- Email: [email protected]
Hypoplastic left heart syndrome: Palliation without cardiopulmonary bypass
J
THORAC CARDIOVASC SURG
1990;99:885-8
Hypoplastic left heart syndrome: Palliation without cardiopulmonary bypass Our 100% mortality rate with first-stage palliation of hypoplastic left heart syndrome performed with cardiopulmonary bypass led us to a procedure not necessitating bypass. In nine neonates with this congenital heart defect, a woven Dacron graft was placed from the main pulmonary artery to the descending thoracic aorta. The patent ductus arteriosus was ligated and the main pulmonary artery banded distal to the graft and proximal to the bifurcation. Five patients were extubated within 4 days. Only low-dose inotropic support was required in eight of the nine. There were no bleeding problems. Four patients died in the hospital: one of Candida sepsis at 81 days, one of low cardiac output at 2 days, and two of restrictive atrial septal defect at 3 and 5 days. The five living patients were discharged 11 to 80 days postoperatively (mean 38 days). We now perform balloon septostomies preoperatively in all patients and believe that this will improve the survival rate. We believe this simpler approach to the treatment of hypoplastic left heart syndrome may allow survival for a cardiac transplant or a staged Fontan procedure at a later date for more definitive treatment.
William Y. Tucker, MDa (by invitation), Robert C. McKone, MD b (by invitation), Kenneth M. Weesner, MDb (by invitation), and Neal D. Kon, MDa (by invitation), Winston-Salem, NiC.
Sponsored by" A. Robert Cordell, MD, Winston-Salem, NiC.
HypoPlastic left heart syndromecontinuesto be a difficult surgical challenge. Norwood's first-stage palliative procedure in whichcardiopulmonarybypassis used with deep hypothermia and circulatory arrest is the only approach that has allowed a significant number of children to survive beyond the neonatal period.1, 2 However, the mortality of this technique remains high, the postoperative problems are difficult to manage, and the success of a later, more functional, definitive second-stage correction remains in question.l-" Cardiac transplantation for hypoplastic left heart syndrome probably offers the patient the best opportunity for normal cardiac function.v" However, transplantation in neonateshas severe limitsof time, donor availability, and patient management. Our 100% mortality rate with the first-stage From the Departments of Cardiothoracic Surgery' and Pediatrics.? Bowman Gray School of Medicine, Wake Forest University Medical Center, Winston-Salem, N.C. Alternate paper for the Sixty-ninth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass., May 8-10, 1989. Address for reprints: William Y. Tucker, MD, Department of Cardiothoracic Surgery, Bowman Gray School of Medicine, 300 South Hawthorne Rd., Winston-Salem, NC 27103.
12/6/18961
repair performed with cardiopulmonary bypass led us to attempt a procedure not necessitating bypass. Methods Patients. We have performed a first-stage palliative procedure without the use of cardiopulmonary bypass in nine neonates with hypoplastic left heart syndrome. Their ages at the time of operation ranged from 2 to 6 days and their weights from 2.3 to 3.7 kg. Eight were clinically normal neonates except for the congenital heart defect. The ninth had ureteral obstruction, which was corrected subsequently. All were admitted in varying states of low cardiac output, with at least mild metabolic acidosis (pH range 6.80 to 7.33). All nine were given prostaglandins and dopamine, with resulting normalization of acid-base status, clinical improvement of cardiac output, and development of adequate urine output. In four patients the diagnosis of hypoplastic left heart syndrome was made by echocardiography, and these were referred for operation without cardiac catheterization or balloon atrial septostomy; two subsequently underwent both procedures. The remaining five patients underwent preoperative cardiac catheterization, three with balloon atrial septostomy as well. At the time of catheterization, two were believed to have a large atrial septal defect and septostomy was not done. Operative procedure. The operative procedure was performed through a left thoracotomy in the fourth intercostal space. The pericardium was opened anterior to the phrenic nerve and the large pulmonary artery was encircled. The pulmonary artery was separated from the small ascending aorta as far
885
The Journal of
8 8 6 Tucker et al.
Thoracic and Cardiovascular Surgery
Vv
Table I. Data on the five surviving patients Patient No.
1 2 3 4 5
Fig. 1. Appearance at end of surgical procedure.
proximally as possible without damaging the left main coronary artery. With the proximal portion of the main pulmonary artery controlled by a partial occlusion clamp, a slightly beveled prosthetic graft was anastomosed to the side of the main pulmonary artery. The grafts used were woven Dacron fabric 10 mm in diameter (n = 1), woven Dacron fabric 8 mm in diameter (n = 7), and polytetrafluoroethylene 8 mm in diameter (n = I). The graft was then looped over the hilum of the left lung and anastomosed to the side of the descending thoracic aorta distal to the patent ductus arteriosus. With the graft open and flowing, the patent ductus arteriosus was ligated. The main pulmonary artery was then banded distal to the origin of the graft and proximal to the branch pulmonary arteries. In each patient we attempted to tighten the band until the pressure distal to it was approximately one half the systemic pressure. After the proper band size was obtained, the band was sutured to the graft at the anastomosis on the pulmonary artery to prevent its distal migration. The final anatomic arrangement is illustrated in Fig. I. Full informed consent was obtained from the parents of each infant before the operative treatment was begun.
Results Operative time was less than 2 hours 30 minutes in every case. All nine patients were stable throughout the procedure and only two required more than low-dose inotropic support at the end of the operation. None of the patients had bleeding problems intraoperatively or postoperatively. Clinical assessment of postoperativecardiac output revealedperfusionto be good in eight patients, and
Preop. diagnostic study
Echocardiography Catheteriza tion Echocardiography Catheterization Catheterization
Extubated Discharged (postop. (postop. day) Septostomy day)
Postop. None Postop. Preop. None
3 3 1 4 59
48 II
24 28 80
it remained good throughout their course. Arterial blood gas pH ranged from 7.33 to 7.67 without the need for sodium bicarbonate. Arterial oxygen tension with the patient breathing 100%inspired oxygen decreased in all patients because of restricted pulmonary bloodflow. Oxygen tension ranged preoperatively from 61 to 154 mm Hg (mean 81 mm Hg) and postoperatively from 40 to 82 mm Hg (mean 57 mm Hg). Urine output was adequate in the eight patients who had clinicallygood cardiac output. Five patients (56%) survived (Table I) and were discharged from the hospital. In two of these the preoperative diagnosis had been made by echocardiography and the operation had been performed without further studies. They subsequently underwent cardiac catheterization and balloon atrial septostomy before discharge. The other three survivors had had preoperativecatheterization, but only one had needed a preoperativeseptostomy; the other two had large left atrial outlets without obstruction. The one survivor not extubated within 4 days had paralysis of the left hemidiaphragm and required ventilatory support for 59 days. Early growth and development have been within the normal range in all five. Patient 1 underwent the operation on July 27,1985, and is alive and well 4V2 years later. Data on the four neonates whodied postoperatively are summarized in Table II. Hemodynamics weregoodpostoperativelyin patient 6, but he could not be weaned from ventilatory support. Cardiac catheterization showed the band to have occluded the right pulmonary artery; an attempt was made to correct this problem, but was unsuccessful. This patient was the second in the series to be operated on. In patients 7 and 8, preoperative echocardiograms had indicated a relatively large patent foramen ovale. In both, we planned to perform cardiac catheterization and balloon atrial septostomy before their discharge. Bothdid extremelywellintraoperativelyand were in hemodynamicallystable condition after the operation. Their conditionsdeteriorated thereafter, and postmortem examinationsshowed both to have small atrial communications. The graft and pulmonary artery band in both patients were free ofproblems.In patient 9,the ST segments
Volume 99 Number 5
Hypoplastic left heart syndrome
May 1990
887
Table II. Data on the four patients who died postoperatively Patient No.
Preop. diagnostic study
Septostomy
Extubated (postop. day)
Died (postop. day)
6 7 8 9
Catheterization Echocardiography Echocardiography Catheterization
Preop. None None Preop.
No No 1 No
81 3
5 2
Cause of death RPA occluded; Candida sepsis Restrictive ASD Restrictive ASD Myocardial ischemia
RPA. Right pulmonary artery; ASD. atrial septal defect.
on the monitor electrocardiogram became elevated at the time of initial band placement. Release of the band resulted in return of the ST segments to baseline. The band was repositioned and constricted a second time without electrocardiographic changes. Postoperatively the patient had low cardiac output, required high-dose inotropic support, and died of myocardial ischemia.
Discussion The requirements of an operation for the successful palliation of hypoplastic left heart syndrome were understood and set forth about 20 years ago,": 8 They include the establishment of a connection between the main pulmonary artery and the aorta, the restriction of pulmonary blood flow,and the creation of a large atrial communication. The procedure reported here is the same in principle as that used with some success in the treatment of neonates with proximal aortic arch interruption." 10 In a report on the early successes of the operation for interrupted arch, Van Praagh and his associates" suggested that the addition of an atrial septal defect to the procedure would make it a palliative, and potentially lifesaving, operation for hypoplastic left heart syndrome. In 1972, discussing their experience with cardiovascular surgery in infants, Bernhard and co-workers 11 mentioned using the interrupted arch procedure in seven patients with aortic atresia, but they concluded, when none of their patients survived, that the procedure was not applicable for treatment of this form of hypoplastic left heart syndrome. In 1980 Levitsky and colleagues12 also found the procedure unsuccessful in the one infant in whom it was used. Doty and Knott 13 described a different approach to the problem of hypoplastic left heart syndrome in 1977-a modified Fontan procedure with cardiopulmonary bypass. All six of their patients died as a result of the high postoperative pulmonary vascular resistance found in these neonates. By 1981 Norwood and associates I began to have success with palliation of hypoplastic left heart syndrome. They have continued to modify and refine their technique and report survival in approximately 60% of a relatively large series of patients.' Their operation alters the anat-
omy to meet all three requirements for palliation of the heart defect but necessitates the use of cardiopulmonary bypass with deep hypothermia and circulatory arrest. Our attempts at palliation of hypoplastic left heart syndrome by means of the procedure of Norwood and colleagues all have been unsuccessful. Sade, Crawford, and Fyfe 3 also have had disappointing results. Bleeding has been frequent and significant; postoperative hemodynamic stability has been difficult to maintain. Cardiac transplantation provides the only opportunity for the patient with hypoplastic left heart syndrome to have normal cardiovascular function. Bailey> Mavroudis," and their associates have had success with this approach. However, the natural history of hypoplastic left heart syndrome leaves little time for a proper donor heart to be found; and infusion of prostaglandin E 1 does not extend survival time substantially. The operation we report here is relatively simple and does not necessitate cardiopulmonary bypass and deep hypothermia, but it does necessitate precise technique. Space is limited in the neonatal thorax, and care must be taken to place the graft and band properly. There is little space in which to work on the main pulmonary artery without comprising the branch arteries. Because of the band constriction seen in patient 6, we now secure the band with sutures to the graft at the anastomosis to prevent its migration. This patient was also the only one having a 10 mm graft inserted. The extra 2 mm of graft diameter occupied much of the available length of the main pulmonary artery, so that it was even more difficult to place the band. We have found the 8 mm graft to be a better choice and no longer use the 10 mm graft. We also try to place the pulmonary artery anastomosis as far proximal as possible to provide space for the band. With the incorporation of these technical changes, none of the last six patients treated has had band obstruction of the right pulmonary artery. The deaths of two patients from lack of an adequate left atrial outlet indicates the importance of a large atrial septal defect being present postoperatively in these patients. Echocardiography has proved to be unreliable in assessing the size of the atrial defect. We now routinely perform cardiac catheterization and balloon atrial sep-
The Journal of Thoracic and Cardiovascular
8 8 8 Tucker et al.
tostomy preoperatively with the hope that the too small atrial septal defect can be avoided and the survival rate improved. This approach to hypoplastic left heart syndrome has allowed the hospital discharge of five of nine infants. Postoperative hemodynamic, respiratory, and bleeding problems have been minimal. The future of these patients remains uncertain, but we believe this approach may allow survival until a proper donor heart can be located for cardiac transplantation. A second-stage Fontan procedure may provide more definitive treatment if pulmonary artery growth is good and pulmonary artery pressures remain normal. REFERENCES 1. Norwood WI, Lang P, Castaneda AR, Campbell ON. Ex-
perience with operations for hypoplastic left heart syndrome. J THORAC CARDIOVASC SURG 1981;82:511-9. 2. Pigott JD, Murphy JD, Barber G, Norwood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 1988;45: 122-8. 3. Sade RM, Crawford FAJr, Fyfe DA. Symposium on hypoplastic left heart syndrome [Letter]. J THORAC CARDIaVASCSURG 1986;91:937-9. 4. Sade RM, Fyfe 0, Alpert Cc. Hypoplastic left heart syndrome: a simplified palliative operation. Ann Thorac Surg 1987;43:309- I 2.
5. Bailey LL, Nehlsen-Cannarella SL, Doroshow RW, et al. Cardiac allotransplantation in newborns as therapy for hy-
Surgery
poplastic left heart syndrome. N Engl J Med 1986;315:94951.
6. Mavroudis C, Harrison H, Klein JB, et al. Infant orthotopic cardiac transplantation. J THORAC CARDIOVASC SURG 1988;96:912-24.
7. Sinha SN, Rusnak SL, Sommers HM, Cole RB, Muster AJ, Paul MH. Hypoplastic left ventricle syndrome: analysis of thirty autopsy cases in infants with surgical considerations. Am J Cardiol 1968;21: 166-73. 8. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, Fyler DC. Interrupted aortic arch: surgical trleatment. Am J Cardiol 1971;27:200-11. 9. Litwin SB, Van Praagh R, Bernhard WF. A palliative operation for certain infants with aortic arch interruption. Ann Thorac Surg 1972;14:369-75. 10. van der Horst R, Hastreiter AR, Levitsky S, Fisher EA, DuBrow IW, Weinberg M. Interrupted aortic arch operation in the first week oflife: hemodynamic and angiographic evaluation one year later. Ann Thorac Surg 1979;27: 11220. 11. Bernhard WF, Litwin SB, Williams WW, Jones JE, Gross
RE. Recent results of cardiovascular surgery in infants in the first year of life. Am J Surg 1972; 123:451-60. 12. Levitsky S, van der Horst RL, Hastreiter AR, Eckner FA, Bennett EJ. Surgical palliation in aortic atresia. J THORAC CARDIOVASC SURG 1980;79:456-61. 13. Doty DB, Knott HW. Hypoplastic left heart syndrome: experience with an operation to establish functionally normal circulation. J THORAC CARDIOVASC SURG 1977;74:624-30.
THORAC CARDIOVASC SURG
1990;99:885-8
Hypoplastic left heart syndrome: Palliation without cardiopulmonary bypass Our 100% mortality rate with first-stage palliation of hypoplastic left heart syndrome performed with cardiopulmonary bypass led us to a procedure not necessitating bypass. In nine neonates with this congenital heart defect, a woven Dacron graft was placed from the main pulmonary artery to the descending thoracic aorta. The patent ductus arteriosus was ligated and the main pulmonary artery banded distal to the graft and proximal to the bifurcation. Five patients were extubated within 4 days. Only low-dose inotropic support was required in eight of the nine. There were no bleeding problems. Four patients died in the hospital: one of Candida sepsis at 81 days, one of low cardiac output at 2 days, and two of restrictive atrial septal defect at 3 and 5 days. The five living patients were discharged 11 to 80 days postoperatively (mean 38 days). We now perform balloon septostomies preoperatively in all patients and believe that this will improve the survival rate. We believe this simpler approach to the treatment of hypoplastic left heart syndrome may allow survival for a cardiac transplant or a staged Fontan procedure at a later date for more definitive treatment.
William Y. Tucker, MDa (by invitation), Robert C. McKone, MD b (by invitation), Kenneth M. Weesner, MDb (by invitation), and Neal D. Kon, MDa (by invitation), Winston-Salem, NiC.
Sponsored by" A. Robert Cordell, MD, Winston-Salem, NiC.
HypoPlastic left heart syndromecontinuesto be a difficult surgical challenge. Norwood's first-stage palliative procedure in whichcardiopulmonarybypassis used with deep hypothermia and circulatory arrest is the only approach that has allowed a significant number of children to survive beyond the neonatal period.1, 2 However, the mortality of this technique remains high, the postoperative problems are difficult to manage, and the success of a later, more functional, definitive second-stage correction remains in question.l-" Cardiac transplantation for hypoplastic left heart syndrome probably offers the patient the best opportunity for normal cardiac function.v" However, transplantation in neonateshas severe limitsof time, donor availability, and patient management. Our 100% mortality rate with the first-stage From the Departments of Cardiothoracic Surgery' and Pediatrics.? Bowman Gray School of Medicine, Wake Forest University Medical Center, Winston-Salem, N.C. Alternate paper for the Sixty-ninth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass., May 8-10, 1989. Address for reprints: William Y. Tucker, MD, Department of Cardiothoracic Surgery, Bowman Gray School of Medicine, 300 South Hawthorne Rd., Winston-Salem, NC 27103.
12/6/18961
repair performed with cardiopulmonary bypass led us to attempt a procedure not necessitating bypass. Methods Patients. We have performed a first-stage palliative procedure without the use of cardiopulmonary bypass in nine neonates with hypoplastic left heart syndrome. Their ages at the time of operation ranged from 2 to 6 days and their weights from 2.3 to 3.7 kg. Eight were clinically normal neonates except for the congenital heart defect. The ninth had ureteral obstruction, which was corrected subsequently. All were admitted in varying states of low cardiac output, with at least mild metabolic acidosis (pH range 6.80 to 7.33). All nine were given prostaglandins and dopamine, with resulting normalization of acid-base status, clinical improvement of cardiac output, and development of adequate urine output. In four patients the diagnosis of hypoplastic left heart syndrome was made by echocardiography, and these were referred for operation without cardiac catheterization or balloon atrial septostomy; two subsequently underwent both procedures. The remaining five patients underwent preoperative cardiac catheterization, three with balloon atrial septostomy as well. At the time of catheterization, two were believed to have a large atrial septal defect and septostomy was not done. Operative procedure. The operative procedure was performed through a left thoracotomy in the fourth intercostal space. The pericardium was opened anterior to the phrenic nerve and the large pulmonary artery was encircled. The pulmonary artery was separated from the small ascending aorta as far
885
The Journal of
8 8 6 Tucker et al.
Thoracic and Cardiovascular Surgery
Vv
Table I. Data on the five surviving patients Patient No.
1 2 3 4 5
Fig. 1. Appearance at end of surgical procedure.
proximally as possible without damaging the left main coronary artery. With the proximal portion of the main pulmonary artery controlled by a partial occlusion clamp, a slightly beveled prosthetic graft was anastomosed to the side of the main pulmonary artery. The grafts used were woven Dacron fabric 10 mm in diameter (n = 1), woven Dacron fabric 8 mm in diameter (n = 7), and polytetrafluoroethylene 8 mm in diameter (n = I). The graft was then looped over the hilum of the left lung and anastomosed to the side of the descending thoracic aorta distal to the patent ductus arteriosus. With the graft open and flowing, the patent ductus arteriosus was ligated. The main pulmonary artery was then banded distal to the origin of the graft and proximal to the branch pulmonary arteries. In each patient we attempted to tighten the band until the pressure distal to it was approximately one half the systemic pressure. After the proper band size was obtained, the band was sutured to the graft at the anastomosis on the pulmonary artery to prevent its distal migration. The final anatomic arrangement is illustrated in Fig. I. Full informed consent was obtained from the parents of each infant before the operative treatment was begun.
Results Operative time was less than 2 hours 30 minutes in every case. All nine patients were stable throughout the procedure and only two required more than low-dose inotropic support at the end of the operation. None of the patients had bleeding problems intraoperatively or postoperatively. Clinical assessment of postoperativecardiac output revealedperfusionto be good in eight patients, and
Preop. diagnostic study
Echocardiography Catheteriza tion Echocardiography Catheterization Catheterization
Extubated Discharged (postop. (postop. day) Septostomy day)
Postop. None Postop. Preop. None
3 3 1 4 59
48 II
24 28 80
it remained good throughout their course. Arterial blood gas pH ranged from 7.33 to 7.67 without the need for sodium bicarbonate. Arterial oxygen tension with the patient breathing 100%inspired oxygen decreased in all patients because of restricted pulmonary bloodflow. Oxygen tension ranged preoperatively from 61 to 154 mm Hg (mean 81 mm Hg) and postoperatively from 40 to 82 mm Hg (mean 57 mm Hg). Urine output was adequate in the eight patients who had clinicallygood cardiac output. Five patients (56%) survived (Table I) and were discharged from the hospital. In two of these the preoperative diagnosis had been made by echocardiography and the operation had been performed without further studies. They subsequently underwent cardiac catheterization and balloon atrial septostomy before discharge. The other three survivors had had preoperativecatheterization, but only one had needed a preoperativeseptostomy; the other two had large left atrial outlets without obstruction. The one survivor not extubated within 4 days had paralysis of the left hemidiaphragm and required ventilatory support for 59 days. Early growth and development have been within the normal range in all five. Patient 1 underwent the operation on July 27,1985, and is alive and well 4V2 years later. Data on the four neonates whodied postoperatively are summarized in Table II. Hemodynamics weregoodpostoperativelyin patient 6, but he could not be weaned from ventilatory support. Cardiac catheterization showed the band to have occluded the right pulmonary artery; an attempt was made to correct this problem, but was unsuccessful. This patient was the second in the series to be operated on. In patients 7 and 8, preoperative echocardiograms had indicated a relatively large patent foramen ovale. In both, we planned to perform cardiac catheterization and balloon atrial septostomy before their discharge. Bothdid extremelywellintraoperativelyand were in hemodynamicallystable condition after the operation. Their conditionsdeteriorated thereafter, and postmortem examinationsshowed both to have small atrial communications. The graft and pulmonary artery band in both patients were free ofproblems.In patient 9,the ST segments
Volume 99 Number 5
Hypoplastic left heart syndrome
May 1990
887
Table II. Data on the four patients who died postoperatively Patient No.
Preop. diagnostic study
Septostomy
Extubated (postop. day)
Died (postop. day)
6 7 8 9
Catheterization Echocardiography Echocardiography Catheterization
Preop. None None Preop.
No No 1 No
81 3
5 2
Cause of death RPA occluded; Candida sepsis Restrictive ASD Restrictive ASD Myocardial ischemia
RPA. Right pulmonary artery; ASD. atrial septal defect.
on the monitor electrocardiogram became elevated at the time of initial band placement. Release of the band resulted in return of the ST segments to baseline. The band was repositioned and constricted a second time without electrocardiographic changes. Postoperatively the patient had low cardiac output, required high-dose inotropic support, and died of myocardial ischemia.
Discussion The requirements of an operation for the successful palliation of hypoplastic left heart syndrome were understood and set forth about 20 years ago,": 8 They include the establishment of a connection between the main pulmonary artery and the aorta, the restriction of pulmonary blood flow,and the creation of a large atrial communication. The procedure reported here is the same in principle as that used with some success in the treatment of neonates with proximal aortic arch interruption." 10 In a report on the early successes of the operation for interrupted arch, Van Praagh and his associates" suggested that the addition of an atrial septal defect to the procedure would make it a palliative, and potentially lifesaving, operation for hypoplastic left heart syndrome. In 1972, discussing their experience with cardiovascular surgery in infants, Bernhard and co-workers 11 mentioned using the interrupted arch procedure in seven patients with aortic atresia, but they concluded, when none of their patients survived, that the procedure was not applicable for treatment of this form of hypoplastic left heart syndrome. In 1980 Levitsky and colleagues12 also found the procedure unsuccessful in the one infant in whom it was used. Doty and Knott 13 described a different approach to the problem of hypoplastic left heart syndrome in 1977-a modified Fontan procedure with cardiopulmonary bypass. All six of their patients died as a result of the high postoperative pulmonary vascular resistance found in these neonates. By 1981 Norwood and associates I began to have success with palliation of hypoplastic left heart syndrome. They have continued to modify and refine their technique and report survival in approximately 60% of a relatively large series of patients.' Their operation alters the anat-
omy to meet all three requirements for palliation of the heart defect but necessitates the use of cardiopulmonary bypass with deep hypothermia and circulatory arrest. Our attempts at palliation of hypoplastic left heart syndrome by means of the procedure of Norwood and colleagues all have been unsuccessful. Sade, Crawford, and Fyfe 3 also have had disappointing results. Bleeding has been frequent and significant; postoperative hemodynamic stability has been difficult to maintain. Cardiac transplantation provides the only opportunity for the patient with hypoplastic left heart syndrome to have normal cardiovascular function. Bailey> Mavroudis," and their associates have had success with this approach. However, the natural history of hypoplastic left heart syndrome leaves little time for a proper donor heart to be found; and infusion of prostaglandin E 1 does not extend survival time substantially. The operation we report here is relatively simple and does not necessitate cardiopulmonary bypass and deep hypothermia, but it does necessitate precise technique. Space is limited in the neonatal thorax, and care must be taken to place the graft and band properly. There is little space in which to work on the main pulmonary artery without comprising the branch arteries. Because of the band constriction seen in patient 6, we now secure the band with sutures to the graft at the anastomosis to prevent its migration. This patient was also the only one having a 10 mm graft inserted. The extra 2 mm of graft diameter occupied much of the available length of the main pulmonary artery, so that it was even more difficult to place the band. We have found the 8 mm graft to be a better choice and no longer use the 10 mm graft. We also try to place the pulmonary artery anastomosis as far proximal as possible to provide space for the band. With the incorporation of these technical changes, none of the last six patients treated has had band obstruction of the right pulmonary artery. The deaths of two patients from lack of an adequate left atrial outlet indicates the importance of a large atrial septal defect being present postoperatively in these patients. Echocardiography has proved to be unreliable in assessing the size of the atrial defect. We now routinely perform cardiac catheterization and balloon atrial sep-
The Journal of Thoracic and Cardiovascular
8 8 8 Tucker et al.
tostomy preoperatively with the hope that the too small atrial septal defect can be avoided and the survival rate improved. This approach to hypoplastic left heart syndrome has allowed the hospital discharge of five of nine infants. Postoperative hemodynamic, respiratory, and bleeding problems have been minimal. The future of these patients remains uncertain, but we believe this approach may allow survival until a proper donor heart can be located for cardiac transplantation. A second-stage Fontan procedure may provide more definitive treatment if pulmonary artery growth is good and pulmonary artery pressures remain normal. REFERENCES 1. Norwood WI, Lang P, Castaneda AR, Campbell ON. Ex-
perience with operations for hypoplastic left heart syndrome. J THORAC CARDIOVASC SURG 1981;82:511-9. 2. Pigott JD, Murphy JD, Barber G, Norwood WI. Palliative reconstructive surgery for hypoplastic left heart syndrome. Ann Thorac Surg 1988;45: 122-8. 3. Sade RM, Crawford FAJr, Fyfe DA. Symposium on hypoplastic left heart syndrome [Letter]. J THORAC CARDIaVASCSURG 1986;91:937-9. 4. Sade RM, Fyfe 0, Alpert Cc. Hypoplastic left heart syndrome: a simplified palliative operation. Ann Thorac Surg 1987;43:309- I 2.
5. Bailey LL, Nehlsen-Cannarella SL, Doroshow RW, et al. Cardiac allotransplantation in newborns as therapy for hy-
Surgery
poplastic left heart syndrome. N Engl J Med 1986;315:94951.
6. Mavroudis C, Harrison H, Klein JB, et al. Infant orthotopic cardiac transplantation. J THORAC CARDIOVASC SURG 1988;96:912-24.
7. Sinha SN, Rusnak SL, Sommers HM, Cole RB, Muster AJ, Paul MH. Hypoplastic left ventricle syndrome: analysis of thirty autopsy cases in infants with surgical considerations. Am J Cardiol 1968;21: 166-73. 8. Van Praagh R, Bernhard WF, Rosenthal A, Parisi LF, Fyler DC. Interrupted aortic arch: surgical trleatment. Am J Cardiol 1971;27:200-11. 9. Litwin SB, Van Praagh R, Bernhard WF. A palliative operation for certain infants with aortic arch interruption. Ann Thorac Surg 1972;14:369-75. 10. van der Horst R, Hastreiter AR, Levitsky S, Fisher EA, DuBrow IW, Weinberg M. Interrupted aortic arch operation in the first week oflife: hemodynamic and angiographic evaluation one year later. Ann Thorac Surg 1979;27: 11220. 11. Bernhard WF, Litwin SB, Williams WW, Jones JE, Gross
RE. Recent results of cardiovascular surgery in infants in the first year of life. Am J Surg 1972; 123:451-60. 12. Levitsky S, van der Horst RL, Hastreiter AR, Eckner FA, Bennett EJ. Surgical palliation in aortic atresia. J THORAC CARDIOVASC SURG 1980;79:456-61. 13. Doty DB, Knott HW. Hypoplastic left heart syndrome: experience with an operation to establish functionally normal circulation. J THORAC CARDIOVASC SURG 1977;74:624-30.