reports
Case
0 Springer-Verlag 1992
Em J Cardio-thorac
Surg (1992) 6:452-454
Large systemic collateral arteries developing late after total repair of tetralogy of Fallot J. T. Lund Department
of Thoracic Surgery RT, Rigshospitalet,
Copenhagen,
Denmark
Abstract. A 22-year-old man with tetralogy of Fallot successfully underwent a corrective operation. Twenty years after total correction, large systemic collateral arteries (SCA) developed. The SCA arose from the right subclavian and internal mammary arteries. The SCA were not present at the time of total repair and thoracotomy had not been performed on this side. A closed-tube thoracostomy for postoperative pneumothorax may be responsible for the development of the large SCA. [Eur J Cardio-thorac Surg (1992) 6:452-4541 Key words: Tetralogy of Fallot - Collaterals - Thoracostomy
Increased collateral pulmonary blood flow is common among patients with tetralogy of Fallot (TF) [5]. Major aortopulmonary collateral arteries are known to exist in cases of TF with pulmonary atresia, but is seldom seen in TF without pulmonary atresia [5]. Development of large systemic collateral arteries @CA) after total correction has not previously been described. A patient who developed large SCA late after an otherwise successful total correction is reported. The development of large SCA may be a rare complication of closed-tube thoracostomy as described by others [l, 2, 41. Case report A male patient with TF was palliatively operated upon in 1949, at the age of 3 years, with establishment of a Blalock-Taussig anastomosis on the left side. When 22 years old, the patient underwent total correction with closure of the ventricular septal defect, pulmonary valvutomy, resection of an infundibular stenosis and insertion of a transvalvular pericardial patch into the outflow tract of the right ventricle (preoperative values, Table 1). The previously established Blalock-Taussig anastomosis was found to be closed. During cardiopulmonary bypass, a large intracardiac return was noted. Postoperatively, a right-sided pneumothorax was treated with closed-tube thoracostomy in the second right intercostal space in the midclavicular line for 3 days (Fig. 1). Two days postoperatively, dye dilution curves were performed demonstrating a left-to-right shunt, 11% of the pulmonary flow. This investigation was repeated 2 years later and no shunt was demonstrated. During the following Received for publication: Accepted for publication:
November 4, 1991 March 5, 1992
3 years, the patient had no cardiopulmonary complaints. Thereafter he was lost to follow-up. Twenty years after the total correction, the patient contacted the hospital because of increasing exertional dyspnoea. Auscultation indicated aortic valve regurgitation and a chest X-ray showed considerable cardiomegaly. Angiography demonstrated aortic valve incompetence grade 2/4, dilatation of a hyperkinetic left ventricle and pulmonary valve incompetence grade 4/4. Catheterization data are shown in Table 1. Incompetence of the valves was considered the main problem though an increased collateral pulmonary circulation to the right upper lobe was suspected due to wash-out of the contrast on angiography. It was therefore decided to explore the right lung for SCA before correction of the pulmonary and aortic valve incompetence. At operation, extensive bleeding and adhesions between the chestwall and the lung were found and further surgery was postponed until the collateral pulmonary circulation was reevaluated. Selective arteriography of the right subclavian artery revealed an extensive network of anastomoses between the right subclavian artery branches and the pulmonary circulation. The collateral arteries resulted in rapid opacification of the branches of the right pulmonary artery to the upper lobe of the right lung (Fig. 2). Open-heart operation was performed preceded by ligation of the right internal mammary, lateral thoracic and subscapular arteries Table 1. Catheterization
1968 1988
data
PA mmHg
RV mmHg
Hb mmol/l
SAT %
IO/4 1214
11015 2414
13.2 9.6
84 94
1968: Prior to total repair. 1988: 20 years after total repair. PA= pulmonary artery, RV = right ventricle, SAT = arterial oxygen saturation
453
which all communicated with right pulmonary artery branches. At the open-heart operation, a minor perforation of the right coronary cusp of the aortic valve was sutured and the pulmonary valve replaced with a homograft pulmonary valve. During the cardiopulmonary bypass, the cardiac return was moderate indicating that no significant collateral pulmonary circulation remained. No major complications occurred postoperatively. The patient was well for the first 6 months after operation. At that time, he complained of mild exertional dyspnoea. X-ray examination of the chest showed an increased vascular pattern in the right upper lobe and reestablished collaterals were suspected. One and a half years after the last operation, angiography was repeated. The valves were well-functioning without regurgitation, but in the right lung a widespread collateral circulation was reestablished, with multiple small systemic arteries arising from the chest wall penetrating the lung surface. However, no major systemic arteries were found to contribute to the collateral blood supply of the right upper lobe. No peripheral pulmonary stenosis was seen at pulmonary angiography.
Discussion
An increased collateral blood flow to the pulmonary circulation is often seen in patients with TF. Most patients have a diffusely enlarged network of bronchial collateral arteries, deeply cyanotic cases in particular. Major aortopulmonary collateral arteries in TF are rare [7], but in patients with TF and pulmonary atresia, the pulmonary circulation may arise directly from aortic collaterals of the descending aorta or indirectly via aortic collaterals arising from major branches of aorta (e.g. internal mammary, subclavian) [5]. In this patient, large SCA were found on the non-operated side (right) 20 years after total correction. During this period there were signs of increased collateral pulmonary circulation, but a postoperative right-sided pneumothorax excluded the presence of the large SCA at that time. Shunt calculation performed 2 days and 2 years after operation supported the absence of the SCA at that time. The observed increased cardiac return during operation and the calculated 11% left-to-right shunt 2 days postoperatively were probably due to diffusely enlarged bronchial collateral arteries, which usually disappear after successful total repair corroborated in this case by dye dilution shunt calculation 2 years after operation.
Development of large SCA after total correction has not been described previously. The pneumothorax excluded the presence of SCA, but the treatment of the pneumothorax may have caused the SCA. Closed-tube thoracostomy has been complicated by development of a systemic-to-pulmonary shunt in two patients with spontaneous pneumothorax [I, 2, 41. In our case, the patient had undergone left-sided thoracotomy in infancy to establish the Blalock-Taussing shunt without developing collaterals on that side, thus special conditions in the right upper lobe may dispose to development of SCA. Persistent hypoperfusion of the lobe may be the explanation. In one case of systemic-to-pulmonary shunt following pneumothorax treated with closed-tube thoracostomy, hypoperfusion of the upper lobe, in which the shunt had developed, was found [2]. Stenosis in the pulmonary arteries is common in patients with TF [6, 81. The most common are central stenosis, very few isolated peripheral stenosis are described [6]. In our case, hypoperfusion might be caused by a peripheral stenosis of the pulmonary artery, though no stenosis was seen on pulmonary angiography. General pulmonary hypoperfusion after total repair due to severe pulmonary regurgitation may dispose to development of SCA. However, pulmonary regurgitation is normally tolerated well after total repair especially in patients without residual pulmonary stenosis [3]. We observed low pulmonary arterial pressure, but there were no other signs of right sided cardiac failure and the patient was without symptoms for 20 years after total correction. The exact mechanism of the SCA development thus remains obscure, but it seems justified to consider adhesions due to the previous pneumothorax and tube thoracostomy to be an important factor. References 1. Cox PA, Keshishian JM, Blades BB (1967) Traumatic arteriovenous tistula of the chest wall and lung. J Thorac Cardiovasc Surg 54: 109-112 2. Fein AB, Godwin JD, Moore AV, Moran JF, Young WG (1983) Systemic artery-to-pulmonary vascular shunt: a complication of closed-tube thoracostomy. AJR 140:917-919
454 3. Kirklin JK, Kirklin JW, Blackstone EH, Milan0 A, Pacific0 AD (1989) Effect of transannular patching on outcome after repair of tetralogy of Fallot. Ann Thorac Surg 48: 783-791 4. Lurus AG, Cowen RL, Eckert JF (1969) Systemic-pulmonary arteriovenous fistula following closed-tube thoracostomy. Radiology 92: 1296- 1298 5. Rabinovitch M, Herrera-Deleon V, Castaneda AR, Reid L (1981) Growth and development of the pulmonary vascular bed in patient with Tetralogy of Fallot with or without pulmonary atresia. Circulation 64:1234-1249 6. Rajani M, Shrivastava S, Tandon R, Bhargava S (1980) Right ventricular cineangiography in tetralogy of Fallot. Cardiovasc Intervent Radio1 3:13-17
7. Ramsay JM, Macartney FJ, Haworth SG (1985) Tetralogy of Fallot with major collateral arteries. Br Heart J 53: 167-172 8. Sharma SN, Sharma S, Shrivastava S, Rajani M, Tandon R (1989) Pulmonary arterial anatomy in tetralogy of Fallot. Int J Cardiol 25:33-38
Jens Teglgaard Lund, MD Department of Thoracic Surgery RT 2102 Rigshospitalet Blegdamsvej 9 DK-2100 Copenhagen Denmark
Case
0 Springer-Verlag 1992
Em J Cardio-thorac
Surg (1992) 6:452-454
Large systemic collateral arteries developing late after total repair of tetralogy of Fallot J. T. Lund Department
of Thoracic Surgery RT, Rigshospitalet,
Copenhagen,
Denmark
Abstract. A 22-year-old man with tetralogy of Fallot successfully underwent a corrective operation. Twenty years after total correction, large systemic collateral arteries (SCA) developed. The SCA arose from the right subclavian and internal mammary arteries. The SCA were not present at the time of total repair and thoracotomy had not been performed on this side. A closed-tube thoracostomy for postoperative pneumothorax may be responsible for the development of the large SCA. [Eur J Cardio-thorac Surg (1992) 6:452-4541 Key words: Tetralogy of Fallot - Collaterals - Thoracostomy
Increased collateral pulmonary blood flow is common among patients with tetralogy of Fallot (TF) [5]. Major aortopulmonary collateral arteries are known to exist in cases of TF with pulmonary atresia, but is seldom seen in TF without pulmonary atresia [5]. Development of large systemic collateral arteries @CA) after total correction has not previously been described. A patient who developed large SCA late after an otherwise successful total correction is reported. The development of large SCA may be a rare complication of closed-tube thoracostomy as described by others [l, 2, 41. Case report A male patient with TF was palliatively operated upon in 1949, at the age of 3 years, with establishment of a Blalock-Taussig anastomosis on the left side. When 22 years old, the patient underwent total correction with closure of the ventricular septal defect, pulmonary valvutomy, resection of an infundibular stenosis and insertion of a transvalvular pericardial patch into the outflow tract of the right ventricle (preoperative values, Table 1). The previously established Blalock-Taussig anastomosis was found to be closed. During cardiopulmonary bypass, a large intracardiac return was noted. Postoperatively, a right-sided pneumothorax was treated with closed-tube thoracostomy in the second right intercostal space in the midclavicular line for 3 days (Fig. 1). Two days postoperatively, dye dilution curves were performed demonstrating a left-to-right shunt, 11% of the pulmonary flow. This investigation was repeated 2 years later and no shunt was demonstrated. During the following Received for publication: Accepted for publication:
November 4, 1991 March 5, 1992
3 years, the patient had no cardiopulmonary complaints. Thereafter he was lost to follow-up. Twenty years after the total correction, the patient contacted the hospital because of increasing exertional dyspnoea. Auscultation indicated aortic valve regurgitation and a chest X-ray showed considerable cardiomegaly. Angiography demonstrated aortic valve incompetence grade 2/4, dilatation of a hyperkinetic left ventricle and pulmonary valve incompetence grade 4/4. Catheterization data are shown in Table 1. Incompetence of the valves was considered the main problem though an increased collateral pulmonary circulation to the right upper lobe was suspected due to wash-out of the contrast on angiography. It was therefore decided to explore the right lung for SCA before correction of the pulmonary and aortic valve incompetence. At operation, extensive bleeding and adhesions between the chestwall and the lung were found and further surgery was postponed until the collateral pulmonary circulation was reevaluated. Selective arteriography of the right subclavian artery revealed an extensive network of anastomoses between the right subclavian artery branches and the pulmonary circulation. The collateral arteries resulted in rapid opacification of the branches of the right pulmonary artery to the upper lobe of the right lung (Fig. 2). Open-heart operation was performed preceded by ligation of the right internal mammary, lateral thoracic and subscapular arteries Table 1. Catheterization
1968 1988
data
PA mmHg
RV mmHg
Hb mmol/l
SAT %
IO/4 1214
11015 2414
13.2 9.6
84 94
1968: Prior to total repair. 1988: 20 years after total repair. PA= pulmonary artery, RV = right ventricle, SAT = arterial oxygen saturation
453
which all communicated with right pulmonary artery branches. At the open-heart operation, a minor perforation of the right coronary cusp of the aortic valve was sutured and the pulmonary valve replaced with a homograft pulmonary valve. During the cardiopulmonary bypass, the cardiac return was moderate indicating that no significant collateral pulmonary circulation remained. No major complications occurred postoperatively. The patient was well for the first 6 months after operation. At that time, he complained of mild exertional dyspnoea. X-ray examination of the chest showed an increased vascular pattern in the right upper lobe and reestablished collaterals were suspected. One and a half years after the last operation, angiography was repeated. The valves were well-functioning without regurgitation, but in the right lung a widespread collateral circulation was reestablished, with multiple small systemic arteries arising from the chest wall penetrating the lung surface. However, no major systemic arteries were found to contribute to the collateral blood supply of the right upper lobe. No peripheral pulmonary stenosis was seen at pulmonary angiography.
Discussion
An increased collateral blood flow to the pulmonary circulation is often seen in patients with TF. Most patients have a diffusely enlarged network of bronchial collateral arteries, deeply cyanotic cases in particular. Major aortopulmonary collateral arteries in TF are rare [7], but in patients with TF and pulmonary atresia, the pulmonary circulation may arise directly from aortic collaterals of the descending aorta or indirectly via aortic collaterals arising from major branches of aorta (e.g. internal mammary, subclavian) [5]. In this patient, large SCA were found on the non-operated side (right) 20 years after total correction. During this period there were signs of increased collateral pulmonary circulation, but a postoperative right-sided pneumothorax excluded the presence of the large SCA at that time. Shunt calculation performed 2 days and 2 years after operation supported the absence of the SCA at that time. The observed increased cardiac return during operation and the calculated 11% left-to-right shunt 2 days postoperatively were probably due to diffusely enlarged bronchial collateral arteries, which usually disappear after successful total repair corroborated in this case by dye dilution shunt calculation 2 years after operation.
Development of large SCA after total correction has not been described previously. The pneumothorax excluded the presence of SCA, but the treatment of the pneumothorax may have caused the SCA. Closed-tube thoracostomy has been complicated by development of a systemic-to-pulmonary shunt in two patients with spontaneous pneumothorax [I, 2, 41. In our case, the patient had undergone left-sided thoracotomy in infancy to establish the Blalock-Taussing shunt without developing collaterals on that side, thus special conditions in the right upper lobe may dispose to development of SCA. Persistent hypoperfusion of the lobe may be the explanation. In one case of systemic-to-pulmonary shunt following pneumothorax treated with closed-tube thoracostomy, hypoperfusion of the upper lobe, in which the shunt had developed, was found [2]. Stenosis in the pulmonary arteries is common in patients with TF [6, 81. The most common are central stenosis, very few isolated peripheral stenosis are described [6]. In our case, hypoperfusion might be caused by a peripheral stenosis of the pulmonary artery, though no stenosis was seen on pulmonary angiography. General pulmonary hypoperfusion after total repair due to severe pulmonary regurgitation may dispose to development of SCA. However, pulmonary regurgitation is normally tolerated well after total repair especially in patients without residual pulmonary stenosis [3]. We observed low pulmonary arterial pressure, but there were no other signs of right sided cardiac failure and the patient was without symptoms for 20 years after total correction. The exact mechanism of the SCA development thus remains obscure, but it seems justified to consider adhesions due to the previous pneumothorax and tube thoracostomy to be an important factor. References 1. Cox PA, Keshishian JM, Blades BB (1967) Traumatic arteriovenous tistula of the chest wall and lung. J Thorac Cardiovasc Surg 54: 109-112 2. Fein AB, Godwin JD, Moore AV, Moran JF, Young WG (1983) Systemic artery-to-pulmonary vascular shunt: a complication of closed-tube thoracostomy. AJR 140:917-919
454 3. Kirklin JK, Kirklin JW, Blackstone EH, Milan0 A, Pacific0 AD (1989) Effect of transannular patching on outcome after repair of tetralogy of Fallot. Ann Thorac Surg 48: 783-791 4. Lurus AG, Cowen RL, Eckert JF (1969) Systemic-pulmonary arteriovenous fistula following closed-tube thoracostomy. Radiology 92: 1296- 1298 5. Rabinovitch M, Herrera-Deleon V, Castaneda AR, Reid L (1981) Growth and development of the pulmonary vascular bed in patient with Tetralogy of Fallot with or without pulmonary atresia. Circulation 64:1234-1249 6. Rajani M, Shrivastava S, Tandon R, Bhargava S (1980) Right ventricular cineangiography in tetralogy of Fallot. Cardiovasc Intervent Radio1 3:13-17
7. Ramsay JM, Macartney FJ, Haworth SG (1985) Tetralogy of Fallot with major collateral arteries. Br Heart J 53: 167-172 8. Sharma SN, Sharma S, Shrivastava S, Rajani M, Tandon R (1989) Pulmonary arterial anatomy in tetralogy of Fallot. Int J Cardiol 25:33-38
Jens Teglgaard Lund, MD Department of Thoracic Surgery RT 2102 Rigshospitalet Blegdamsvej 9 DK-2100 Copenhagen Denmark
