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Images in Cardiovascular Medicine Three-Dimensional Echocardiography in Criss-Cross Heart Could a Specimen Be Better? Alessia Del Pasqua, MD; Stephen Pruett Sanders, MD; Gabriele Rinelli, MD A Downloaded from http://circ.ahajournals.org/ by guest on June 17, 2017 9-day-old neonate weighing 3 kg was referred to our institution for cyanosis and a systolic murmur. A 2-dimensional echocardiogram suggested SDL, levocardia, criss-cross heart with double-outlet right ventricle, hypoplasia of the tricuspid valve and right ventricle, and severe pulmonary stenosis. A real-time 3-dimensional echocardiogram (Sonos 7500, Philips Medical Systems, Andover, Mass) demonstrated the complex anatomy of the criss-cross heart as clearly as if one were viewing an anatomic specimen. The atria were situated in relatively normal positions, with normal venous connections. The left atrium was posterior and communicated via the mitral valve with the morphologically left ventricle situated inferiorly and extending to the right (Figure 1). The right atrium was anterior and communicated by means of a tricuspid valve with the hypoplastic morphologically right ventricle, positioned superiorly and extending to the left (Figure 2). The right atrium–right ventricle axis was nearly orthogonal to, rather than parallel to, the left atrium–left ventricle axis so that the atrioventricular valves were seen to cross each other, as viewed in the frontal plane (Figure 3 and Movies I and II). The ventricles appeared to have been twisted clockwise about their long axes when viewed from the apex, whereas the base of the heart remained fixed. This gave the characteristic appearance of each atrium emptying into the contralateral ventricle. Given the rarity of this cardiac defect and the specimen-like quality of the images, the 3-dimensional echocardiogram was very useful not only for clinical diagnosis but also for didactic purposes. Disclosures None. From Università degli Studi di Siena (A.D.P.), Cardiology Department, Siena, Italy, and Ospedale Pediatrico Bambino Gesù (S.P.S., G.R.), Paediatric Cardiology Department, Rome, Italy. The online-only Data Supplement, which consists of 2 movies, is available with this article at http://circ.ahajournals.org/cgi/content/full/116/17/ e414/DC1. Correspondence to Gabriele Rinelli, MD, Ospedale Pediatrico Bambino Gesù, Piazza S. Onofrio 1, 00165 Rome, Italy. E-mail [email protected] (Circulation. 2007;116:e414-e415.) © 2007 American Heart Association, Inc. Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.107.717991 e414 Del Pasqua et al Downloaded from http://circ.ahajournals.org/ by guest on June 17, 2017 Figure 1. Three-dimensional echocardiographic subcostal view (posterior cut): The arrow traces the route from the left atrium to the left ventricle through the mitral valve. The left ventricle is normal in size, whereas the right ventricle is clearly hypoplastic. A indicates aorta; RV, right ventricle. Spatial orientation symbols: R indicates right; L, left; S, superior; I, inferior; A, anterior (toward observer); and P, posterior (away from observer). *Ventricular septal defect. Figure 2. Three-dimensional echocardiographic subcostal view (more anterior cut with respect to Figure 1): The arrow traces the route from the right atrium to the right ventricle through the tricuspid valve. Note that the axis of the tricuspid valve is nearly orthogonal to the axis of the mitral valve. The subpulmonary stenosis resulting from deviation of the infundibular septum is demonstrated. A indicates aorta; P, pulmonic valve; and I, infundibular septum. *Coronary sinus ostium; °atrial septal defect. Spatial orientation symbols as in Figure 1. 3D Echocardiography in Criss-Cross Heart e415 Figure 3. Three-dimensional echocardiographic subcostal view (a more inferior view obtained by rotating Figure 2): Both ventricles and atrioventricular valves are visualized. The mitral valve is seen en face (small arrow), whereas the tricuspid valve is seen in long-axis view (the big arrow traces the route from the right atrium to the right ventricle), demonstrating the “criss-cross.” The hypoplastic right ventricle is now evident, with only the inlet and outlet portions well represented. Small arrow indicates mitral valve orifice; S, interventricular septum; RV, right ventricle; A, aorta; P, pulmonic valve; and I, infundibular septum. Spatial orientation symbols as in Figure 1. *Ventricular septal defect. Three-Dimensional Echocardiography in Criss-Cross Heart: Could a Specimen Be Better? Alessia Del Pasqua, Stephen Pruett Sanders and Gabriele Rinelli Circulation. 2007;116:e414-e415 doi: 10.1161/CIRCULATIONAHA.107.717991 Downloaded from http://circ.ahajournals.org/ by guest on June 17, 2017 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2007 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/116/17/e414 Data Supplement (unedited) at: http://circ.ahajournals.org/content/suppl/2007/10/17/116.17.e414.DC1 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/