging mode (66 ± 17% vs. 46 ± 14%, P < 0.05). DATA CONCLUSION: MRI-guided catheterizations were carried out safely and is feasible in children and adults with CHD. The pSAT sequence offered robust and simultaneous high contrast visualization of the catheter and cardiac anatomy. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY STAGE: 1.

Imaging findings of multisystem inflammatory syndrome in children associated with COVID-19.

BACKGROUND: A hyperinflammatory immune-mediated shock syndrome has been recognised in children exposed to the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus that causes coronavirus disease 2019 (COVID-19). OBJECTIVE: To describe typical imaging findings in children with multisystem inflammatory syndrome associated with COVID-19. MATERIALS AND METHODS: During the first wave of the COVID-19 pandemic, imaging studies and clinical data from children treated for multisystem inflammatory syndrome were collected from multiple centres. Standardised case templates including demographic, biochemical and imaging information were completed by participating centres and reviewed by paediatric radiologists and paediatricians. RESULTS: We included 37 children (21 boys; median age 8.0 years). Polymerase chain reaction (PCR) testing was positive for SARS-CoV-2 in 15/37 (41%) children and immunoglobulins in 13/19 children (68%). Common clinical presentations were fever (100%), abdominal pain (68%), rash (54%), conjunctivitis (38%) and cough (32%). Thirty-three children (89%) showed laboratory or imaging findings of cardiac involvement. Thirty of the 37 children (81%) required admission to the intensive care unit, with good recovery in all cases. Chest radiographs demonstrated cardiomegaly in 54% and signs of pulmonary venous hypertension/congestion in 73%. The most common chest CT abnormalities were ground-glass and interstitial opacities (83%), airspace consolidation (58%), pleural effusion (58%) and bronchial wall thickening (42%). Echocardiography revealed impaired cardiac function in half of cases (51%) and coronary artery abnormalities in 14%. Cardiac MRI showed myocardial oedema in 58%, pericardial effusion in 42% and decreased left ventricular function in 25%. Twenty children required imaging for abdominal symptoms, the commonest abnormalities being free fluid (71%) and terminal ileum wall thickening (57%). Twelve children underwent brain imaging, showing abnormalities in two cases. CONCLUSION: Children with multisystem inflammatory syndrome showed pulmonary, cardiac, abdominal and brain imaging findings, reflecting the multisystem inflammatory disease. Awareness of the imaging features of this disease is important for early diagnosis and treatment.

Comparison of stenosis models for usage in the estimation of pressure gradient across aortic coarctation.

Non-invasive estimation of the pressure gradient in cardiovascular stenosis has much clinical importance in assisting the diagnosis and treatment of stenotic diseases. In this research, a systematic comparison is conducted to investigate the accuracy of a group of stenosis models against the MRI- and catheter-measured patient data under the aortic coarctation condition. Eight analytical stenosis models, including six from the literature and two proposed in this study, are investigated to examine their prediction accuracy against the clinical data. The two improved models proposed in this study consider comprehensively the Poiseuille loss, the Bernoulli loss in its exact form, and the entrance effect, of the blood flow. Comparison of the results shows that one of the proposed models demonstrates a cycle-averaged mean prediction error of -0.15 ± 3.03 mmHg, a peak-to-peak prediction error of -1.8 ± 6.89 mmHg, which is the best among the models studied.

Echocardiographic examination of mitral valve abnormalities in the paediatric population: current practices.

We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.

Nomograms for Cardiovascular Magnetic Resonance Measurements in the Pediatric Age Group: To Define the Normal and the Expected Abnormal Values in Corrected/Palliated Congenital Heart Disease: A Systematic Review.

Our purpose is to provide an overview and to systematically review the strengths and limitations of studies on pediatric and adolescent normal values for cardiovascular MRI parameters. A literature search was performed within the National Library of Medicine using the following keywords: normal, reference values, cardiovascular magnetic resonance imaging, and children/pediatric. Eleven published studies evaluating cardiovascular MRI measurements in normal children were included in the present analysis. Our results revealed reasonable consistencies in the protocols employed for cardiovascular MRI. Inter- and intraobserver variability analyses were performed in most studies and generally showed acceptable reproducibility. However, several numerical and methodological limitations emerged. Besides small sample sizes (the largest study enrolled 114 subjects), data for some structures (pulmonary arteries, aortic arch) were limited, and neonates/infants were poorly represented (eg, only two studies). There was heterogeneity regarding measurement normalization (eg, for gender, age, or both), and data were mostly expressed as mean values, while z-scores (commonly used in pediatric echocardiography) were rarely employed. Theoretically, a z-score or a standard deviation of ±2 is considered pathological. Furthermore, differences among races and ethnic groups were not evaluated. In conclusion, our analyses revealed an important need for generation of pediatric and adolescent cardiovascular MRI nomograms built over a wide population of healthy children, using consistent methodologies and with consideration of potentially relevant confounders. More data on expected abnormal values in specific CHD populations (eg, univentricular hearts) also need to be defined. Level of Evidence: 2 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2019;49:1222-1235.

Visualization of coronary arteries in paediatric patients using whole-heart coronary magnetic resonance angiography: comparison of image-navigation and the standard approach for respiratory motion compensation.

AIMS: To investigate the use of respiratory motion compensation using image-based navigation (iNAV) with constant respiratory efficiency using single end-expiratory thresholding (CRUISE) for coronary magnetic resonance angiography (CMRA), and compare it to the conventional diaphragmatic navigator (dNAV) in paediatric patients with congenital or suspected heart disease. METHODS: iNAV allowed direct tracking of the respiratory heart motion and was generated using balanced steady state free precession startup echoes. Respiratory gating was achieved using CRUISE with a fixed 50% efficiency. Whole-heart CMRA was acquired with 1.3 mm isotropic resolution. For comparison, CMRA with identical imaging parameters were acquired using dNAV. Scan time, visualization of coronary artery origins and mid-course, imaging quality and sharpness was compared between the two sequences. RESULTS: Forty patients (13 females; median weight: 44 kg; median age: 12.6, range: 3 months-17 years) were enrolled. 25 scans were performed in awake patients. A contrast agent was used in 22 patients. The scan time was significantly reduced using iNAV for awake patients (iNAV 7:48 ± 1:26 vs dNAV 9:48 ± 3:11, P = 0.01) but not for patients under general anaesthesia (iNAV = 6:55 ± 1:50 versus dNAV = 6:32 ± 2:16; P = 0.32). In 98% of the cases, iNAV image quality had an equal or higher score than dNAV. The visual score analysis showed a clear difference, favouring iNAV (P = 0.002). The right coronary artery and the left anterior descending vessel sharpness was significantly improved (iNAV: 56.8% ± 10.1% vs dNAV: 53.7% ± 9.9%, P < 0.002 and iNAV: 55.8% ± 8.6% vs dNAV: 53% ± 9.2%, P = 0.001, respectively). CONCLUSION: iNAV allows for a higher success-rate and clearer depiction of the mid-course of coronary arteries in paediatric patients. Its acquisition time is shorter in awake patients and image quality score is equal or superior to the conventional method in most cases.

Living the heart in three dimensions: applications of 3D printing in CHD.

Advances in biomedical engineering have led to three-dimensional (3D)-printed models being used for a broad range of different applications. Teaching medical personnel, communicating with patients and relatives, planning complex heart surgery, or designing new techniques for repair of CHD via cardiac catheterisation are now options available using patient-specific 3D-printed models. The management of CHD can be challenging owing to the wide spectrum of morphological conditions and the differences between patients. Direct visualisation and manipulation of the patients' individual anatomy has opened new horizons in personalised treatment, providing the possibility of performing the whole procedure in vitro beforehand, thus anticipating complications and possible outcomes. In this review, we discuss the workflow to implement 3D printing in clinical practice, the imaging modalities used for anatomical segmentation, the applications of this emerging technique in patients with structural heart disease, and its limitations and future directions.

Three-dimensional quantification of vorticity and helicity from 3D cine PC-MRI using finite-element interpolations.

PURPOSE: We propose a 3D finite-element method for the quantification of vorticity and helicity density from 3D cine phase-contrast (PC) MRI. METHODS: By using a 3D finite-element method, we seamlessly estimate velocity gradients in 3D. The robustness and convergence were analyzed using a combined Poiseuille and Lamb-Ossen equation. A computational fluid dynamics simulation was used to compared our method with others available in the literature. Additionally, we computed 3D maps for different 3D cine PC-MRI data sets: phantom without and with coarctation (18 healthy volunteers and 3 patients). RESULTS: We found a good agreement between our method and both the analytical solution of the combined Poiseuille and Lamb-Ossen. The computational fluid dynamics results showed that our method outperforms current approaches to estimate vorticity and helicity values. In the in silico model, we observed that for a tetrahedral element of 2 mm of characteristic length, we underestimated the vorticity in less than 5% with respect to the analytical solution. In patients, we found higher values of helicity density in comparison to healthy volunteers, associated with vortices in the lumen of the vessels. CONCLUSIONS: We proposed a novel method that provides entire 3D vorticity and helicity density maps, avoiding the used of reformatted 2D planes from 3D cine PC-MRI. Magn Reson Med 79:541-553, 2018. © 2017 International Society for Magnetic Resonance in Medicine.

Variability of 4D flow parameters when subjected to changes in MRI acquisition parameters using a realistic thoracic aortic phantom.

PURPOSE: To assess the variability of peak flow, mean velocity, stroke volume, and wall shear stress measurements derived from 3D cine phase contrast (4D flow) sequences under different conditions of spatial and temporal resolutions. METHODS: We performed controlled experiments using a thoracic aortic phantom. The phantom was connected to a pulsatile flow pump, which simulated nine physiological conditions. For each condition, 4D flow data were acquired with different spatial and temporal resolutions. The 2D cine phase contrast and 4D flow data with the highest available spatio-temporal resolution were considered as a reference for comparison purposes. RESULTS: When comparing 4D flow acquisitions (spatial and temporal resolution of 2.0 × 2.0 × 2.0 mm3 and 40 ms, respectively) with 2D phase-contrast flow acquisitions, the underestimation of peak flow, mean velocity, and stroke volume were 10.5, 10 and 5%, respectively. However, the calculated wall shear stress showed an underestimation larger than 70% for the former acquisition, with respect to 4D flow, with spatial and temporal resolution of 1.0 × 1.0 × 1.0 mm3 and 20 ms, respectively. CONCLUSIONS: Peak flow, mean velocity, and stroke volume from 4D flow data are more sensitive to changes of temporal than spatial resolution, as opposed to wall shear stress, which is more sensitive to changes in spatial resolution. Magn Reson Med 79:1882-1892, 2018. © 2017 International Society for Magnetic Resonance in Medicine.

Hypertension in Coarctation of the Aorta: Challenges in Diagnosis in Children.

Evidence indicates that patients with coarctation of the aorta (COA) suffer from increased cardiovascular morbidity and mortality in later life despite successful repair of COA in childhood. Systolic arterial hypertension is common, presenting in up to one-third of patients, and is regarded as the main driver of premature cardiovascular events in this group of patients. In this review, we discuss the prevalence and pathophysiology of hypertension in children following successful COA repair with no residual arch obstruction. The challenges in accurate blood pressure assessment at this early phase are considered and non-invasive measures of central blood pressure are discussed. Although the pathways for investigations in adults are well defined, we highlight the need to address the issues of cardiovascular surveillance in children and describe techniques which can provide complementary information for cardiovascular assessment in this group of patients such that timely treatment can occur.

NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

The goal of this study is to evaluate whether NT-proBNP plasma levels may help as a screening biomarker for monitoring right ventricular dilatation, pulmonary regurgitation and the onset of heart failure in patients with repaired Tetralogy of Fallot. Our single-centre observational prospective study involved 43 patients (15.1 years, SD = 8) with corrected Tetralogy of Fallot. Data collection included: clinical parameters (electrocardiogram, chest X-ray, NYHA scale, time since last surgery), biochemistry (NT-proBNP levels) and MRI values (ventricular volumetry, pulmonary flow assessment). Mean time since last surgery was 13.5 years (SD = 7.8). There was a statistically significant correlation between the NT-proBNP levels (187.4 pg/ml, SD = 154.9) and right ventricular dilatation for both the right ventricular end-diastolic volume (124.9 ml/m2, SD = 31.2) (Pearson = 0.19, p < 0.01) and end-systolic volume (56.1 ml/m2, SD = 18.8) (Pearson = 0.21, p < 0.01) and also with the pulmonary regurgitation fraction (36.5%, SD = 16, Pearson = 0.12, p < 0.01). No significant correlation was found between NT-proBNP and right ventricular ejection fraction (54.6%, SD = 10.6, Pearson = -0.07), left ventricular ejection fraction (59.9%, SD = 7.1, Pearson = -0.18) or any clinical parameters. The receiver operating curve analysis evidenced that a NT-proBNP cut-off value above 133.2 pg/ml predicted the presence of dilated right ventricular end-diastolic and end-systolic volumes over centile 95 (sensitivity 82 and 83% and specificity 93 and 79%, respectively). In conclusion, in patients with surgically corrected Tetralogy of Fallot, NT-proBNP levels correlate with right ventricular dilatation and the degree of pulmonary regurgitation. Ambulatory determination of NT-proBNP might be an easy, readily available and cost-effective alternative for MRI follow-up evaluation of these patients.

Realistic aortic phantom to study hemodynamics using MRI and cardiac catheterization in normal and aortic coarctation conditions.

PURPOSE: To design and characterize a magnetic resonance imaging (MRI)-compatible aortic phantom simulating normal and aortic coarctation (AoCo) conditions and to compare its hemodynamics with healthy volunteers and AoCo patients. MATERIALS AND METHODS: The phantom is composed of an MRI-compatible pump, control unit, aortic model, compliance chamber, nonreturn, and shutoff valves. The phantom without and with AoCo (13, 11, and 9 mm) was studied using 2D and 3D phase-contrast data and with a catheterization unit to measure pressures. The phantom data were compared with the mean values of 10 healthy volunteers and two AoCo patients. RESULTS: Hemodynamic parameters in the normal phantom and healthy volunteers were: heart rate: 68/61 bpm, cardiac output: 3.5/4.5 L/min, peak flow and peak velocity (Vpeak) in the ascending aorta (AAo): 270/357 mL/s (significantly, P < 0.05) and 97/107 cm/s (not significantly, P = 0.16), and pressure in the AAo of the normal phantom of 131/58 mmHg. Hemodynamic parameters in the 13, 11, and 9 mm coarctation phantoms and Patients 1 and 2 were: heart rate: 75/75/75/97/78 bpm, cardiac output: 3.3/3.0/2.9/4.0/5.8 L/min, peak flow in the AAo: 245/265/215/244/376 mL/s, Vpeak in the AAo: 96/95/81/196/187 cm/s, Vpeak after the AoCo: 123/187/282/247/165 cm/s, pressure in the AAo: 124/56, 127/51, 133/50, 120/51 and 87/39 mmHg, and a trans-coarctation systolic pressure gradient: 7, 10, 30, 20, and 11 mmHg. CONCLUSION: We propose and characterize a normal and an AoCo phantom, whose hemodynamics, including velocity, flow, and pressure data are within the range of healthy volunteers and patients with AoCo. J. Magn. Reson. Imaging 2016;44:683-697.

3D printed models for planning endovascular stenting in transverse aortic arch hypoplasia.

OBJECTIVES: To evaluate whether three-dimensional (3D) printed models can be used to improve interventional simulation and planning in patients with aortic arch hypoplasia. BACKGROUND: Stenting of a hypoplastic transverse arch is technically challenging, and complications such as stent migration and partial obstruction of the origin of the head and neck vessels are highly dependent on operator skills and expertise. METHODS: Using magnetic resonance imaging (MRI) data, a 3D model of a repaired aortic coarctation of a 15-year-old boy with hypoplastic aortic arch was printed. Simulation of the endovascular stenting of the hypoplastic arch was carried out under fluoroscopic guidance in the 3D printed model, and subsequently in the patient. A Bland-Altman analysis was used to evaluate the agreement between measurements of aortic diameter in the 3D printed model and the patient's MRI and X-ray angiography. RESULTS: The 3D printed model proved to be radio-opaque and allowed simulation of the stenting intervention. The assessment of optimal stent position, size, and length was found to be useful for the actual intervention in the patient. There was excellent agreement between the 3D printed model and both MRI and X-ray angiographic images (mean bias and standard deviation of 0.36 ± 0.45 mm). CONCLUSIONS: 3D printed models accurately replicate patients' anatomy and are helpful in planning endovascular stenting in transverse arch hypoplasia. This opens a door for potential simulation applications of 3D models in the field of catheterization and cardiovascular interventions.