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Isolated congenitally corrected transposition of the great arteries with dextroversion discovered incidentally in a patient with cocaine-induced acute myocardial infarction Anumeha Tandon, MD, Rahul Bose, MD, Anthony D. Yoon, MD, and Jeffrey M. Schussler, MD Complex cardiac congenital anomalies can occasionally be found in adult patients who have no knowledge of their condition. Here we present the case of a 27-year-old man with cocaine-induced acute myocardial infarction in whom an isolated congenitally corrected transposition of the great arteries with dextroversion was discovered incidentally. W hile congenital anomalies are often left to subspecialists in the adult congenital arena, familiarity with them is important for Figure 1. Electrocardiogram demonstrating a reversal of the P-wave axis (inverted P waves in leads I, AVR, and AVF; arrow) the general cardiologist. When with reversal of progression of R waves across the entire precordial leads (V1–V6). these patients present in association with other, more common conditions, evaluation can anterior descending artery, but in a conformation that would be be more challenging. considered “opposite” of normal anatomy. The opposite coronary artery appeared similar in conformation to a right coronary CASE DESCRIPTION artery, but also had an “opposite” course (Figure 3). A 27-year-old man presented with chest pain. A loud systolic A transthoracic echocardiogram (with images obtained primurmur was heard at the right sternal border, and a rightward marily from right-sided windows) revealed an aorta originating displaced point of maximal impact was noted. The admission from a morphologic right ventricle (systemic ventricle) and the electrocardiogram showed prominent R waves in V1, with an pulmonary trunk from a morphologic left ventricle (pulmonary inverted P-wave axis, and reverse R wave progression across the ventricle). These findings were confirmed by cardiac computed precordium (Figure 1). A chest radiograph demonstrated a righttomography (Figure 4). Abdominal ultrasound showed normally ward-pointed heart, prominent right-sided chambers, and the oriented viscera confirming situs solitus (normal orientation absence of the usual aortic and pulmonary contours (Figure 2). of the viscera). The patient was treated medically for cocaine The urine was positive for cocaine. The patient’s troponin level intoxication and was ultimately discharged in good condition. was 55 ng/mL. At urgent cardiac catheterization, the ventriculogram in the left anterior oblique position disclosed the heart From the Department of Internal Medicine, Division of Cardiology, Baylor University pointing toward the right hemithorax, suggesting dextrocardia Medical Center at Dallas (Tandon, Bose, Yoon, Schussler); the Jack and Jane or dextroversion. Angiography showed no coronary narrowing, Hamilton Heart and Vascular Hospital, Dallas, Texas (Tandon, Yoon, Schussler); but demonstrated “mirror image” epicardial coronary arteries. and Texas A&M College of Medicine (Schussler). The most anterior coronary artery coursed toward the right side Corresponding author: Jeffrey M. Schussler, MD, 621 N. Hall Street, Suite 400, of the body and covered a distribution similar to that of a left Dallas, TX 75226 (e-mail: [email protected]). Proc (Bayl Univ Med Cent) 2016;29(2):171–173 171 Figure 2. Chest radiograph demonstrating a rightward-oriented heart, with the morphologic left ventricle (LV) pointed towards the right. There is loss of the normal aortopulmonary contours (arrowhead), and this area appears “flat.” The gastric bubble is noted under the left hemidiaphragm, indicative of situs solitus (normally oriented viscera). DISCUSSION Congenitally corrected transposition of the great arteries (CCTGA) is a rare (<1% of all congenital heart disease) anomaly where the great arteries are transposed and the ventricles, ventricular septum, atrioventricular valves, epicardial coronary arteries, and the conduction system are inverted. Other congenital heart defects such as ventricular septal defect, pulmonary stenosis, anomalies of the systemic atrioventricular valve (commonly, Ebstein’s anomaly), and conduction defects are present in approximately 98% of these patients. Most patients are identified in childhood, usually due to complications from the associated abnormalities. A minority of patients (particularly those without associated anomalies) may be asymptomatic for many years and present later in life due to findings on physical or radiologic exams, or if they experience systemic ventricular failure (1). Dextrocardia, or a rightward-pointing heart, is seen in up to 20% of these patients, and CCTGA should be strongly suspected when dextrocardia is observed (2). Dextrocardia with situs solitus (normally oriented viscera) and no other cardiac anomalies is rare, with an incidence of 1 live birth in 30,000 (3). a b c d Figure 3. Systemic ventriculogram (morphologic right ventricle) with a rightward-oriented apex. (a) Standard left anterior oblique imaging demonstrates a systemic ventricle that points towards the right side of the body. (b) Injection of the ascending aorta (Ao) fills the left main (LM) coronary artery. (c) Selective injection of the LM fills arteries that are equivalent to the left anterior descending (LAD) and left circumflex (LCx) arteries, in that they supply blood to the anterior and lateral portions of the systemic ventricle. (d) Injection of the posteriorly located coronary artery, equivalent to the right coronary artery (RCA), demonstrates that it supplies blood to the posterior portions of the systemic ventricle. 172 Baylor University Medical Center Proceedings Volume 29, Number 2 a b c d cardiac magnetic resonance imaging, and invasive coronary angiography can all be used in conjunction to allow for accurate diagnosis. Coronary anatomy, in particular, is variable. In up to 50% of patients, there may be associated coronary anomalies (4). Patients who present with coronary atherosclerosis or acute coronary syndromes are uncommon, as these patients usually present with systemic ventricular failure early in life. Coronary revascularization is appropriate and feasible, although it may be technically challenging due to abnormal conformation of the coronary anatomy (5, 6). 1. Wissocque L, Mondesert B, Dubart AE. Late diagnosis of isolated congenitally corrected transposition of the great arteries in a 92-year old woman. Eur J Cardiothorac Surg 2015 Nov 15 [Epub ahead of print]. 2. Warnes CA. Transposition of the great arteries. Circulation 2006;114(24):2699–2709. 3. Solzbach U, Beuter M, Hartig B, Haas H. Isolated dextrocardia Figure 4. (a) Computed tomography of the heart with 3D rendering demonstrating the course of the coronary arteries as with situs solitus (dextroversion). they supply blood to the systemic ventricle (SV). (b) A fully rendered 3D representation of the heart shows the relationship Herz 2010;35(3):207–210. between the SV and the pulmonary ventricle (PV) as well as the left anterior descending (LAD). (c) A short-axis view of the 4. Ismat FA, Baldwin HS, Karl heart demonstrates that the aorta (Ao) is positioned anterior to the pulmonary artery (PA); (d) rather than being oriented TR, Weinberg PM. Coronary 90° to each other, they are oriented in parallel. anatomy in congenitally corrected transposition of the great arteries. Int J Cardiol 2002;86(2– 3):207–216. The approach to evaluating a patient with dextrocardia, 5. Baltalarli A, Tanriverdi H, Goksin I, Onem G, Rendeci O, Sacar M. once the condition has been identified, should include idenCoronary arterial revascularization in an adult with congenitally cortification of visceral situs, atrioventricular concordance, venrected transposition of great arteries and dextrocardia. J Card Surg tricular morphology and situs, relation of the great arteries, 2006;21(3):296–297. 6. Mehrotra P, Choi JW, Flaherty J, Davidson CJ. Percutaneous coronary and finally associated abnormalities (4). Imaging modalities intervention in a patient with cardiac dextroversion. Proc (Bayl Univ Med such as echocardiography, cardiac computed tomography, Cent) 2006;19(3):226–228. April 2016 Isolated congenitally corrected transposition of the great arteries with dextroversion discovered incidentally 173