Asian Cardiovascular and Thoracic Annals http://aan.sagepub.com/
Percutaneous closure of atrial septal defect with situs solitus and dextrocardia Mohammed Omar Galal, Muhammad Arif Khan and Milad El-Segaier Asian Cardiovascular and Thoracic Annals published online 29 October 2013 DOI: 10.1177/0218492313500498 The online version of this article can be found at: http://aan.sagepub.com/content/early/2013/10/28/0218492313500498
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Case Study
Percutaneous closure of atrial septal defect with situs solitus and dextrocardia
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313500498 aan.sagepub.com
Mohammed Omar Galal1,2, Muhammad Arif Khan1 and Milad El-Segaier1,3
Abstract Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia.
Keywords Dextrocardia, situs solitus, Atrial septal defect secundum, percutaneous closure, technical difficulties
Introduction Percutaneous closure of secundum atrial septal defect (ASD) in cases of dextrocardia and situs inversus has been reported in only a few cases.1 To the best of our knowledge, secundum ASD device closure in a case of isolated dextrocardia with situs solitus has never been reported.
Case report Echocardiography in a 19-month-old girl, weighing 9.5 kg, showed situs solitus, dextrocardia, and a moderate secundum ASD (Figure 1a). Transesophageal echocardiography (TEE) showed a fenestrated ASD of 13 mm with acceptable rims. The right femoral vein and right femoral artery were accessed with a 5F sheath and 20G Vygon catheter, respectively. We decided to close the ASD using an Amplatzer Cribriform Occluder, size 18 mm (AGA Medical, Plymouth, MN, USA). The device was selected according to standard guidelines, understanding that this device, unlike the regular one, does not stent the ASD. Hence, 18 mm is the diameter of the device itself and not of the central part. We assumed that in the lateral view, the left atrium could be reached by orienting the catheter posteriorly, but we discovered that this was not the case in our patient. Only after several attempts did we find that the orientation of the interatrial septum was oblique (from posteroinferior to anterosuperior), thus the left
atrium was positioned more anterosuperiorly than expected. Therefore, when probing the left pulmonary veins in lateral fluoroscopy views, they appeared more anterior than anticipated (Figure 2c). Although confirming the position of the device, TEE could not help us to determine how the interatrial septum was oriented. Nevertheless, it confirmed that the device was not impinging on the atrioventricular valves, and away from the aorta and pulmonary veins. Despite these difficulties, we could deploy and release the device in a stable and safe position (Figure 3a). Postcatheterization chest radiography showed the device in a good position. An electrocardiogram showed sinus rhythm and no heart block, and echocardiography showed the device in a good position with no residual shunt (Figure 4). The device was away from venae cavae, aorta, and the atrioventricular valves. The patient was discharged home on aspirin with general post-ASD device instructions and the need for endocarditis prophylaxis for 6 months. 1 Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia 2 University of Essen, Essen, Germany 3 Skane University Hospital, Lund, Sweden
Corresponding author: Muhammad Arif Khan, FCPS, King Fahad Medical City, Prince Salman Heart Center, PO Box 59046, Riyadh 11525, Saudi Arabia. Email: [email protected]
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Figure 1. (a) Subcostal view showing dextrocardia and a fenestrated secundum ASD. (b) Subcostal view colour image showing dextrocardia and fenestrated ASD secundum with left to right shunt. ASD: atrial septal defect; LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle.
Figure 2. Frozen-frame angiograms of our patient: (a) anteroposterior view showing dextrocardia and the direction of the device during deployment, with the sheath pointing superiorly; (b) anteroposterior view showing dextrocardia with the long sheath and exchange wire positioned in the left upper pulmonary vein; (c) lateral view showing the long sheath and exchange wire in the left upper pulmonary vein, pointing anteriorly. Frozen-frame angiograms of a patient with levocardia: (d) anteroposterior view showing the exchange wire in the left lower pulmonary vein; (e) lateral view showing the exchange wire in the left lower pulmonary vein, but in contrast to dextrocardia, pointing posteriorly.
Discussion Only a few cases have been reported of interventional closure of secundum ASD associated with situs inversus and dextrocardia.2–4 Percutaneous device closure of
multiple ASD associated with situs inversus and dextrocardia has also been reported.4 In addition, device closure of a secundum ASD has been reported in a case of dextrocardia with a systemic venous anomaly in the form of interrupted inferior vena cava.5 We assumed
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Figure 2. Continued.
Figure 3. (a) Chest radiograph of our patient in lateral view, showing vertical orientation of the atrial septal defect device in situs solitus, dextrocardia. (b) Chest radiograph in lateral view of a patient with levocardia, showing horizontal orientation of the atrial septal defect device. The arrow in each figure indicates orientation of the centre of the device, notice the difference in orientation between (a) (Situs solitus, dextrocardia) and (b) (situs solitus, levocardia).
that we would have some technical issues similar to those in situs inversus, and with the use of lateral fluoroscopy for guidance, we might overcome these difficulties. While trying to access the left atrium, we thought that in the lateral view, the orientation of the tip of the catheter should point posteriorly. However, the orientation of the catheter in our patient was more anterosuperior. Additionally, to our surprise, every time when we advanced the catheter over the exchange wire to position it in the upper left pulmonary vein, in the lateral view, the catheter tip ended up anterosuperiorly. TEE could not help to overcome this situation. We needed to orient ourselves by the pressure tracing and some hand contrast injections. In our case with multiple
small holes in the atrial septum, this helped us to position the device, without much fear. As the ASD was a fenestrated type, positioning the device through a small hole gave us reassurance that the probability of embolization is extremely low. The interatrial septum plane is oblique in cases of levocardia, with the left atrium more posterior than the right atrium. This relationship changes in certain conditions such as dextrocardia and scoliosis.6 In dextrocardia, the interatrial septum is directed anteriorly and to the right, with the morphologic right atrium situated to the right and slightly posteriorly, and the morphologic left atrium to the left and slightly anteriorly.7 Our case highlights the technical difficulties encountered
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Figure 4. Parasternal short-axis view showing the device in position, sandwiching the interatrial septum well, with normal superior vena caval (SVC) flow without obstruction.
during device closure of ASD secundum in situs solitus associated with dextrocardia. Attention should be paid to the abnormal orientation of the interatrial septum and its relationship to the right and left atria. Guidance by hand injections and pressure tracing may be more helpful than TEE. To the best of our knowledge, this is the first report of successful ASD device closure in a patient with situs solitus and dextrocardia. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Masura J, Gavora P and Podnar T. Long-term outcome of transcatheter secundum-type atrial septal defect closure using Amplatzer septal occluders. J Am Coll Cardiol 2005; 45: 505–507.
2. Hakim F, Madani A, Samara Y, Ata IA, et al. Transcatheter closure of atrial septal defect in a patient with dextrocardia using the Amplatzer septal occluder. Cathet Cardiovasc Diagn 1998; 43: 291–294. 3. Chen CY, Lee CH, Yang MW, Chung HT, Hsieh IC and Ho AC. Usefulness of transesophageal echocardiography for transcatheter closure of ostium secundum atrial septum defect with the Amplatzer septal occluder. Chang Gung Med J 2005; 28: 837–845. 4. Raj BS, George OK and Chandy ST. Transcatheter closure of multiple atrial septal defects in situs inversus with dextrocardia. Indian Heart J 2008; 60: 152–154. 5. Kashour TS, Latroche B, Elhoury ME and Galal MO. Successful percutaneous closure of a secundum atrial septal defect through femoral approach in a patient with interrupted inferior vena cava. Congenit Heart Dis 2010; 5: 620–623. 6. Ho SY, Mccarthy KP and Faletra FF. Anatomy of left atrium for interventional echocardiography. Eur J Echocardiogr 2011; 12: i11–i15. 7. Lev M, Liberthson RR, Eckner FA and Arcilla RA. Pathologic anatomy of dextrocardia and its clinical implications. Circulation 1968; 37: 975–999.
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Percutaneous closure of atrial septal defect with situs solitus and dextrocardia Mohammed Omar Galal, Muhammad Arif Khan and Milad El-Segaier Asian Cardiovascular and Thoracic Annals published online 29 October 2013 DOI: 10.1177/0218492313500498 The online version of this article can be found at: http://aan.sagepub.com/content/early/2013/10/28/0218492313500498
Published by: http://www.sagepublications.com
On behalf of:
The Asian Society for Cardiovascular Surgery
Additional services and information for Asian Cardiovascular and Thoracic Annals can be found at: Email Alerts: http://aan.sagepub.com/cgi/alerts Subscriptions: http://aan.sagepub.com/subscriptions Reprints: http://www.sagepub.com/journalsReprints.nav Permissions: http://www.sagepub.com/journalsPermissions.nav
>> OnlineFirst Version of Record - Oct 29, 2013 What is This?
Downloaded from aan.sagepub.com at King Mongkut's University of Technology,Thonburi on October 19, 2014
XML Template (2013) [27.7.2013–9:40am] //blrnas3/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/130399/APPFile/SG-AANJ130399.3d
(AAN)
[1–4] [PREPRINTER stage]
Case Study
Percutaneous closure of atrial septal defect with situs solitus and dextrocardia
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313500498 aan.sagepub.com
Mohammed Omar Galal1,2, Muhammad Arif Khan1 and Milad El-Segaier1,3
Abstract Percutaneous closure of secundum atrial septal defect associated with situs solitus and dextrocardia has not been reported previously. We describe the technical difficulties encountered during transcatheter closure of a secundum atrial septal defect in a 19-month-old girl with situs solitus and dextrocardia.
Keywords Dextrocardia, situs solitus, Atrial septal defect secundum, percutaneous closure, technical difficulties
Introduction Percutaneous closure of secundum atrial septal defect (ASD) in cases of dextrocardia and situs inversus has been reported in only a few cases.1 To the best of our knowledge, secundum ASD device closure in a case of isolated dextrocardia with situs solitus has never been reported.
Case report Echocardiography in a 19-month-old girl, weighing 9.5 kg, showed situs solitus, dextrocardia, and a moderate secundum ASD (Figure 1a). Transesophageal echocardiography (TEE) showed a fenestrated ASD of 13 mm with acceptable rims. The right femoral vein and right femoral artery were accessed with a 5F sheath and 20G Vygon catheter, respectively. We decided to close the ASD using an Amplatzer Cribriform Occluder, size 18 mm (AGA Medical, Plymouth, MN, USA). The device was selected according to standard guidelines, understanding that this device, unlike the regular one, does not stent the ASD. Hence, 18 mm is the diameter of the device itself and not of the central part. We assumed that in the lateral view, the left atrium could be reached by orienting the catheter posteriorly, but we discovered that this was not the case in our patient. Only after several attempts did we find that the orientation of the interatrial septum was oblique (from posteroinferior to anterosuperior), thus the left
atrium was positioned more anterosuperiorly than expected. Therefore, when probing the left pulmonary veins in lateral fluoroscopy views, they appeared more anterior than anticipated (Figure 2c). Although confirming the position of the device, TEE could not help us to determine how the interatrial septum was oriented. Nevertheless, it confirmed that the device was not impinging on the atrioventricular valves, and away from the aorta and pulmonary veins. Despite these difficulties, we could deploy and release the device in a stable and safe position (Figure 3a). Postcatheterization chest radiography showed the device in a good position. An electrocardiogram showed sinus rhythm and no heart block, and echocardiography showed the device in a good position with no residual shunt (Figure 4). The device was away from venae cavae, aorta, and the atrioventricular valves. The patient was discharged home on aspirin with general post-ASD device instructions and the need for endocarditis prophylaxis for 6 months. 1 Prince Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia 2 University of Essen, Essen, Germany 3 Skane University Hospital, Lund, Sweden
Corresponding author: Muhammad Arif Khan, FCPS, King Fahad Medical City, Prince Salman Heart Center, PO Box 59046, Riyadh 11525, Saudi Arabia. Email: [email protected]
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Figure 1. (a) Subcostal view showing dextrocardia and a fenestrated secundum ASD. (b) Subcostal view colour image showing dextrocardia and fenestrated ASD secundum with left to right shunt. ASD: atrial septal defect; LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle.
Figure 2. Frozen-frame angiograms of our patient: (a) anteroposterior view showing dextrocardia and the direction of the device during deployment, with the sheath pointing superiorly; (b) anteroposterior view showing dextrocardia with the long sheath and exchange wire positioned in the left upper pulmonary vein; (c) lateral view showing the long sheath and exchange wire in the left upper pulmonary vein, pointing anteriorly. Frozen-frame angiograms of a patient with levocardia: (d) anteroposterior view showing the exchange wire in the left lower pulmonary vein; (e) lateral view showing the exchange wire in the left lower pulmonary vein, but in contrast to dextrocardia, pointing posteriorly.
Discussion Only a few cases have been reported of interventional closure of secundum ASD associated with situs inversus and dextrocardia.2–4 Percutaneous device closure of
multiple ASD associated with situs inversus and dextrocardia has also been reported.4 In addition, device closure of a secundum ASD has been reported in a case of dextrocardia with a systemic venous anomaly in the form of interrupted inferior vena cava.5 We assumed
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Figure 2. Continued.
Figure 3. (a) Chest radiograph of our patient in lateral view, showing vertical orientation of the atrial septal defect device in situs solitus, dextrocardia. (b) Chest radiograph in lateral view of a patient with levocardia, showing horizontal orientation of the atrial septal defect device. The arrow in each figure indicates orientation of the centre of the device, notice the difference in orientation between (a) (Situs solitus, dextrocardia) and (b) (situs solitus, levocardia).
that we would have some technical issues similar to those in situs inversus, and with the use of lateral fluoroscopy for guidance, we might overcome these difficulties. While trying to access the left atrium, we thought that in the lateral view, the orientation of the tip of the catheter should point posteriorly. However, the orientation of the catheter in our patient was more anterosuperior. Additionally, to our surprise, every time when we advanced the catheter over the exchange wire to position it in the upper left pulmonary vein, in the lateral view, the catheter tip ended up anterosuperiorly. TEE could not help to overcome this situation. We needed to orient ourselves by the pressure tracing and some hand contrast injections. In our case with multiple
small holes in the atrial septum, this helped us to position the device, without much fear. As the ASD was a fenestrated type, positioning the device through a small hole gave us reassurance that the probability of embolization is extremely low. The interatrial septum plane is oblique in cases of levocardia, with the left atrium more posterior than the right atrium. This relationship changes in certain conditions such as dextrocardia and scoliosis.6 In dextrocardia, the interatrial septum is directed anteriorly and to the right, with the morphologic right atrium situated to the right and slightly posteriorly, and the morphologic left atrium to the left and slightly anteriorly.7 Our case highlights the technical difficulties encountered
Downloaded from aan.sagepub.com at King Mongkut's University of Technology,Thonburi on October 19, 2014
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Figure 4. Parasternal short-axis view showing the device in position, sandwiching the interatrial septum well, with normal superior vena caval (SVC) flow without obstruction.
during device closure of ASD secundum in situs solitus associated with dextrocardia. Attention should be paid to the abnormal orientation of the interatrial septum and its relationship to the right and left atria. Guidance by hand injections and pressure tracing may be more helpful than TEE. To the best of our knowledge, this is the first report of successful ASD device closure in a patient with situs solitus and dextrocardia. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Masura J, Gavora P and Podnar T. Long-term outcome of transcatheter secundum-type atrial septal defect closure using Amplatzer septal occluders. J Am Coll Cardiol 2005; 45: 505–507.
2. Hakim F, Madani A, Samara Y, Ata IA, et al. Transcatheter closure of atrial septal defect in a patient with dextrocardia using the Amplatzer septal occluder. Cathet Cardiovasc Diagn 1998; 43: 291–294. 3. Chen CY, Lee CH, Yang MW, Chung HT, Hsieh IC and Ho AC. Usefulness of transesophageal echocardiography for transcatheter closure of ostium secundum atrial septum defect with the Amplatzer septal occluder. Chang Gung Med J 2005; 28: 837–845. 4. Raj BS, George OK and Chandy ST. Transcatheter closure of multiple atrial septal defects in situs inversus with dextrocardia. Indian Heart J 2008; 60: 152–154. 5. Kashour TS, Latroche B, Elhoury ME and Galal MO. Successful percutaneous closure of a secundum atrial septal defect through femoral approach in a patient with interrupted inferior vena cava. Congenit Heart Dis 2010; 5: 620–623. 6. Ho SY, Mccarthy KP and Faletra FF. Anatomy of left atrium for interventional echocardiography. Eur J Echocardiogr 2011; 12: i11–i15. 7. Lev M, Liberthson RR, Eckner FA and Arcilla RA. Pathologic anatomy of dextrocardia and its clinical implications. Circulation 1968; 37: 975–999.
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