Chantale Lapierre

Fetal hydronephrosis: is there hope for consensus?

This review article aims at summarizing the data regarding fetal and neonatal hydronephrosis, at correlating controversial data with the differences in the practice of obstetrical sonography from one country to another, and finally, at presenting our own criteria for fetal renal collecting system dilatation along with our own guidelines of postnatal investigation.

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.

Imaging of biliary disorders in children.

Biliary atresia and related disorders of the biliary tree, such as choledochal cyst, must be considered in the differential diagnosis of prolonged conjugated hyperbilirubinemia in infants and children. Pediatric biliary tract diseases include a variety of entities with a wide range of clinical presentations. Radiology plays an important role in the diagnosis and management of these pathologies. Unrecognized causes of biliary disease, like biliary atresia, can lead to liver transplantation during the first year of life. The aim of this article is to review the imaging of pediatric biliary disorders, including the implications of interventional radiology in some biliary diseases.

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.

Follow-Up Chest X-Ray in Patients with Kawasaki Disease: The Significance and Clinical Application of Coronary Artery Macro-Calcification

Kawasaki disease (KD) related coronary artery (CA) aneurysms may lead to significant and potentially insidious progressive stenosis. It is also well recognized that CA scarring leads to heavy calcification in KD. We intended to correlate the angiographic anomalies associated with coronary calcifications in KD and to evaluate the chronology and the detection rate of KD-related CA calcification on plain chest X-ray. Between 1992 and 2006, 65 CA angiograms were performed in 50 KD patients. Chest fluoroscopies and angiograms were retrospectively reviewed. When angiograms were abnormal, chest X-rays were reviewed by two radiologists blinded to the results of angiograms. CA lesions were identified in 18/50 (36%) patients, including isolated CA aneurysms in 10. All 8 patients who had CA aneurysms associated with stenosis and/or occlusion had CA calcification identifiable on chest X-ray. All significant stenotic lesions were concomitant with calcification. Plain chest X-ray, a simple inexpensive low dose mean, easily identifies KD patients at risk for serious CA stenosis when specific search for CA calcification is pursued. When detected, a closer tracking of coronary artery patency is warranted via other imaging techniques, usually expensive, invasive, requiring sedation in children or exposing to high radiation.

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.

The Role of Cardiac Magnetic Resonance in the Diagnosis of Anomalous Pulmonary Venous Return with Subsequent Amplatzer Device Treatment

We report the case of a young girl with a mixed total anomalous pulmonary venous return (cardiac and supracardiac) treated sequentially by partial neonatal surgery, and then catheterization at age 19 with installation of an Amplatzer device as a treatment of the remaining anomaly. We describe the usefulness of magnetic resonance imaging in both the diagnosis and follow-up of this anomaly.

Partial anomalous pulmonary venous return to the azygos vein: an unusual case.

A 13-year-old boy with a heart murmur was referred for cardiac evaluation. The patient had previously been in good health except for recurrent pulmonary infections. The electrocardiogram showed right atrial hypertrophy. Chest radiography (Fig. 1) showed normal heart size, increased pulmonary vascularity, and prominence of the azygos vein. Transthoracic echocardiography demonstrated normalsized cardiac cavities with good biventricular function and standard connection of left pulmonary veins into the left atrium (the right pulmonary veins were not visualized). Magnetic resonance imaging (MRI) showed upper and lower right pulmonary veins connected to an enlarged azygos vein, which drained into the superior vena cava (Fig. 2). Pulmonary venous return from the left lung was normal. The right cardiac cavities were enlarged. Calculated pulmonary-to-systemic flow (Qp/Qs) was 2.0. The patient was referred for surgical correction because of enlarged right ventricular volume and pressure overload. Pulmonary veins connection to the brachiocephalic veins, portal vein, azygos vein, and coronary sinus is rarely seen [1]. According to Toyoshima et al. [2], in an exhaustive review of the world literature, only 14 cases of partial anomalous pulmonary venous return (PAPVR) showing anomalous pulmonary venous return to the azygos vein, were reported, but none of them were associated with atrial septal defect, which is frequently the case in PAPVR (90%). Transthoracic echocardiography can demonstrate the normal connection and drainage of the pulmonary veins with the left atrium in children, but it is less sensitive in adolescents and adults. MRI and computed tomography have been shown to be accurate noninvasive methods for the evaluation of cardiac structures in congenital heart disease [3–5]. MRI has a higher sensitivity for the detection

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in a newborn

A 5-day-old child was referred to the department of cardiology for bradycardia without any other cardiac symptoms. ECG was normal. Cardiac echography demonstrated a reverse flow inside the interventricular branch of the left coronary artery suggesting ALCAPA. ECG-gated multidetector CT angiography, with oblique axial view (a) and 3-D volume-rendered images (b), confirmed the diagnosis of ALCAPA (DLP=99 mGy-cm) (Fig. 1). Reimplantation of the left coronary artery into the aorta was performed promptly and with success. ALCAPA is a rare congenital anomaly but is one of the most common causes of myocardial ischemia in children [1]. Usually, symptoms occur around 8 weeks of life. If untreated, most patients will die within the first year of life. Cardiac echography alone permits the diagnosis of ALCAPA. Nevertheless, ECG-gated multidetector CT angiography can be used instead of conventional angiography to clarify the anatomy or confirm an unclear diagnosis [2]. In neonates, early surgical correction allows improvement of ventricular function.

Infracardiac total anomalous pulmonary venous return (TAPVR)

A 1-day-old boy presented with tachypnea and cyanosis and required immediate intubation and inotropic support. Because the transthoracic echocardiography suggested infracardiac TAPVR connecting to the ductus venosus but failed to show the left upper pulmonary vein, we performed CTA, which allows accurate and noninvasive evaluation of all pulmonary veins [1]. CTA confirmed infracardiac TAPVR to the ductus venosus via a descending vein and revealed stenoses in the proximal part of the ductus venosus and in the distal aspect of the descending vein (Fig. 1). The infant was operated on without delay. In TAPVR the pulmonary veins drain in an infracardiac fashion in approximately 25% of cases [2]. The usual site is the portal vein. Among the multiple mechanisms of obstruction, increased resistance in the connecting venous structure is the most significant. Pulmonary venous connection to the ductus venosus might not be obstructive during the first days of life, but as the ductus venosus closes, obstruction develops. Obstructed TAPVR requires immediate surgical intervention.