Situs Inversus Totalis and Single Coronary Ostium

Situs Inversus Totalis and Single Coronary Ostium

Situs Inversus Totalis and Single Coronary Ostium: A Coincidence or a Pattern? Alexander Turchin, MD,* Stephen S. Radentz, MD,† and Allen Burke, MD‡ *...

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Situs Inversus Totalis and Single Coronary Ostium: A Coincidence or a Pattern? Alexander Turchin, MD,* Stephen S. Radentz, MD,† and Allen Burke, MD‡ *New England Medical Center, Boston, Massachusetts, USA; †Office of the Chief Medical Examiner, Baltimore, Maryland, USA; ‡Armed Forces Institute of Pathology, Washington, DC, USA

ⴙⴙ Situs inversus totalis and single coronary ostium are rare congenital anomalies, and no ontogenic connection has been described between them. Only three cases of association of single coronary ostium and situs inversus have been reported in the literature, all found on angiography. Here we present the first case of this association discovered at autopsy. Based on the apparently higher than expected frequency of this finding, an underlying pathologic connection between these conditions is proposed. Cardiovasc Pathol 2000;9:127–129 © 2000 by Elsevier Science Inc.

Situs inversus totalis is a rare condition in which orientation of all asymmetrical organs in the body is a mirror image of the normal morphology, known as situs solitus. Its prevalence in the general population is estimated to be 1 in 10,000 (1). Solitary coronary ostium is nearly as uncommon, with suggested incidence of c. 1:2,500 (2). While incidence of congenital cardiac defects is increased in patients with situs inversus totalis (1,3), coronary artery abnormalities are not commonly found (4). To our knowledge, there are only three descriptions in the literature of the association of complete situs inversus with single coronary ostium (5–7), all detected by angiography. Here we report the first case of situs inversus totalis with a single coronary ostium found at necropsy.

Case Report A 40-year-old male with history of alcohol and cocaine abuse as well as several recent episodes of seizures was found dead in his bedroom. The day before he complained vaguely of not feeling well. He was last seen alive in the evening before going to bed; his body was found the next morning sitting on the bed with his head resting on an adjacent coffee table. Manuscript received November 24, 1999; accepted December 14, 1999. Address for correspondence: Alexander Turchin, MD, 224 Kennedy Drive, #806, Malden, MA 02148, USA. Tel: (781) 322-9180; E-mail: . Cardiovascular Pathology Vol. 9, No. 2, March/April 2000:127–129  2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010

Over the year prior to his death, the patient had two episodes of witnessed tonic-clonic seizures, which were thought to be due to alcohol withdrawal; otherwise his past medical history was unremarkable. There was no history of sinusitis or respiratory tract infections; he had one child.

Pathology On autopsy, the patient was found to have situs inversus totalis (Figure 1). Examination of the heart showed that he had only a single coronary ostium in the anatomical left aortic sinus (Figure 2). There was an anomalous right coronary artery coursing in front of the pulmonary trunk, consistent with the IB1-type pattern, according to the classification proposed by Shirani and Roberts (8). There was no significant coronary atherosclerosis. The patient was also found to have chronic active hepatitis with bridging fibrosis and microvesicular steatosis. No significant pathology was found in other organs, including brain. It was concluded that the patient most likely died from a cardiac arrhythmia, to which he was predisposed by his hepatic cirrhosis (9), seizure disorder (10), and cocaine abuse.

Discussion Situs inversus totalis was first described by Baillie in 1785 (11). It is thought to be multifactorial in etiology, and

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Figure 1. Thoracic and abdominal cavity, as opened in the course of the autopsy. Both supra- and subdiaphragmatic viscera are seen in reverse orientation, consistent with situs inversus totalis.

both sporadic and inherited cases have been reported. While most patients with situs inversus have no other associated pathology, and enjoy a normal life span, the incidence of congenital cardiac malformations is significantly increased (1)—up to 3%. Most commonly reported defects are tetralogy of Fallot and interventricular septal defect (4); association with coronary artery abnormalities has not been described. Single coronary ostium was first reported by Hyrtl in 1841 (12). It is a rare finding and usually does not affect life expectancy. The latest classification of the resulting distribution patterns of coronary vessels, proposed by Shirani and Roberts (8), is based on the location of the ostium and presence of aberrant coronary arteries. While several cases of myocardial ischemia and/or sudden death associated with single coronary ostium have been described (8) (in most cases there was an aberrant coronary artery coursing between aorta and pulmonary trunk), no evidence of myocardial ischemia could be demonstrated in our patient. To the best of our knowledge, this is the fourth case of association of single coronary ostium and situs inversus totalis described in the literature. If these conditions were completely independent from each other, the expected fre-

Figure 2. (A) Epicardial surface of the heart from the anterior aspect. The pulmonary trunk is arising from the left side, and the relationship of the aorta and pulmonary trunk is inverted, indicating situs inversus. The right-sided coronary sinus (physiologic left coronary sinus) gives rise to a large single proximal artery, which courses leftward around the pulmonary trunk. The three branch vessels are the left circumflex, ramus intermedius, and left anterior descending artery, respectively. The continuation of the artery, in front of the pulmonary trunk, becomes the right coronary artery. (B) The left ventricular outflow tract demonstrating the aortic valve. The anterior leaflet of the mitral valve (right) is connected to the physiologic left coronary cusp and the posterior (“noncoronary”) cusp (cut through, seen on both sides of the valve). The dilated single ostium is seen in the (physiologic) left coronary cusp. The (physiologic) right coronary cusp is in the center of the photography.

quency of finding them in the same person would have been c. 1:25,000,000. It is therefore unlikely that so many cases of this coincidence would be described over just 20 years, especially taking into account that neither condition causes symptoms forcing the patient to seek medical attention. In addition, out of approximately 200 cases of single coronary ostium reported in the literature (2,8), four are in association with situs inversus totalis. Even taking into account the ex-

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pected reporting bias, the difference between the observed (1:50) and expected (1:10,000) frequency is significant. We thus proposed that there is an underlying association between situs inversus totalis and single coronary ostium. Further studies would, of course, be required to confirm this hypothesis and elucidate possible mechanisms.

References 1. Torgensen J. Genic factors in visceral asymmetry in the development and pathological changes of the lungs, heart and abdominal organs. Archives of Pathology 1949;47:566–593. 2. Sharbaugh AH, White RS. Single coronary artery: Analysis of the anatomic variation, clinical importance, and report of five cases. JAMA 1974;230:243–246. 3. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births: Incidence and natural history. Circulation 1971;43:323– 332. 4. Merklin RJ, Varano NR. Situs inversus and cardiac defects. A study of 111 cases of reversed asymmetry. J Thoracic Cardiovasc Surgery 1963;45:334–342. 5. Kleinfeld M, Rozanski JJ, Brumlik JV. Situs inversus, subaortic and






11. 12.


subpulmonic stenosis, ventricular septal defect, and single coronary artery. Chest 1976;70:391–393. Wester JP, Ernst JPMG, Mast EG, Plokker HWM, Bal ET, Verzijlbergen JF. Coronary angioplasty in a patient with situs inversus totalis and a single coronary artery. Catheterization and Cardiovascular Diagnosis 1994;31:304–308. Yip ASB, Chow WH, Cheung KL. Dextrocardia with single coronary artery ostium in a patient with aortic regurgitation and supraventricular tachycardia. Angiology 1994;45:907–910. Shirani J, Roberts WC. Solitary Coronary ostium in the aorta in the absence of other major congenital cardiovascular anomalies. J Amer Coll Cardiol 1993;21:137–143. Bernardi M, Calandra S, Colantoni A, Trevisani F, Raimondo ML, Sica G, Schepis F, Mandini M, Simoni P, Contin M, Raimondo G. Q-T interval prolongation in cirrhosis: Prevalence, relationship with severity, and etiology of the disease and possible pathogenetic factors. Hepatology 1998;27:28–34. Nilsson L, Farahmand BY, Persson PG, Thiblin I, Tomson T. Risk factors for sudden unexpected death in epilepsy: A case-control study. Lancet 1999;353:888–893. Baillie, M. Of a remarkable transposition of the viscera. Phil Trans Royal Soc London 1785–1790;16:483. Hyrtl J. Einige in chirurgischer Hinsicht wichtige Gefässvarietäten. Med Jahrb Österr Staats 1841;33:17–38.