Case Study
A rare variety of mixed total anomalous pulmonary venous connection
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(7) 849–851 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313486120 aan.sagepub.com
Amit Mishra, Pranav Sharma, Ramesh Patel, Jigar Panchal and Atul Solanki
Abstract We describe a rare case of mixed supra- and infracardiac total anomalous pulmonary venous connection in a 2-month-old boy weighing 2.3 kg who was admitted with central cyanosis. Although the surgical repair was successful, the child died due to pulmonary hypertensive crisis.
Keywords Cardiovascular surgical procedures, heart defects, congenital, pulmonary veins, vascular malformations, vascular surgical procedures
Introduction Total anomalous pulmonary venous connection (TAPVC) accounts for approximately 2% of congenital cardiac anomalies, and 5%–10% of total anomalous pulmonary venous connections are a mixed type.1,2 Several varieties of mixed TAPVC have been described, but mixed supra- and infracardiac TAPVC has not been reported so far in the English literature.
Case report A 2-month-old boy weighing 2.3 kg was admitted to our institute with central cyanosis, tachypnea, and failure to thrive. He was electively intubated. Arterial blood gases indicated severe metabolic acidosis. The total white blood cell count was 16,000 mm 3, and quantitative C-reactive protein was positive. Chest radiography showed cardiomegaly with features of pulmonary venous congestion. Echocardiography showed situs solitus, atrioventricular and ventriculoarterial concordance, normally related great arteries, a small patent ductus arteriosus with a bidirectional shunt, a small atrial septal defect with a right-to-left shunt, and severe pulmonary arterial hypertension. The right-sided pulmonary veins formed a confluence and drained via the vertical vein to the innominate vein, but the leftsided pulmonary venous anatomy was not clear. Computed tomography with 3-dimensional reconstruction revealed 2 right upper lobe pulmonary veins
forming a confluence and draining into a left-sided ascending vertical vein to the innominate vein which finally drained into a right-sided superior vena cava. On the left side, the left upper and lower pulmonary veins and right lower pulmonary vein formed a confluence that drained into the descending vertical vein and finally into the portal vein (Figure 1). Emergency surgery was undertaken. After sternotomy and thymectomy, the left-sided vertical vein was looped. Under cardiopulmonary bypass with aortic and bicaval venous cannulation and moderate hypothermia, the patent ductus arteriosus was ligated. Under cardioplegic arrest, the heart was deflected into the right pleural cavity, and the pulmonary veins were identified (Figure 2A). Both lower and upper common chambers were opened transversely and anastomosed to each other, forming a single common pulmonary venous chamber (Figure 2B). The vertical vein draining below diaphragm was also opened up to the level of the diaphragm. The left atrium was opened and the atrial Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center, New Civil Hospital Campus, Asarwa, India Corresponding author: Amit Mishra, Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center, New Civil Hospital Campus, Asarwa, Ahmedabad 380016, Gujarat, India. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
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Asian Cardiovascular & Thoracic Annals 22(7)
Figure 1. Helical contrast computed tomography with 3-dimensional reconstruction, showing mixed supra- and infracardiac total anomalous pulmonary venous connection with an ascending and descending vertical vein. LT: left; RT PV: right pulmonary vein.
septal defect was closed directly. The left atrium was anastomosed to the common chamber (Figure 2C). The child was weaned off cardiopulmonary bypass with the support of milrinone and dobutamine. The vertical vein draining to the innominate vein was ligated, while the descending vein draining below diaphragm was left open. The child was moved to the intensive care unit with stable hemodynamic parameters. Postoperative echocardiography revealed a widely open pulmonary venous confluence draining into the left atrium, and an intact intraatrial septum with good biventricular function. The patient was electively ventilated for 72 h, during which, he had 2 episodes of pulmonary hypertensive crisis. He was extubated and remained stable for 24 h, but required reintubation for left lung collapse. He was extubated again after 60 h and maintained good hemodynamics for 24 h, but suffered an episode of pulmonary hypertensive crisis from which he could not be revived.
Figure 2. Operative steps: (A) mixed supra- and infracardiac total anomalous pulmonary venous connection; (B) common chamber after anastomosis of upper and lower chambers; (C) anastomosis between the new common chamber and the left atrium.
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
Mishra et al.
851
Discussion The surgical classification and various types of mixed TAPVC have been described.3–5 Correct identification of the pulmonary venous anatomy is extremely important for optimal surgical correction. Echocardiography is a subjective investigation and may not be able to correctly identify the pulmonary venous connection in many instances. Computed tomography is the gold standard in such situations, and it can delineate the exact pulmonary venous anatomy. When there is the slightest doubt about the drainage of pulmonary venous chambers, it is always advisable to get a computed tomography scan to avoid on-table surprises. There is no consensus regarding management of the descending vertical vein in intracardiac TAPVC. Many surgeons ligate or divide the vertical vein, but we always leave the descending vertical vein open in infracardiac TAPVC because it is almost always obstructed distally. We have found that the dilated supradiaphragmatic portion of the descending vertical vein can be utilized in the construction of a wide anastomosis between the left atrium and the common chamber, which is useful in neonates and premature babies with a very small common chamber. It is extremely important to emphasize the postoperative management for preventing pulmonary hypertensive crisis. This includes elective ventilation, minimal suction, and use of sildenafil and nitrous oxide ventilation, as required. Late presentation of cases of mixed TAPVC with an infracardiac component (which is invariably obstructive) and severe pulmonary arterial hypertension is associated with high mortality. Late presentation, poor
preoperative general condition, an ongoing septic process, obstruction in the pulmonary venous system, and severe pulmonary hypertension leading to pulmonary hypertensive crisis were important factors in determining the unfavorable outcome in our patient. 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. Bharati S and Lev M. Congenital anomalies of the pulmonary veins. Cardiovasc Clin 1973; 5: 23–41. 2. Total anomalous pulmonary venous connection. Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Kirklin/Barratt-Boyes Cardiac Surgery, 4th ed. Philadelphia: Elsevier, 2013:1183. 3. Chowdhury UK, Airan B, Malhotra A, et al. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg 2008; 135: 106–116. 4. Wang Z, Qi W, Hu Y, et al. Mixed-type total anomalous pulmonary venous connection in an adult. Ann Thorac Surg 2011; 91: 610–612. 5. Chowdhury UK, Malhotra A, Kothari SS, et al. A suggested new surgical classification for mixed totally anomalous pulmonary venous connection. Cardiol Young 2007; 17: 342–353.
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
A rare variety of mixed total anomalous pulmonary venous connection
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(7) 849–851 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313486120 aan.sagepub.com
Amit Mishra, Pranav Sharma, Ramesh Patel, Jigar Panchal and Atul Solanki
Abstract We describe a rare case of mixed supra- and infracardiac total anomalous pulmonary venous connection in a 2-month-old boy weighing 2.3 kg who was admitted with central cyanosis. Although the surgical repair was successful, the child died due to pulmonary hypertensive crisis.
Keywords Cardiovascular surgical procedures, heart defects, congenital, pulmonary veins, vascular malformations, vascular surgical procedures
Introduction Total anomalous pulmonary venous connection (TAPVC) accounts for approximately 2% of congenital cardiac anomalies, and 5%–10% of total anomalous pulmonary venous connections are a mixed type.1,2 Several varieties of mixed TAPVC have been described, but mixed supra- and infracardiac TAPVC has not been reported so far in the English literature.
Case report A 2-month-old boy weighing 2.3 kg was admitted to our institute with central cyanosis, tachypnea, and failure to thrive. He was electively intubated. Arterial blood gases indicated severe metabolic acidosis. The total white blood cell count was 16,000 mm 3, and quantitative C-reactive protein was positive. Chest radiography showed cardiomegaly with features of pulmonary venous congestion. Echocardiography showed situs solitus, atrioventricular and ventriculoarterial concordance, normally related great arteries, a small patent ductus arteriosus with a bidirectional shunt, a small atrial septal defect with a right-to-left shunt, and severe pulmonary arterial hypertension. The right-sided pulmonary veins formed a confluence and drained via the vertical vein to the innominate vein, but the leftsided pulmonary venous anatomy was not clear. Computed tomography with 3-dimensional reconstruction revealed 2 right upper lobe pulmonary veins
forming a confluence and draining into a left-sided ascending vertical vein to the innominate vein which finally drained into a right-sided superior vena cava. On the left side, the left upper and lower pulmonary veins and right lower pulmonary vein formed a confluence that drained into the descending vertical vein and finally into the portal vein (Figure 1). Emergency surgery was undertaken. After sternotomy and thymectomy, the left-sided vertical vein was looped. Under cardiopulmonary bypass with aortic and bicaval venous cannulation and moderate hypothermia, the patent ductus arteriosus was ligated. Under cardioplegic arrest, the heart was deflected into the right pleural cavity, and the pulmonary veins were identified (Figure 2A). Both lower and upper common chambers were opened transversely and anastomosed to each other, forming a single common pulmonary venous chamber (Figure 2B). The vertical vein draining below diaphragm was also opened up to the level of the diaphragm. The left atrium was opened and the atrial Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center, New Civil Hospital Campus, Asarwa, India Corresponding author: Amit Mishra, Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center, New Civil Hospital Campus, Asarwa, Ahmedabad 380016, Gujarat, India. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
850
Asian Cardiovascular & Thoracic Annals 22(7)
Figure 1. Helical contrast computed tomography with 3-dimensional reconstruction, showing mixed supra- and infracardiac total anomalous pulmonary venous connection with an ascending and descending vertical vein. LT: left; RT PV: right pulmonary vein.
septal defect was closed directly. The left atrium was anastomosed to the common chamber (Figure 2C). The child was weaned off cardiopulmonary bypass with the support of milrinone and dobutamine. The vertical vein draining to the innominate vein was ligated, while the descending vein draining below diaphragm was left open. The child was moved to the intensive care unit with stable hemodynamic parameters. Postoperative echocardiography revealed a widely open pulmonary venous confluence draining into the left atrium, and an intact intraatrial septum with good biventricular function. The patient was electively ventilated for 72 h, during which, he had 2 episodes of pulmonary hypertensive crisis. He was extubated and remained stable for 24 h, but required reintubation for left lung collapse. He was extubated again after 60 h and maintained good hemodynamics for 24 h, but suffered an episode of pulmonary hypertensive crisis from which he could not be revived.
Figure 2. Operative steps: (A) mixed supra- and infracardiac total anomalous pulmonary venous connection; (B) common chamber after anastomosis of upper and lower chambers; (C) anastomosis between the new common chamber and the left atrium.
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
Mishra et al.
851
Discussion The surgical classification and various types of mixed TAPVC have been described.3–5 Correct identification of the pulmonary venous anatomy is extremely important for optimal surgical correction. Echocardiography is a subjective investigation and may not be able to correctly identify the pulmonary venous connection in many instances. Computed tomography is the gold standard in such situations, and it can delineate the exact pulmonary venous anatomy. When there is the slightest doubt about the drainage of pulmonary venous chambers, it is always advisable to get a computed tomography scan to avoid on-table surprises. There is no consensus regarding management of the descending vertical vein in intracardiac TAPVC. Many surgeons ligate or divide the vertical vein, but we always leave the descending vertical vein open in infracardiac TAPVC because it is almost always obstructed distally. We have found that the dilated supradiaphragmatic portion of the descending vertical vein can be utilized in the construction of a wide anastomosis between the left atrium and the common chamber, which is useful in neonates and premature babies with a very small common chamber. It is extremely important to emphasize the postoperative management for preventing pulmonary hypertensive crisis. This includes elective ventilation, minimal suction, and use of sildenafil and nitrous oxide ventilation, as required. Late presentation of cases of mixed TAPVC with an infracardiac component (which is invariably obstructive) and severe pulmonary arterial hypertension is associated with high mortality. Late presentation, poor
preoperative general condition, an ongoing septic process, obstruction in the pulmonary venous system, and severe pulmonary hypertension leading to pulmonary hypertensive crisis were important factors in determining the unfavorable outcome in our patient. 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. Bharati S and Lev M. Congenital anomalies of the pulmonary veins. Cardiovasc Clin 1973; 5: 23–41. 2. Total anomalous pulmonary venous connection. Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Kirklin/Barratt-Boyes Cardiac Surgery, 4th ed. Philadelphia: Elsevier, 2013:1183. 3. Chowdhury UK, Airan B, Malhotra A, et al. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg 2008; 135: 106–116. 4. Wang Z, Qi W, Hu Y, et al. Mixed-type total anomalous pulmonary venous connection in an adult. Ann Thorac Surg 2011; 91: 610–612. 5. Chowdhury UK, Malhotra A, Kothari SS, et al. A suggested new surgical classification for mixed totally anomalous pulmonary venous connection. Cardiol Young 2007; 17: 342–353.
Downloaded from aan.sagepub.com at UNIV OF MONTANA on April 4, 2015
