Ann Thorac Surg 2000;69:635-637
© 2000 The Society of Thoracic Surgeons
Case Reports
Aorticoright atrial tunnel
Afksendiyos Kalangos, MD, PhDa,
Maurice Beghetti, MDb,
Dominique Vala, MDa,
Said Chraibi, MDb,
Bernard Faidutti, MDa
a Clinics for Cardiovascular Surgery, University Cantonal Hospital of Geneva, Geneva, Switzerland
b Clinics for Pediatric Cardiology, University Cantonal Hospital of Geneva, Geneva, Switzerland
Address reprint requests to Dr Kalangos, Clinic For Cardiovascular Surgery, University Cantonal Hospital of Geneva, 24, rue Micheli-du-Crest, 1211 Geneva 14, Switzerland
 |
Abstract
|
|---|
Two unusual cases of aorticoright atrial tunnel are described. Both patients were referred to our institution for evaluation of a continuous heart murmur best heard along the right upper sternal border. Ascending aortography showed the tunnel taking its origin from the aortic root and entering the right atrium through a tortuous link. Both patients underwent surgical closure. In addition, a review of similar cases in the literature is presented.
|
Introduction
|
|---|
The aorticoright atrial tunnel is a rare congenital anomaly first described by Otero Coto and colleagues in 1980 [1]. In this pathology, contrary to congenital coronary arteriovenous and coronary–cameral fistulas, the shunt is established through a vascular link arising from the aortic root and terminating in the right atrium (RA). We present two new patients with this unusual communication and review the five previously reported patients to better clarify its characteristics.
|
Case reports
|
|---|
Patient 1
An 18-year-old Ethiopian man was referred to our institution for evaluation of a continuous grade 4/6 heart murmur best heard along the right upper sternal border. A two-dimensional echocardiography identified a structure coursing from the left sinus of Valsalva toward the dilated RA. Aortography revealed moderate aortic insufficiency and the presence of a large tunnel originating from the left sinus of Valsalva, passing posterior to the aortic root, and terminating in the roof of the RA. QP/QS was estimated at 1.5/1. Coronary angiography revealed normal coronary arteries, although no sinus node artery could be identified. During operation, exploration of the aortic root revealed a tricuspid aortic valve with a moderately dilated aortic annulus and left sinus of Valsalva. The orifice of the tunnel, 7 mm in diameter, was located in the left sinus of Valsalva, 7 mm to the right of the left coronary ostium. An oblique right atriotomy revealed an orifice 5 mm in diameter in the superior aspect of the interatrial septum, 1.5 cm below the orifice of the superior vena cava (SVC). This orifice was obliterated by a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected up to its aortic origin and cut from the wall of the dilated sinus of Valsalva. The defect was then sutured with a 4-0 monofilament running suture.
Patient 2
An asymptomatic 7-year-old Moroccan boy was referred to our institution for evaluation of a heart murmur. Physical examination was normal except for a grade 2/6 continuous murmur best heard along the right upper sternal border. A two-dimensional echocardiogram revealed a large tunnel originating from the left sinus of Valsalva, then taking an abnormal course to end in the RA. Diagnosis was confirmed by aortography and coronary angiography. Injection of contrast material into the aortic root resulted in rapid filling of a large canal taking origin from the left sinus of Valsalva (Fig 1A). This canal passed posterior to the aortic root, terminating in the roof of the RA. Selective injection of contrast material into both coronary arteries appeared normal, although no sinus node artery could be identified. QP/QS was estimated at 1.3/1. During operation, exploration of the aortic root revealed a normal tricuspid aortic valve and a slightly dilated left sinus of Valsalva. From the wall of the left sinus of Valsalva, an orifice located 3 mm to the right of the left coronary artery ostium was identified. This orifice, 5 mm in diameter, was the origin of a tunnel running posterior to the aortic root along the roof of the left atrium before disappearing near the RA–SVC junction (Fig 1B). An oblique right atriotomy revealed a windsocklike orifice 3 mm in diameter, in the superior aspect of the interatrial septum, 1 cm below the orifice of the SVC. This orifice was then longitudinally incised, revealing an aneurysm of 2 to 2.5 cm in diameter located in the superior aspect of the interatrial septum, extending posteriorly toward the junction of the SVC with the right atrial roof. First, the aneurysmal formation was obliterated by sewing its anterior and posterior walls together with a 4-0 monofilament running suture. Thereafter, the tunnel was completely dissected from its origin at the left sinus of Valsalva to the RA–SVC junction and then ligated by a 5-0 monofilament at both ends.
View larger version (75K):
[in this window]
[in a new window]
|
Fig 1. (A) Selective injection of contrast material into the aorta shows a large tortuous communication (white arrow) coursing rightward and posteriorly to connect with the right atrium. Note the aneurysmal dilatation of the tunnel (black arrows) before it enters the right atrium. (B) Intraoperative photograph showing the aorticoright atrial tunnel (white arrow) dissected along the left atrial roof. (AA = ascending aorta; VCS = cannulated superior vena cava.)
|
|
|
Comment
|
|---|
Literature concerning aorticoright atrial communications is limited to 7 patients, including both of ours. The communication between the aortic root and the RA arose from above the left sinus of Valsalva in 4 patients [2], from above the noncoronary sinus in 1 patient [1], and from inside the left sinus in both of our patients. The communication terminated in the lateral aspect of the RA in 1 patient, in the SVC near its junction with the RA in 2 patients, and in the superior aspect of the RA in 4 patients, including ours. We believe that the communication arising from a diverticulum of the left sinus of Valsalva, which also gave rise to the left main coronary artery in 2 patients and independently to the left circumflex and anterior descending arteries in 2 other patients [2], cannot be considered as a true connection of the left coronary artery with the RA, therefore, contesting the use of the term coronary-cameral fistula. Moreover, the communication arose independently from the left coronary artery in the other 3 patients and the histologic examination in our patients demonstrated that its tissue is similar to the aortic one. The distribution of both coronary arteries appeared normal in all patients, although no sinus node artery could be identified in 4 patients on selective coronary angiograms, including our patients [1, 2]. No comment about its presence was made in the remaining 3 patients. This sinus node artery, originating in about 55% of hearts from the right coronary artery and in 45% of hearts from the left circumflex artery, in rare instances may take its origin directly from the aortic root [3]. Although no sinus node dysfunction was present in any patient, fistulous involvement of the sinus node artery arising from the left sinus of Valsalva or above was speculated on explaining the course of the tunnel in some patients [2]. The tunnel showed aneurysmal dilatation in 2 patients. In our second patient, the tunnel presented an aneurysmal dilatation in the superior aspect of the interatrial septum before entering the RA through a stenotic ostium of 3 mm in diameter. In the remaining patients, the tunnel was large and tortuous. The wall of the tunnel presented calcifications in 2 patients [1, 2]. In only 1 patient was there an associated cardiac lesion, which was that of an absent right SVC and the presence of a large left SVC [1].
All patients were essentially asymptomatic. A continuous murmur was best heard at the right upper sternal border in 6 patients including both of ours, and in the left infraclavicular area in 1 patient [1, 2]. This murmur led cardiologists to further investigate and hence establish diagnosis in 6 patients, including ours [2]. In the other patient, the discovery of an anomalous mediastinal structure on chest roentgenogram forced surgeons to perform exploratory thoracotomy during which they noted the presence of a mediastinal mass with a continuous thrill requiring additional investigations [1]. Although the tunnel could be readily identified by two-dimensional echocardiography, retrograde aortography combined with selective coronary angiography was the most appropriate diagnostic tool [2].
Surgical treatment was performed in 6 patients, including ours [1, 2]. Although the need for operative closure in asymptomatic patients remains controversial, we recommend the closure of an aorticoright atrial tunnel, as we do for all congenital coronary fistulas. We stress the low rate of surgical complications in the asymptomatic patient. The risk of continued patency of the communication with age could become manifest by volume overload of both ventricles, bacterial endocarditis, aneurysm formation, or spontaneous rupture. Although coil embolization could be a possible alternative treatment for this condition, surgical treatment also has the benefit of reconstructing the dilated sinus of Valsalva or minimizing the likelihood of further sinus dilatation by ligating the tunnel close to the sinus.
The aortic origin of the tunnel was confirmed by aortotomy and the atrial end by right atriotomy in 5 patients, including ours [2]. In 1 patient with aneurysmal dilatation of the tunnel, the aortic and right atrial orifices were confirmed and closed through aneurysmotomy [1]. In 2 patients, the tunnel was opened to the right of the aorta and the incision was then extended toward its aortic origin [2]. The orifice and tunnel were oversewn flush with the sinus, with care taken to protect the coronary ostium. In none of the patients was the dilated sinus of Valsalva obliterated to avoid compromising the coronary blood flow or distorting the adjacent aortic valve [1, 2]. However, the persistence of a dilated sinus of Valsalva constitutes concern and requires further follow-up to determine its evolution.
According to the above described anatomic grounds of an aorticoright atrial tunnel, the differential diagnosis can only include the possibility of a ruptured aneurysm of the sinus of Valsalva or the right coronary artery [4], which can become more confounding if the tunnel presents aneurysmal dilatation and has its origin inside the sinus.
In conclusion, the aorticoright atrial tunnel should be included in the clinical differential diagnosis of continuous murmurs usually best heard at the right upper sternal border. Ascending aortography combined with selective coronary angiography is the best diagnostic tool, showing the tunnel taking its origin from the aortic root and entering the RA through a tortuous link. Surgical closure should be recommended soon after diagnosis, as the continued patency of the tunnel may predispose patients to increasing risks and surgical mortality with age.
|
References
|
|---|
-
Otero Coto E., Caffarena J.M., Such M., Marques J.L. Aorta-right atrial communication. Report of an unusual case. J Thorac Cardiovasc Surg 1980;80:941-944.[Abstract]
-
Rosenberg H., Williams W.G., Trusler G.A., et al. Congenital aortico-right atrial communication. The dilemma of differentiation from coronary-cameral fistula. J Thorac Cardiovasc Surg 1986;91:841-847.[Abstract]
-
James T.N. The sinus node. Am J Cardiol 1977;40:965-986.[Medline]
-
Gray L.A., McMartin D.E. Surgical treatment of coronary artery aneurysm with rupture into the right atrium. J Thorac Cardiovasc Surg 1977;74:455-460.[Abstract]
Accepted for publication July 1, 1999.
This article has been cited by other articles:
|
|
|
|
 
C. Turkay, I. Golbasi, A. Belgi, S. Tepe, and O. Bayezid
Aorta-right atrial tunnel
J. Thorac. Cardiovasc. Surg.,
May 1, 2003;
125(5):
1058 - 1060.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
