Ann Thorac Surg 1998;65:1110-1114
© 1998 The Society of Thoracic Surgeons
Atrial Septal Displacement for Repair of Anomalous Pulmonary Venous Return Into the Right Atrium
Takeshi Hiramatsu, MDaa,
Yoshinori Takanashi, MDaa,
Yasuharu Imai, MDaa,
Shuichi Hoshino, MDaa,
Kazuhiro Seo, MDaa,
Masatsugu Terada, MDaa,
Yusuke Iwata, MDaa,
Hirofumi Tomimatsu, MDbb
a Department of Pediatric Cardiac Surgery, Tokyo Women’s Medical College, Heart Institute of Japan, Tokyo, Japan
b Department of Pediatric Cardiology, Tokyo Women’s Medical College, Heart Institute of Japan, Tokyo, Japan
Accepted for publication October 4, 1997.
Address reprint requests to Dr Hiramatsu, Department of Pediatric Cardiac Surgery, Tokyo Women’s Medical College, Heart Institute of Japan, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, Japan 162
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Abstract
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Background. In the repair of anomalous connection of the pulmonary veins to the right atrium, the use of a baffle of pericardium to divert the pulmonary venous blood into the left atrium could cause pulmonary venous obstruction as a result of thickening of the pericardial patch. Anomalous pulmonary venous drainage to the right atrium caused by malposition of the atrial septum primum can be repaired by displacing the shifted septum primum to the normal position.
Methods. In 5 patients with total (n = 2) or partial (n = 3) anomalous pulmonary venous drainage into the right atrium, the septum primum was shifted toward the left atrium and the pulmonary veins drained into the anatomic right atrium despite their normal connection with the posterior wall of the left atrium. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum between the anomalous pulmonary veins and both venae cavae. No patch was used.
Results. Postoperative echocardiography showed a wide pathway from the pulmonary veins to the left atrium with no stenotic portions. No atrial arrhythmias occurred after the operation.
Conclusions. This technique may be advantageous because it allows for future growth of the route of the pulmonary venous pathway and avoids postoperative supraventricular arrhythmias.
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Introduction
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The traditional approach to the repair of anomalous connection of the pulmonary veins to the right atrium (RA) has been an intraatrial procedure in which the remnant of the atrial septum primum is excised and a baffle of pericardium is placed to divert the pulmonary venous blood across the large defect in the atrial septum and into the left atrium (LA). The occasional occurrence of late retraction and thickening of the pericardial patch with this technique can result in pulmonary venous obstruction [1]. Another approach to the repair of this anomaly is the right atrial flap method [2, 3]. However, this technique may be associated with atrial arrhythmias in the same manner that the atrial switch operation for repair of transposition of the great arteries is associated with supraventricular tachycardia. Sinus node dysfunction also can be encountered later because the suture line inevitably lies close to this structure.
Van Praagh and colleagues [4] recently reported partial or total pulmonary venous drainage to the RA resulting from malposition of the atrial septum primum predominantly in patients with visceral heterotaxy. They observed that poor development or absence of the septum secundum appeared to be responsible for the malposition of the septum primum. We have treated 5 patients with total (n = 2) or partial (n = 3) pulmonary venous drainage directly into the RA in whom the atrial septum primum was shifted to the left and the pulmonary veins returned to the normal posterior portion of the LA. This type of anomaly can be repaired if the leftward-shifted atrial septum is displaced to the normal position using an atrial septal displacement technique. We used this technique in all 5 of our patients without any external materials. Herein, we present our operative technique and the postoperative course of these patients.
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Patients and methods
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Five girls, 3 with partial pulmonary venous drainage (right pulmonary veins returned to the RA) and 2 with total pulmonary venous drainage (Darling’s classification IIa), underwent successful surgical repair of their defects. At operation, these patients were found to have the following features in common: the superior limbic band of the atrial septum secundum was hypoplastic or absent, the atrial septum primum was shifted to the left, and anomalous pulmonary venous return was present, although all or some of the pulmonary veins returned to the normal posterior portion of the LA under the terminal sulcus (Fig 1A). We repaired these anomalies using an atrial septal displacement technique without any patches. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum along the line between the anomalous pulmonary veins and both venae cavae(Figs 1B, 1C). Although all the patients had an atrial septal defect (secundum type), it was possible to connect the incised atrial septum to the RA wall directly, because the RA was relatively large in this type of anomaly, with a right-to-left shunt at the atrial level and an abundant RA wall. When the shifted septum was incised at the posterior edge, special care was taken not to create an extracardiac hole and the incised endocardium was reendothelialized to avoid tissue overgrowth in the future. No pericardial or external patch was used for the reconstruction in this technique.
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Fig 1. (A) The right pulmonary veins (PV) returned to the normal posterior portion of the left atrium (LA) under the terminal sulcus. (B) The posterosuperior border of the atrial septum primum was incised and detached. (C) The incised septum was displaced between the pulmonary veins and the venae cavae. (RA = right atrium; SVC = superior vena cava.)
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The anomalous pulmonary venous return was diagnosed by cardiac catheterization in 2 patients and by echocardiography in 3 patients. A preoperative four-chamber view in 1 patient revealed the leftward-shifted atrial septum primum and the partial anomalous pulmonary venous drainage that returned to the normal posterior portion of the LA (Fig 2). The patients ranged in age from 27 days to 3 years (mean, 13.1 months) and in body weight from 3.0 to 14.3 kg (mean, 6.5 kg). All the patients had an atrial septal defect of the secundum type. One patient had a ventricular septal defect of the perimembranous type and 1 had a patent ductus arteriosus. One patient had valvular pulmonary stenosis, and polysplenia was diagnosed (Table 1).
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Fig 2. On preoperative echocardiographic evaluation, the atrial septum primum was shifted to the left and the right pulmonary veins returned to the right atrium (RA) as a result. (LA = left atrium; LV = left ventricle; RV = right ventricle.)
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A regular sinus rhythm was present in all the patients before operation. After operation, the flow acceleration between the pulmonary veins and the atrial septum was evaluated by echocardiography. Electrocardiography was performed continuously during the intensive care unit stay and daily thereafter.
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Results
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The duration of cardiopulmonary bypass ranged from 63 to 107 minutes (mean, 82 minutes) and the duration of aortic cross-clamping ranged from 31 to 63 minutes (mean, 48 minutes). Concomitant surgical procedures performed included ventricular septal defect closure, patent ductus arteriosus ligation, and pulmonary valvotomy. All patients were weaned from bypass with minimal inotropic support and a postoperative central venous pressure ranging from 6 to 9 cm H2O. They were extubated within 2 to 5 hours after the operation and were discharged from the hospital within 2 weeks without any significant problems.
Postoperative echocardiography showed that the atrial septum primum was displaced to the normal position and aligned with the septum secundum, there was a wide space from the pulmonary veins to the LA, and there was no flow acceleration at the anastomotic site. None of the patients had either pulmonary venous obstruction or vena caval stenosis after the operation. The typical four-chambers view of 1 patient is presented in Figure 3.
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Fig 3. On postoperative echocardiographic evaluation, the displaced atrial septum primum was aligned with the atrial septum (arrow) secundum and all the pulmonary veins (*) returned to the left atrium (LA). (LV = left ventricle; RA = right atrium; RV = right ventricle.)
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All the patients had a regular sinus rhythm after the operation and no evidence of a supraventricular arrhythmia or sinoatrial block at any time during their hospital stay. Outpatient follow-up ranged from 7 to 25 months (mean, 12 months) after the operation and all patients were alive and doing well. No cardiac symptoms occurred in any of the patients and chest roentgenography and electrocardiography did not reveal any problems. None of the patients underwent postoperative cardiac catheterization. Two patients underwent echocardiography 6 months after the operation and neither had any evidence of pulmonary venous obstruction or vena caval stenosis.
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Comment
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Normally, the septum primum grows from sinus venosus tissue adjacent to the inferior vena cava-RA junction parallel to the left venous valve [5]. When it completes its normal growth, it is attached on the LA side of the superior limbic band. It is the valve of the foramen ovale for the fetal circulation and forms part of the interatrial septum for the postnatal heart. Its normal growth and attachment on the septum secundum are essential for alignment of the common pulmonary vein with the cavity of the LA. If the superior limbic band of the septum secundum fails to develop, the cephalad crescent border of the septum primum remains unattached and could be carried leftward in cases of atrial situs solitus or rightward in cases of atrial situs inversus by the bloodstream of the fetal circulation, which proceeds from the RA toward the LA. Depending on the degree of septum primum displacement toward the LA, half or all of the pulmonary veins may drain into the cavity of the anatomic RA despite their normal connection with the posterior wall of the LA. Van Praagh and associates [4] speculated that the reason for the malposition of the septum primum may be poor development or absence of the superior limbic band of the septum secundum.
Historically, this anomaly was reported by Edwards [6] in 1953 and confirmed by Moller and co-workers [7] in 1967, but it did not attain its deserved recognition in the diagnosis of partial or total pulmonary return to the RA until 1993 [7]. The high incidence of visceral heterotaxy with polysplenia in affected patients is consistent because polysplenia is known to be associated with absence of the superior limbic band [8]. In our series, absence or hypoplasia of the superior limbic band was observed in all patients during operation. These findings appear to confirm this theory, although polysplenia was diagnosed in only 1 patient.
Two-dimensional echocardiography with Doppler interrogation is a reliable method of diagnosing this malformation. A deviated atrial septum is a characteristic feature. Preoperative recognition of the true nature of this anomaly facilitates its successful surgical management.
There are two other malformations in which the right pulmonary veins drain into the RA: (1) sinus venosus defects of the RA type with unroofing of the right pulmonary veins [9], and (2) large ostium secundum defects extending into the posterior border of the atrial septum that allow the right pulmonary veins to drain into the RA, although they normally are connected with the LA [10]. These defects should be differentiated from atrial septal malposition because they require different surgical treatment. In the case of the sinus venosus defects, a patch should be sutured between the upper border of the septum primum and the right border of the pulmonary veins. For the large atrial septal secundum defects with posterior extension, a patch should be used to close the defect, placing the pulmonary veins into the LA.
In the surgical treatment of atrial septal malposition, Van Praagh and colleagues [4] recommended that the shifted septum primum be resected completely and a new, appropriately positioned septum be constructed or that the pulmonary veins be baffled to the LA, because incomplete resection of the septum primum may result in pulmonary venous obstruction. However, incising the shifted septum primum at the posterior edge and displacing it to the normal position can allow for anatomic repair and is not associated with postoperative pulmonary venous obstruction in our experience. This technique does not require a pericardial or artificial baffle and therefore avoids the possibility of late retraction and thickening of the baffle, which can cause pulmonary venous obstruction. Reconstruction without baffling also allows for future growth of the anastomotic site.
Although the follow-up period in our series was relatively short, there was no postoperative evidence of supraventricular arrhythmias or pulmonary venous obstruction at the anastomotic site. This atrial septal displacement technique may be advantageous because it allows for future growth of the route from the pulmonary veins to the LA and is not associated with postoperative supraventricular arrhythmias.
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References
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- In: Kirklin J.W., Barrett-Boyes B.G., eds. Cardiac surgery, 2nd ed. New York: Churchill Livingstone Inc, 1993:609-673.
- Muraoka R., Yokota M., Aoshima M. A new technique for correction of total anomalous pulmonary venous connection to right atrium using a flap of the right atrial wall. Arch Jpn Chir 1981;50:860-866.
- Sawatari K., Imai Y., Kurosawa H., et al. A new technique for correction of right pulmonary venous connection to superior venae cavae. Jpn J Cardiovasc Surg 1988;17:622-629.
- Van Praagh S., Carrera M.E., Sanders S., Mayer J.E., Jr, Van Praagh R. Partial or total direct pulmonary venous drainage to right atrium due to malposition of septum primum. Chest 1995;107:1488-1498.[Abstract/Free Full Text]
- Van Praagh R., Corsini I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13 postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart J 1969;78:379-405.[Medline]
- Edwards J.E. Symposium on anomalous pulmonary venous connection (drainage): pathologic and developmental considerations in anomalous pulmonary venous connection. Proc Staff Meetings Mayo Clin 1953;28:441-452.
- Moller J.H., Nakib A., Anderson R.C., et al. Congenital cardiac disease associated with polysplenia: a developmental complex of bilateral "left-sidedness". Circulation 1967;36:789-799.[Abstract/Free Full Text]
- Cochrane A.D., Menahem S., Mee R.B.B. Divided left atrium with absence of the interatrial septum in monozygotic twins. Cardiol Young 1993;3:51-54.
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Abstract
Introduction
