doi:10.1093/mmcts/mmt001 published online 7 February 2013.
Partial anomalous pulmonary venous connection (including scimitar syndrome) Pieter C. van de Woestijne*, Niels Verberkmoes and Ad J.J.C. Bogers Department of Cardiothoracic Surgery, Erasmus University Medical Center, Rotterdam, Netherlands *Corresponding author. Department of Cardio-thoracic Surgery, Room Bd 573, Erasmus University Medical Center, PO Box 2040, 3000 CA Rotterdam, Netherlands. Tel: +31-10-7035412; fax: +31-10-7033993; e-mail: [email protected] (P. van de Woestijne). Received 6 July 2011; revised 17 October 2012; accepted 13 November 2012
Summary Partial anomalous pulmonary venous connection (PAPVC) is defined to exist when some but not all venous drainage enters the left atrium, while the remaining veins connect to the right-sided circulation. Scimitar syndrome is a specialized example, in which an anomalous pulmonary vein descends from the right lung and drains into the inferior caval vein. PAPVC is associated with sinus venosus-type atrial septal defect (ASD). Diagnosis was, in the past, based on echocardiographic imaging and could be difficult. Multislice spiral computed tomography and magnetic resonance imaging improved the imaging quality. The surgical correction is dependent on the type of anomalous connection and the presence of an ASD. Outcome is good but obstructed venous return is an important issue. Keywords: Partial anomalous pulmonary venous connection • Scimitar syndrome • Surgical correction
INTRODUCTION Partial anomalous pulmonary venous connection (PAPVC) is a congenital pulmonary venous anomaly that involves drainage of one to three pulmonary veins into the right-sided circulation. This creates a partial left-to-right shunt. Scimitar syndrome consists of a descending pulmonary vein from the right lung draining into the inferior caval vein, right atrium, coronary sinus, azygos vein, portal vein or hepatic vein. This condition is often associated with hypoplastic right lung and pulmonary artery, sequestration of the right lower lobe, congenital heart defects and diaphragmatic and musculoskeletal anomalies. PAPVC occurs when there are disturbances during the development of the common pulmonary vein while the pulmonary–splanchnic connections are still present [1]. Symptoms may occur mostly when more than one pulmonary vein is connected to the right-sided circulation. This can finally result in right heart failure. Diagnosis is often made with (transoesophageal) echocardiography (Videos 1 and 2). However others report that multislice CT scan or magnetic resonance imaging (MRI) has more sensitivity and specificity for this diagnosis and gives more anatomical information before surgical correction (Photo 1 and Video 3) [1, 2]. Surgery is the best option and specific corrections must be performed based on the type or location of the PAPVC and the presence of an atrial septal defect (ASD). Several techniques are described, varying from one or double-patch closure of the sinus venosus ASD, reimplantation of pulmonary veins, tube interposition, Warden’s technique, and have been described previously by other groups [3].
We report the types of surgery and the results of surgical correction in patients with PAPVC in our hospital. This study was approved by the medical ethical committee (MEC-2011-239).
PATIENTS AND METHODS From 1969 to 2011, we operated 83 patients (55% male) with PAPVC. In the same period, we operated on another 103 patients with sinus venosus ASD with normal pulmonary vein connections below the level of the intra-atrial septum. The mean age at correction was 18 years (range 0–72 years), and the median age was 6 years (Graph 1). PAPVC was right-sided in 70 patients (84%), left-sided in 11 patients and bilateral in 2 patients. In the patients with abnormal connections from the right lung, 41% were accompanied by a sinus venosus-type ASD, 29% with a normal ASD (secundam type) and in 29% isolated PAPVC without ASD. Compared with patients with abnormal connections from the left lung, there was no accompanying sinus venosus-type ASD but secundam-type ASD was present in 55% of the cases. Surgical treatment consists of several techniques and is shown in Table 1. The two ‘other’ types of surgery were one patient with surgery for VSD, where rerouting was not possible, and one patient underwent surgery to create a palliative Fontan circulation. We explain the two most common surgical methods with illustrations and photos.
Rerouting technique The first method is rerouting the venous return from the right lung with an intracardiac tunnel, using a pericardial patch
© The Author 2013. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Video 1: Transoesophageal echocardiography in a 30-year-old woman with right-sided partial anomalous pulmonary venous connection with a common pulmonary vein connecting to the caudal part of the right atrium next to the inferior caval vein. This was accompanied by a sinus venosus-type atrial septal defect.
Video 3: Magnetic resonance image series with contrast of the partial anomalous pulmonary venous connection connecting the right atrium just above the inferior caval vein (same patient as the transoesophageal echocardiography).
Video 2: Same image as Video 1 with colour Doppler mode.
Graph 1: Age distribution of 83 patients with partial anomalous pulmonary venous connection and surgical correction.
Table 1: Surgical methods used in partial anomalous pulmonary venous connection correction Type of surgery
Photo 1: Three-dimensional reconstructed magnetic resonance image of the right-sided partial anomalous pulmonary venous connection to the inferior right atrium.
Rerouting of pulmonary veins Rerouting of pulmonary veins with patch or tube material Rerouting of pulmonary veins and closure of the sinus venosus ASD with patch Closure of sinus venosus ASD with patch (intracardiac baffle) Other
Number of patients 22 17 7 24 2
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P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
(Schematics 1–4). We use Prolene running sutures for the patch. With this method, an existing ASD could be used or enlarged or an ASD could be created if there is none. In case of a pulmonary venous connection to the superior caval vein, rerouting can be performed by using one patch as described and illustrated before. Sometimes, a double-patch technique can be used by placing a second pericardial patch to enlarge the superior caval vein at the right lateral side. This can be necessary if an incision is made into the lateral side of the superior caval vein when the pulmonary venous connection is at a very high level towards the azygos vein. Through this extra incision, the rerouting patch can be easily sutured.
Schematic 3: An autologous pericardial patch is used to correct this defect with a running suture.
Schematic 1: Surgical view of the heart. Patient on extracorporeal circulation with selective cannulation of both caval veins. The right atrium is opened. At the bottom of the picture, the right pulmonary veins debouch in the right atrium. The dotted line indicates the foramen ovale.
Schematic 4: Final result after inserting the pericardial patch.
Replantation technique The second method is rerouting the venous return by replantation of the pulmonary veins into the left atrium (Schematics 5–11 and Photos 2–4). Hereby, an existing ASD could be used or enlarged for the exposure of the left atrial wall or an ASD could be created to obtain access to the left atrium. Pericardial patches could be used for the closure of the remaining defect(s) in the right atrial wall and ASD if there is one.
Scimitar syndrome
Schematic 2: Detailed view of the intra-atrial septum where an atrial septal defect can be created or an existing atrial septal defect can be used or enlarged for rerouting the blood from the pulmonary veins into the left atrium.
In scimitar syndrome, the venous drainage of the right lung forms one vessel which enters the inferior caval vein. On X-ray, this imposes as a round sword (scimitar). Somewhat similar to the rerouting method, this anomaly can be corrected by suturing an autologous pericardial patch from inside the inferior caval vein to an ASD (Schematics 12–15). This can be an existing ASD which often has to be enlarged at the caudal part or an ASD can be created.
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 5: Surgical view of the opened right atrium. The dotted line indicates the opening of the intra-atrial septum. Sometimes, an existing atrial septal defect could also be used or enlarged. The pulmonary veins are coming out into the right atrium above the intra-atrial septum.
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Schematic 8: Creation of an opening in the back wall of the left atrium.
Schematic 6: Detailed view of the opening of the intra-atrial septum. Mostly, the septum does not have to be removed but just incised to get a good exposure of the left atrial wall. Schematic 9: Removal of the pulmonary veins as an island (asterisk) from the right atrial lateral wall.
Schematic 7: The intra-atrial septum is partially removed and the back wall of the left atrium is visible.
Schematic 10: The pulmonary veins are being sutured into the opening of the left atrial wall and the gap in the right atrium is closed with a pericardial patch. Sometimes, this defect can be closed primarily.
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P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 11: Final result after closing the intra-atrial septum also with a pericardial patch. The dotted lines mark the position of the pulmonary veins.
Photo 4: Postoperative image after the closure of the right atrium. The suture on the right side of the heart indicates the place of the abnormal connection which is now draining into the left atrium. The atrial septal defect was closed with an autologous pericardial patch.
Photo 2: Photo from the operating room from the same patient mentioned on the transoesophageal echocardiography prior to correction with a vessel loop around the abnormal pulmonary vein connection to the right atrium.
Schematic 12: Surgical view of the opened right atrium in scimitar syndrome. The right pulmonary veins are draining directly to the inferior caval vein.
Photo 3: The right atrium is opened, the abnormal connection excised from the free wall and reimplanted into the lateral wall of the left atrium, performed through the atrial septal defect.
Schematic 13: Creation of a hole in the intra-atrial septum, sometimes an existing atrial septal defect can be used or enlarged.
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 14: A pericardial patch is used for rerouting the blood flow towards the left atrium.
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Graph 2: Kaplan–Meier curve of the survival after partial anomalous pulmonary venous connection correction.
Schematic 15: Final situation after suturing the pericardial patch.
Left-sided PAPVC Left-sided PAPVC is rare and consists mostly of a connection from the upper left pulmonary vein to the anonymous vein or persistent left superior caval vein. Surgery consists of replantation of that vein into the left atrial appendage (LAA). This can be difficult depending on the distance to the LAA and the (im)mobility of the pulmonary vein.
RESULTS In our series of patients with PAPVC, follow-up was complete for 94% of the patients until 1 June 2011. Five patients were lost for the follow-up. The mean follow-up was 14 years (range 2 weeks to 38 years). There was no operative mortality. Long-term survival was 88% and shown as a Kaplan–Meier curve in Graph 2. Five patients needed a reoperation due to pulmonary vein obstruction and two patients underwent balloon dilatation of a pulmonary vein stenosis. Freedom from reintervention is shown in Graph 3.
Graph 3: Kaplan–Meier curve of the freedom from reintervention after partial anomalous pulmonary venous connection correction.
DISCUSSION PAPVC is an uncommon congenital anomaly which is undiagnosed because of an asymptomatic course of the disease. It is strongly associated with a sinus venosus-type ASD. Diagnosis is based on chest radiography and echocardiography but even more on multislice spiral computed tomography or MRI. Surgical correction varies but has the purpose of creating an unobstructed flow from the pulmonary veins to the left atrium. This could be performed with good success and long-term survival. Sometimes, reinterventions are necessary for pulmonary vein obstructions. Scimitar syndrome has been reported as a continuing management challenge.
Acknowledgement We thank C.F. Wilbrink for his clear illustrations.
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P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Conflict of interest: none declared.
References [1] Ho ML, Bhalla S, Bierhals A, Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging 2009;24:89–95.
[2] Amat F, Le Bret E, Sigal-Cinqualbre A, Coblence M, Lambert V, Rohnean A et al. Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact CardioVasc Thorac Surg 2011;12:179–82. [3] Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg 2007;84:2020–6; Discussion 2020–6.
Partial anomalous pulmonary venous connection (including scimitar syndrome) Pieter C. van de Woestijne*, Niels Verberkmoes and Ad J.J.C. Bogers Department of Cardiothoracic Surgery, Erasmus University Medical Center, Rotterdam, Netherlands *Corresponding author. Department of Cardio-thoracic Surgery, Room Bd 573, Erasmus University Medical Center, PO Box 2040, 3000 CA Rotterdam, Netherlands. Tel: +31-10-7035412; fax: +31-10-7033993; e-mail: [email protected] (P. van de Woestijne). Received 6 July 2011; revised 17 October 2012; accepted 13 November 2012
Summary Partial anomalous pulmonary venous connection (PAPVC) is defined to exist when some but not all venous drainage enters the left atrium, while the remaining veins connect to the right-sided circulation. Scimitar syndrome is a specialized example, in which an anomalous pulmonary vein descends from the right lung and drains into the inferior caval vein. PAPVC is associated with sinus venosus-type atrial septal defect (ASD). Diagnosis was, in the past, based on echocardiographic imaging and could be difficult. Multislice spiral computed tomography and magnetic resonance imaging improved the imaging quality. The surgical correction is dependent on the type of anomalous connection and the presence of an ASD. Outcome is good but obstructed venous return is an important issue. Keywords: Partial anomalous pulmonary venous connection • Scimitar syndrome • Surgical correction
INTRODUCTION Partial anomalous pulmonary venous connection (PAPVC) is a congenital pulmonary venous anomaly that involves drainage of one to three pulmonary veins into the right-sided circulation. This creates a partial left-to-right shunt. Scimitar syndrome consists of a descending pulmonary vein from the right lung draining into the inferior caval vein, right atrium, coronary sinus, azygos vein, portal vein or hepatic vein. This condition is often associated with hypoplastic right lung and pulmonary artery, sequestration of the right lower lobe, congenital heart defects and diaphragmatic and musculoskeletal anomalies. PAPVC occurs when there are disturbances during the development of the common pulmonary vein while the pulmonary–splanchnic connections are still present [1]. Symptoms may occur mostly when more than one pulmonary vein is connected to the right-sided circulation. This can finally result in right heart failure. Diagnosis is often made with (transoesophageal) echocardiography (Videos 1 and 2). However others report that multislice CT scan or magnetic resonance imaging (MRI) has more sensitivity and specificity for this diagnosis and gives more anatomical information before surgical correction (Photo 1 and Video 3) [1, 2]. Surgery is the best option and specific corrections must be performed based on the type or location of the PAPVC and the presence of an atrial septal defect (ASD). Several techniques are described, varying from one or double-patch closure of the sinus venosus ASD, reimplantation of pulmonary veins, tube interposition, Warden’s technique, and have been described previously by other groups [3].
We report the types of surgery and the results of surgical correction in patients with PAPVC in our hospital. This study was approved by the medical ethical committee (MEC-2011-239).
PATIENTS AND METHODS From 1969 to 2011, we operated 83 patients (55% male) with PAPVC. In the same period, we operated on another 103 patients with sinus venosus ASD with normal pulmonary vein connections below the level of the intra-atrial septum. The mean age at correction was 18 years (range 0–72 years), and the median age was 6 years (Graph 1). PAPVC was right-sided in 70 patients (84%), left-sided in 11 patients and bilateral in 2 patients. In the patients with abnormal connections from the right lung, 41% were accompanied by a sinus venosus-type ASD, 29% with a normal ASD (secundam type) and in 29% isolated PAPVC without ASD. Compared with patients with abnormal connections from the left lung, there was no accompanying sinus venosus-type ASD but secundam-type ASD was present in 55% of the cases. Surgical treatment consists of several techniques and is shown in Table 1. The two ‘other’ types of surgery were one patient with surgery for VSD, where rerouting was not possible, and one patient underwent surgery to create a palliative Fontan circulation. We explain the two most common surgical methods with illustrations and photos.
Rerouting technique The first method is rerouting the venous return from the right lung with an intracardiac tunnel, using a pericardial patch
© The Author 2013. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
2
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Video 1: Transoesophageal echocardiography in a 30-year-old woman with right-sided partial anomalous pulmonary venous connection with a common pulmonary vein connecting to the caudal part of the right atrium next to the inferior caval vein. This was accompanied by a sinus venosus-type atrial septal defect.
Video 3: Magnetic resonance image series with contrast of the partial anomalous pulmonary venous connection connecting the right atrium just above the inferior caval vein (same patient as the transoesophageal echocardiography).
Video 2: Same image as Video 1 with colour Doppler mode.
Graph 1: Age distribution of 83 patients with partial anomalous pulmonary venous connection and surgical correction.
Table 1: Surgical methods used in partial anomalous pulmonary venous connection correction Type of surgery
Photo 1: Three-dimensional reconstructed magnetic resonance image of the right-sided partial anomalous pulmonary venous connection to the inferior right atrium.
Rerouting of pulmonary veins Rerouting of pulmonary veins with patch or tube material Rerouting of pulmonary veins and closure of the sinus venosus ASD with patch Closure of sinus venosus ASD with patch (intracardiac baffle) Other
Number of patients 22 17 7 24 2
3
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
(Schematics 1–4). We use Prolene running sutures for the patch. With this method, an existing ASD could be used or enlarged or an ASD could be created if there is none. In case of a pulmonary venous connection to the superior caval vein, rerouting can be performed by using one patch as described and illustrated before. Sometimes, a double-patch technique can be used by placing a second pericardial patch to enlarge the superior caval vein at the right lateral side. This can be necessary if an incision is made into the lateral side of the superior caval vein when the pulmonary venous connection is at a very high level towards the azygos vein. Through this extra incision, the rerouting patch can be easily sutured.
Schematic 3: An autologous pericardial patch is used to correct this defect with a running suture.
Schematic 1: Surgical view of the heart. Patient on extracorporeal circulation with selective cannulation of both caval veins. The right atrium is opened. At the bottom of the picture, the right pulmonary veins debouch in the right atrium. The dotted line indicates the foramen ovale.
Schematic 4: Final result after inserting the pericardial patch.
Replantation technique The second method is rerouting the venous return by replantation of the pulmonary veins into the left atrium (Schematics 5–11 and Photos 2–4). Hereby, an existing ASD could be used or enlarged for the exposure of the left atrial wall or an ASD could be created to obtain access to the left atrium. Pericardial patches could be used for the closure of the remaining defect(s) in the right atrial wall and ASD if there is one.
Scimitar syndrome
Schematic 2: Detailed view of the intra-atrial septum where an atrial septal defect can be created or an existing atrial septal defect can be used or enlarged for rerouting the blood from the pulmonary veins into the left atrium.
In scimitar syndrome, the venous drainage of the right lung forms one vessel which enters the inferior caval vein. On X-ray, this imposes as a round sword (scimitar). Somewhat similar to the rerouting method, this anomaly can be corrected by suturing an autologous pericardial patch from inside the inferior caval vein to an ASD (Schematics 12–15). This can be an existing ASD which often has to be enlarged at the caudal part or an ASD can be created.
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 5: Surgical view of the opened right atrium. The dotted line indicates the opening of the intra-atrial septum. Sometimes, an existing atrial septal defect could also be used or enlarged. The pulmonary veins are coming out into the right atrium above the intra-atrial septum.
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Schematic 8: Creation of an opening in the back wall of the left atrium.
Schematic 6: Detailed view of the opening of the intra-atrial septum. Mostly, the septum does not have to be removed but just incised to get a good exposure of the left atrial wall. Schematic 9: Removal of the pulmonary veins as an island (asterisk) from the right atrial lateral wall.
Schematic 7: The intra-atrial septum is partially removed and the back wall of the left atrium is visible.
Schematic 10: The pulmonary veins are being sutured into the opening of the left atrial wall and the gap in the right atrium is closed with a pericardial patch. Sometimes, this defect can be closed primarily.
5
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 11: Final result after closing the intra-atrial septum also with a pericardial patch. The dotted lines mark the position of the pulmonary veins.
Photo 4: Postoperative image after the closure of the right atrium. The suture on the right side of the heart indicates the place of the abnormal connection which is now draining into the left atrium. The atrial septal defect was closed with an autologous pericardial patch.
Photo 2: Photo from the operating room from the same patient mentioned on the transoesophageal echocardiography prior to correction with a vessel loop around the abnormal pulmonary vein connection to the right atrium.
Schematic 12: Surgical view of the opened right atrium in scimitar syndrome. The right pulmonary veins are draining directly to the inferior caval vein.
Photo 3: The right atrium is opened, the abnormal connection excised from the free wall and reimplanted into the lateral wall of the left atrium, performed through the atrial septal defect.
Schematic 13: Creation of a hole in the intra-atrial septum, sometimes an existing atrial septal defect can be used or enlarged.
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Schematic 14: A pericardial patch is used for rerouting the blood flow towards the left atrium.
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Graph 2: Kaplan–Meier curve of the survival after partial anomalous pulmonary venous connection correction.
Schematic 15: Final situation after suturing the pericardial patch.
Left-sided PAPVC Left-sided PAPVC is rare and consists mostly of a connection from the upper left pulmonary vein to the anonymous vein or persistent left superior caval vein. Surgery consists of replantation of that vein into the left atrial appendage (LAA). This can be difficult depending on the distance to the LAA and the (im)mobility of the pulmonary vein.
RESULTS In our series of patients with PAPVC, follow-up was complete for 94% of the patients until 1 June 2011. Five patients were lost for the follow-up. The mean follow-up was 14 years (range 2 weeks to 38 years). There was no operative mortality. Long-term survival was 88% and shown as a Kaplan–Meier curve in Graph 2. Five patients needed a reoperation due to pulmonary vein obstruction and two patients underwent balloon dilatation of a pulmonary vein stenosis. Freedom from reintervention is shown in Graph 3.
Graph 3: Kaplan–Meier curve of the freedom from reintervention after partial anomalous pulmonary venous connection correction.
DISCUSSION PAPVC is an uncommon congenital anomaly which is undiagnosed because of an asymptomatic course of the disease. It is strongly associated with a sinus venosus-type ASD. Diagnosis is based on chest radiography and echocardiography but even more on multislice spiral computed tomography or MRI. Surgical correction varies but has the purpose of creating an unobstructed flow from the pulmonary veins to the left atrium. This could be performed with good success and long-term survival. Sometimes, reinterventions are necessary for pulmonary vein obstructions. Scimitar syndrome has been reported as a continuing management challenge.
Acknowledgement We thank C.F. Wilbrink for his clear illustrations.
7
P.C. van de Woestijne et al. / Multimedia Manual of Cardio-Thoracic Surgery
Conflict of interest: none declared.
References [1] Ho ML, Bhalla S, Bierhals A, Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging 2009;24:89–95.
[2] Amat F, Le Bret E, Sigal-Cinqualbre A, Coblence M, Lambert V, Rohnean A et al. Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children. Interact CardioVasc Thorac Surg 2011;12:179–82. [3] Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg 2007;84:2020–6; Discussion 2020–6.
