Current Surgical Technique to Repair Fallot’s Tetralogy With Absent Pulmonary Valve Syndrome E. Snir, MD, M. R. de Leval, MD, FRCS, M. J. Elliott, MD, FRCS, and J. Stark, MD, FRCS The Cardiothoracic Unit, The Hospital for Sick Children, London, England
The experience with surgical repair of Fallot’s tetralogy and absent pulmonary valve syndrome is reviewed. Twenty-two patients aged 1 day to 8 years were treated between 1982 and 1989 using one surgical technique. This consisted of resection of the main pulmonary artery and large parts of the anterior wall of the right and left pulmonary artery. The ventricular septal defect was closed with a patch, and an aortic or pulmonary homograft (size, 8 to 24 mm) was interposed between the
right ventricle and the pulmonary artery. Two of the 8 infants operated on died; there was one late death. All 14 older children survived the operation. All survivors are well up to 7 years follow-up (mean follow-up, 3.6 years). We recommend early treatment of infants; older children can be treated electively. The technique used in our series gives excellent results.
A
8 years). Two infants and 2 older children were included in our previous communication [16]. All patients in the infant group had severe respiratory symptoms and congestive heart failure. Several infants did not respond to medical therapy and were admitted on an emergency or semiemergency basis for urgent treatment. Older children had mild respiratory or cardiac symptoms or both. They were managed medically, and operation was performed electively at an older age. Clinical findings included a pansystolic murmur and low-pitched diastolic murmur at the left sternal border. Second pulmonary sound was absent. Chest roentgenography showed cardiomegaly and gross dilatation of the main, left, and right pulmonary arteries in all. Pulmonary vascularity was normal or slightly reduced. Four of 8 infants had severe air trapping on chest roentgenography (Table 1).Two of them had to be mechanically ventilated before operation; 1 underwent lobectomy in another hospital before admission to our department. All infants and children underwent two-dimensional echocardiography and Doppler assessment. In addition, cardiac catheterization and angiography was performed on all but 1 sick neonate. This, in retrospect, proved to be an error. This patient had grossly hypoplastic peripheral pulmonary arteries with branching anomalies and clearly inoperable disease. He died on the operating table. Abnormalities of his pulmonary vascular tree were elucidated by postmortem angiocardiography (Fig 1). Angiography confirmed the diagnosis by demonstrating the ventricular septal defect and gross dilatation of the pulmonary arteries. Peak systolic right ventricular to pulmonary arterial pressure gradient varied from 32 to 85 mm Hg (mean, 56 mm Hg) in the group of infants and 50 to 110 mm Hg (mean, 72 mm Hg)in the group of older children. Associated cardiac anomalies included absence
bsent pulmonary valve syndrome (APVS) is a rare variant of Fallot’s tetralogy. It is characterized by a ventricular septal defect that is usually located in the outlet septum; the pulmonary valve is either absent or rudimentary but there is usually a degree of obstruction at the pulmonary valve ring level or within the outflow tract of the right ventricle. Pulmonary arteries are grossly dilated with exaggerated pulsations; this often causes severe obstruction of one or both main bronchi. Patients are seen in two well-defined groups: infants with severe respiratory symptoms often are seen in the neonatal period. Morbidity and mortality in this group is high [ M I . The second group is composed of older children whose symptoms are less severe. Elective operations can usually be delayed. Medical treatment of sick infants is usually unsuccessful [7, 81. In the past various surgical techniques were suggested and tried. They included palliative [9-141 and corrective [2, 15-19] procedures. We have favored total repair of patients with APVS, irrespective of age and symptoms, and have published our results using several surgical techniques in 1986 (161. The purpose of this communication is to review our results since January 1982 using a standardized approach.
Material and Methods Patients Twenty-two patients with APVS were operated on between January 1982 and December 1989. Eight were infants younger than 1 year (aged 1 day to 11 months). Fourteen were children older than 1 year (age range, 2 to Accepted for publication Feb 14, 1991. Address reprint requests to Dr Stark, The Hospital for Sick Children, Great Ormond St, London WClN 3JH, England. 0 1991 by The Society of Thoracic Surgeons
(Ann Thoruc Surg 2991;52:979-82)
980
Ann Thorac Surg
SNIRETAL TETRALOGY AND ABSENT PULMONARY VALVE
1991;51:979-82
Table 1. Data on 8 lnfant Patients Patient
No.
Age
Symptoms
1
5 mo
Severe respiratory failure; recurrent chest infection
Hyperinflated right middle lobe
Large MPA
2
3 mo
3
27 d
Severe respiratory failure; cyanosis Ventilated since birth
Hyperinflated right lower lobe Hyperinflated right upper and middle lobe
Aneurysmal dilated PAS Large right PA with abnormal branching
4
9 mo
11 mo
Enlarged heart, enlarged central PAS Enlarged central PAS
Large PAS
5 6
6 mo
7
I d
8
7 mo
Heart failure, respiratory failure Recurrent chest infection, cyanosis Severe respiratory failure Cyanosis, low cardiac output, acidosis; ventilated Severe respiratory, cyanosis; lobectomy elsewhere age 6 mo
PA
=
Chest Roentgenogram
Angiogram
Outcome
Extubated
Right middle lobectomy 4 days after operation; well Well
14 d
Right upper and middle lobectomy 7 days after operation; died at 3 mo Well
...
Grossly dilated PAS
Died at 14 d
...
Hyperinflation both lungs Enlarged heart, large central PAS
Huge main PA
Well
4d
Died on table
...
Large PA
Large PAS
Well
2d
...
3d
2d
pulmonary artery.
of left pulmonary artery in 1, major aortopulmonary collaterals in 1, and DiGeorge syndrome in 2 patients.
Results Infants
Surgical Technique
Two of 8 infants operated on died. One was a 1-day-old neonate who was operated on without cardiac catheterization and angiocardiography. Right and left pulmonary arteries were grossly dilated. Dilatation ended abruptly in the hilum, and intrapulmonary branches of both right and left pulmonary arteries were severely hypoplastic. The patient could not be weaned off cardiopulmonary bypass. Postmortem angiography showed severe hypoplasia of peripheral pulmonary arteries on both sides with gross abnormalitiesin pulmonary branching pattern (see Fig 1). There was no blood supply to the right lower lobe, and branching into the right upper lobe and the left lung was abnormal. The second death occurred in a 3-week-old infant who suffered renal failure after operation. He died 2 weeks later in multiorgan failure. In addition, there was one late death. A premature infant, aged 1 month and weighing 1.8 kg, was treated in another hospital abroad. After 1 month of intermittent positive-pressure ventilation he
The main pulmonary artery was resected and the anterior wall of the right and left pulmonary artery was resected to reduce their size. Resection was carried out to the hilum of both lungs, close to the origin of upper lobe arteries (Fig 2). Suturing the edges of the resected right and left pulmonary arteries considerably reduced their size. Plication of the posterior wall of the right and left pulmonary artery was not performed in any of our patients. Incision was then extended into the right ventricular outflow tract. Ventricular septa1 defect was closed with a polytetrafluoroethylene (Gore-Tex) patch and running 4.0 or 5.0 polypropylene suture. Aortic or pulmonary homograft was interposed between the right ventricle and the bifurcation of the pulmonary artery. Right ventricular outflow tract was reconstructed either with the anterior cusp of the mitral valve of the homograft or with a pericardial patch. The size of the homograft varied between 8 and 24 mm.
Ann Thorac Surg 1991:51:979-62
SNlRETAL
TETRALOGY AND ABSENT PULMONARY VALVE
981
gradually improved and he was weaned off the ventilator to continuous positive airway pressure. He was then transferred back to the refemng hospital. Unfortunately, extubation was not possible. The child gradually required increased ventilation, and 2 months after operation the child was restudied. Aneurysm of the homograft was found with severe reduction of the peripheral pulmonary artery sizes. The patient underwent reoperation, and the homograft was exchanged for a new one (12 mm). Unfortunately, the suprasystemic pressure in the right ventricle was reduced to the systemic level and the patient died 2 days later, presumably because of gross abnormalities in the size and branching pattern of intraparenchymal pulmonary arteries.
Children All 14 children survived the operation. Their postoperative course was generally uneventful, and they were extubated after 1 to 3 days (mean, 1.2 days).
Late Results Fig I . Postmortem pulmona ry angiogram shows grossly dilated main, right, and left pulmonary arteries. There is no blood suwly to the right lower lobe, and intraparenchymal branches to the rest of the lung are abnormal, narrow, and “spidery.”
was transferred to us for surgical treatment. On admission, there was severe air trapping in the right upper and middle lobes. The patient underwent uneventful repair: the ventricular septa1 defect was closed with a Gore-Tex patch, and an 8-mm pulmonary homograft was interposed between the right ventricle and the pulmonary artery after reduction of the size of both right and left pulmonary arteries. Seven days after the operation, the air trapping caused severe respiratory embarrassment and emergency right upper and middle lobectomy had to be performed. After this operation the child’s condition
Fig 2 . Important features of surgical technique, namely, the resection of the main pulmonary artery and the anterior wall of the right and left pulmonary artery (indicated by dotted line), are illustrated. The ascending aorta is shown as transected; this is for the purpose of illustration only-to show the full extent of the pulmonary artery excision.
Follow-up up to 7 years (mean, 3.6 years) was available in all survivors. All patients operated on after the age of 1 year were symptom-free. No reoperations have been required so far. One of the 5 surviving infants has severe developmental retardation. This patient was recatheterized in December 1988. The conduit was unobstructed, and the right ventricular to left ventricular ratio was 0.5. His symptoms were obviously related to the developmental retardation rather than to any residual or recurrent hemodynamic lesion.
Comment Absent pulmonary valve syndrome can be diagnosed accurately with two-dimensional echocardiography. We use echocardiography without additional invasive investigation for an increasing number of children with various congenital heart defects (201. There are, however, certain limitations to this technique, and evaluation of peripheral and intraparenchymal pulmonary arteries is one of them. The 1 infant in our series who did not undergo preoperative angiography (Fig 1) is a good example. This confirms our belief that angiography is a useful, and in some cases essential, investigation of infants with APVS. Abnormalities of hilar branches of the pulmonary artery in APVS were demonstrated on postmortem pulmonary angiography by Rabinovitch and associates (211. They found that instead of single segmental arteries there were tufts of arteries that entwined and compressed interpulmonary bronchi. Rabinovitch and associates also found impaired alveolar multiplication. On the basis of their data they [21] suggest that these abnormalities of interparenchymal pulmonary arteries may be responsible for continuing symptoms in some infants even after relief of compression of the main bronchi. The difference in the severity of symptoms between infants and older children with APVS was observed by several authors [2,4,6,22]. High mortality rates in infants with APVS led several surgeons to try various palliative or
982
SNIRETAL TETRALOGY AND ABSENT PULMONARY VALVE
two-stage procedures [l,8,9,13]. Byrne and co-workers [lo] successfully treated a 6-week-old infant by ligation of the pulmonary artery and creation of a central shunt. The same basic approach using a modified Blalock-Taussig shunt was used by Ilbawi and associates [ll]in 4 infants; 2 survived. Opie and colleagues [141 and Park and Trinkle [121 banded the pulmonary artery successfully in 3 infants with APVS. The authors recommending total repair of this anomaly agree that the ventricular septal defect should be closed and right ventricular outflow tract obstruction relieved. There is, however, no general consensus whether a pulmonary valve should be inserted and the size of the pulmonary arteries should be reduced. McCaughan and co-workers [2] recommended insertion of a pulmonary valve and plication of aneurysmal pulmonary arteries in infants with APVS. In older children they believe both these steps are not necessary. Ilbawi and associates [4, 111 recommended pulmonary valve insertion in the older group. For infants they prefer palliation first. Insertion of a pulmonary valve was also recommended by Layton and co-workers [ 151 and Mavroudis and colleagues [ 181, whereas Stellin and associates [17] place more emphasis on reduction of pulmonary artery size by anterior resection and posterior plication. In our previous experience we have treated patients with APVS by closing the ventricular septal defect, but the approach to the pulmonary valve varied. We have placed some outflow tract patches, heterograft valves, and aortic or pulmonary homografts. In general, pulmonary arterial size was not reduced. Since 1982 we have used a fairly uniform technique consisting of ventricular septal defect closure, placement of a homograft, and reduction of the size of the pulmonary arteries, although we have not used posterior wall plication as suggested by Stellin and associates [lq. We continue using this technique. The problems are encountered in the group of youngest infants who may have gross anatomical anomalies in the size and distribution of the pulmonary arteries. In some of them early bronchomalacia develops, and this may necessitate lobectomy in addition to the intracardiac repairs. Although we are pleased with the overall results (19 long-term survivors among 22 patients operated on), we cannot draw definite conclusions about the group of young infants as the numbers are too small.
References 1. Waldhausen JA, Friedman S, Nicodemus H, Miller WW, Rashkind W, Johnson J. Absence of the pulmonary valve in patients with tetralogy of Fallot. Surgical management. J Thorac Cardiovasc Surg 1%9;5766%74. 2. McCaughan BC, Danielson GK, Driscoll DJ, McGoon DC. Tetralogy of Fallot with absent pulmonary valve. Early and late results of surgical treatment. J Thorac Cardiovasc Surg 1985;89:280-7. 3. Fixher DR, Neches WH, Beerman LB, et al. Tetralogy of F d o t with absent pulmonic valve. Analysis of 17 patients. Ann J Cardiol 1984;531433-7. 4. Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, &Leon SY. Tetralogy of Fallot with absent pulmonary valve. J Thorac Cardiovasc Surg 1981;81:906-15.
Ann Thorac Surg 1991;51:979-82
5. Emmanoulides GC, Thanopoulos B, Siassi 8, Fishbeii M. "Agenesis" of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol 1976;3740>9. 6. Pinsky WW, Nihill MR, Mullins CE, Harrison G, McNamara DG. The absent pulmonary valve syndrome. Circulation 1978;57159-62. 7. DCruz IA, Arcilla RA, Agustsson MH. Dilatation of the pulmonary trunk in stenosis of the pulmonary valve and of the pulmonary arteries in children. Am Heart J 1964;68:612. 8. Osman MZ, Merg CCL, Girdany BR. Congenital absence of the pulmonary valve. Report of eight cases with review of the literature. AJR 1%9;106:5fM9. 9. Litwin SB, Rosenthal A, Fellows K. Surgical management of young infants with tetralogy of Fallot, absence of the pulmonary valve and respiratory distress. J Thorac Cardiovasc Surg 1973;65:5524. 10. Byme JP, Hawkins JA, Battiste CE, Khoury GH. Palliative procedures in tetralogy of Fallot with absent pulmonary valve. A new approach. Ann Thorac Surg 1982;33:49A502. 11. Ilbawi MN, Fedorchik J, Muster AJ, et al. Surgical approach to severely symptomatic newborn infants with tetralogy of Fallot and absent pulmonary valve. J Thorac Cardiovasc Surg 1986;91:58&9. 12. Park MK, Trinkle JK. Absent pulmonary valve syndrome: a two stage operation. Ann Thorac Surg 1986;41:669-71. 13. Bove EL, Shaher RM, Alley R, McKneally M. Tetralogy of Fallot with absent pulmonary valve and aneurysm of the pulmonary artery: report of two cases presenting as obstructive lung disease. J Pediatr 1972;81:33943. 14. Opie JC, Sandor GG, Ashmore PG, Patterson MW. Successful palliation by pulmonary artery banding in absent pulmonary valve syndrome with aneurysmal pulmonary arteries. J Thorac Cardiovasc Surg 1983;8512%3. 15. Layton A, McDonald A, McDonald L, Towers M, Weaver D, Yacoub M. The syndrome of absent pulmonary valve. Total correction with aortic vascular homografts. J Thorac Cardiovasc Surg 1972;63800-8. 16. Karl TR, Musumeci F, de Leval M, Pincott JR, Taylor JFN, Stark J. Surgical treatment of absent pulmonary valve syndrome. J Thorac Cardiovasc Surg 1986;91:590-7. 17. Stellin G, Jonas RA, Goh TH, Brawn WJ, Venables AW, Mee RB. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg 1983;36:468-75. 18. Mavroudis C, Turley K, Stanger P, Ebert PA. Surgical management of tetralogy of Fallot with absent pulmonary valve. J Cardiovasc Surg 1983;24:603-9. 19. Arensman FW, Francis PD, Helmsworth JA, Benzing G. Early medical and surgical intervention for tetralogy of Fallot with absence of pulmonic valve. J Thorac Cardiovasc Surg 1982;&4:430-6. 20. Stark J, Smallhom J, Huhta J, de Leval M. Surgery for congenital heart defects diagnosed with cross-sectional echocardiography. Circulation 1983;68(Suppl2):129-38. 21. Rabinovitch M, Grady S, David I, et al. Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves. Am J Cardiol 1982;50:8op13. 22. Lakier JB, Stranter P, Heymann MA, Hoffman JI, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve. Natural history and haemodynamic considerations. Circulation 1974;50:167-75.
The experience with surgical repair of Fallot’s tetralogy and absent pulmonary valve syndrome is reviewed. Twenty-two patients aged 1 day to 8 years were treated between 1982 and 1989 using one surgical technique. This consisted of resection of the main pulmonary artery and large parts of the anterior wall of the right and left pulmonary artery. The ventricular septal defect was closed with a patch, and an aortic or pulmonary homograft (size, 8 to 24 mm) was interposed between the
right ventricle and the pulmonary artery. Two of the 8 infants operated on died; there was one late death. All 14 older children survived the operation. All survivors are well up to 7 years follow-up (mean follow-up, 3.6 years). We recommend early treatment of infants; older children can be treated electively. The technique used in our series gives excellent results.
A
8 years). Two infants and 2 older children were included in our previous communication [16]. All patients in the infant group had severe respiratory symptoms and congestive heart failure. Several infants did not respond to medical therapy and were admitted on an emergency or semiemergency basis for urgent treatment. Older children had mild respiratory or cardiac symptoms or both. They were managed medically, and operation was performed electively at an older age. Clinical findings included a pansystolic murmur and low-pitched diastolic murmur at the left sternal border. Second pulmonary sound was absent. Chest roentgenography showed cardiomegaly and gross dilatation of the main, left, and right pulmonary arteries in all. Pulmonary vascularity was normal or slightly reduced. Four of 8 infants had severe air trapping on chest roentgenography (Table 1).Two of them had to be mechanically ventilated before operation; 1 underwent lobectomy in another hospital before admission to our department. All infants and children underwent two-dimensional echocardiography and Doppler assessment. In addition, cardiac catheterization and angiography was performed on all but 1 sick neonate. This, in retrospect, proved to be an error. This patient had grossly hypoplastic peripheral pulmonary arteries with branching anomalies and clearly inoperable disease. He died on the operating table. Abnormalities of his pulmonary vascular tree were elucidated by postmortem angiocardiography (Fig 1). Angiography confirmed the diagnosis by demonstrating the ventricular septal defect and gross dilatation of the pulmonary arteries. Peak systolic right ventricular to pulmonary arterial pressure gradient varied from 32 to 85 mm Hg (mean, 56 mm Hg) in the group of infants and 50 to 110 mm Hg (mean, 72 mm Hg)in the group of older children. Associated cardiac anomalies included absence
bsent pulmonary valve syndrome (APVS) is a rare variant of Fallot’s tetralogy. It is characterized by a ventricular septal defect that is usually located in the outlet septum; the pulmonary valve is either absent or rudimentary but there is usually a degree of obstruction at the pulmonary valve ring level or within the outflow tract of the right ventricle. Pulmonary arteries are grossly dilated with exaggerated pulsations; this often causes severe obstruction of one or both main bronchi. Patients are seen in two well-defined groups: infants with severe respiratory symptoms often are seen in the neonatal period. Morbidity and mortality in this group is high [ M I . The second group is composed of older children whose symptoms are less severe. Elective operations can usually be delayed. Medical treatment of sick infants is usually unsuccessful [7, 81. In the past various surgical techniques were suggested and tried. They included palliative [9-141 and corrective [2, 15-19] procedures. We have favored total repair of patients with APVS, irrespective of age and symptoms, and have published our results using several surgical techniques in 1986 (161. The purpose of this communication is to review our results since January 1982 using a standardized approach.
Material and Methods Patients Twenty-two patients with APVS were operated on between January 1982 and December 1989. Eight were infants younger than 1 year (aged 1 day to 11 months). Fourteen were children older than 1 year (age range, 2 to Accepted for publication Feb 14, 1991. Address reprint requests to Dr Stark, The Hospital for Sick Children, Great Ormond St, London WClN 3JH, England. 0 1991 by The Society of Thoracic Surgeons
(Ann Thoruc Surg 2991;52:979-82)
980
Ann Thorac Surg
SNIRETAL TETRALOGY AND ABSENT PULMONARY VALVE
1991;51:979-82
Table 1. Data on 8 lnfant Patients Patient
No.
Age
Symptoms
1
5 mo
Severe respiratory failure; recurrent chest infection
Hyperinflated right middle lobe
Large MPA
2
3 mo
3
27 d
Severe respiratory failure; cyanosis Ventilated since birth
Hyperinflated right lower lobe Hyperinflated right upper and middle lobe
Aneurysmal dilated PAS Large right PA with abnormal branching
4
9 mo
11 mo
Enlarged heart, enlarged central PAS Enlarged central PAS
Large PAS
5 6
6 mo
7
I d
8
7 mo
Heart failure, respiratory failure Recurrent chest infection, cyanosis Severe respiratory failure Cyanosis, low cardiac output, acidosis; ventilated Severe respiratory, cyanosis; lobectomy elsewhere age 6 mo
PA
=
Chest Roentgenogram
Angiogram
Outcome
Extubated
Right middle lobectomy 4 days after operation; well Well
14 d
Right upper and middle lobectomy 7 days after operation; died at 3 mo Well
...
Grossly dilated PAS
Died at 14 d
...
Hyperinflation both lungs Enlarged heart, large central PAS
Huge main PA
Well
4d
Died on table
...
Large PA
Large PAS
Well
2d
...
3d
2d
pulmonary artery.
of left pulmonary artery in 1, major aortopulmonary collaterals in 1, and DiGeorge syndrome in 2 patients.
Results Infants
Surgical Technique
Two of 8 infants operated on died. One was a 1-day-old neonate who was operated on without cardiac catheterization and angiocardiography. Right and left pulmonary arteries were grossly dilated. Dilatation ended abruptly in the hilum, and intrapulmonary branches of both right and left pulmonary arteries were severely hypoplastic. The patient could not be weaned off cardiopulmonary bypass. Postmortem angiography showed severe hypoplasia of peripheral pulmonary arteries on both sides with gross abnormalitiesin pulmonary branching pattern (see Fig 1). There was no blood supply to the right lower lobe, and branching into the right upper lobe and the left lung was abnormal. The second death occurred in a 3-week-old infant who suffered renal failure after operation. He died 2 weeks later in multiorgan failure. In addition, there was one late death. A premature infant, aged 1 month and weighing 1.8 kg, was treated in another hospital abroad. After 1 month of intermittent positive-pressure ventilation he
The main pulmonary artery was resected and the anterior wall of the right and left pulmonary artery was resected to reduce their size. Resection was carried out to the hilum of both lungs, close to the origin of upper lobe arteries (Fig 2). Suturing the edges of the resected right and left pulmonary arteries considerably reduced their size. Plication of the posterior wall of the right and left pulmonary artery was not performed in any of our patients. Incision was then extended into the right ventricular outflow tract. Ventricular septa1 defect was closed with a polytetrafluoroethylene (Gore-Tex) patch and running 4.0 or 5.0 polypropylene suture. Aortic or pulmonary homograft was interposed between the right ventricle and the bifurcation of the pulmonary artery. Right ventricular outflow tract was reconstructed either with the anterior cusp of the mitral valve of the homograft or with a pericardial patch. The size of the homograft varied between 8 and 24 mm.
Ann Thorac Surg 1991:51:979-62
SNlRETAL
TETRALOGY AND ABSENT PULMONARY VALVE
981
gradually improved and he was weaned off the ventilator to continuous positive airway pressure. He was then transferred back to the refemng hospital. Unfortunately, extubation was not possible. The child gradually required increased ventilation, and 2 months after operation the child was restudied. Aneurysm of the homograft was found with severe reduction of the peripheral pulmonary artery sizes. The patient underwent reoperation, and the homograft was exchanged for a new one (12 mm). Unfortunately, the suprasystemic pressure in the right ventricle was reduced to the systemic level and the patient died 2 days later, presumably because of gross abnormalities in the size and branching pattern of intraparenchymal pulmonary arteries.
Children All 14 children survived the operation. Their postoperative course was generally uneventful, and they were extubated after 1 to 3 days (mean, 1.2 days).
Late Results Fig I . Postmortem pulmona ry angiogram shows grossly dilated main, right, and left pulmonary arteries. There is no blood suwly to the right lower lobe, and intraparenchymal branches to the rest of the lung are abnormal, narrow, and “spidery.”
was transferred to us for surgical treatment. On admission, there was severe air trapping in the right upper and middle lobes. The patient underwent uneventful repair: the ventricular septa1 defect was closed with a Gore-Tex patch, and an 8-mm pulmonary homograft was interposed between the right ventricle and the pulmonary artery after reduction of the size of both right and left pulmonary arteries. Seven days after the operation, the air trapping caused severe respiratory embarrassment and emergency right upper and middle lobectomy had to be performed. After this operation the child’s condition
Fig 2 . Important features of surgical technique, namely, the resection of the main pulmonary artery and the anterior wall of the right and left pulmonary artery (indicated by dotted line), are illustrated. The ascending aorta is shown as transected; this is for the purpose of illustration only-to show the full extent of the pulmonary artery excision.
Follow-up up to 7 years (mean, 3.6 years) was available in all survivors. All patients operated on after the age of 1 year were symptom-free. No reoperations have been required so far. One of the 5 surviving infants has severe developmental retardation. This patient was recatheterized in December 1988. The conduit was unobstructed, and the right ventricular to left ventricular ratio was 0.5. His symptoms were obviously related to the developmental retardation rather than to any residual or recurrent hemodynamic lesion.
Comment Absent pulmonary valve syndrome can be diagnosed accurately with two-dimensional echocardiography. We use echocardiography without additional invasive investigation for an increasing number of children with various congenital heart defects (201. There are, however, certain limitations to this technique, and evaluation of peripheral and intraparenchymal pulmonary arteries is one of them. The 1 infant in our series who did not undergo preoperative angiography (Fig 1) is a good example. This confirms our belief that angiography is a useful, and in some cases essential, investigation of infants with APVS. Abnormalities of hilar branches of the pulmonary artery in APVS were demonstrated on postmortem pulmonary angiography by Rabinovitch and associates (211. They found that instead of single segmental arteries there were tufts of arteries that entwined and compressed interpulmonary bronchi. Rabinovitch and associates also found impaired alveolar multiplication. On the basis of their data they [21] suggest that these abnormalities of interparenchymal pulmonary arteries may be responsible for continuing symptoms in some infants even after relief of compression of the main bronchi. The difference in the severity of symptoms between infants and older children with APVS was observed by several authors [2,4,6,22]. High mortality rates in infants with APVS led several surgeons to try various palliative or
982
SNIRETAL TETRALOGY AND ABSENT PULMONARY VALVE
two-stage procedures [l,8,9,13]. Byrne and co-workers [lo] successfully treated a 6-week-old infant by ligation of the pulmonary artery and creation of a central shunt. The same basic approach using a modified Blalock-Taussig shunt was used by Ilbawi and associates [ll]in 4 infants; 2 survived. Opie and colleagues [141 and Park and Trinkle [121 banded the pulmonary artery successfully in 3 infants with APVS. The authors recommending total repair of this anomaly agree that the ventricular septal defect should be closed and right ventricular outflow tract obstruction relieved. There is, however, no general consensus whether a pulmonary valve should be inserted and the size of the pulmonary arteries should be reduced. McCaughan and co-workers [2] recommended insertion of a pulmonary valve and plication of aneurysmal pulmonary arteries in infants with APVS. In older children they believe both these steps are not necessary. Ilbawi and associates [4, 111 recommended pulmonary valve insertion in the older group. For infants they prefer palliation first. Insertion of a pulmonary valve was also recommended by Layton and co-workers [ 151 and Mavroudis and colleagues [ 181, whereas Stellin and associates [17] place more emphasis on reduction of pulmonary artery size by anterior resection and posterior plication. In our previous experience we have treated patients with APVS by closing the ventricular septal defect, but the approach to the pulmonary valve varied. We have placed some outflow tract patches, heterograft valves, and aortic or pulmonary homografts. In general, pulmonary arterial size was not reduced. Since 1982 we have used a fairly uniform technique consisting of ventricular septal defect closure, placement of a homograft, and reduction of the size of the pulmonary arteries, although we have not used posterior wall plication as suggested by Stellin and associates [lq. We continue using this technique. The problems are encountered in the group of youngest infants who may have gross anatomical anomalies in the size and distribution of the pulmonary arteries. In some of them early bronchomalacia develops, and this may necessitate lobectomy in addition to the intracardiac repairs. Although we are pleased with the overall results (19 long-term survivors among 22 patients operated on), we cannot draw definite conclusions about the group of young infants as the numbers are too small.
References 1. Waldhausen JA, Friedman S, Nicodemus H, Miller WW, Rashkind W, Johnson J. Absence of the pulmonary valve in patients with tetralogy of Fallot. Surgical management. J Thorac Cardiovasc Surg 1%9;5766%74. 2. McCaughan BC, Danielson GK, Driscoll DJ, McGoon DC. Tetralogy of Fallot with absent pulmonary valve. Early and late results of surgical treatment. J Thorac Cardiovasc Surg 1985;89:280-7. 3. Fixher DR, Neches WH, Beerman LB, et al. Tetralogy of F d o t with absent pulmonic valve. Analysis of 17 patients. Ann J Cardiol 1984;531433-7. 4. Ilbawi MN, Idriss FS, Muster AJ, Wessel HU, Paul MH, &Leon SY. Tetralogy of Fallot with absent pulmonary valve. J Thorac Cardiovasc Surg 1981;81:906-15.
Ann Thorac Surg 1991;51:979-82
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