Ann Thorac Surg 1995;59:1019-1021
© 1995 The Society of Thoracic Surgeons
Case Reports
Isolated Atrial Inversion in Situs Inversus: A Rare Anatomic Arrangement
Giuseppe Santoro, MD,
Paolo Masiello, MD,
Rosario Farina, MD,
Cesare Baldi, MD,
Leonardo Di Leo, MD,
Giuseppe Di Benedetto, MD
Divisions of Cardiac Surgery and Cardiology, Ospedale S. Leonardo, Salerno, Italy
Accepted for publication August 23, 1994.
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Abstract
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Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.
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Introduction
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One of the fascinations of the congenital cardiac malformations is that the heart can show virtually any possible connection or relationship between its main segments (ie, atria, ventricles, and great arteries). One of the rarest combinations is that in which a discordant atrioventricular connection accompanies a concordant ventriculoarterial connection with normally related great arteries. Based on the spatial relationship between the great arteries, ``isolated atrial inversion'' {SLI} or {IDS} and ``isolated ventricular inversion'' {SLS} or {IDI} have been distinguished [1]. This article describes an exceedingly rare case of isolated atrial inversion in concordance with visceral situs inversus and reviews the literature about this issue.
A 1-month-old infant was admitted to our hospital because of mild cyanosis since birth. Physical examination revealed a 2/6 systolic murmur at the second left intercostal space. The liver edge was 2 cm below the left costal margin. Chest roentgenogram showed levocardia and visceral situs inversus (Fig 1
). Electrocardiography showed a clockwise deviation of the P-wave axis with a physiologic sequence of ventricular depolarization. Abdominal ultrasound examination showed a normally formed right-sided single spleen. Bidimensional echocardiography and angiocardiography clarified the diagnosis. Systemic venous blood drained through a left-sided inferior vena cava and a left superior vena cava into a left-sided morphologically right atrium. It entered through a mitral valve a left-sided morphologically left ventricle. A right-sided, morphologically left atrium received all pulmonary veins and entered through a tricuspid valve a right-sided morphologically right ventricle. Right and left ventricles showed topology and relationship usual for situs solitus. Concordant ventriculoarterial connection, normally related great arteries, and right aortic arch were found (Fig 2). A moderate-sized atrial septal defect allowed intercirculatory mixing. Systemic arterial saturation was 81%.
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Fig 2. . Right (A) and left (B) ventriculograms in anteroposterior views, showing ventriculoarterial relationships.
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As the baby grew up duly, a corrective operation was performed successfully when the patient was 8 months of age. After a median sternotomy, aortic cannulation was performed in the usual manner. Venous cannulation of the heart was achieved easily by lifting the heart from the apex and placing the cannula in the inferior vena cava, after the superior vena cava had been cannulated. A ``specular'' Senning procedure then was performed.
Postoperative course was uneventful. At discharge Holter electrocardiography and echocardiography did not show any significant anomaly.
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Comment
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Hearts with discordant atrioventricular and concordant ventriculoarterial connections from an anatomically heterogeneous but physiologically homogeneous entity in which the blood circulation resembles classic transposition of the great arteries. Isolated atrial inversion ({SLI} or {IDS}) is a rare condition that may be distinguished from the isolated ventricular inversion ({SLS} or {IDI}) based on the ventriculoarterial relationships [1]. Its anatomic pattern is characterized by discordant atrioventricular connection, normal right/left ventricular relationship, concordant ventriculoarterial connection, and normally related great arteries. Few cases of this anomalous anatomic arrangement have been reported, some of which had some degree of heterotaxy [2–4], whereas a few others were in visceroatrial situs solitus. To date, only 4 cases of this anomaly in situs inversus have been reported [585--8]. As expected, isolated atrial inversion shows a circulatory pattern resembling classic transposition of the great arteries with intercirculatory mixing at the atrial level through an atrial septal defect. Nevertheless, its physiologic and anatomic surgical repair can be achieved by an atrial switch procedure, by which the morphologically left ventricle serves the systemic circulation and the morphologically right ventricle serves the pulmonary circulation [7]. In our patient, a Senning procedure was chosen to avoid using prosthetic material.
In conclusion, a precise segmental diagnostic approach by echocardiography and angiocardiography is mandatory to define this rare anatomic arrangement and to make easier appropriate surgical management of these patients.
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Footnotes
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Address reprint requests to Dr Di Benedetto, Division of Cardiac Surgery, Ospedale ``S. Leonardo,'' Via S. Leonardo 1, 84100 Salerno, Italy.
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References
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- Pasquini L, Sanders SP, Parness I, et al. Echocardiographic and anatomic findings in atrioventricular discordance with ventriculoarterial concordance. Am J Cardiol 1988;62:1256–62.[Medline]
- Akita H, Matsouka S, Kuroda Y. A rare case of isolated atrial discordance with primary atrial situs inversus. Tokushima J Exp Med 1993;40:113–7.[Medline]
- Leijala MA, Lincoln CR, Shinebourn EA, Nellen L. A rare congenital cardiac malformation with situs inversus and discordant atrioventricular and concordant ventriculoarterial connections: diagnosis and surgical treatment. Am Heart J 1981;101:355–6.[Medline]
- Clarkson PM, Brandt PWT, Barratt-Boyes BG, Neutze JM. Isolated atrial inversion. Visceral situs solitus, visceroatrial discordance, discordant ventricular d-loop without transposition, dextrocardia: diagnosis and surgical correction. Am J Cardiol 1972;29:877–81.[Medline]
- Espino-Vela J, de la Cruz MV, Munoz-Castellanos L, Plaza L, Attie F. Ventricular inversion without transposition of the great vessels in situs inversus. Br Heart J 1970;37:443–50.
- Squarcia U, Ritter DG, Kincaid OW. Dextrocardia: angiographic study and classification. Am J Cardiol 1973;32:965–77.[Medline]
- Fox LS, Kirklin JW, Pacifico AD, Waldo AL, Bargeron LM. Intracardiac repair of cardiac malformations with atrioventricular discordance. Circulation 1976;54:123–7.[Abstract/Free Full Text]
- Calcaterra G, Anderson RH, Lau KC, Shinebourne EA. Dextrocardia: value of segmental analysis in its categorization. Br Heart J 1979;32:497–507.
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