Catheterization and Cardiovascular Interventions 86:773–776 (2015)
Percutaneous Closure of a Hemodynamically Significant Connection Between the Inferior Vena Cava and the Left Atrium Jens Wiebe, MD, Johannes Rixe, MD, and Holger Nef,* MD Objective and Background: A connection between the inferior vena cava (IVC) and the left atrium (LA) can occur as a rare complication after surgical atrial septum defect (ASD) repair. We demonstrate the first case of a percutaneous closure of this connection. Methods and Results: A 67-year-old female was admitted to hospital due to exertional dyspnea. A history of a surgical ASD repair in 1960 and 1966 with a residual shunt was already known. Transesophageal echocardiography and a CT scan revealed a hemodynamically significant drainage of the IVC into the LA. This connection was successfully closed percutaneously with an AMPLATEZR Duct Occluder I (St. Jude Medical, St. Paul, MN). Post-procedural CT-scan and transthoracic echocardiography demonstrated a stable position and there was also no evidence of a residual shunt. The patient reported a significant reduction of exertional dyspnea. Conclusion: Percutaneous closure of an IVC to LA connection in this case was safe and feasible. The decision about which device is optimal must be made on an individual basis. VC 2015 Wiley Periodicals, Inc.
Key words: vena cava; closure; AV fistula/AVM; interventional devices/innovation
INTRODUCTION
A rare complication after surgical atrial septal defect (ASD) repair is drainage of the inferior vena cava (IVC) into the left atrium (LA), which has been described only anecdotally in case reports [1–4]. We present the first documented case in which a connection between the IVC and the LA was closed percutaneously.
CASE REPORT
We describe the case a 67-year-old female with exertional dyspnea (New York Heart Association Class III) for several years. The patient has a history of a surgical ASD repair in 1960 and 1966 with a residual shunt. Although attempts were made to retrieve the records, these were unsuccessful and thus the exact technique used and the reason for the second surgery are unknown. Furthermore, the patient suffered from atrial fibrillation and was treated with a vitamin K antagonist, a beta-blocker, and digoxin. No other rhythm disturbances have been noted. Transesophageal echocardiography (TEE) confirmed the residual ASD, which was located inferiorly, and a subsequent CT scan specified it as a connection between the IVC and C 2015 Wiley Periodicals, Inc. V
the LA (Fig. 1). The ASD was fully visible in both imaging modalities and its size was 10 mm measured by TEE as well as by CT (Fig. 2). In addition, TEE demonstrated slight right heart dilatation (diameter right ventricle 33 mm, right atrium 36 mm) and a normal systolic pulmonary pressure of 27 mm Hg. Due to the hemodynamically relevant shunt volume (Qp:Qs 1.8) and the increase in exertional dyspnea, we decided to treat the connection percutaneously. The AMPLATZER Duct Occluder I (St. Jude Medical, St. Paul, MN) appeared to be suitable for closure. The procedure was performed under TEE and fluoroscopic guidance. Vessel access was gained via the right femoral vein. The Department of Cardiology, Medizinische Klinik I, University of Giessen, Giessen 35392, Germany Conflict of interest: Nothing to report. *Correspondence to: Prof. Dr. med. Holger Nef, Medizinische Klinik I, Department of Cardiology, University of Giessen, Klinikstrasse 33, 35392 Giessen, Germany E-mail: [email protected] Received 10 May 2014; Revision accepted 3 January 2015 DOI: 10.1002/ccd.25825 Published online 12 February 2015 in Wiley Online Library (wileyonlinelibrary.com)
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Wiebe et al.
Fig. 1. Pre-procedural CT images illustrating the connection (*) between the left atrium and the inferior vena cava. A: Three-dimensional volume-rendering technique (VRT) image of the ASD. B: Multiplanar reformat (MPR) image of the defect (*) with a reconstructed slice thickness of 0.75 mm. LA 5 left atrium, RA 5 right atrium, IVC 5 inferior vena cava. *Marks connection between IVC and LA. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. Sizing of the defect on CT and TEE. Sizing was performed on CT by adjusting two planes (sagittal and transversal) in the orthogonal and longitudinal orientation to the defect, which yielded an in-plane view of the defect in the coronal view (A). Defect size was 0.91 cm 3 1.21 cm. Additional TEE sizing was performed in an up-esophageal view at 75 degrees (B) and the defect was measured at 1.03 cm
defect was measured with a sizing balloon and had a diameter of 12.7 mm and a length of 6.6 mm. The margin of such defects could consist of soft tissue in which sufficient stabilization of the device is not possible. This discrimination is not possible by TEE or CT and thus additional balloon sizing was necessary. The first attempt to place a 10/12 mm occluder failed, but after another attempt a 12/14 mm occluder was
implanted (Fig. 3). The wiggle test confirmed a stable position and the device was released successfully. Predischarge TTE displayed no residual shunt and CT scan approved the correct position. There was also no evidence of LA protrusion, although some insignificant protrusion was observed at the IVC (Fig. 4). At the first follow-up examination, which took place after 30 days, the patient reported a significant reduction of
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Percutaneous Closure of an IVC to LA Connection
775
Fig. 3. Fluoroscopic images obtained during the PDA occluder implantation procedure. A: First, the size of the PDA-occluder system is accurately determined using a sizing balloon. B: The PDA occluder is visible at the tip of the delivery catheter.
Fig. 4. Post-procedural CT images showing the PDA occluder in the correct position. A: VRT image in three dimensions. B: MPR image with a reconstructed slice thickness of 0.75 mm. There is no relevant transfer of contrast agent from the left atrium to the inferior vena cava/right atrium. LA 5 left atrium, RA 5 right atrium, IVC 5 inferior vena cava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
exertional dyspnea. There was no evidence of a residual flow between the IVC and the LA as determined by TTE. DISCUSSION
There are only few cases known in which drainage of the IVC into the LA after surgical repair of an inferior ASD has occurred [1–4]. An explanation is that a
prominent Eustachian valve was unintentionally included into closure. By misinterpreting the Eustachian valve as being the inferior rim of the ASD, the superior rim was sutured with the Eustachian valve instead of the true inferior rim. Consequently, the true inferior rim was not affected by the closure and a shunt remained, resulting in a flow from the IVC to the LA [1]. Typically, this becomes apparent by cyanosis, dyspnea, or hypoxemia after a relatively short time
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
776
Wiebe et al.
[1,2]. There are reports, however, of a delayed diagnosis of up to 36 years [3,4]. In our case it was 45 years before the final diagnosis was made, although there were a “residual ASD” and exertional dyspnea already noted. The current treatment of choice would have been another surgical repair [1–3]. We describe the first case in which a connection between the IVC and the LA was closed percutaneously. Due to the anatomy of the tunnel (length and diameter), we used an AMPLATZER Duct Occluder I. This device consists of a self-expanding nitinol mesh with a polyester fabric and a retention skirt. Based on the design of the device, it appeared to be optimal to ensure complete and stable closure of this gap. Established ASD and PFO closure devices did not appear to be suitable, since their waist length is too short. Furthermore, the discs of these systems were judged to be unfavorable because of possible protrusion into the LA.
CONCLUSION
In the present case, percutaneous closure of an IVC to LA connection was feasible and safe. Nevertheless, further experience is needed and the decision concerning the optimal device must be made on an individual basis. REFERENCES 1. Sapin PM, Salley RK. Arterial desaturation and orthodeoxia after atrial septal defect repair: Demonstration of the mechanism by transesophageal and contrast echocardiography. J Am Soc Echocardiogr 1997;10:588–592. 2. Thompson E, Moritz D, Perdue R, Cansino S. Diversion of the inferior vena cava following repair of atrial septal defect causing hypoxemia. Echocardiography 2004;21:329–332. 3. Beitzke D, Koestenberger M, Knez I, Beitzke A. Anomalous connection of the inferior vena cava to the left atrium: A surgical error in closing an atrial septal defect. Clin Res Cardiol 2008;97:191–193. 4. Fouty BW, Lynch DA, Fontenot AP, Schwarz MI. Hypoxemia explained 36 years later. Chest 2001;120:1739–1740.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Percutaneous Closure of a Hemodynamically Significant Connection Between the Inferior Vena Cava and the Left Atrium Jens Wiebe, MD, Johannes Rixe, MD, and Holger Nef,* MD Objective and Background: A connection between the inferior vena cava (IVC) and the left atrium (LA) can occur as a rare complication after surgical atrial septum defect (ASD) repair. We demonstrate the first case of a percutaneous closure of this connection. Methods and Results: A 67-year-old female was admitted to hospital due to exertional dyspnea. A history of a surgical ASD repair in 1960 and 1966 with a residual shunt was already known. Transesophageal echocardiography and a CT scan revealed a hemodynamically significant drainage of the IVC into the LA. This connection was successfully closed percutaneously with an AMPLATEZR Duct Occluder I (St. Jude Medical, St. Paul, MN). Post-procedural CT-scan and transthoracic echocardiography demonstrated a stable position and there was also no evidence of a residual shunt. The patient reported a significant reduction of exertional dyspnea. Conclusion: Percutaneous closure of an IVC to LA connection in this case was safe and feasible. The decision about which device is optimal must be made on an individual basis. VC 2015 Wiley Periodicals, Inc.
Key words: vena cava; closure; AV fistula/AVM; interventional devices/innovation
INTRODUCTION
A rare complication after surgical atrial septal defect (ASD) repair is drainage of the inferior vena cava (IVC) into the left atrium (LA), which has been described only anecdotally in case reports [1–4]. We present the first documented case in which a connection between the IVC and the LA was closed percutaneously.
CASE REPORT
We describe the case a 67-year-old female with exertional dyspnea (New York Heart Association Class III) for several years. The patient has a history of a surgical ASD repair in 1960 and 1966 with a residual shunt. Although attempts were made to retrieve the records, these were unsuccessful and thus the exact technique used and the reason for the second surgery are unknown. Furthermore, the patient suffered from atrial fibrillation and was treated with a vitamin K antagonist, a beta-blocker, and digoxin. No other rhythm disturbances have been noted. Transesophageal echocardiography (TEE) confirmed the residual ASD, which was located inferiorly, and a subsequent CT scan specified it as a connection between the IVC and C 2015 Wiley Periodicals, Inc. V
the LA (Fig. 1). The ASD was fully visible in both imaging modalities and its size was 10 mm measured by TEE as well as by CT (Fig. 2). In addition, TEE demonstrated slight right heart dilatation (diameter right ventricle 33 mm, right atrium 36 mm) and a normal systolic pulmonary pressure of 27 mm Hg. Due to the hemodynamically relevant shunt volume (Qp:Qs 1.8) and the increase in exertional dyspnea, we decided to treat the connection percutaneously. The AMPLATZER Duct Occluder I (St. Jude Medical, St. Paul, MN) appeared to be suitable for closure. The procedure was performed under TEE and fluoroscopic guidance. Vessel access was gained via the right femoral vein. The Department of Cardiology, Medizinische Klinik I, University of Giessen, Giessen 35392, Germany Conflict of interest: Nothing to report. *Correspondence to: Prof. Dr. med. Holger Nef, Medizinische Klinik I, Department of Cardiology, University of Giessen, Klinikstrasse 33, 35392 Giessen, Germany E-mail: [email protected] Received 10 May 2014; Revision accepted 3 January 2015 DOI: 10.1002/ccd.25825 Published online 12 February 2015 in Wiley Online Library (wileyonlinelibrary.com)
774
Wiebe et al.
Fig. 1. Pre-procedural CT images illustrating the connection (*) between the left atrium and the inferior vena cava. A: Three-dimensional volume-rendering technique (VRT) image of the ASD. B: Multiplanar reformat (MPR) image of the defect (*) with a reconstructed slice thickness of 0.75 mm. LA 5 left atrium, RA 5 right atrium, IVC 5 inferior vena cava. *Marks connection between IVC and LA. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 2. Sizing of the defect on CT and TEE. Sizing was performed on CT by adjusting two planes (sagittal and transversal) in the orthogonal and longitudinal orientation to the defect, which yielded an in-plane view of the defect in the coronal view (A). Defect size was 0.91 cm 3 1.21 cm. Additional TEE sizing was performed in an up-esophageal view at 75 degrees (B) and the defect was measured at 1.03 cm
defect was measured with a sizing balloon and had a diameter of 12.7 mm and a length of 6.6 mm. The margin of such defects could consist of soft tissue in which sufficient stabilization of the device is not possible. This discrimination is not possible by TEE or CT and thus additional balloon sizing was necessary. The first attempt to place a 10/12 mm occluder failed, but after another attempt a 12/14 mm occluder was
implanted (Fig. 3). The wiggle test confirmed a stable position and the device was released successfully. Predischarge TTE displayed no residual shunt and CT scan approved the correct position. There was also no evidence of LA protrusion, although some insignificant protrusion was observed at the IVC (Fig. 4). At the first follow-up examination, which took place after 30 days, the patient reported a significant reduction of
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
Percutaneous Closure of an IVC to LA Connection
775
Fig. 3. Fluoroscopic images obtained during the PDA occluder implantation procedure. A: First, the size of the PDA-occluder system is accurately determined using a sizing balloon. B: The PDA occluder is visible at the tip of the delivery catheter.
Fig. 4. Post-procedural CT images showing the PDA occluder in the correct position. A: VRT image in three dimensions. B: MPR image with a reconstructed slice thickness of 0.75 mm. There is no relevant transfer of contrast agent from the left atrium to the inferior vena cava/right atrium. LA 5 left atrium, RA 5 right atrium, IVC 5 inferior vena cava. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
exertional dyspnea. There was no evidence of a residual flow between the IVC and the LA as determined by TTE. DISCUSSION
There are only few cases known in which drainage of the IVC into the LA after surgical repair of an inferior ASD has occurred [1–4]. An explanation is that a
prominent Eustachian valve was unintentionally included into closure. By misinterpreting the Eustachian valve as being the inferior rim of the ASD, the superior rim was sutured with the Eustachian valve instead of the true inferior rim. Consequently, the true inferior rim was not affected by the closure and a shunt remained, resulting in a flow from the IVC to the LA [1]. Typically, this becomes apparent by cyanosis, dyspnea, or hypoxemia after a relatively short time
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
776
Wiebe et al.
[1,2]. There are reports, however, of a delayed diagnosis of up to 36 years [3,4]. In our case it was 45 years before the final diagnosis was made, although there were a “residual ASD” and exertional dyspnea already noted. The current treatment of choice would have been another surgical repair [1–3]. We describe the first case in which a connection between the IVC and the LA was closed percutaneously. Due to the anatomy of the tunnel (length and diameter), we used an AMPLATZER Duct Occluder I. This device consists of a self-expanding nitinol mesh with a polyester fabric and a retention skirt. Based on the design of the device, it appeared to be optimal to ensure complete and stable closure of this gap. Established ASD and PFO closure devices did not appear to be suitable, since their waist length is too short. Furthermore, the discs of these systems were judged to be unfavorable because of possible protrusion into the LA.
CONCLUSION
In the present case, percutaneous closure of an IVC to LA connection was feasible and safe. Nevertheless, further experience is needed and the decision concerning the optimal device must be made on an individual basis. REFERENCES 1. Sapin PM, Salley RK. Arterial desaturation and orthodeoxia after atrial septal defect repair: Demonstration of the mechanism by transesophageal and contrast echocardiography. J Am Soc Echocardiogr 1997;10:588–592. 2. Thompson E, Moritz D, Perdue R, Cansino S. Diversion of the inferior vena cava following repair of atrial septal defect causing hypoxemia. Echocardiography 2004;21:329–332. 3. Beitzke D, Koestenberger M, Knez I, Beitzke A. Anomalous connection of the inferior vena cava to the left atrium: A surgical error in closing an atrial septal defect. Clin Res Cardiol 2008;97:191–193. 4. Fouty BW, Lynch DA, Fontenot AP, Schwarz MI. Hypoxemia explained 36 years later. Chest 2001;120:1739–1740.
Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).
