Case Study
Partial anomalous pulmonary venous connection with acquired valve disease
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(8) 966–969 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314527767 aan.sagepub.com
Masaki Hamamoto, Taira Kobayashi and Hiroshi Kodama
Abstract We describe the rare case of a 76-year-old man with left partial anomalous pulmonary venous connection with intact atrial septum. He experienced dyspnea due to significant aortic stenosis and mitral regurgitation, and required valve repair. Preoperative computed tomography revealed connection of the left upper pulmonary vein to the innominate vein. Although right heart overload was not recognized, we repaired the left partial anomalous pulmonary venous connection with concomitant aortic valve replacement and mitral annuloplasty, because of the potential for unpredictable harmful effects of a residual left-to-right shunt. The patient recovered well with no signs of left upper lung congestion.
Keywords Aortic valve stenosis, Cardiac surgical procedures, Heart defects, congenital, Pulmonary veins, Vascular surgical procedures
Introduction Partial anomalous pulmonary venous connection (PAPVC) is a congenital anomaly that exhibits direct return of blood flow from the pulmonary veins to the systemic circulation. PAPVC accounts for less than 1% of all congenital cardiac diseases.1 In the spectrum of PAPVC, left-sided PAPVC belongs to an uncommon subgroup compared to right-sided PAPVC.2 While PAPVC is frequently accompanied by an atrial septal defect, left-sided PAPVC with an intact atrial septum occurs less often.2 We describe a rare case of left-sided PAPVC with an intact atrial septum in an elderly patient with degenerative heart valve disease. We discuss the surgical indications and procedures for left PAPVC.
Case report A 76-year-old man who presented with frequent episodes of dyspnea on effort, was diagnosed as having severe aortic stenosis and moderate mitral regurgitation. He was admitted to our hospital for surgical intervention. Chest radiography showed a cardiothoracic ratio of 53% with bilateral increased pulmonary vasculature (Figure 1A). Electrocardiography revealed normal sinus rhythm with no right bundle branch
block. Echocardiography demonstrated severe aortic stenosis with an aortic valve area of 0.73 cm2 and moderate mitral regurgitation with no leaflet prolapse. Cardiac catheterization revealed no significant stenosis of the coronary arteries or any evidence of right heart overload. On contrast-enhanced computed tomography, there was a connection between the left upper pulmonary vein and the innominate vein, but not to the left atrium (Figure 1B, 1C). Transesophageal echocardiography showed no other anomalies such as atrial septal defect or pulmonary valve stenosis. We diagnosed this case as left PAPVC with intact atrial septum. We decided to repair the left PAPVC concomitant with double-valve surgery even though there was no sign of right-side volume overload including pulmonary hypertension. The heart was exposed through a median sternotomy. The right side of the heart was not dilated (Figure 2A). The left PAPVC was exposed from the junction with the innominate vein to the left Department of Cardiovascular Surgery, JA Hiroshima General Hospital, Hiroshima, Japan Corresponding author: Masaki Hamamoto, MD, PhD, Department of Cardiovascular Surgery, JA Hiroshima General Hospital, 1-3-3 Zigozen, Hatsukaichi, Hiroshima 738-8503, Japan. Email: [email protected]
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Figure 1. Preoperative (A–C) and postoperative (D, E) examination findings. (A) Chest radiograph. (B, C) Computed tomography showing anomalous connection of the left upper pulmonary vein to the innominate vein (white arrowhead). (D) Intraoperative transesophageal echocardiography showing blood flow in the left atrial appendage from the reconstructed left upper pulmonary vein. (E) Computed tomography showing anastomosis of the left upper pulmonary vein to the left atrial appendage (white arrowhead). Ao: ascending aorta; IV: innominate vein; LA: left atrium; LAA: left atrial appendage; LLPA: left lower pulmonary vein; PA: pulmonary artery; RUPA: RLPA: right lower pulmonary vein; right upper pulmonary vein.
pulmonary hilum, with special care to avoid injury to the left phrenic nerve (Figure 2B). Intraoperative blood sampling revealed an oxygen step-up in the innominate vein, indicating a left-to-right shunt. Cardiopulmonary bypass (CPB) was established with ascending aortic and bicaval cannulation. After cardioplegic arrest, the left PAPVC was transected at its junction with the innominate vein, and clamped proximally at the hilum. The left atrial appendage (LAA) was also clamped at its base and the anastomotic orifice was created (Figure 2C). There was no sign of thrombus inside the LAA. The left PAPVC was anastomosed to the LAA with a continuous suture using 6/0 polypropylene, with care taken to avoid tearing the fragile venous wall with the suture (Figure 2D, 2E). Aortic valve replacement with a bioprosthesis and mitral annuloplasty with a rigid band were performed. Transesophageal echocardiography showed blood flow within the LAA from the
reconstructed pulmonary vein (Figure 1D). The postoperative clinical course was uneventful with no evidence of congestion of the left upper lung. Computed tomography revealed a patent left PAPVC with no anastomotic stenosis or kinking of the reconstructed upper pulmonary vein (Figure 1E).
Discussion Surgical repair of PAPVC is necessary when patients exhibit symptoms or demonstrate right heart failure, pulmonary hypertension, or atrial arrhythmia.3 Right heart failure depends on the magnitude of shunting. Patients with PAPVC with intact atrial septum exhibit a physiological condition similar to secundum atrial septal defect with a left-to-right shunt. The traditional surgical indication for repair of a left-to-right shunt at the atrial level is a pulmonary-to-systemic blood flow
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
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Asian Cardiovascular & Thoracic Annals 23(8)
Figure 2. (A, B, D) Intraoperative photographs and (C, E) schema. (A) The whole heart. (B) Close-up picture of the left partial anomalous pulmonary venous connection (white arrowhead) to the innominate vein. (C) The left upper pulmonary vein (LUPV) was transected and taken down to the left atrial appendage (LAA). The LAA was clamped and the anastomotic orifice was created. (D) Anastomosis between the left upper pulmonary vein and the left atrial appendage with no kinking. (E) The left upper pulmonary vein was anastomosed to the LAA with a running suture using 6/0 polypropylene. Ao: ascending aorta; IV: innominate vein; LAA: left atrial appendage; LUPV: left upper pulmonary vein; PA: pulmonary artery; SVC: superior vena cava.
ratio >1.5.4 On the other hand, Babb and colleagues5 proposed that surgical correction should be considered whenever PAPVC is found because of the potential for this anomaly to develop into advanced pulmonary vascular obstructive disease. ElBardissi and colleagues3 also reported that surgical intervention should be considered at an early stage to prevent the progression of right heart failure. In our case, we performed surgical correction of left PAPVC concomitant with aortic valve replacement and mitral annuloplasty, despite a small shunt with no sign of right-side overload, because the natural course of untreated PAPVC is unpredictable. There are two potential approaches to repair of a left PAPVC. A median sternotomy is the primary surgical approach, which makes it possible to perform concomitant procedures for coexisting cardiac diseases during CPB. A left thoracotomy offers a minimally invasive off-pump repair.3 However, concomitant procedures for intracardiac anomalies or valve diseases could be restricted. In our case, we selected to perform a
median sternotomy and repair of the left PAPVC on CPB because aortic valve replacement and mitral annuloplasty were the major procedures requiring CPB. Several operative techniques have been reported for left PAPVC: end-to-end anastomosis of the left PAPVC to the LAA, side-to-end anastomosis of the left PAPVC to the LAA and subsequent ligation of the vertical vein near the innominate vein,6 and endto-side anastomosis of the left PAPVC to the left atrium.7 The first procedure has been applied most often and provides the option for less-invasive offpump anastomosis.3,4,8 However, several drawbacks have been reported, including thrombus formation, kinking of the reconstructed vein, and anastomotic stenosis. The second procedure has been indicated for total unilateral left PAPVC. The third procedure has the advantage of creating a wide anastomotic orifice at the left atrial wall with no trabeculae. The disadvantage of this procedure is the anastomotic site on the posterolateral left atrial wall, which is less visible and
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
Hamamoto et al.
969
technically demanding for anastomosis or hemostasis via a median sternotomy. In our case, we applied the first procedure because the length of the left upper pulmonary vein was too short to reach the posterolateral left atrial wall. Considering the potential for thrombus formation and venous kinking, we avoided the use of vascular prostheses or an autologous pericardial roll for extension. Various procedures have been used to overcome anastomotic stenosis. Ban and colleagues8 described patch augmentation of the anastomotic site using the patient’s own pericardium. Ports and colleagues7 reported the creation of a wide anastomotic orifice of the left PAPVC, using a cobra head configuration.7 Van Meter and colleagues4 recommended the use of interrupted sutures at the anterior wall of the anastomosis to prevent a ‘‘purse-string effect’’. In our case, the left upper pulmonary vein, which was 10 mm in diameter, was thin and fragile. Therefore, we used a continuous suture for end-to-end anastomosis without applying pericardial augmentation because the frail venous wall could lead to suture bleeding with many stitches, and simple anastomosis with a continuous suture might decrease the chance of anastomotic failure in the deep surgical field via a median sternotomy. Postoperative anticoagulation after PAPVC repair is controversial. In their report of pericardial patch augmentation of the anastomotic site, Ban and colleagues8 indicated that postoperative anticoagulation was not necessary. However, many surgeons remain concerned that thrombus formation could develop at the suture line or the LAA trabeculae in the low-flow state of the pulmonary venous circulation. Therefore, we used oral anticoagulant postoperatively for 3 months with a target international normalized ratio of around 2.0.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Herlong JR, Jaggers JJ and Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies [Review]. Ann Thorac Surg 2000; 69: S56–S69. 2. Kalke BR, Carlson RG, Ferlic RM, Sellers RD and Lillehei CW. Partial anomalous pulmonary venous connections. Am J Cardiol 1967; 20: 90–101. 3. ElBardissi AW, Dearani JA, Suri RM and Danielson GK. Left-sided partial anomalous pulmonary venous connections. Ann Thorac Surg 2008; 85: 1007–1014. 4. Van Meter C Jr, LeBlanc JG, Culpepper WS 3rd and Ochsner JL. Partial anomalous pulmonary venous return. Circulation 1990; 82(Suppl IV): 195–198. 5. Babb JD, McGlynn TJ, Pierce WS and Kirkman PM. Isolated partial anomalous venous connection: a congenital defect with late and serious complications. Ann Thorac Surg 1981; 31: 540–541. 6. Bello AG, Daggett WM, Hutter AM Jr, Desanctis RW, Buckley MJ and Austen WG. Surgical correction of partial anomalous venous drainage with intact atrial septum: association with acquired valve lesions. J Cardiovasc Surg 1973; 14: 357–364. 7. Ports TA, Turley K, Brundage BH and Ebert PA. Operative correction of total left anomalous pulmonary venous return. Ann Thorac Surg 1978; 27: 246–249. 8. Ban T, Sakata R and Hirata K. Surgical treatment of partial anomalous pulmonary venous connection of the left lung (lt-PAPVC). J Cardiac Surg 1987; 2: 369–373.
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
Partial anomalous pulmonary venous connection with acquired valve disease
Asian Cardiovascular & Thoracic Annals 2015, Vol. 23(8) 966–969 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314527767 aan.sagepub.com
Masaki Hamamoto, Taira Kobayashi and Hiroshi Kodama
Abstract We describe the rare case of a 76-year-old man with left partial anomalous pulmonary venous connection with intact atrial septum. He experienced dyspnea due to significant aortic stenosis and mitral regurgitation, and required valve repair. Preoperative computed tomography revealed connection of the left upper pulmonary vein to the innominate vein. Although right heart overload was not recognized, we repaired the left partial anomalous pulmonary venous connection with concomitant aortic valve replacement and mitral annuloplasty, because of the potential for unpredictable harmful effects of a residual left-to-right shunt. The patient recovered well with no signs of left upper lung congestion.
Keywords Aortic valve stenosis, Cardiac surgical procedures, Heart defects, congenital, Pulmonary veins, Vascular surgical procedures
Introduction Partial anomalous pulmonary venous connection (PAPVC) is a congenital anomaly that exhibits direct return of blood flow from the pulmonary veins to the systemic circulation. PAPVC accounts for less than 1% of all congenital cardiac diseases.1 In the spectrum of PAPVC, left-sided PAPVC belongs to an uncommon subgroup compared to right-sided PAPVC.2 While PAPVC is frequently accompanied by an atrial septal defect, left-sided PAPVC with an intact atrial septum occurs less often.2 We describe a rare case of left-sided PAPVC with an intact atrial septum in an elderly patient with degenerative heart valve disease. We discuss the surgical indications and procedures for left PAPVC.
Case report A 76-year-old man who presented with frequent episodes of dyspnea on effort, was diagnosed as having severe aortic stenosis and moderate mitral regurgitation. He was admitted to our hospital for surgical intervention. Chest radiography showed a cardiothoracic ratio of 53% with bilateral increased pulmonary vasculature (Figure 1A). Electrocardiography revealed normal sinus rhythm with no right bundle branch
block. Echocardiography demonstrated severe aortic stenosis with an aortic valve area of 0.73 cm2 and moderate mitral regurgitation with no leaflet prolapse. Cardiac catheterization revealed no significant stenosis of the coronary arteries or any evidence of right heart overload. On contrast-enhanced computed tomography, there was a connection between the left upper pulmonary vein and the innominate vein, but not to the left atrium (Figure 1B, 1C). Transesophageal echocardiography showed no other anomalies such as atrial septal defect or pulmonary valve stenosis. We diagnosed this case as left PAPVC with intact atrial septum. We decided to repair the left PAPVC concomitant with double-valve surgery even though there was no sign of right-side volume overload including pulmonary hypertension. The heart was exposed through a median sternotomy. The right side of the heart was not dilated (Figure 2A). The left PAPVC was exposed from the junction with the innominate vein to the left Department of Cardiovascular Surgery, JA Hiroshima General Hospital, Hiroshima, Japan Corresponding author: Masaki Hamamoto, MD, PhD, Department of Cardiovascular Surgery, JA Hiroshima General Hospital, 1-3-3 Zigozen, Hatsukaichi, Hiroshima 738-8503, Japan. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
Hamamoto et al.
967
Figure 1. Preoperative (A–C) and postoperative (D, E) examination findings. (A) Chest radiograph. (B, C) Computed tomography showing anomalous connection of the left upper pulmonary vein to the innominate vein (white arrowhead). (D) Intraoperative transesophageal echocardiography showing blood flow in the left atrial appendage from the reconstructed left upper pulmonary vein. (E) Computed tomography showing anastomosis of the left upper pulmonary vein to the left atrial appendage (white arrowhead). Ao: ascending aorta; IV: innominate vein; LA: left atrium; LAA: left atrial appendage; LLPA: left lower pulmonary vein; PA: pulmonary artery; RUPA: RLPA: right lower pulmonary vein; right upper pulmonary vein.
pulmonary hilum, with special care to avoid injury to the left phrenic nerve (Figure 2B). Intraoperative blood sampling revealed an oxygen step-up in the innominate vein, indicating a left-to-right shunt. Cardiopulmonary bypass (CPB) was established with ascending aortic and bicaval cannulation. After cardioplegic arrest, the left PAPVC was transected at its junction with the innominate vein, and clamped proximally at the hilum. The left atrial appendage (LAA) was also clamped at its base and the anastomotic orifice was created (Figure 2C). There was no sign of thrombus inside the LAA. The left PAPVC was anastomosed to the LAA with a continuous suture using 6/0 polypropylene, with care taken to avoid tearing the fragile venous wall with the suture (Figure 2D, 2E). Aortic valve replacement with a bioprosthesis and mitral annuloplasty with a rigid band were performed. Transesophageal echocardiography showed blood flow within the LAA from the
reconstructed pulmonary vein (Figure 1D). The postoperative clinical course was uneventful with no evidence of congestion of the left upper lung. Computed tomography revealed a patent left PAPVC with no anastomotic stenosis or kinking of the reconstructed upper pulmonary vein (Figure 1E).
Discussion Surgical repair of PAPVC is necessary when patients exhibit symptoms or demonstrate right heart failure, pulmonary hypertension, or atrial arrhythmia.3 Right heart failure depends on the magnitude of shunting. Patients with PAPVC with intact atrial septum exhibit a physiological condition similar to secundum atrial septal defect with a left-to-right shunt. The traditional surgical indication for repair of a left-to-right shunt at the atrial level is a pulmonary-to-systemic blood flow
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
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Asian Cardiovascular & Thoracic Annals 23(8)
Figure 2. (A, B, D) Intraoperative photographs and (C, E) schema. (A) The whole heart. (B) Close-up picture of the left partial anomalous pulmonary venous connection (white arrowhead) to the innominate vein. (C) The left upper pulmonary vein (LUPV) was transected and taken down to the left atrial appendage (LAA). The LAA was clamped and the anastomotic orifice was created. (D) Anastomosis between the left upper pulmonary vein and the left atrial appendage with no kinking. (E) The left upper pulmonary vein was anastomosed to the LAA with a running suture using 6/0 polypropylene. Ao: ascending aorta; IV: innominate vein; LAA: left atrial appendage; LUPV: left upper pulmonary vein; PA: pulmonary artery; SVC: superior vena cava.
ratio >1.5.4 On the other hand, Babb and colleagues5 proposed that surgical correction should be considered whenever PAPVC is found because of the potential for this anomaly to develop into advanced pulmonary vascular obstructive disease. ElBardissi and colleagues3 also reported that surgical intervention should be considered at an early stage to prevent the progression of right heart failure. In our case, we performed surgical correction of left PAPVC concomitant with aortic valve replacement and mitral annuloplasty, despite a small shunt with no sign of right-side overload, because the natural course of untreated PAPVC is unpredictable. There are two potential approaches to repair of a left PAPVC. A median sternotomy is the primary surgical approach, which makes it possible to perform concomitant procedures for coexisting cardiac diseases during CPB. A left thoracotomy offers a minimally invasive off-pump repair.3 However, concomitant procedures for intracardiac anomalies or valve diseases could be restricted. In our case, we selected to perform a
median sternotomy and repair of the left PAPVC on CPB because aortic valve replacement and mitral annuloplasty were the major procedures requiring CPB. Several operative techniques have been reported for left PAPVC: end-to-end anastomosis of the left PAPVC to the LAA, side-to-end anastomosis of the left PAPVC to the LAA and subsequent ligation of the vertical vein near the innominate vein,6 and endto-side anastomosis of the left PAPVC to the left atrium.7 The first procedure has been applied most often and provides the option for less-invasive offpump anastomosis.3,4,8 However, several drawbacks have been reported, including thrombus formation, kinking of the reconstructed vein, and anastomotic stenosis. The second procedure has been indicated for total unilateral left PAPVC. The third procedure has the advantage of creating a wide anastomotic orifice at the left atrial wall with no trabeculae. The disadvantage of this procedure is the anastomotic site on the posterolateral left atrial wall, which is less visible and
Downloaded from aan.sagepub.com at UNIV NEBRASKA LIBRARIES on September 26, 2015
Hamamoto et al.
969
technically demanding for anastomosis or hemostasis via a median sternotomy. In our case, we applied the first procedure because the length of the left upper pulmonary vein was too short to reach the posterolateral left atrial wall. Considering the potential for thrombus formation and venous kinking, we avoided the use of vascular prostheses or an autologous pericardial roll for extension. Various procedures have been used to overcome anastomotic stenosis. Ban and colleagues8 described patch augmentation of the anastomotic site using the patient’s own pericardium. Ports and colleagues7 reported the creation of a wide anastomotic orifice of the left PAPVC, using a cobra head configuration.7 Van Meter and colleagues4 recommended the use of interrupted sutures at the anterior wall of the anastomosis to prevent a ‘‘purse-string effect’’. In our case, the left upper pulmonary vein, which was 10 mm in diameter, was thin and fragile. Therefore, we used a continuous suture for end-to-end anastomosis without applying pericardial augmentation because the frail venous wall could lead to suture bleeding with many stitches, and simple anastomosis with a continuous suture might decrease the chance of anastomotic failure in the deep surgical field via a median sternotomy. Postoperative anticoagulation after PAPVC repair is controversial. In their report of pericardial patch augmentation of the anastomotic site, Ban and colleagues8 indicated that postoperative anticoagulation was not necessary. However, many surgeons remain concerned that thrombus formation could develop at the suture line or the LAA trabeculae in the low-flow state of the pulmonary venous circulation. Therefore, we used oral anticoagulant postoperatively for 3 months with a target international normalized ratio of around 2.0.
Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement None declared.
References 1. Herlong JR, Jaggers JJ and Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies [Review]. Ann Thorac Surg 2000; 69: S56–S69. 2. Kalke BR, Carlson RG, Ferlic RM, Sellers RD and Lillehei CW. Partial anomalous pulmonary venous connections. Am J Cardiol 1967; 20: 90–101. 3. ElBardissi AW, Dearani JA, Suri RM and Danielson GK. Left-sided partial anomalous pulmonary venous connections. Ann Thorac Surg 2008; 85: 1007–1014. 4. Van Meter C Jr, LeBlanc JG, Culpepper WS 3rd and Ochsner JL. Partial anomalous pulmonary venous return. Circulation 1990; 82(Suppl IV): 195–198. 5. Babb JD, McGlynn TJ, Pierce WS and Kirkman PM. Isolated partial anomalous venous connection: a congenital defect with late and serious complications. Ann Thorac Surg 1981; 31: 540–541. 6. Bello AG, Daggett WM, Hutter AM Jr, Desanctis RW, Buckley MJ and Austen WG. Surgical correction of partial anomalous venous drainage with intact atrial septum: association with acquired valve lesions. J Cardiovasc Surg 1973; 14: 357–364. 7. Ports TA, Turley K, Brundage BH and Ebert PA. Operative correction of total left anomalous pulmonary venous return. Ann Thorac Surg 1978; 27: 246–249. 8. Ban T, Sakata R and Hirata K. Surgical treatment of partial anomalous pulmonary venous connection of the left lung (lt-PAPVC). J Cardiac Surg 1987; 2: 369–373.
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