Ann Thorac Surg 1996;62:1529-1532
© 1996 The Society of Thoracic Surgeons
Case Report
Intraatrial Baffle Repair of Isolated Ventricular Inversion With Left Atrial Isomerism
Doff B. McElhinney, MS,
V. Mohan Reddy, MD,
Norman H. Silverman, MD,
Frank L. Hanley, MD
Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of California, San Francisco, San Francisco, California
Accepted for publication June 7, 1996.
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Abstract
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Isolated ventricular inversion with left atrial isomerism, partial anomalous pulmonary venous connection, and interruption of the inferior vena cava with azygos continuation to a right superior vena cava was diagnosed by echocardiography in a neonate. At 48 days of age, the patient underwent successful anatomic correction with redirection of flow from the superior vena cava and hepatic veins to the left-sided tricuspid valve, and flow from the pulmonary veins to the right-sided mitral valve. In the present report, the surgical techniques of this case are described, along with a survey of the surgical literature covering anatomic repair of isolated ventricular inversion.
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Introduction
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Discordant atrioventricular connection with concordant ventriculoarterial connection, otherwise known as isolated ventricular inversion [1], is an extremely rare congenital cardiac malformation. Previous reports in the English-language literature describe only 14 patients with this malformation who have undergone anatomic correction (Table 1
). In this report, we present the case of an infant with isolated ventricular inversion and left atrial isomerism who was diagnosed by echocardiography to have inferior vena cava discontinuity with azygos continuation to a right superior vena cava (SVC), direct drainage of two hepatic veins into the right-sided systemic venous atrium, and partial anomalous pulmonary venous connection of the right pulmonary veins to the systemic venous atrium. She was referred to our institution and underwent successful atrial baffle repair at 48 days of age.
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Table 1. . Reported Cases of Anatomic and Physiologic Correction of Atrioventricular Discordance With Ventriculoarterial Concordancea
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The patient was the product of a full-term pregnancy and weighted 3,350 g at birth. She was noted to be cyanotic on the first day of life, and was shown by echocardiography to have isolated ventricular inversion, partial anomalous pulmonary venous connection, and interrupted inferior vena cava with azygos continuation to the right SVC. She remained stable on no medications, with systemic arterial oxygen saturations varying around 75% on room air, and was feeding well until referral to our institution for repair.
On preoperative echocardiographic evaluation at our institution (Fig 1), the diagnosis of isolated ventricular inversion was confirmed. The morphologic left ventricle was posterior and to the patient's right side, with the morphologic right ventricle anterior and to the left. The ventriculoarterial relation was concordant, with the aorta positioned posterior and to the left of the pulmonary artery. Left atrial isomerism was present with multiple systemic and pulmonary venous abnormalities. There was partial anomalous pulmonary venous connection with all of the right pulmonary veins draining to the right-sided atrium, all of the left pulmonary veins draining to the left-sided atrium, interrupted inferior vena cava with right azygos continuation, and separate drainage of the hepatic veins to the right-sided atrium. A tiny apical muscular ventricular septal defect and a tiny patent foramen ovale were also present. Her electrocardiogram was consistent with right ventricular hypertrophy, and showed no disturbances of rate or rhythm. Chest roentgenography showed prominent pulmonary vascular markings and mild cardiomegaly. An abdominal computed tomographic scan was read as situs inversus, with a right-sided stomach, left-sided liver extending across the midline, and a midline single spleen.
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Fig 1. . Echocardiogram performed 1 day before operation. (A) Subcostal coronal view showing the main pulmonary artery (PA) arising from the morphologic right ventricle (MRV), which is anterosuperior to the morphologic left ventricle (MLV). (B) Subcostal coronal view showing the right-sided systemic venous atrium (RA) draining to the MLV. The MLV is concordant with the aorta (AO), from which a coronary artery (CA) is shown to arise (arrows). (C) Pulmonary veins (PV) connect to both the left atrium (LA) and RA. The LA connection with the MRV also can be visualized.
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Operation was performed when the patient was 48 days of age. Venous cannulation of the SVC and both hepatic veins was performed, and the repair was carried out at moderate hypothermia with continuous cardiopulmonary bypass and aortic cross-clamping with cardioplegia. The external appearance of the atria showed bilateral fingerlike projections of the appendages, consistent with the diagnosis of left atrial isomerism. Atriotomy of the right-sided atrium revealed entrance of the SVC, two hepatic veins, and two right pulmonary veins. The internal morphologic features of this atrium were ambiguous, but there was no coronary sinus ostium visible, and there did not appear to be extension of pectinate muscles around the posterior muscular atrioventricular vestibule in either atrium, which is a distinctive characteristic of left atrial isomerism [12]. After excision of the atrial septum, intraatrial baffle rerouting of pulmonary and systemic venous return was performed using a fresh autologous pericardial patch. The patch was attached with running 7-0 monofilament sutures around the orifices of the SVC, hepatic veins, and pulmonary veins to redirect blood from the SVC and hepatic veins to the left-sided tricuspid valve, and blood from the left pulmonary veins, which had previously drained into the left-sided atrium, to the right-sided mitral valve (Fig 2). The operation was completed without event.
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Fig 2. . Technique of intraatrial baffle placement, viewed through a right atriotomy. A fresh autologous pericardial patch is sutured around the orifices of the superior vena cava (SVC), hepatic veins, and left pulmonary veins (LPV) to redirect pulmonary venous return to the right-sided mitral valve (MV), and systemic venous return to the left-sided tricuspid valve (TV). Note the unconventional venous cannulation of the SVC and both hepatic veins.
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Postoperatively, the patient had an uneventful course. She was extubated the day after the operation and required low-dose inotropic support for 2 days. Arterial oxygen saturation was 99% on the day of extubation, and left and right atrial pressures varied around 3 to 5 mm Hg. Discharge echocardiogram performed 6 days postoperatively showed normal biventricular function and an intact atrial baffle with no venoatrial or atrioventricular obstruction. A very small diastolic right-to-left shunt through the apical ventricular septal defect remained.
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Comment
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Can "true" isolated ventricular inversion exist in a situation of visceral heterotaxy? Isolated ventricular inversion, or discordant atrioventricular connection with concordant ventriculoarterial connection, is a rare alignment of the cardiac segments that has been shown to take many forms, but technically requires a definitive segmental diagnosis, including atrial situs, ventricular loop, and relation of the great arteries [10]. Among the English-language reports of surgical repair of isolated ventricular inversion, 3 [2, 5, 10] expressly exclude patients with atrial situs ambiguous from discussion, whereas only 2 [6, 7] explicitly include a patient with undefined atrial situs. However, descriptions of 3 other patients reported as having atrial situs solitus or inversus suggest that they may have had situs ambiguous as well (Table 1
) [8]. This is by no means a straightforward issue, as is clear from the controversy among cardiac morphologists regarding the developmental significance of and criteria for determining atrial situs [12]. Another interesting observation is that all patients had a significant ventricular septal defect except the 4 patients (including the patient in the present report) who had azygos or hemiazygos continuation.
In the present case, diagnosis by preoperative echocardiography was left atrial isomerism with only the left pulmonary veins draining to the left-sided atrium. Despite minimal intracardiac shunting, the anomalous drainage of the right pulmonary veins to the right atrium and left ventricle permitted satisfactory oxygenation and a stable preoperative course. Of concern in this patient was the fact that the right ventricle had been chronically preload reduced, receiving only right lung pulmonary venous return. At operation, observation of the atrial appendages and internal atrial morphology supported the preoperative diagnosis. Anatomically and physiologically, however, the situation is similar to one of situs solitus, with all systemic venous return opening into the right-sided atrium, and partial anomalous pulmonary venous connection, with the two right pulmonary veins draining into the right-sided atrium and the two left pulmonary veins entering to the left of a well-formed but malpositioned atrial septum. From a surgical point of view, this is a case of atrioventricular discordance with ventriculoarterial concordance that was corrected anatomically and physiologically by rerouting all systemic venous return to the left-sided tricuspid valve and blood from the left pulmonary veins to the right-sided mitral valve.
The underlying issue of distinguishing situs solitus or inversus from atrial isomerism can be of importance surgically, especially with respect to the cardiac conduction system [12]. In the present case, the diagnosis of left atrial isomerism was of minimal help surgically, insofar as anomalies of the conduction system in circumstances of left atrial isomerism are not well mapped out. If this had been a case of right atrial isomerism, however, this information might have been valuable, given the evidence that bilateral sinus nodes are commonly present with this malformation [12]. In a number of the surgical cases reported in the literature, patients had postoperative rhythm disturbances, ranging from first-degree atrioventricular block to complete heart block (see Table 1). In the present case, the patient had nodal reentry tachycardia, but this resolved after 1 day of atrial pacing.
As a review of the surgical literature shows (see Table 1), anatomic and physiologic repair with an intraatrial baffle or Senning procedure has been successful in all but 1 of the 15 cases (including the present case) reported. Little has been reported in the way of long-term follow-up of these patients, but because atrial baffle repair achieves anatomic and physiologic correction, it is likely that the primary long-term issues will be limited to disturbances of cardiac rhythm, and possibly venous obstruction, without concern over ventricular function.
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Footnotes
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Address reprint requests to Dr Reddy, Division of Cardiothoracic Surgery, ucsF, 505 Parnassus Ave, M593, San Francisco, CA 94143-0118.
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References
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- Van Praagh R, Van Praagh S. Isolated ventricular inversion: a consideration of the morphogenesis, definition and diagnosis of nontransposed and transposed great arteries. Am J Cardiol 1966;17:395–406.[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]
- Hazan E, Baillot F, Rey C, Dupuis C. Isolated ventricular discordance and complete atrioventricular canal in situs inversus. Report of successful surgical repair. Am J Cardiol 1977;40:463–6.[Medline]
- Leijala MA, Lincoln CR, Shinebourne EA, Nellen M. 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]
- Ostermeyer J, Bircks W, Krian A, Sievers G, Hilgenberg F. Isolated atrioventricular discordance: report of two surgical cases with isolated ventricular inversion. J Thorac Cardiovasc Surg 1983;86:926–9.[Abstract]
- Snider AR, Enderlein MA, Teitel DF, Hirji M, Heymann MA. Isolated ventricular inversion: two-dimensional echocardiographic findings and a review of the literature. Pediatr Cardiol 1984;5:27–33.[Medline]
- Park SC, Siewers RD, Neches WH, et al. Ventricular inversion with normal ventriculoarterial connection and left atrial isomerism: correction by the Mustard operation. J Am Coll Cardiol 1984;4:136–40.[Abstract]
- Arciprete P, Macartney FJ, de Leval M, Stark J. Mustard's operation for patients with ventriculoarterial concordance: report of two cases and a cautionary tale. Br Heart J 1985;53:443–50.[Abstract/Free Full Text]
- Baudet EM, Hafez A, Choussat A, Roques X. Isolated ventricular inversion with situs solitus: successful surgical repair. Ann Thorac Surg 1986;41:91–4.[Abstract]
- 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]
- Ranjit MS, Wilkinson JL, Mee RB. Discordant atrioventricular connexion with concordant ventriculo-arterial connexion (so-called "isolated ventricular inversion") with usual atrial arrangement (situs solitus). Int J Cardiol 1991;31:114–7.[Medline]
- Uemura H, Ho SY, DeVine WA, Kilpatrick LL, Anderson RH. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995;60:561–9.[Abstract/Free Full Text]
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Abstract
Introduction
