Julie Déry

Segmental Approach to Imaging of Congenital Heart Disease

The segmental approach, which is widely used in the imaging work-up of congenital heart disease, consists of a three-step evaluation of the cardiac anatomy. In step 1, the visceroatrial situs is determined. Visceroatrial situs refers to the position of the atria in relation to the nearby anatomy (including the stomach, liver, spleen, and bronchi). Three different anatomic configurations may be observed: situs solitus (normal), situs inversus (inverted), or situs ambiguus (ambiguous). In step 2, the left- or rightward orientation of the ventricular loop is evaluated, and the positions of the ventricles are identified on the basis of their internal morphologic features. In step 3, the position of the great vessels is determined first, and any abnormalities are noted. Abnormalities in the origin of the great vessels, or conotruncal anomalies, are predominantly of three types: D-transposition (dextrotransposition), L-transposition (levotransposition), and D-malposition with double outlet right ventricle. Next, the relationships between the atria and ventricles and the ventricles and great vessels are determined at two levels: atrioventricular (concordant, discordant, ambiguous, double inlet, absence of right or left connection) and ventriculoarterial (concordant, discordant, double outlet). Last, a search is performed for any associated abnormalities of the cardiac chambers, septa, outflow tract, and great vessels. By executing these steps sequentially during image review, the radiologist can achieve a more accurate interpretation. Multiplanar reconstructions of cross-sectional image data obtained with computed tomography or magnetic resonance imaging are particularly useful for evaluating congenital heart disease.

Coronary Wall Structural Changes in Patients With Kawasaki Disease: New Insights From Optical Coherence Tomography (OCT).

Background Coronary artery aneurysms (CAA) are serious complications of Kawasaki disease (KD). Optical coherence tomography (OCT) is a high-resolution intracoronary imaging modality that characterizes coronary artery wall structure. The purpose of this work was to describe CAA wall sequelae after KD. Methods and Results KD patients scheduled for routine coronary angiography underwent OCT imaging between March 2013 and August 2014. Subjects’ clinical courses, echocardiography, and coronary angiography examinations were reviewed retrospectively. OCT was performed in 18 patients aged 12.4±5.5 years, 9.0±5.1 years following onset of KD. Of those, 14 patients (77.7%) had a history of CAA (7 with giant CAA and 7 with regressed CAA at time of OCT). Intracoronary nitroglycerin was given to all patients (88.4±45.5 μg/m2). Mean radiation dose was 10.9±5.2 mGy/kg. One patient suffered from a transitory uneventful vasospasm at the site of a regressed CAA; otherwise no major procedural complications occurred. The most frequent abnormality observed on OCT was intimal hyperplasia (15 patients, 83.3%) seen at both aneurysmal sites and angiographically normal segments amounting to 390.8±166.0 μm for affected segments compared to 61.7±17 μm for unaffected segments (P<0.001). Disappearance of the media, and presence of fibrosis, calcifications, macrophage accumulation, neovascularization, and white thrombi were seen in 72.2%, 77.8%, 27.8%, 44.4%, and 33.3% of patients. Conclusions In this study, OCT proved safe and insightful in the setting of KD, with the potential to add diagnostic value in the assessment of coronary abnormalities in KD. The depicted coronary structural changes correspond to histological findings previously described in KD.

Prenatal ultrasound screening of congenital heart disease in the general population: general concepts, guidelines, differential diagnoses.

Abstract Congenital heart diseases (CHDs) carry a high prevalence rate in the general population (0.8%–1%). Most fetal CHDs occur in patients without any risk factors. The prenatal recognition of CHD has major impacts on the pregnancy and its outcome. The aforementioned data justify prenatal ultrasound (US) screening of CHD in the general low-risk population. As demonstrated in the literature, the application of an extended basic US cardiac examination improves the detection of CHD, in particular the conotruncal anomalies. The stepwise method suggested for fetal heart US screening during the mid–second trimester sonogram is based on 4 routine axial views of heart and great vessels: (1) a transverse view of the superior abdomen, (2) a 4-chamber view, (3) a 3-vessel view, and (4) a transverse view of the aortic arch. This protocol can be obtained rapidly because these scans are easy to perform. Despite the fact that the sequential segmental approach universally used in the postnatal diagnosis of CHD is not specifically addressed here, the detected anomalies can be categorized according to these views, and a short differential diagnosis proposed. Abnormal cardiac and/or vascular landmarks shown on these key scans should lead to a referral in the fetal cardiac center for a more precise evaluation, as well as for counseling.

Importance of anatomical dominance in the evaluation of coronary dilatation in Kawasaki disease

Introduction In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease. METHODS We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance. RESULTS Of 28 patients (2.6 [0.2-10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3-6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation. CONCLUSION Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.

Long-Term Follow-Up of Large Atrial Septal Occluder (Amplatzer Device) With Cardiac MRI in a Pediatric Population

OBJECTIVE The purpose of this study is to evaluate the position and the progress of large Amplatzer septal occluder (ASO) devices relevant to adjacent cardiac structures in growing children using MRI. MATERIALS AND METHODS Institutional review board approval for this study was obtained. Twenty-five children who underwent large ASO implantation were evaluated using MRI. All subjects were initially imaged 7 years earlier using the same protocol. Spatial and dynamic relationships between the ASO and the adjacent cardiac structures were compared to assess the progress over the 7-year growth span with respect to protrusion, contact, and extrinsic deformity of the mitral valve, the left atrial roof, the aortic annulus and root, and the ostia of adjacent veins. RESULTS No long-term device-related complications were documented during follow-up. Impingement of the ASO into the opening of the right superior and inferior pulmonary veins and the superior and inferior vena cava resolved completely, compared with initial observations, in five of 16, three of three, five of 10, and three of nine patients, respectively, and significantly regressed in the remaining patients. The devices contact with the mitral valve and the left atrial roof and the aortic root deformity resolved in seven of 10, 11 of 20, and 10 of 18 cases, respectively. CONCLUSION The distance between the ASO and the surrounding structures increases with time in growing children who require large ASO devices, likely decreasing the risk of long-term complications.

Right pulmonary artery torsion following surgical reimplantation.

We report the case of a 3-month-old boy with an anomalous origin of right pulmonary artery (AORPA) from the ascending aorta who presented postoperatively with torsion of the right pulmonary artery demonstrated on 3-D volume-rendered CT angiogram. To our knowledge, CT images of this entity have not been reported. This case illustrates a rare surgical complication that can be easily imaged by CT.

CV-WS-20 Tetralogie de fallot : evaluation radiologique postoperatoire

Objectifs pedagogiques Revoir et illustrer les principaux types de chirurgie correctrice et leurs complications. Demontrer le role des differentes modalites radiologiques. Messages a retenir L’echographie cardiaque transthoracique demeure la modalite diagnostique de premier choix dans le suivi des cardiopathies congenitales. La radiographie pulmonaire, le CT et l’IRM sont des examens complementaires incontournables dans l’evaluation postoperatoire des TOF, particulierement lorsque la fenetre acoustique est limitee.

Visibilite des coronaires a la TDM sans synchronisation cardiaque chez les nourrissons porteurs d’une cardiopathie

Objectifs Evaluer la visibilite des trajets proximaux des coronaires a la TDM sans synchronisation cardiaque. Comparer et correler les donnees obtenues aux donnees de l’angiographie conventionnelle et/ou de la chirurgie. Materiels et methodes Etude retrospective (2007-2008) de 22 patients âges Resultats Les branches coronariennes identifiees ont ete de 3 chez 10 pts, 2 chez 5 pts (2 de ces 5 pts avaient une hypoplasie ventriculaire), 1 chez 3 pts, aucune chez 4 patients. Les donnees chirurgicales et/ou angiographiques etaient disponibles pour fins de correlation dans 12 cas et concordaient a 100 % avec celles de la TDM. Conclusion Malgre qu’il ne s’agisse pas de son indication primaire, la TDM sans synchronisation cardiaque permet de visualiser de facon appropriee les coronaires proximalement. Le choix de la technique utilisee demeure cependant crucial.

RP-WP-6 Approche segmentaire des cardiopathies congenitales

Objectifs Acquerir les notions essentielles de l’embryologie cardiaque. Connaitre les etapes sequentielles de l’approche segmentaire des cardiopathies congenitales (CC). Savoir appliquer l’approche segmentaire lors de l’analyse de cas cliniques. Points cles Le CT et l’IRM sont des examens indispensables pour l’evaluation des CC. L’utilisation de l’approche segmentaire permet au radiopediatre general de faire une analyse schematique, ordonnee et complete de ces cas complexes.