Hopewell Ntsinjana

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)

BackgroundAnomalous 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.Methods6 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).ResultsThe 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).ConclusionsCMR 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.

A statistical shape modelling framework to extract 3D shape biomarkers from medical imaging data: assessing arch morphology of repaired coarctation of the aorta

BackgroundMedical image analysis in clinical practice is commonly carried out on 2D image data, without fully exploiting the detailed 3D anatomical information that is provided by modern non-invasive medical imaging techniques. In this paper, a statistical shape analysis method is presented, which enables the extraction of 3D anatomical shape features from cardiovascular magnetic resonance (CMR) image data, with no need for manual landmarking. The method was applied to repaired aortic coarctation arches that present complex shapes, with the aim of capturing shape features as biomarkers of potential functional relevance. The method is presented from the user-perspective and is evaluated by comparing results with traditional morphometric measurements.MethodsSteps required to set up the statistical shape modelling analyses, from pre-processing of the CMR images to parameter setting and strategies to account for size differences and outliers, are described in detail. The anatomical mean shape of 20 aortic arches post-aortic coarctation repair (CoA) was computed based on surface models reconstructed from CMR data. By analysing transformations that deform the mean shape towards each of the individual patient’s anatomy, shape patterns related to differences in body surface area (BSA) and ejection fraction (EF) were extracted. The resulting shape vectors, describing shape features in 3D, were compared with traditionally measured 2D and 3D morphometric parameters.ResultsThe computed 3D mean shape was close to population mean values of geometric shape descriptors and visually integrated characteristic shape features associated with our population of CoA shapes. After removing size effects due to differences in body surface area (BSA) between patients, distinct 3D shape features of the aortic arch correlated significantly with EF (r = 0.521, p = .022) and were well in agreement with trends as shown by traditional shape descriptors.ConclusionsThe suggested method has the potential to discover previously unknown 3D shape biomarkers from medical imaging data. Thus, it could contribute to improving diagnosis and risk stratification in complex cardiac disease.

Using 4D Cardiovascular Magnetic Resonance Imaging to Validate Computational Fluid Dynamics: A Case Study

Computational fluid dynamics (CFD) can have a complementary predictive role alongside the exquisite visualization capabilities of 4D cardiovascular magnetic resonance (CMR) imaging. In order to exploit these capabilities (e.g., for decision-making), it is necessary to validate computational models against real world data. In this study, we sought to acquire 4D CMR flow data in a controllable, experimental setup and use these data to validate a corresponding computational model. We applied this paradigm to a case of congenital heart disease, namely, transposition of the great arteries (TGA) repaired with arterial switch operation. For this purpose, a mock circulatory loop compatible with the CMR environment was constructed and two detailed aortic 3D models (i.e., one TGA case and one normal aortic anatomy) were tested under realistic hemodynamic conditions, acquiring 4D CMR flow. The same 3D domains were used for multi-scale CFD simulations, whereby the remainder of the mock circulatory system was appropriately summarized with a lumped parameter network. Boundary conditions of the simulations mirrored those measured in vitro. Results showed a very good quantitative agreement between experimental and computational models in terms of pressure (overall maximum % error = 4.4% aortic pressure in the control anatomy) and flow distribution data (overall maximum % error = 3.6% at the subclavian artery outlet of the TGA model). Very good qualitative agreement could also be appreciated in terms of streamlines, throughout the cardiac cycle. Additionally, velocity vectors in the ascending aorta revealed less symmetrical flow in the TGA model, which also exhibited higher wall shear stress in the anterior ascending aorta.

Validation of an animal model of right ventricular dysfunction and right bundle branch block to create close physiology to postoperative tetralogy of Fallot

BACKGROUND In the past 5 years a few number of studies and case reports have come out focusing on biventricular (BiV) stimulation for treatment of congenital heart disease related ventricular dysfunction. The few available studies include a diverse group of pathophysiological entities ranging from a previously repaired tetralogy of Fallot (TOF) to a functional single ventricle anatomy. Patients status is too heterogeneous to build important prospective study. To well understand the implication of prolonged electromechanical dyssynchrony we performed a chronic animal model that mimics essential parameters of postoperative TOF. METHODS Significant pulmonary regurgitation, mild stenosis, as well as right ventricular outflow tract (RVOT) scars were induced in 15 piglets to mimic repaired TOF. 4 months after hemodynamics and dyssynchrony parameters were compared with a control group and with a population of symptomatic adult with repaired TOF. RESULTS Comparing the animal model with the animal control group on echocardiography, RV dilatation, RV and LV dysfunction, broad QRS complex and dyssynchrony were observed on the animal model piglets. Moreover, epicardial electrical mapping showed activation consistent with a right bundle branch block. The animal models displayed the same pathophysiological parameters as the post TOF repair patients in terms of QRS duration, pulmonary regurgitation biventricular dysfunction and dyssynchrony. CONCLUSION This chronic swine model mimics electromechanical ventricular activation delay, RV and LV dysfunction, as in adult population of repair TOF. It does appear to be a very useful and interesting model to study the implication of dyssynchrony and the interest of resynchronization therapy in TOF failing ventricle.

A Non-parametric Statistical Shape Model for Assessment of the Surgically Repaired Aortic Arch in Coarctation of the Aorta: How Normal is Abnormal?

Coarctation of the Aorta CoA is a cardiac defect that requires surgical intervention aiming to restore an unobstructed aortic arch shape. Many patients suffer from complications post-repair, which are commonly associated with arch shape abnormalities. Determining the degree of shape abnormality could improve risk stratification in recommended screening procedures. Yet, traditional morphometry struggles to capture the highly complex arch geometries. Therefore, we use a non-parametric Statistical Shape Model based on mathematical currents to fully account for 3D global and regional shape features. By computing a template aorta of a population of healthy subjects and analysing its transformations towards CoA arch shape models using Partial Least Squares regression techniques, we derived a shape vector as a measure of subject-specific shape abnormality. Results were compared to a shape ranking by clinical experts. Our study suggests Statistical Shape Modelling to be a promising diagnostic tool for improved screening of complex cardiac defects.

Ventriculoarterial coupling in palliated hypoplastic left heart syndrome: Noninvasive assessment of the effects of surgical arch reconstruction and shunt type.

OBJECTIVE To assess the coupling efficiency in hypoplastic left heart syndrome, considering the effect of surgical arch reconstruction and the shunt type received during the Norwood procedure. METHODS Ventriculoarterial coupling was assessed before Fontan completion in 32 patients with hypoplastic left heart syndrome (19 modified Blalock-Taussig and 13 Sano shunts at stage 1). Cardiovascular magnetic resonance data were analyzed, deriving functional parameters and 3-dimensional volumes. Dimensional indexes were computed from 3-dimensional data sets as the area ratio of the isthmus to the descending aorta (Risthmus) and the isthmus to surgically enlarged transverse arch (Rarch). Wave intensity was calculated from cardiac magnetic resonance, using the peaks of the forward compression and expansion waves in early and late systole as surrogate indicators of ventriculoarterial coupling. RESULTS Aortic distensibility (3.6±2.7×10(-3) 1/mm Hg) was not associated with the time elapsed from stage 1 palliation (P=.94), suggesting an early loss of elasticity that did not progress thereafter. Risthmus was 1.0±0.4, and Rarch was 0.3±0.1, indicating the dilated reconstructed arch was the main anatomic feature. The forward compression wave correlated significantly with Rarch (R2=0.23, P=.006) but not with Risthmus (R2<0.01, P=.63). Patients with a reduced ejection fraction exhibited a larger ventricular mass (R2=0.28, P=.003). The Sano shunt patients had a lower ejection fraction (51%±6% vs 57%±6%, P=.02); however, neither the forward compression nor expansion wave varied significantly between shunt type or the other functional parameters. CONCLUSIONS Ventriculoarterial coupling in operated hypoplastic left heart syndrome was affected by aortic arch size mismatch but not by the type of shunt placed at the Norwood operation.

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.

Detecting Clinically Meaningful Shape Clusters in Medical Image Data: Metrics Analysis for Hierarchical Clustering Applied to Healthy and Pathological Aortic Arches

Objective: Todays growing medical image databases call for novel processing tools to structure the bulk of data and extract clinically relevant information. Unsupervised hierarchical clustering may reveal clusters within anatomical shape data of patient populations as required for modern precision medicine strategies. Few studies have applied hierarchical clustering techniques to three-dimensional patient shape data and results depend heavily on the chosen clustering distance metrics and linkage functions. In this study, we sought to assess clustering classification performance of various distance/linkage combinations and of different types of input data to obtain clinically meaningful shape clusters. Methods: We present a processing pipeline combining automatic segmentation, statistical shape modeling, and agglomerative hierarchical clustering to automatically subdivide a set of 60 aortic arch anatomical models into healthy controls, two groups affected by congenital heart disease, and their respective subgroups as defined by clinical diagnosis. Results were compared with traditional morphometrics and principal component analysis of shape features. Results: Our pipeline achieved automatic division of input shape data according to primary clinical diagnosis with high F-score (0.902 ± 0.042) and Matthews correlation coefficient (0.851 ± 0.064) using the correlation/weighted distance/linkage combination. Meaningful subgroups within the three patient groups were obtained and benchmark scores for automatic segmentation and classification performance are reported. Conclusion: Clustering results vary depending on the distance/linkage combination used to divide the data. Yet, clinically relevant shape clusters and subgroups could be found with high specificity and low misclassification rates. Significance: Detecting disease-specific clusters within medical image data could improve image-based risk assessment, treatment planning, and medical device development in complex disease.

Residual Shunt After Ductus Arteriosus Occluder Implantation Complicated by Late Endocarditis

We report the case of a 20-year-old woman who had percutaneous closure of a patent ductus arteriosus (PDA) with a 17-mm Rashkind occluder when she was 4 years of age. A small residual shunt was noted on color-flow Doppler echocardiography immediately after the procedure and at 6-month follow-up. Because of the lack of hemodynamic complications, no additional intervention was performed. Sixteen years later, she presented with recurrent bilateral bronchopneumonia that required antibiotic treatment. Chest radiography during the third episode showed a right bronchopneumonia (Figure 1). At that time, new treatment with amoxicillin and clavulanic acid was given for 10 days, but fever persisted despite this course of antibiotics. Figure 1. Chest radiograph showed right upper lobe consolidation suggestive of infective bronchopneumonia (arrow). The Rashkind occluder device is visible in the duct area (dotted arrow). Results of the physical examination showed blood pressure of …