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Extracardiac Interatrial Tunnel and Atrial Septal Defect Closed With a Single Atrial Septal Occluder in an Adolescent Girl
Canadian Journal of Cardiology 31 (2015) 1073.e17e1073.e19 www.onlinecjc.ca
Case Report
Extracardiac Interatrial Tunnel and Atrial Septal Defect Closed With a Single Atrial Septal Occluder in an Adolescent Girl Tsukasa Torigoe, MD, PhD,a Seiichi Sato, MD, PhD,a and Akiko Sanada, MD, PhDb a
Department of Pediatrics and Neonatology, Niigata City General Hospital, Niigata, Japan b
Department of Cardiology, Niigata City General Hospital, Niigata, Japan
ABSTRACT
RESUM E
In contrast to the atrial septal defect (ASD) as a congenital interatrial shunt, the extracardiac interatrial tunnel is extremely rare. We report the first percutaneous closure of this entity during closure of a secundum ASD after careful investigation of ischemic risk in a 15-year-old girl. The extracardiac interatrial tunnel could entail interatrial shunt, and, if misidentified as an ASD, could result in lethal procedural complications during surgical or catheter interventions. In our case, the tunnel was also morphologically similar to the cardiac arterial/venous system. Therefore, careful investigation of its potential relationship with coronary circulation was required before its closure.
Contrairement à la communication interauriculaire (CIA) comme le nital, le tunnel interauriculaire extrashunt interauriculaire conge cardiaque est extrêmement rare. Nous rapportons la première fere de cette entite durant la fermeture d’une meture par voie percutane CIA de type ostium secundum après l’examen minutieux du risque mique chez une fille de 15 ans. Le tunnel interauriculaire ische extracardiaque pourrait entraîner le shunt interauriculaire, et s’il est ment identifie comme e tant une CIA, il pourrait provoquer des errone tales durant la chirurgie ou les complications interventionnelles le ter. Dans le cas que nous pre sentons, le tunnel interventions par cathe tait e galement similaire sur le plan morphologique aux systèmes e riel et veineux. Par conse quent, l’examen minutieux de son lien arte tait ne cessaire avant sa potentiel avec la circulation coronarienne e fermeture.
We report the first percutaneous closure of an extremely rare entity, the extracardiac interatrial tunnel, during closure of an atrial septal defect (ASD) after careful investigation of the coronary circulation.
imaging showed that the tunnel connected the left atrium (LA) orifice and the right atrium (RA) anterior side, running between the RA and sinus of Valsalva and along the right atrioventricular groove and right coronary artery (Fig. 1C and D). Cardiac catheterization revealed a pulmonary/systemic flow ratio of 1.9 without pulmonary hypertension. The catheters were safely inserted into the ASD and the tunnel using careful TEE examination to avoid misidentification. Selective angiography in the tunnel and coronary angiography showed the tunnel had no connection to the coronary artery or vein (Fig. 1E and F). Therefore, a 9-mm septal occluder was deployed to close the ASD and the tunnel simultaneously. Before and after releasing it from the delivery wire, we carefully reconfirmed normal coronary angiography and electrocardiography. TEE showed the orifice was almost closed, without ASD leakage (Fig. 1B). At the latest follow-up, transthoracic echocardiography (TTE) and electrocardiography showed no residual flow or ischemic findings.
Case Report A 15-year-old girl was evaluated for indicated closure of a secundum ASD, diagnosed at 1 month of age, using an Amplatzer septal occluder (St Jude Medical, St Paul, MN). A 2/6 systolic murmur at the left second intercostal space was observed, without cyanosis. Transesophageal echocardiography (TEE) revealed not only the 6-mm diameter ASD at the anterosuperior septum but also an abnormal 3.8-mm diameter tunnel orifice at the superior septum to the ASD (Fig. 1A). The distance between them was 5.1 mm. Flow direction in the tunnel was largely left-to-right. Computed tomography Received for publication November 25, 2014. Accepted March 3, 2015. Corresponding author: Dr Tsukasa Torigoe, Department of Pediatrics and Neonatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata 950-1197, Japan. Tel.: þ81-25-281-5151; fax: þ81-25-281-5187. E-mail: [email protected] See page 1073.e19 for disclosure information.
Discussion The extracardiac interatrial tunnel associated with ASD was safely closed with careful investigation of ischemic risk. If
http://dx.doi.org/10.1016/j.cjca.2015.03.002 0828-282X/Ó 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
1073.e18
Canadian Journal of Cardiology Volume 31 2015
Figure 1. Transesophageal echocardiography showing (A) the atrial septal defect (arrow), the tunnel orifice (arrow head), and long sheath (dotted arrow); and (B) a nearly closed orifice after occluder device implantation. (C, D) Computed tomography image shows the atrial septal defect (arrow) and the tunnel (arrow head) connecting the left atrium (LA) and right atrium (RA), running parallel to the right coronary artery (dotted arrow). (E, F) Selective angiography in the tunnel revealed the tunnel between the LA and RA but no connection to the coronary artery or vein. Ao, aorta; RV, right ventricle.
extracardiac interatrial tunnels are misidentified as ASDs, there is a lethal risk of tunnel rupture during surgical or catheter intervention.1 In our case, TEE facilitated safe catheter manipulation. Only 2 cases with similar anatomical characteristics have been reported, both with no intervention. One was an adult with exertional dyspnea who had previously undergone surgical ASD closure. Cardiac catheterization revealed a tunnel connecting the posterior RA and LA, with RA and right ventricle volume overload (pulmonary/systemic flow ratio of 2.1).2 The other case was an infant with a chromosomal anomaly. Ventricular septal defect, patent ductus arteriosus, patent foramen ovale, and left superior vena cava were recognized using transthoracic echocardiography. The RA to LA appendage tunnel was not revealed until autopsy.1 These cases were associated with ASD,1,2 but the relation of the ASD to the aetiology of the tunnel is unknown. In our case, we considered that the tunnel could be the coronary venous system because of similar morphological features. The literature describes in some patients a RA coronary sinus for venous drainage of part of the RA and anterior right
ventricle, running parallel and 1-2 cm cephalad to the posterior or posterolateral tricuspid orifice and draining into the RA. The septal LA vein, running through the interatrial groove or superior interatrial muscle connection, also drains into the RA.3 An RA coronary sinus could explain the aetiology of the tunnel in the adult with dyspnea because of a similar tunnel course (posterior to the RA), and a septal LA vein would have a location similar to the location of the RA tunnel orifice (inferior to the fossa ovalis) in the infant with the chromosomal anomaly. However, each possibility differs from the actual case with respect to having a large LA or left atrial appendage orifice, respectively. Our case also differs from both previous cases because the tunnel in our case had a course anterior to the tricuspid orifice and a large LA orifice. However, because there are many variations in the coronary venous system and some anomalies, such as a coronary venous fistula, were also suspected, we carefully investigated the coronary circulation and ischemia during the procedure using angiography and electrocardiography. However, it was serendipitous that the tunnel in this case opened adequately into the RA and that the area of
Torigoe et al. Extracardiac Interatrial Tunnel
myocardium it drained was small because, otherwise, its closure on the LA aspect might have caused myocardial ischemia until venous collaterals developed. The extracardiac interatrial tunnel is poorly recognized but could entail hemodynamic and lethal procedural complications. The aetiology is unknown, but in our case its morphological feature required careful investigation of its potential relationship with coronary circulation during its closure. Disclosures The authors have no conflicts of interest to disclose.
1073.e19
References 1. Waldman JD, McFeeley P, Bornikova L. Right atrial tunnel to the left atrial appendage: a danger during balloon septostomy. Pediatr Cardiol 2001;22:397-9. 2. Kanadas¸i M, Ozbarlas N, Demirtas¸ M, et al. ‘Natural conduit’ between two atria associated with atrial septal defect. Int J Cardiovasc Imaging 2005;21:379-82. 3. Saremi F, Muresian H, Sánchez-Quintana D. Coronary veins: comprehensive CT-anatomic classification and review of variants and clinical implications. Radiographics 2012;32:E1-32.
Case Report
Extracardiac Interatrial Tunnel and Atrial Septal Defect Closed With a Single Atrial Septal Occluder in an Adolescent Girl Tsukasa Torigoe, MD, PhD,a Seiichi Sato, MD, PhD,a and Akiko Sanada, MD, PhDb a
Department of Pediatrics and Neonatology, Niigata City General Hospital, Niigata, Japan b
Department of Cardiology, Niigata City General Hospital, Niigata, Japan
ABSTRACT
RESUM E
In contrast to the atrial septal defect (ASD) as a congenital interatrial shunt, the extracardiac interatrial tunnel is extremely rare. We report the first percutaneous closure of this entity during closure of a secundum ASD after careful investigation of ischemic risk in a 15-year-old girl. The extracardiac interatrial tunnel could entail interatrial shunt, and, if misidentified as an ASD, could result in lethal procedural complications during surgical or catheter interventions. In our case, the tunnel was also morphologically similar to the cardiac arterial/venous system. Therefore, careful investigation of its potential relationship with coronary circulation was required before its closure.
Contrairement à la communication interauriculaire (CIA) comme le nital, le tunnel interauriculaire extrashunt interauriculaire conge cardiaque est extrêmement rare. Nous rapportons la première fere de cette entite durant la fermeture d’une meture par voie percutane CIA de type ostium secundum après l’examen minutieux du risque mique chez une fille de 15 ans. Le tunnel interauriculaire ische extracardiaque pourrait entraîner le shunt interauriculaire, et s’il est ment identifie comme e tant une CIA, il pourrait provoquer des errone tales durant la chirurgie ou les complications interventionnelles le ter. Dans le cas que nous pre sentons, le tunnel interventions par cathe tait e galement similaire sur le plan morphologique aux systèmes e riel et veineux. Par conse quent, l’examen minutieux de son lien arte tait ne cessaire avant sa potentiel avec la circulation coronarienne e fermeture.
We report the first percutaneous closure of an extremely rare entity, the extracardiac interatrial tunnel, during closure of an atrial septal defect (ASD) after careful investigation of the coronary circulation.
imaging showed that the tunnel connected the left atrium (LA) orifice and the right atrium (RA) anterior side, running between the RA and sinus of Valsalva and along the right atrioventricular groove and right coronary artery (Fig. 1C and D). Cardiac catheterization revealed a pulmonary/systemic flow ratio of 1.9 without pulmonary hypertension. The catheters were safely inserted into the ASD and the tunnel using careful TEE examination to avoid misidentification. Selective angiography in the tunnel and coronary angiography showed the tunnel had no connection to the coronary artery or vein (Fig. 1E and F). Therefore, a 9-mm septal occluder was deployed to close the ASD and the tunnel simultaneously. Before and after releasing it from the delivery wire, we carefully reconfirmed normal coronary angiography and electrocardiography. TEE showed the orifice was almost closed, without ASD leakage (Fig. 1B). At the latest follow-up, transthoracic echocardiography (TTE) and electrocardiography showed no residual flow or ischemic findings.
Case Report A 15-year-old girl was evaluated for indicated closure of a secundum ASD, diagnosed at 1 month of age, using an Amplatzer septal occluder (St Jude Medical, St Paul, MN). A 2/6 systolic murmur at the left second intercostal space was observed, without cyanosis. Transesophageal echocardiography (TEE) revealed not only the 6-mm diameter ASD at the anterosuperior septum but also an abnormal 3.8-mm diameter tunnel orifice at the superior septum to the ASD (Fig. 1A). The distance between them was 5.1 mm. Flow direction in the tunnel was largely left-to-right. Computed tomography Received for publication November 25, 2014. Accepted March 3, 2015. Corresponding author: Dr Tsukasa Torigoe, Department of Pediatrics and Neonatology, Niigata City General Hospital, 463-7 Shumoku, Chuo-ku, Niigata 950-1197, Japan. Tel.: þ81-25-281-5151; fax: þ81-25-281-5187. E-mail: [email protected] See page 1073.e19 for disclosure information.
Discussion The extracardiac interatrial tunnel associated with ASD was safely closed with careful investigation of ischemic risk. If
http://dx.doi.org/10.1016/j.cjca.2015.03.002 0828-282X/Ó 2015 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
1073.e18
Canadian Journal of Cardiology Volume 31 2015
Figure 1. Transesophageal echocardiography showing (A) the atrial septal defect (arrow), the tunnel orifice (arrow head), and long sheath (dotted arrow); and (B) a nearly closed orifice after occluder device implantation. (C, D) Computed tomography image shows the atrial septal defect (arrow) and the tunnel (arrow head) connecting the left atrium (LA) and right atrium (RA), running parallel to the right coronary artery (dotted arrow). (E, F) Selective angiography in the tunnel revealed the tunnel between the LA and RA but no connection to the coronary artery or vein. Ao, aorta; RV, right ventricle.
extracardiac interatrial tunnels are misidentified as ASDs, there is a lethal risk of tunnel rupture during surgical or catheter intervention.1 In our case, TEE facilitated safe catheter manipulation. Only 2 cases with similar anatomical characteristics have been reported, both with no intervention. One was an adult with exertional dyspnea who had previously undergone surgical ASD closure. Cardiac catheterization revealed a tunnel connecting the posterior RA and LA, with RA and right ventricle volume overload (pulmonary/systemic flow ratio of 2.1).2 The other case was an infant with a chromosomal anomaly. Ventricular septal defect, patent ductus arteriosus, patent foramen ovale, and left superior vena cava were recognized using transthoracic echocardiography. The RA to LA appendage tunnel was not revealed until autopsy.1 These cases were associated with ASD,1,2 but the relation of the ASD to the aetiology of the tunnel is unknown. In our case, we considered that the tunnel could be the coronary venous system because of similar morphological features. The literature describes in some patients a RA coronary sinus for venous drainage of part of the RA and anterior right
ventricle, running parallel and 1-2 cm cephalad to the posterior or posterolateral tricuspid orifice and draining into the RA. The septal LA vein, running through the interatrial groove or superior interatrial muscle connection, also drains into the RA.3 An RA coronary sinus could explain the aetiology of the tunnel in the adult with dyspnea because of a similar tunnel course (posterior to the RA), and a septal LA vein would have a location similar to the location of the RA tunnel orifice (inferior to the fossa ovalis) in the infant with the chromosomal anomaly. However, each possibility differs from the actual case with respect to having a large LA or left atrial appendage orifice, respectively. Our case also differs from both previous cases because the tunnel in our case had a course anterior to the tricuspid orifice and a large LA orifice. However, because there are many variations in the coronary venous system and some anomalies, such as a coronary venous fistula, were also suspected, we carefully investigated the coronary circulation and ischemia during the procedure using angiography and electrocardiography. However, it was serendipitous that the tunnel in this case opened adequately into the RA and that the area of
Torigoe et al. Extracardiac Interatrial Tunnel
myocardium it drained was small because, otherwise, its closure on the LA aspect might have caused myocardial ischemia until venous collaterals developed. The extracardiac interatrial tunnel is poorly recognized but could entail hemodynamic and lethal procedural complications. The aetiology is unknown, but in our case its morphological feature required careful investigation of its potential relationship with coronary circulation during its closure. Disclosures The authors have no conflicts of interest to disclose.
1073.e19
References 1. Waldman JD, McFeeley P, Bornikova L. Right atrial tunnel to the left atrial appendage: a danger during balloon septostomy. Pediatr Cardiol 2001;22:397-9. 2. Kanadas¸i M, Ozbarlas N, Demirtas¸ M, et al. ‘Natural conduit’ between two atria associated with atrial septal defect. Int J Cardiovasc Imaging 2005;21:379-82. 3. Saremi F, Muresian H, Sánchez-Quintana D. Coronary veins: comprehensive CT-anatomic classification and review of variants and clinical implications. Radiographics 2012;32:E1-32.