Ronald Guérin

Surgical Treatment of Partial Anomalous Pulmonary Venous Drainage A Long-Term Follow-up Study

Fourteen children who had been operated on for partial anomalous pulmonary venous return from the right lung to the vena cava or right atrium have been submitted to a follow-up study 1 to 9 years after surgery. This included clinical, hemodynamic, and angiographic data. All patients were doing well and had normal exercise tolerance at the time of follow-up examination. Electrocardiograms and chest roentgenograms had returned to normal in 10 and were improved in three. Hemodynamic and angiographic data demonstrated perfect correction in eight. Of the remaining six, three had an obstructed superior vena cava (SVC) with collateral pathways to a persistent left superior vena cava in two and to the inferior vena cava in one. Three had significant pressure gradients between the SVC and the right atrium, two with a small right-to-left shunt from the SVC to the left atrium, and one with an additional small left-to-right shunt. None had obstruction to the pulmonary venous return. These complications occurred exclusively in cases in which at least one anomalous vein drained high into the superior vena cava. Enlargement of the SVC with a patch of pericardium gave good results in three patients with this type of anomalous drainage but did not prevent obstruction or severe narrowing of the SVC in two other cases. It is concluded that the present corrective technics are not adequate for cases in which an anomalous vein drains high in the SVC, especially in the presence of a left superior vena cava.

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.

Subxiphoid two-dimensional echocardiographic diagnosis of double-chambered right ventricle.

The echocardiographic features of 14 patients with double-chambered right ventricle are presented. Diagnosis was confirmed in 13 patients at cardiac catheterization and in one at necropsy. The ultrasonic diagnosis was based on the visualization in subxiphoid short-axis view of an anomalous muscle bundle at the lower margin of the right ventricular infundibulum; this structure was present in diastole and systole and the distal portion of infundibulum was wide and free of obstruction. Associated lesions were very frequent (especially ventricular septal defects, present in 13 patients). Subxiphoid two-dimensional echocardiography is an easy, reliable, noninvasive means of assessing this disease in infants and children.

Echocardiographic manifestations of persistence of the right sinus venosus valve

The echocardiographic features of a neonate born with a persistent right sinus venosus valve are presented. Because surgical correction of this serious anomaly may be possible, the diagnosis should be made soon after birth. An echocardiogram suggesting a right atrial myxoma, an unlikely finding in a neonate, should be an important clue to the diagnosis of this anomaly.

Echocardiographic and angiographic findings in superior-inferior cardiac ventricles.

The anatomy of superior-inferior ventricles was studied in 17 patients, aged 1 day to 22 years, using echocardiography and angiography. In all patients, the right ventricle was located superiorly and the left ventricle inferiorly. The right ventricular sinus was underdeveloped in 14 of the 17 patients. Conversely, the right ventricular outflow tract was normally developed in all 17 patients. The visceroatrial situs was solitus in all patients, and it was associated with a concordant D-loop in 9 patients and with a discordant L-loop in 8. There was a high incidence of associated transposition of the great arteries (9 patients) or double-outlet right ventricle (5). Segmental combination was unpredictable, D-loop being associated with L-position of the great arteries in 4 of 8 patients and L-loop being associated with D-position of the great arteries in 4 of 9. There were only 3 concordant ventriculoarterial connections. Frequently associated anomalies included ventricular septal defect (17 patients), atrioventricular valve malformations (17) subaortic conus (14) and pulmonary outflow tract stenosis or atresia (11). Criss-cross hearts were present in only 7 patients.

Inferior Vena Cava Stenosis in Scimitar Syndrome: A Case Report

The scimitar syndrome, first described by Chassinat in 1836, consists aessentially of an anomalous pulmonary vein draining whole or part of the right lung into the inferior vena cava. Associated anomalies are frequent, such as hypoplasia of the right lung, dextrocardia, malformations of the right pulmonary artery and bronchial tree, and abnormal arterial supply of the right lung (the so-called sequestration). This article describes a scimitar syndrome associated with stenosis of the inferior vena cava, whose initial diagnosis was made by two-dimensional echocardiographic Doppler color flow mapping. To our knowledge this is the first description of such an unusual association.

True Diverticulum of the Right Ventricle: Two Cases Associated with Tetralogy of Fallot

A true right ventricular diverticulum is a rare malformation consisting of an accessory ventricular outpouching communicating with the right ventricle through a muscular annulus and located at the right superior margin of the right ventricle. Its normal myocardial structure causes it to function as normal ventricle and in itself does not cause functional disturbances. In all reported instances, the malformation was associated with a ventricular septal defect and obstructive lesions of the right ventricular outflow tract. The 2 patients we describe had partial resection at the time of repair of tetralogy of Fallot and are doing well one and four years postoperatively.

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.

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.

Surgical Repair of Superoinferior Ventricles: Experience with 3 Patients

Superoinferior ventricles are a rare anomaly characterized by a horizontal ventricular septum and a hypoplastic right ventricular sinus localized anterosuperiorly to the left ventricle. This anomaly frequently is accompanied by malformation of the atrioventricular valves. A large ventricular septal defect is always present, and anomalies of the ventriculoarterial relations are common. The results of surgical repair of this complex lesion have been poor. Our recent surgical experience with 3 patients, 2 of whom are well 36 and 38 months postoperatively, suggests a hopeful outcome for the repair of this complex anomaly. The surgical approach was different in each of the 3 patients, demonstrating the need for a precise echocardiographic and angiocardiographic preoperative description of the cardiac anatomy to appropriately repair the multiple variants of this complex anomaly.