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.

Computed tomography in paediatric heart disease.

Cardiovascular CT (CCT) is an important imaging modality in congenital and acquired paediatric heart disease. Technological advances have resulted in marked improvements in spatial and temporal resolution of CCT with a concomitant increase in speed of data acquisition and a decrease in radiation dose. This has elevated CCT from being sparingly used to an essential diagnostic tool in the daily multimodality imaging practice alongside echocardiography, cardiovascular MR and invasive angiography. The application of CCT in paediatric congenital and acquired heart disease can be both technically and diagnostically challenging. This review highlights important considerations for current state of the art CCT across the spectrum of heart disease encountered in children.

Utility of adenosine stress perfusion CMR to assess paediatric coronary artery disease.

AIMS: Cardiovascular magnetic resonance (CMR), using adenosine stress perfusion and late-gadolinium enhancement (LGE), is becoming the 'gold standard' non-invasive imaging modality in the assessment of adults with coronary artery disease (CAD). However, despite its proved feasibility in paediatric patients, clinical utility has not been demonstrated. Therefore, this study aims to establish the role of adenosine stress perfusion CMR as a screening test in paediatric patients with acquired or congenital CAD. METHODS AND RESULTS: A total of 58 paediatric patients underwent 61 consecutive clinically indicated coronary artery assessments for diagnostic and clinical decision-making purposes. The diagnosis was based on X-ray or computed tomography coronary angiography for anatomy, adenosine stress CMR imaging for myocardial perfusion and LGE for tissue characterization. Two studies were aborted because of unwanted side effects of adenosine stress, thus 59 studies were completed in 56 patients [median age 14.1 years (interquartile range 10.9-16.2)]. When compared with coronary anatomical imaging, adenosine stress perfusion CMR performed as follows: sensitivity 100% (95% confidence interval, CI: 71.6-100%), specificity 98% (95% CI: 86.7-99.9%), positive predictive value (PPV) 92.9% (95% CI: 64.2-99.6%), and negative predictive value 100% (95% CI: 89.9-100%). CONCLUSION: In paediatric CAD, adenosine stress perfusion CMR imaging is adequate as an initial, non-invasive screening test for the identification of significant coronary artery lesions, with anatomical imaging used to confirm the extent of the culprit lesion.

Echocardiographic arterial measurements in complex congenital diseases before bidirectional Glenn: comparison with cardiovascular magnetic resonance imaging.

AIMS: This study sought to investigate diagnostic accuracy of echocardiographic measures of great vessels in patients before bidirectional cavopulmonary connection (BCPC) compared with cardiovascular magnetic resonance (CMR). METHODS AND RESULTS: Seventy-two patients (61% after Norwood operation) undergoing BCPC between 2007 and 2012 were assessed pre-operatively using echocardiography and CMR. Bland-Altman analysis and correlation coefficients were used for comparison of echocardiography and CMR measurements. Sensitivity, specificity, and positive and negative predictive values were calculated to assess the ability of echocardiography to detect vessel stenosis. Twenty-four percent of all vessel measurements could not be made by echocardiography due to poor image quality. Acquisition of unsatisfactory images was higher in non-sedated patients. Although there was a reasonable correlation (0.68-0.90) and low bias (-0.8 to 0.5), there were wide limits of agreement between echocardiography and CMR demonstrating poor agreement. Sensitivity and specificity for pulmonary branches were moderate [sensitivity for right pulmonary artery (RPA) 67%, left pulmonary artery (LPA) 54%, specificity for RPA 65%, LPA 72%] with low levels of accuracy (RPA and LPA 42%). Sensitivity, specificity, and accuracy were better for aorta (82, 86, and 63%, respectively). CONCLUSION: This study demonstrates modest agreement between echocardiographic and CMR measures of vessel diameter and stenosis detection. Approximately a quarter of all vessel segments could not be measured using echocardiography due to poor image quality, which was significantly lower in non-sedated patients. These findings show that echocardiography cannot substitute CMR for reliable identification of great vessel stenoses in complex patients prior to the BCPC, particularly those with Blalock-Taussig shunts.

Free breathing contrast-enhanced time-resolved magnetic resonance angiography in pediatric and adult congenital heart disease.

BACKGROUND: Contrast enhanced magnetic resonance angiography (MRA) is generally performed during a long breath-hold (BH), limiting its utility in infants and small children. This study proposes a free-breathing (FB) time resolved MRA (TRA) technique for use in pediatric and adult congenital heart disease (CHD). METHODS: A TRA sequence was developed by combining spiral trajectories with sensitivity encoding (SENSE, x4 kx-ky and x2 kz) and partial Fourier (75% in kz). As no temporal data sharing is used, an independent 3D data set was acquired every ~1.3s, with acceptable spatial resolution (~2.3x2.3x2.3 mm). The technique was tested during FB over 50 consecutive volumes. Conventional BH-MRA and FB-TRA data was acquired in 45 adults and children with CHD. We calculated quantitative image quality for both sequences. Diagnostic accuracy was assessed in all patients from both sequences. Additionally, vessel measurements were made at the sinotubular junction (N = 43), proximal descending aorta (N = 43), descending aorta at the level of the diaphragm (N = 43), main pulmonary artery (N = 35), left pulmonary artery (N = 35) and the right pulmonary artery (N = 35). Intra and inter observer variability was assessed in a subset of 10 patients. RESULTS: BH-MRA had significantly higher homogeneity in non-contrast enhancing tissue (coefficient of variance, P <0.0001), signal-to-noise ratio (P <0.0001), contrast-to-noise ratio (P <0.0001) and relative contrast (P = 0.02) compared to the FB-TRA images. However, homogeneity in the vessels was similar in both techniques (P = 0.52) and edge sharpness was significantly (P <0.0001) higher in FB-TRA compared to BH-MRA. BH-MRA provided overall diagnostic accuracy of 82%, and FB-TRA of 87%, with no statistical difference between the two sequences (P = 0.77). Vessel diameter measurements showed excellent agreement between the two techniques (r = 0.98, P <0.05), with no bias (0.0 mm, P = 0.71), and clinically acceptable limits of agreement (-2.7 to +2.8 mm). Inter and intra observer reproducibility showed good agreement of vessel diameters (r>0.988, P<0.0001), with negligible biases (between -0.2 and +0.1mm) and small limits of agreement (between -2.4 and +2.5mm). CONCLUSIONS: We have described a FB-TRA technique that is shown to enable accurate diagnosis and vessel measures compared to conventional BH-MRA. This simplifies the MRA technique and will enable angiography to be performed in children and adults whom find breath-holding difficult.

Assessment of cardiac time intervals using high temporal resolution real-time spiral phase contrast with UNFOLDed-SENSE.

PURPOSE: To develop a real-time phase contrast MR sequence with high enough temporal resolution to assess cardiac time intervals. METHODS: The sequence utilized spiral trajectories with an acquisition strategy that allowed a combination of temporal encoding (Unaliasing by fourier-encoding the overlaps using the temporal dimension; UNFOLD) and parallel imaging (Sensitivity encoding; SENSE) to be used (UNFOLDed-SENSE). An in silico experiment was performed to determine the optimum UNFOLD filter. In vitro experiments were carried out to validate the accuracy of time intervals calculation and peak mean velocity quantification. In addition, 15 healthy volunteers were imaged with the new sequence, and cardiac time intervals were compared to reference standard Doppler echocardiography measures. For comparison, in silico, in vitro, and in vivo experiments were also carried out using sliding window reconstructions. RESULTS: The in vitro experiments demonstrated good agreement between real-time spiral UNFOLDed-SENSE phase contrast MR and the reference standard measurements of velocity and time intervals. The protocol was successfully performed in all volunteers. Subsequent measurement of time intervals produced values in keeping with literature values and good agreement with the gold standard echocardiography. Importantly, the proposed UNFOLDed-SENSE sequence outperformed the sliding window reconstructions. CONCLUSION: Cardiac time intervals can be successfully assessed with UNFOLDed-SENSE real-time spiral phase contrast. Real-time MR assessment of cardiac time intervals may be beneficial in assessment of patients with cardiac conditions such as diastolic dysfunction.

Ventriculovascular interactions late after atrial and arterial repair of transposition of the great arteries.

OBJECTIVE: The aim of this study was to compare atrial switch and arterial switch operations for the repair of transposition of the great arteries (TGA), assessing cardiac function and ventriculoarterial (VA) coupling based on cardiac magnetic resonance (CMR) data. Using CMR-derived wave intensity analysis, this study provides a noninvasive comparison of the 2 systemic ventricles and evaluates the subclinical hemodynamic burden of these operations. METHODS: Fifty-four patients (18 controls, 18 atrial switches, 18 arterial switches) were studied. Dimensional and functional data, including the ejection fraction (EF) and end-diastolic volume, were derived from CMR, as well as aortic distensibility. Wave intensity was computed from CMR according to a formulation based on changes in area and velocity, and the peaks of the 2 major systolic waves (forward compression wave [FCW], and forward expansion wave [FEW]) were measured as indicators of ventricular function. RESULTS: Both switches exhibited significantly increased end-diastolic volume and enlarged aortic root areas, and atrial switches were also characterized by significantly lower EF. Patients with TGA presented stiffer ascending aortas, particularly those repaired with an arterial switch. Both FCW and FEW were significantly lower in both TGA cohorts than the controls, suggesting compromised VA coupling, likely a result of increased impedance caused by the stiffer ascending aorta. A significant difference between switch type was noticed in terms of the FEW peak, which was lower in the atrial switch group. CONCLUSIONS: These data highlight reduced aortic distensibility and abnormal VA coupling in repaired TGA. An intraoperational difference was noted in terms of EF and peak FEW, likely reflecting the different nature of the subaortic ventricle.

3D morphometric analysis of the arterial switch operation using in vivo MRI data.

The arterial switch operation (ASO) is widely used nowadays as the surgical strategy of choice to repair transposition of the great arteries (TGA). Residual morphological and geometrical abnormalities of the aorta, pulmonary arteries and coronary arteries, however, have not been fully studied in a three-dimensional (3D) domain. These morphometric complications might have implications on long-term outcomes of ASO patients, hence the need to explore them in detail and study them with reference to healthy controls of comparable age and body surface area. These anatomical characteristics were examined using 3D patient-specific anatomical models reconstructed from cardiovascular magnetic resonance (CMR) images of 20 ASO patients (mean age 14.4 ± 2.4 years, 16 males and 4 females) compared with healthy controls (mean age 15.2 ± 2.0 years, 17 males and 3 females). It was found that the aorta, pulmonary arteries and re-implanted coronary arteries of ASO patients were significantly different morphologically and geometrically to those of healthy controls. In particular, the aortic root was dilated, with abnormal 3D angulation and additional acute angulation of the curvature of the aortic arch in the ASO group compared with controls. This could theoretically impinge on aortic flow profiles and physiological stresses, which can act as a primer for the development of early atherosclerotic disease in the ASO population.

Update of the European Association of Cardiovascular Imaging (EACVI) Core Syllabus for the European Cardiovascular Magnetic Resonance Certification Exam.

An updated version of the European Association of Cardiovascular Imaging (EACVI) Core Syllabus for the European Cardiovascular Magnetic Resonance (CMR) Certification Exam is now available online. The syllabus lists key elements of knowledge in CMR. It represents a framework for the development of training curricula and provides expected knowledge-based learning outcomes to the CMR trainees, in particular those intending to demonstrate CMR knowledge in the European CMR exam, a core requirement in the CMR certification process.

Aortic arch shape is not associated with hypertensive response to exercise in patients with repaired congenital heart diseases.

BACKGROUND: Aortic arch geometry is linked to abnormal blood pressure (BP) response to maximum exercise. This study aims to quantitatively assess whether aortic arch geometry plays a role in blood pressure (BP) response to exercise. METHODS: 60 age- and BSA-matched subjects--20 post-aortic coarctation (CoA) repair, 20 transposition of great arteries post arterial switch operation (ASO) and 20 healthy controls--had a three-dimensional (3D), whole heart magnetic resonance angiography (MRA) at 1.5 Tesla, 3D geometric reconstructions created from the MRA. All subjects underwent cardiopulmonary exercise test on the same day as MRA using an ergometer cycle with manual BP measurements. Geometric analysis and their correlation with BP at peak exercise were assessed. RESULTS: Arch curvature was similarly acute in both the post-CoA and ASO cases [0.05 ± 0.01 vs. 0.05 ± 0.01 (1/mm/m²); p = 1.0] and significantly different to that of normal healthy controls [0.05 ± 0.01 vs. 0.03 ± 0.01 (1/mm/m²), p < 0.001]. Indexed transverse arch cross sectional area were significantly abnormal in the post-CoA cases compared to the ASO cases (117.8 ± 47.7 vs. 221.3 ± 44.6; p < 0.001) and controls (117.8 ± 47.7 vs. 157.5 ± 27.2 mm²; p = 0.003). BP response to peak exercise did not correlate with arch curvature (r = 0.203, p = 0.120), but showed inverse correlation with indexed minimum cross sectional area of transverse arch and isthmus (r = -0.364, p = 0.004), and ratios of minimum arch area/ descending diameter (r = -0.491, p < 0.001). CONCLUSION: Transverse arch and isthmus hypoplasia, rather than acute arch angulation plays a role in the pathophysiology of BP response to peak exercise following CoA repair.

Obstructive left heart disease in neonates with a "borderline" left ventricle: diagnostic challenges to choosing the best outcome.

In most newborns with left heart obstruction, the choice between a single-ventricle or biventricular management pathway is clear. However, in some neonates with a "borderline" left ventricle, this decision is difficult. Existing criteria do not reliably identify neonates who will have a good long-term outlook after biventricular repair (BVR). The objective of this study was prospective assessment of the outcome after BVR for newborns in whom the left ventricle (LV) was considered "borderline" by an expert group. This study was a prospective follow-up evaluation of neonates with obstructive left heart disease related to a "borderline" LV who underwent biventricular management between January 2005 and April 2011. Of 154 neonates who required intervention for left heart obstruction, 13 (7.8 %) met the echocardiographic (echo) inclusion criteria. At the first and last echo, the z-scores were respectively -1.76 ± 1.37 and -0.66 ± 1.47 (p = 0.013) for the mitral valve, -1.02 ± 1.57 and -0.23 ± 1.78 (p = 0.056) for the aortic valve, and 13.77 ± 5.8 and 20.85 ± 8.9 ml/m(2) (p = 0.006) for the LV end-diastolic volume. At this writing, all 12 survivors are clinically well. However, LV diastolic dysfunction and pulmonary artery hypertension was present in 5 (36 %) of 12 patients. Endocardial fibroelastosis (EFE) was detected in five patients at the last follow-up echo, but only in two patients preoperatively. Cardiac magnetic resonance imaging did not confirm EFE in any of assessed patients. The study authors could not reliably predict the outcome after BVR for neonates with left heart obstruction and a "borderline" LV. The presence of EFE with consequent diastolic dysfunction is more important than LV volume in determining the outcome. Prospective identification of EFE remains challenging.

Trends in pediatric cardiovascular magnetic resonance imaging.

Cardiac magnetic resonance (CMR) imaging has significantly evolved over the last decade, becoming an integral part of the contemporary assessment of both congenital and acquired pediatric heart disease. Recent trends show that there is a growing interest in clinical applications and research in this field. An attempt to discuss the evolving technologies, techniques, and applications of CMR in pediatrics is not complete without understanding the current strengths of the modality. CMR complements readily available echocardiography, in many cases information from CMR can remove the need for invasive angiographic catheterization, and in other cases can be used to augment cardiac catheterisation.

Systemic to pulmonary collateral blood flow influences early outcomes following the total cavopulmonary connection.

BACKGROUND: Systemic to pulmonary collaterals (SPCs) represent an additional and unpredictable source of pulmonary blood flow in patients with single ventricle physiology following bidirectional superior cavopulmonary connection (BCPC). Understanding their influence on patient outcomes has been hampered by uncertainty about the optimal method of quantifying SPC flow. OBJECTIVE: To quantify SPC flow by cardiac magnetic resonance (CMR) prior to total cavopulmonary connection (TCPC) in order to identify preoperative risk factors and determine influence on postoperative outcomes. DESIGN: Single centre prospective cohort study. SETTING: Tertiary referral centre. PATIENTS: 65 patients with single ventricle physiology undergoing CMR for preoperative assessment of TCPC completion underwent quantification of SPC flow. Clinical outcomes of 41 patients in whom TCPC was completed were obtained. MAIN OUTCOME MEASURES: Early post-TCPC clinical outcomes associated with SPC flow were assessed, including postoperative chest drainage volume, postoperative chest drainage duration and length of intensive care and hospital stays. Additionally preoperative covariates associated with SPC flow were assessed including age at BCPC and CMR, SpO(2) at BCPC and CMR, ventricle type, pulmonary artery (PA) cross-sectional area and PA pulsatility. Different methods of CMR SPC flow quantification were compared. RESULTS: Higher SPC flow was associated with increased postoperative chest drain volume (r=0.51, p=0.001), chest drain duration (r=0.43, p=0.005), and intensive care unit (r=0.32, p=0.04) and log-transformed hospital stays (r=0.31, p=0.048). The effect of SPC flow on outcome was independent of fenestration, ventricle type and function. Preoperative covariates associated with SPC flow included age at BCPC (ß=-0.34, p=0.008), SpO(2) at time of CMR (ß=0.34, p=0.004) and branch PA cross-sectional area (ß=-0.26, p=0.036), model R(2)=0.34. Moreover, patients with pulsatile pulmonary blood flow had lower SPC flow than those without (0.8 vs 1.3 l/min/m(2) p=0.012). SPC flow calculated by the difference between pulmonary venous return and pulmonary artery flow (l/min/m(2)) showed greatest association with preoperative covariates and strongest correlation with postoperative outcomes compared with other methods of quantification. CONCLUSIONS: CMR can provide an effective measurement of SPC flow prior to TCPC. Young age at BCPC, high preoperative oxygen saturation and smaller PAs are associated with increased SPC flow, which may promote increased postoperative pleural drainage and lengthen recovery.

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.

MRI may be sufficient for noninvasive assessment of great vessel stents: an in vitro comparison of MRI, CT, and conventional angiography.

OBJECTIVE: The purpose of this study was to compare the diagnostic performance of MRI and CT assessment of great vessel stents in an in vitro model. MATERIALS AND METHODS: Three contemporary great vessel stent materials (nitinol, platinum-iridium, and stainless steel) were assessed with three luminal conditions: no stenosis, internal stenosis, and external stenosis. Stents of the same material were implanted into an aorta model that was attached to an animal bypass pump with pulsatile flow. Each stent was imaged with conventional angiography as reference standard, 10 different MRI sequences, and CT. The sensitivity and specificity for the identification of stent stenosis was determined and stent lumen measurements compared. RESULTS: Of the investigated MRI sequences, three had the highest overall sensitivity and specificity for the identification of stent stenosis in all studied materials: through-plane gradientrecalled echo (GRE) with 75° flip angle (100% and 95%, respectively), in- and through-plane steady-state free precession (SSFP) (99% and 90%) and MR angiography (MRA) with 75° flip angle (93% and 85%). Comparable sensitivity and specificity were achieved with CT (98% and 93%). GRE, SSFP, and MRA sequences tended to underestimate stent lumen diameter in externally nonstenosed stents and overestimate diameter in internally stenosed stents (p < 0.05). CT slightly underestimated external stenoses in all stent types (p < 0.05). CONCLUSION: Defined MRI sequences are feasible to assess nitinol, platinum-iridium, and stainless steel great vessel stents with diagnostic performance comparable with CT.

Takayasu's disease: a review.

Takayasu's disease is a rare chronic vasculitis of unknown aetiology. Initial symptoms and signs are non-specific, and a high index of suspicion is needed to make the correct diagnosis. The disease is associated with a high incidence of morbidity, and a significant risk of premature death. Serological tests have proved unreliable in distinguishing active from quiescent disease, with non-invasive imaging currently offering the best option for early diagnosis, and monitoring the response to treatment. In this review, we detail the epidemiology, pathophysiology, clinical features, imaging characteristics, and currently available treatments.