Case Study
Transposition of great arteries and partial anomalous pulmonary venous drainage
Asian Cardiovascular & Thoracic Annals 21(6) 713–716 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312463217 aan.sagepub.com
Sachin Talwar, Palleti Rajashekar, Veeram Arun Reddy, Shiv Kumar Choudhary and Balram Airan
Abstract We describe the technical aspects of performing an atrial switch operation in a 4-year-old boy with d-transposition of the great arteries and partial anomalous drainage of the left-sided pulmonary veins. The rarity of the condition is discussed.
Keywords Heart defects, congenital, heart septal defects, atrial, pulmonary veins, pulmonary circulation, transposition of great vessels
Introduction The arterial switch operation is the procedure of choice for patients with d-transposition of great arteries (d-TGA) when no contraindications exist. However, an atrial switch operation is an important tool in the armamentarium of pediatric cardiac surgeons because it is considered definite palliation for isolated atrioventricular discordance and is a part of the anatomic repair for congenitally corrected TGA. In countries where patients with d-TGA present late in life, with regressed left ventricle (LV), this operation offers lower-risk palliation over the strategy of a staged arterial switch after LV preparation, or an arterial switch operation with LV preparation using mechanical circulatory assist. However, the atrial switch operation may need to be modified when additional defects coexist, or in the presence of anatomical variations. In a recent review, we discussed the indications, techniques, and pitfalls of this operation in detail.1 In this case report, we focus on the technical aspects of this operation in the setting of dTGA, intact ventricular septum, a regressed LV, and associated partial anomalous pulmonary venous drainage (PAPVC) of the entire left lung.
grade II clubbing were present. A loud 2nd heart sound was heard in the right 2nd intercostal space, just lateral to the right sternal border. Chest radiography showed mild cardiomegaly with a typical ‘‘egg on side’’ cardiac shadow with increased pulmonary plethora, more marked on the left side. Transthoracic echocardiography suggested situs solitus, levocardia, atrioventricular concordance, ventriculoarterial discordance, a small patent foramen ovale, and dilated main and branch pulmonary arteries. The LV was regressed and the right ventricle was normal. The pulmonary venous drainage was not clear. On cardiac catheterization, the saturation in the upper part of the superior vena cava above the innominate vein was 63% while in the lower part, it was 76%. The saturation in the vertical vein on the left side was 97%. The mean pulmonary venous pressure was 18 mm Hg; d-TGA was confirmed. Injection of the vertical vein showed the dye filling the vertical vein to innominate vein to superior vena cava to right atrium (Figure 1). The right-sided pulmonary veins drained normally to the left atrium (LA).
Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi, India
Case report A 4-year-old boy weighing 13 kg presented with cyanosis for 3 years, poor weight gain, easy fatigability, and effort intolerance. On examination, mild cyanosis and
Corresponding author: Sachin Talwar, MCh, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi 110029, India. Email: [email protected]
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
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Asian Cardiovascular & Thoracic Annals 21(6)
Figure 1. Angiogram shows the left sided pulmonary valve pulmonary veins (PV) into vertical vein (VV) that drains into the innominate vein (IV).
Computed tomography-angiography confirmed PAPVC of the left-sided pulmonary veins to the left brachiocephalic vein via an ascending vertical vein on the left side (Figure 2). An atrial switch operation with PAPVC repair was planned. The surgical approach was via a standard median sternotomy and vertical pericardiotomy. After systemic heparinization, both venae cavae were cannulated in their extrapericardial course. Extrapericardial cannulation was desirable because it leaves the pericardial reflection intact for reconstruction of the pulmonary venous baffle by the in-situ pedicled pericardium technique.1 Under cardiopulmonary bypass and aortic crossclamping, cold blood cardioplegia was delivered through the aortic root. The right atrium was opened, the atrial septal defect was enlarged. Left-sided pulmonary veins draining into the vertical vein and thereby to the innominate vein were noted. The vertical vein was freed from the surrounding structures, ligated, and divided flush with its junction with the innominate vein (Figure 3). The LA appendage was opened between stay sutures, and the divided and filleted end of the vertical vein was anastomosed to the LA appendage in end-to-side fashion (Figure 3). This procedure directed the left-sided pulmonary venous return to the LA via the LA appendage. Dissection was carried out in the interatrial groove on the right side to expose the LA completely. A large opening was made in the LA, and its margins were excised. A 1.5 1.5-cm Dacron patch was sutured behind the mitral valve and in front of the orifice of the right-sided pulmonary veins and the orifice of the LA appendage. When the suture line was continued along the right edge of the atrial septal defect,
Figure 2. Computed tomography-angiogram in coronal view. The pulmonary veins (arrows) from the left upper and some of the basal segments are draining into the left superior vena cava (*). #: right superior vena cava; CV: connecting vein.
it separated the pulmonary veins from the mitral valve and redirected pulmonary venous return into the pericardial cavity through the opening in the LA. The coronary sinus was cut back and sutured to the LA wall to redirect it to the mitral valve. The right edge of the right atriotomy was sutured to the left edge of the atrial septal defect (Figure 3). In this manner, the venae cavae and the coronary sinus return were routed to the mitral valve. The left edge of the right atriotomy was sutured to the edges of the pericardium on the right side. The pulmonary venous return hence exited into the pericardial cavity and was routed to the tricuspid valve. The patient was weaned from cardiopulmonary bypass. The aortic crossclamp time was 63 min and cardiopulmonary bypass time was 136 min. His postoperative recovery was uneventful. At the 6-month follow-up, echocardiography showed unobstructed systemic and pulmonary venous baffles and good ventricular function. The systemic saturation on room air is 100%.
Discussion The combination of d-TGA and partial anomalous PAPVC is rare. There are few case reports of TGA with TAPVC, but not with PAPVC.2,3 Therefore, it is important to report the technical considerations in performing an atrial switch in this setting. The important step in this patient was the dissection and flush ligation and division of the vertical vein from the innominate vein. This ensured that we were able to fillet the vertical vein so that an anastomosis between this vein and the LA appendage provided unobstructed pulmonary venous drainage because of the close proximity of
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
Talwar et al.
715
Figure 3. Schematic diagrams showing surgical reconstruction. (a) The vertical vein (VV) has been transfixed and divided (D) flush with the innominate vein (IV) and is anastomosed to the left atrial appendage (LAA). (b) The completed anastomosis between the VV and LAA. (c) The right atrium has been opened. The arrow shows the passage of pulmonary venous return into the LAA. The left atrium (LA) has been opened and its edges excised. (d) A Dacron patch (D) has been sutured in front of pulmonary vein the openings (dotted structures) behind the mitral valve. (e) The right edge of the right atriotomy has been sutured to the left edge of the atrial septal defect to direct the SVC and IVC to the MV. (f) The pulmonary venous chamber reconstruction is completed by suturing the left edge of the right atriotomy to the edges of the pericardium (P) on the right side. Ao: aorta; PV: pulmonary vein; IVC: inferior vena cava; MV: mitral valve; SVC: superior vena cava.
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Asian Cardiovascular & Thoracic Annals 21(6)
both structures, and a tension-free widely patent anastomosis. The remaining steps of the operation were fairly standard and combined the principles of the Senning, Mustard, and Schumacher modifications for construction of various baffles.1 We feel that with the dwindling numbers of atrial switch operations these days, this report would benefit the less experienced surgeon who may occasionally encounter such patients. Another important point in this patient was survival into childhood. In d-TGA, the circulations are in parallel, and an unobstructed interatrial communication is essential for survival. However, this patient survived into childhood despite a restrictive interatrial communication because the PAPVC ensured mixing of oxygenated and deoxygenated blood at the level of the innominate vein.
Conflicts of interest statement None declared.
References 1. Talwar S, Nair VV, Choudhary SK, Airan B. Arterial switch operation in the current era: modifications and pitfalls. World J Pediatr Congenit Heart Surg 2012; 3: 96–103. Available at: http://pch.sagepub.com/content/3/1/96.full. pdfþhtml. Accessed July 31, 2012. 2. Sapsford RN, Aberdeen E, Watson DA and Crew AD. Transposed great arteries combined with totally anomalous Pulmonary veins: a report of a successful correction. J Thorac Cardiovasc Surg 1972; 63: 360–366. 3. Barbero-Marcial M, Verginelli G, Vila J and Zerbini EJ. Transposition of the great arteries associated with total anomalous pulmonary venous connection: a surgical approach. Ann Thorac Surg 1984; 37: 92–94.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
Transposition of great arteries and partial anomalous pulmonary venous drainage
Asian Cardiovascular & Thoracic Annals 21(6) 713–716 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312463217 aan.sagepub.com
Sachin Talwar, Palleti Rajashekar, Veeram Arun Reddy, Shiv Kumar Choudhary and Balram Airan
Abstract We describe the technical aspects of performing an atrial switch operation in a 4-year-old boy with d-transposition of the great arteries and partial anomalous drainage of the left-sided pulmonary veins. The rarity of the condition is discussed.
Keywords Heart defects, congenital, heart septal defects, atrial, pulmonary veins, pulmonary circulation, transposition of great vessels
Introduction The arterial switch operation is the procedure of choice for patients with d-transposition of great arteries (d-TGA) when no contraindications exist. However, an atrial switch operation is an important tool in the armamentarium of pediatric cardiac surgeons because it is considered definite palliation for isolated atrioventricular discordance and is a part of the anatomic repair for congenitally corrected TGA. In countries where patients with d-TGA present late in life, with regressed left ventricle (LV), this operation offers lower-risk palliation over the strategy of a staged arterial switch after LV preparation, or an arterial switch operation with LV preparation using mechanical circulatory assist. However, the atrial switch operation may need to be modified when additional defects coexist, or in the presence of anatomical variations. In a recent review, we discussed the indications, techniques, and pitfalls of this operation in detail.1 In this case report, we focus on the technical aspects of this operation in the setting of dTGA, intact ventricular septum, a regressed LV, and associated partial anomalous pulmonary venous drainage (PAPVC) of the entire left lung.
grade II clubbing were present. A loud 2nd heart sound was heard in the right 2nd intercostal space, just lateral to the right sternal border. Chest radiography showed mild cardiomegaly with a typical ‘‘egg on side’’ cardiac shadow with increased pulmonary plethora, more marked on the left side. Transthoracic echocardiography suggested situs solitus, levocardia, atrioventricular concordance, ventriculoarterial discordance, a small patent foramen ovale, and dilated main and branch pulmonary arteries. The LV was regressed and the right ventricle was normal. The pulmonary venous drainage was not clear. On cardiac catheterization, the saturation in the upper part of the superior vena cava above the innominate vein was 63% while in the lower part, it was 76%. The saturation in the vertical vein on the left side was 97%. The mean pulmonary venous pressure was 18 mm Hg; d-TGA was confirmed. Injection of the vertical vein showed the dye filling the vertical vein to innominate vein to superior vena cava to right atrium (Figure 1). The right-sided pulmonary veins drained normally to the left atrium (LA).
Cardiothoracic Center, All India Institute of Medical Sciences, New Delhi, India
Case report A 4-year-old boy weighing 13 kg presented with cyanosis for 3 years, poor weight gain, easy fatigability, and effort intolerance. On examination, mild cyanosis and
Corresponding author: Sachin Talwar, MCh, Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi 110029, India. Email: [email protected]
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
714
Asian Cardiovascular & Thoracic Annals 21(6)
Figure 1. Angiogram shows the left sided pulmonary valve pulmonary veins (PV) into vertical vein (VV) that drains into the innominate vein (IV).
Computed tomography-angiography confirmed PAPVC of the left-sided pulmonary veins to the left brachiocephalic vein via an ascending vertical vein on the left side (Figure 2). An atrial switch operation with PAPVC repair was planned. The surgical approach was via a standard median sternotomy and vertical pericardiotomy. After systemic heparinization, both venae cavae were cannulated in their extrapericardial course. Extrapericardial cannulation was desirable because it leaves the pericardial reflection intact for reconstruction of the pulmonary venous baffle by the in-situ pedicled pericardium technique.1 Under cardiopulmonary bypass and aortic crossclamping, cold blood cardioplegia was delivered through the aortic root. The right atrium was opened, the atrial septal defect was enlarged. Left-sided pulmonary veins draining into the vertical vein and thereby to the innominate vein were noted. The vertical vein was freed from the surrounding structures, ligated, and divided flush with its junction with the innominate vein (Figure 3). The LA appendage was opened between stay sutures, and the divided and filleted end of the vertical vein was anastomosed to the LA appendage in end-to-side fashion (Figure 3). This procedure directed the left-sided pulmonary venous return to the LA via the LA appendage. Dissection was carried out in the interatrial groove on the right side to expose the LA completely. A large opening was made in the LA, and its margins were excised. A 1.5 1.5-cm Dacron patch was sutured behind the mitral valve and in front of the orifice of the right-sided pulmonary veins and the orifice of the LA appendage. When the suture line was continued along the right edge of the atrial septal defect,
Figure 2. Computed tomography-angiogram in coronal view. The pulmonary veins (arrows) from the left upper and some of the basal segments are draining into the left superior vena cava (*). #: right superior vena cava; CV: connecting vein.
it separated the pulmonary veins from the mitral valve and redirected pulmonary venous return into the pericardial cavity through the opening in the LA. The coronary sinus was cut back and sutured to the LA wall to redirect it to the mitral valve. The right edge of the right atriotomy was sutured to the left edge of the atrial septal defect (Figure 3). In this manner, the venae cavae and the coronary sinus return were routed to the mitral valve. The left edge of the right atriotomy was sutured to the edges of the pericardium on the right side. The pulmonary venous return hence exited into the pericardial cavity and was routed to the tricuspid valve. The patient was weaned from cardiopulmonary bypass. The aortic crossclamp time was 63 min and cardiopulmonary bypass time was 136 min. His postoperative recovery was uneventful. At the 6-month follow-up, echocardiography showed unobstructed systemic and pulmonary venous baffles and good ventricular function. The systemic saturation on room air is 100%.
Discussion The combination of d-TGA and partial anomalous PAPVC is rare. There are few case reports of TGA with TAPVC, but not with PAPVC.2,3 Therefore, it is important to report the technical considerations in performing an atrial switch in this setting. The important step in this patient was the dissection and flush ligation and division of the vertical vein from the innominate vein. This ensured that we were able to fillet the vertical vein so that an anastomosis between this vein and the LA appendage provided unobstructed pulmonary venous drainage because of the close proximity of
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
Talwar et al.
715
Figure 3. Schematic diagrams showing surgical reconstruction. (a) The vertical vein (VV) has been transfixed and divided (D) flush with the innominate vein (IV) and is anastomosed to the left atrial appendage (LAA). (b) The completed anastomosis between the VV and LAA. (c) The right atrium has been opened. The arrow shows the passage of pulmonary venous return into the LAA. The left atrium (LA) has been opened and its edges excised. (d) A Dacron patch (D) has been sutured in front of pulmonary vein the openings (dotted structures) behind the mitral valve. (e) The right edge of the right atriotomy has been sutured to the left edge of the atrial septal defect to direct the SVC and IVC to the MV. (f) The pulmonary venous chamber reconstruction is completed by suturing the left edge of the right atriotomy to the edges of the pericardium (P) on the right side. Ao: aorta; PV: pulmonary vein; IVC: inferior vena cava; MV: mitral valve; SVC: superior vena cava.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
716
Asian Cardiovascular & Thoracic Annals 21(6)
both structures, and a tension-free widely patent anastomosis. The remaining steps of the operation were fairly standard and combined the principles of the Senning, Mustard, and Schumacher modifications for construction of various baffles.1 We feel that with the dwindling numbers of atrial switch operations these days, this report would benefit the less experienced surgeon who may occasionally encounter such patients. Another important point in this patient was survival into childhood. In d-TGA, the circulations are in parallel, and an unobstructed interatrial communication is essential for survival. However, this patient survived into childhood despite a restrictive interatrial communication because the PAPVC ensured mixing of oxygenated and deoxygenated blood at the level of the innominate vein.
Conflicts of interest statement None declared.
References 1. Talwar S, Nair VV, Choudhary SK, Airan B. Arterial switch operation in the current era: modifications and pitfalls. World J Pediatr Congenit Heart Surg 2012; 3: 96–103. Available at: http://pch.sagepub.com/content/3/1/96.full. pdfþhtml. Accessed July 31, 2012. 2. Sapsford RN, Aberdeen E, Watson DA and Crew AD. Transposed great arteries combined with totally anomalous Pulmonary veins: a report of a successful correction. J Thorac Cardiovasc Surg 1972; 63: 360–366. 3. Barbero-Marcial M, Verginelli G, Vila J and Zerbini EJ. Transposition of the great arteries associated with total anomalous pulmonary venous connection: a surgical approach. Ann Thorac Surg 1984; 37: 92–94.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Downloaded from aan.sagepub.com at MICHIGAN STATE UNIV LIBRARIES on June 11, 2015
