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MMCTS (October 18, 2007). doi:10.1510/mmcts.2006.002386
Copyright © 2007 European Association for Cardio-thoracic Surgery

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Procedure


The surgical anatomy and treatment of interatrial communications{star}

Christian Schreibera,*, Jürgen Hörera, Manfred Vogtb, Andreas Kühnb, Paul Liberaa, Rüdiger Langea and Robert H. Andersonc

a German Heart Center Munich, Clinic of Cardiovascular Surgery at the Technical University, Lazarettstrasse 36, 80636 Munich, Germany
b Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technical University, Lazarettstrasse 36, 80636 Germany
c Cardiac Unit, Institute of Child Health, University College, London, UK

* Corresponding author: * Tel.: +49-89-1218 4629; fax: +49-89-1218 4113schreiber{at}dhm.mhn.de


   Summary
Top
 Summary
Introduction
 Current approach
 Discussion
 References
 
Holes permitting shunting between the atrial chambers can take various anatomical forms, varying from the patent oval foramen, which shunts only from right-to-left, to the so-called sinus venosus defect, which is associated with anomalous connection of the pulmonary veins. Our review deals with all forms of interatrial communications, except for the so-called ‘primum’ defect, since although the lesion produces interatrial shunting of blood, the atrioventricular septal defect with common atrioventricular junction but separate valvar orifices for the right and left ventricles, is strictly an atrioventricular septal defect. In addition, the review illustrates in detail the morphological features of interatrial communications, and describes surgical challenges and approaches.

Key Words: Atrial septal defect • Interatrial communications • Surgery


   Introduction
Top
 Summary
 Introduction
Current approach
 Discussion
 References
 
Holes between the atrial chambers make up the third most common congenital cardiac malformation seen in adults [1]. Such communication provides the situation for increased flow of blood to the lungs, and the consequences of prolonged exposure to such increased flow are often the reason for identification of the lesion, but the malformations often remain clinically silent, and are identified only at physical examination. Their true incidence in the population, therefore, remains unknown. Whereas anatomic closure of such holes between the atriums is clearly indicated when the lesion is diagnosed in childhood, and shows excellent long-term results [2], the indications for closure in older patients remain controversial. Recent clinical trials have favored anatomic closure at the time of diagnosis, since it has been shown to be safe and efficacious [3,4,5,6], and superior to medical treatment [7]. Although mortality after surgical closure is low, some patients do die late after surgery as a consequence of arrhythmias and cardiac failure. The need for closure, therefore, needs to be weighed against what is known of natural history. According to Campbell [8], the mortality rate for patients with an unrepaired atrial septal defect is low in the first three decades, at 0.6, 0.7, and 2.7% per annum, respectively. In the next three decades, in contrast, the rate increases to 4.5, 5.4 and 7.5% per annum, indicating that the prolonged elevation of flow of blood to the lungs might have already led to elevated pulmonary arterial pressures and right ventricular impairment. It is these cardiopulmonary pathologies that are the potential risk factors for operative and long-term mortality. In this review, therefore, we discuss the surgical options for closure of holes between the atrial chambers. We set the morphology of the lesions in the context of normal structure of the atrial septum, since as we will show, some of the holes permitting shunting between the atriums are outside the confines of the true septum [9].

History
After success with extracardiac operations, such as the ligation of the persistently patent arterial duct, correction of aortic coarctation, and construction of systemic-to-pulmonary arterial shunts, cardiac surgeons began to develop operations demanding access to the inside of the heart. The hole between the atrial chambers through the oval fossa, because of its simplicity and location, was the logical starting point. The first experimental efforts to close such holes surgically date back to the year 1926 [10]. Then, as early as 1950, Swan and colleagues showed that the defects could be closed by undertaking surgery during occlusion of the caval venous inflows [11]. Subsequent to this, Sondergaard repaired his first clinical case on 11 November, 1952 [12], while Derra and colleagues, using hypothermia combined with inflow occlusion, reported surgical closure in 1957 for eight patients, with one death [13]. Following the development of the heart-lung machine, closure of an atrial septal defect was again an obvious procedure to attempt under direct vision, and this was achieved by Gibbon and his colleagues in an 18-year-old patient on 6 May, 1953 [14].

Indications
Atrial septal defects smaller than 4 mm in size are reported to undergo spontaneous closure [15], whereas defects larger than 8 mm are unlikely to close spontaneously [16]. Because of the known potential complications of untreated atrial septal defects, such as late arrhythmia, right heart failure, and pulmonary hypertension, atrial septal defects should be closed either surgically or via a transcatheter approach at the time of diagnosis, including patients who do not exhibit symptoms of heart failure. If the defect is diagnosed in infancy, most centers prefer to close an atrial septal defect before a child starts school. Very rarely symptoms of heart failure are observed in patients presenting with isolated defect before the age of five years. In these patients, closure should be performed at the time of diagnosis, since the likelihood of achieving good results in terms of normalization of right ventricular dimensions decreases with further delay.


   Current approach
Top
 Summary
 Introduction
 Current approach
Discussion
 References
 
Nowadays, surgical closure of a hole between the atrial chambers is generally considered a ‘simple’ procedure, with a very low morbidity and mortality even for those variants that exist outside the confines of the oval fossa. When we analyzed our own experience, in 2001, freedom from reoperation 15 years after closure of a defect in over 1000 patients was as high as 99% [17]. The surgical approach can be through a median sternotomy, a right anterolateral thoracotomy (Photos 1 and 2), or a midaxillary thoracotomy (Photo 3).


Figure 1
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  		Photo 1 Cosmetic result in male patient after minimal invasive surgery.

 

Figure 2
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  		Photo 2 Cosmetic result in female patient after minimal invasive surgery.

 

Figure 3
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  		Photo 3 Midaxillary thoracotomy in prepubescent patient.

 
In our opinion, a median sternotomy should be avoided when closing atrial defects, other than those associated with partially anomalous connection of a left pulmonary vein, or for the atrioventricular septal defect with exclusively atrial shunting in the setting of insufficiency of the left atrioventricular valve. Since our long-term follow-up in prepubescent female patients after using the approach through a right anterolateral thoracotomy revealed significantly impaired unilateral development of the breasts, we suggested that this approach should be abandoned in this subgroup of patients [18]. We now reserve the anterolateral thoracotomy for adult patients.

So as to make a midaxillary thoracotomy [19], the patient is placed in an anterior oblique position at an angle of 45°, and the arm is suspended at a right angle (Video 1).


Figure 1
Click on image to view video		 Video 1 Steps involved in a midaxillary thoracotomy followed by closure of an atrial septal defect in the oval fossa: first, the patient is placed in an anterior oblique position at an angle of 45°, and the arm is suspended at a right angle. The skin incision is performed near the midaxillary line.
 
The skin incision is performed near the midaxillary line. It begins at the height of the mammary areola, and passes posteriorly towards the tip of the scapula, ranging in length from 5 to 6.5 cm. Although the latissimus dorsi muscle inserts on the posterior aspect of the iliac crest, in the lateral position the anterior margin is usually found on the line between the posterior axillary fold and the anterior-superior iliac spine. The subcutaneous attachments of the latissimus dorsi muscle, therefore, are mobilized with electrocautery by dissecting just superficial to the fascia (Video 2).


Figure 2
Click on image to view video		 Video 2 Mobilization of the latissimus dorsi muscle. The muscle can then be retracted posteriorly, exposing the serratus anterior muscle. This muscle is dissected by dividing the fascia along its posterior border up to, and just above, the tip of the scapula, and down to the lowest attachment on the anterior aspect of the sixth rib. The thorax is opened in the bed of the fourth rib.
 
The entire anterior border of the muscle is thus freed. The muscle can now be retracted posteriorly, exposing the serratus anterior muscle. This muscle is dissected by dividing the fascia along its posterior border up to, and just above, the tip of the scapula, and down to the lowest attachment on the anterior aspect of the sixth rib. The muscle is retracted forward, and the thorax is opened in the bed of the fourth rib. The heart is then exposed by opening the pericardium, and placing suitable stay sutures (Video 3).


Figure 3
Click on image to view video		 Video 3 Exposition of the heart by opening the pericardium, and placement of suitable stay sutures.
 
Tapes are passed around the caval veins, and two standard purse-string sutures are placed on the ascending aorta, permitting direct cannulation (Video 4). We have used DLP® cannulas (Medtronic – MMCTSLink 74).


Figure 4
Click on image to view video		 Video 4 Direct cannulation of the aorta and both caval veins. Cardiopulmonary bypass is instituted under normothermia. The operating table is then tilted, keeping down the head of the patient. The caval venous tapes are snared, fibrillation is induced electrically, and the right atrium is opened. Two pump suckers, of which one is placed in the coronary sinus, allow clear exposure of the right atrial cavity. The intraatrial defect is either closed with direct suture or patch material.
 
After placing standard purse-string sutures at the junctions of the caval veins with the right atrium, we perform bicaval cannulation using straight cannulas, currently employing wire-reinforced cannulas from Stöckert® – MMCTSLink 156. The use of angled cannulas may also be considered. We have not used femoral or iliac cannulation in any patient in our series. We then institute cardiopulmonary bypass. The caval venous tapes are snared, the right atrium is opened under normothermia, and fibrillation is induced electrically. We perform a vertical incision in the mid portion of the atrium in contrast to the primary standard oblique atrial incision, believing this to facilitate subsequent closure after the modified thoracotomy. Two pump suckers, one placed in the coronary sinus, allow clear exposure of the right atrial cavity. The intraatrial defect, when within the oval fossa, is either closed by direct suture, or using a patch. The pericardial stay-sutures are then removed before gradually inflating the lungs for de-airing. With a needle and a syringe, blood is withdrawn from the pulmonary veins, or directly from the left atrium and the ascending aorta. The operating table is then rotated, keeping down the head of the patient, and the heart is defibrillated. After a short period of reperfusion, the patient is weaned from bypass. To close, the retractors are removed, pericostal sutures are placed, and the muscles are allowed to return to their normal position. Single sutures can be placed on the posterior border of the serratus muscle, and another on the anterior border of the latissimus muscle, thus anchoring them to the surrounding fascia. It is, of course, possible to use other minimally invasive approaches, such as a mini-sternotomy [20, 21], a transxyphoid approach without sternotomy [22, 23], or a posterolateral thoracotomy [24, 25].

Varied anatomy of interatrial communications
The surgical procedure illustrated thus far has been the closure of a simple defect in the floor of the oval fossa. It is well recognised, however, that holes permitting shunting between the atrial chambers can take various anatomical forms, varying from the patent oval foramen, which shunts only from right-to-left, to the so-called sinus venosus defect, which is associated with anomalous connection of the pulmonary veins. So as to explain the surgical approaches to these lesions, it is necessary first to consider the structure of the normal atrial septum, and then to demonstrate how defects can produce the potential for interatrial shunting when they are without the confines of the normal septum.

Structure of the normal atrial septum
When viewing the posterior wall of the right atrium, it seems at first sight that a large proportion separates the cavity from the left atrium (Photo 4). Sectioning the heart, however, shows that this initial impression is misleading, since it is only the floor of the oval fossa and its immediate antero-inferior rim that can be removed without exiting from the heart (Photo 5).


Figure 4
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  		Photo 4 The right atrium has been opened to show its posterior wall, in which is seen the oval fossa. At first sight, the larger part of this wall seems to interpose between the right and left atriums. As shown in Photo 5, this appearance is deceptive.

 

Figure 5
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  		Photo 5 The heart shown in Photo 4 has been transected through the middle of the oval fossa. It can now be seen that the rims of the fossa are no more than infoldings of the atrial wall.

 
The other rims of the fossa are no more than infoldings containing extracardiac fat, particularly the supero-posterior rim, which constitutes the deep interatrial groove known as Waterston's or Sondergaard's groove (Photo 6). Although often considered a ‘secondary septum’, and certainly separating the atrial cavities, it is necessary for the surgeon to recognise that the rim is an infolding, and that incisions in this area extend into extracardiac space (Schematic 1).


Figure 6
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  		Photo 6 This four-chamber section is across an adult heart. It shows that the superior rim of the oval fossa is also an infolding, and is filled with fat.

 

Figure 1
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  		Schematic 1 The left-hand panel (A) shows the arrangement of the atrial septum. As is shown in the middle panel (B), it is possible to remove the floor of the oval fossa without passing outside the heart. Removal of the so-called ‘septum secundum’ (C) creates a communication with extracardiac space, because the alleged septum is, in reality, the superior interatrial fold (see Photo 6).

 
Morphology of interatrial communications
Recognition that the true atrial septum is the floor of the oval fossa leads to the morphological distinction between atrial septal defects and interatrial communications. Interatrial communications are the holes that permit shunting between the atrial chambers. But not all such holes are produced by deficiencies of the atrial septum! The essence of the atrial septal defect is deficiency (Photo 7) or fenestration (Photo 8) of the floor of the oval fossa. It is a lesion of this type that is present in the patient illustrated in Videos 1 through 4.


Figure 7
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  		Photo 7 The intraoperative photograph shows deficiency of the entire floor of the oval fossa. The photograph was taken in the operating room by Benson Wilcox, University of North Carolina, and is reproduced with his permission.

 

Figure 8
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  		Photo 8 The intraoperative photograph shows fenestration of the floor of the oval fossa. The photograph was again taken in the operating room by Benson Wilcox, University of North Carolina, and is reproduced with his permission.

 
We show the lesion as diagnosed echocardiographically in Videos 5, 6, 7, 8, 9, 10.


Figure 5
Click on image to view video		 Video 5 Defect within the oval fossa.
 

Figure 6
Click on image to view video		 Video 6 The transesophageal echocardiogram is taken at an angle of 70° to show the details of the interatrial septum. There is a long tunnel-type patent foramen ovale.
 

Figure 7
Click on image to view video		 Video 7 This transesophageal echocardiogram is taken in a cranio-caudal view at 90°, and shows a large aneurysm of the atrial septum, with the septum itself showing an excursion of about 2.5 cm.
 

Figure 8
Click on image to view video		 Video 8 This apical four-chamber is taken from transthoracic examination. The right atrium and ventricle are enlarged as sign of significant volume load at atrial level. In the central portion of the fossa ovalis, there is a moderate sized defect, with the so-called T-phenomenon.
 

Figure 9
Click on image to view video		 Video 9 Transesophageal echocardiography from the midesophagus. A large atrial septal defect is shown within the fossa ovalis, with nearly no rim posteriorly, but with a good rim antero-inferior to the atrioventricular valves. Due to its large size, this defect was not deemed suitable for interventional occlusion, and the patient underwent surgery.
 

Figure 10
Click on image to view video		 Video 10 Transesophageal four-chamber view from the midesophagus. It shows the leaflets of a common atrioventricular valve fixed to the crest of the ventricular septum. Both ventricles are of good size, and the atrioventricular junction is equally connected to both ventricles. There is lack of septation at atrial level, giving a common atrium, but morphologically right and left atrial appendages could be identified.
 
Holes within the oval fossa should also be distinguished from patency of the oval foramen. The normal arrangement of the oval fossa is such that the wall forming its floor, the so-called flap valve, is of greater area than the rim seen through the right atrium (Photo 4). With this arrangement, as long as left atrial pressure exceeds the pressure in the right atrium, there will be no shunting from left-to-right, even if the flap valve is not anatomically fused to the left atrial side of the fossa. Such failure of anatomic fusion of the flap valve with the left atrial side of the rims is found in up to one-third of the normal population [26]. These probe patent foramens (Photo 9), however, are not usually of clinical significance except in deep-sea divers, those at risk of paradoxical embolus, and now as a potential cause of migraine. Because of this recent evidence of clinical significance, there can now be a need for surgical closure of the probe patent foramen. It is also possible for the flap valve of the fossa to be aneurysmal, yet still close the interatrial communication.


Figure 9
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  		Photo 9 The picture shows the left atrial aspect of a probe patent oval foramen. The flap valve overlaps the rims of the fossa, but is not fused to the rims.

 
Of more interest in the past were the variants of lesions permitting interatrial shunting, but outside the confines of the oval foramen. The example of this arrangement is the so-called sinus venosus defect (Videos 11 and 12).


Figure 11
Click on image to view video		 Video 11 The transesophageal clip is taken at an angle of 0° to show the right atrium. The two right pulmonary veins are entering its posterior and lateral wall. The sinus venosus defect, outside the confines of the oval fossa, is a typical finding in patients with such partially anomalous pulmonary venous connection, although the pulmonary veins can also drain anomalously in partial fashion when the atrial septum is intact.
 

Figure 12
Click on image to view video		 Video 12 Cranio-caudal view, at 130°, through right atrium and its atrial appendage, shows the connection of the superior caval vein to the right atrium. The sinus venosus defect is clearly outside the normal interatrial septum, and shows how the superior caval vein opens to the left, as well as the right atrium.
 
This exists in the mouth of either the superior or inferior caval vein, most usually the superior vein [27, 28], and is associated with anomalous connection of the right upper pulmonary veins. It is the anomalous connection of the pulmonary veins that creates an extracardiac conduit outside the confines of the oval fossa (Schematic 2) so that, in most instances, the mouth of the superior caval vein looks directly into the cavities of both the right and the left atriums (Photo 10, Video 13).


Figure 2
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  		Schematic 2 The cartoons show how the sinus venosus defect (arrow in lower panel) creates a conduit between the atrial chambers, converting the superior rim of the oval fossa into a tube of atrial tissue filled with extracardiac fat. SCV – superior caval vein.

 

Figure 10
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  		Photo 10 The picture shows a superior sinus venosus defect viewed from the right atrium. Note the biatrial connection of the superior caval vein, the integrity of the oval fossa, and the probe passed through the atrial tube forming the superior rim of the oval fossa (see Schematic 2).

 

Figure 13
Click on image to view video		 Video 13 Progressive stages of surgical correction of a sinus venosus defect.
 
In rare circumstances, however, the sinus venosus defect can exist without biatrial connection of the superior caval vein (Photo 11).


Figure 11
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  		Photo 11 In this much rarer example of a superior sinus venosus defect, there is no overriding of the oval fossa by the mouth of the superior caval vein. The anomalous connection of the right pulmonary veins, however, still creates an interatrial communication outside the confines of the oval fossa.

 
The defect can also be found in the mouth of the inferior caval vein [29], with anomalous connection of the right lower pulmonary vein producing the necessary extraseptal communication, but this is much rarer than the superior defect.

The sinus venosus defect is closed via a right-sided atriotomy. After identification of all anomalously connected pulmonary veins, the suture line starts between the orifice of the most cranially connected pulmonary vein and the mouth of the superior caval vein. The suture line is then continued in such a fashion as to connect all pulmonary veins to the defect between the atrial chambers. In some cases, it may be necessary to enlarge the defect to avoid obstruction of the pulmonary venous pathway. When the orifice of the pulmonary veins is located in the superior caval vein, the patch has to be extended into the caval vein. To avoid obstruction of the systemic venous return, it may also be necessary to enlarge the superior caval vein itself with a patch. When inserting such a patch, the incision across the junction of the caval vein to the atrium should be avoided whenever possible, since the artery supplying the sinus node can be located in this area. Care has also to be taken to avoid the sinus node itself, placing the sutures relatively superficially.

The final defect permitting interatrial shunting outside the confines of the oval fossa is the so-called ‘coronary sinus defect’. Most usually associated with persistence of the left superior caval vein, the defect is produced by disappearance of the walls that usually separate the coronary sinus from the cavity of the left atrium [30]. Thus, the hole is, in effect, the mouth of the coronary sinus.

It is of surgical significance to establish whether or not the left superior caval vein is present and connects to the left atrium, and also whether there is a bridging vein joining the left caval vein, if present, to the superior caval vein. If such a bridging vein is present, and is of good calibre, then the mouth of the coronary sinus can be closed and the left caval vein ligated. In such an instance, coronary venous blood must be able to drain into the left atrium, as is always the case when the coronary sinus itself is unroofed. In case the coronary sinus is fenestrated rather than completely unroofed, however, it may be wise for the surgeon to completely unroof the coronary sinus. If a bridging vein is not present, an intraatrial repair should be performed, connecting the persistent left superior caval vein to the right atrium. In such a case, the precise technique used depends on the location of the orifice of the left caval vein. If it drains into a fenestrated coronary sinus, the fenestrations can be closed with a patch. If it drains into the roof of the left atrium, in contrast, the venous return must be redirected by tunnelling the orifice with a patch to the right atrium (Photo 12).


Figure 12
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  		Photo 12 The illustration shows a coronary sinus defect as viewed from the left atrium. There is total absence of the two walls that normally interpose between the coronary sinus and the cavity of the left atrium. In absence of a bridging vein, it would be wise for the surgeon to create a wall within the left atrium so as to join the mouth of the persistent left superior caval vein to the orifice of the coronary sinus (red dotted lines).

 

   Discussion
Top
 Summary
 Introduction
 Current approach
 Discussion
References
 
Interatrial communications may exist within the oval fossa, and outside the confines of the oval foramen. Whereas the surgical technique of closure of defects within the oval fossa is exclusively performed by direct suture or patch closure, there are a variety of technical approaches proposed for correction of sinus venosus defects and ostium primum defects. Outcome of surgical closure of holes within the oval fossa has been reported to be excellent in large series. Early mortality was <1.5% [20, 31, 32], and there was no residual shunt or recurrence of a shunt observed during follow-up examinations applying either minimally invasive strategies or conventional repair via midline sternotomy [33,34,35]. Data from a population-based study conducted in Finland demonstrate equal survival of 812 patients who had undergone surgical closure of an atrial septal defect during childhood compared to that of the general population [36]. The experience of the Mayo Clinic shows that if the defect is closed before the age of 25 years, there was no difference in survival rate up to 27 years postoperatively compared to that of an age-matched control population [2]. We could affirm these findings in our study population including 281 patients who underwent surgical secundum ASD closure when they were older than 30 years. Overall survival up to 29 years postoperatively was not different from an age and sex matched control population [6].

Whereas the results of repair of sinus venosus defect in terms of early and late mortality is similar to those of closure of defects within the oval fossa, the incidence of complications is higher. Stenoses of the superior caval vein and/or of the right pulmonary vein have been reported to occur in 10% of the patients. In addition, dysfunction of the sinus node has been observed in 7% of the patients, however, without the need for pacemaker implantation [37]. To avoid stenoses of the venous inflow, the double patch technique has shown to produce better results in terms of superior vena cava narrowing and gradient across the pulmonary vein without any increase in complications compared to correction by the use of a single intra-luminal patch [38]. Since sinus node dysfunction can occur postoperatively, particularly when an incision has been made across the superior vena cava/right atrial junction [28], some authors prefer the caval division technique (Warden procedure) for repair of sinus venosus atrial septal defect with partial anomalous pulmonary venous connection [39].



   Footnotes
 
{star} The authors have chosen to retain the copyright on this work. Back


   References
Top
 Summary
 Introduction
 Current approach
 Discussion
 References
 

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