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Ann Thorac Surg 2004;78:1951-1958
© 2004 The Society of Thoracic Surgeons
a Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, Okayama-City, Japan
b Department of Cardiovascular Surgery, Fukuoka Children's Hospital, Fukuoka-City, Japan
c Department of Cardiovascular Surgery, Shizuoka Children's Hospital, Shizuoka-City, Japan
Accepted for publication May 19, 2004.
* Address reprint requests to Dr Sano, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, 251 Shikata-cho, Okayama-City 7008558, Japan
s_sano{at}cc.okayama-u.ac.jp
Presented at the Thirty-ninth Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 31Feb 2, 2003.
| Abstract |
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METHODS: Procedures performed included an aortopulmonary neoaortic reconstruction and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 6 patients, 5 mm in 41, and 6 mm in 26. Continuous cerebral perfusion was used in all patients and an additional descending aortic perfusion was used in 39. Postoperative management was basically the same as that for infants undergoing other types of operations.
RESULTS: There were 61 hospital survivors (84%), including 5 of 6 patients weighing less than 2 kg, with 8 late deaths. Risk factors for hospital mortality include preoperative treatment without ventilatory support and surgeon's experience (first 10 cases). Three patients underwent a primary Fontan operation at 5, 9, and 10 months of age, with one late death. Forty-one patients underwent the bidirectional Glenn shunt after a mean interval of 6.9 months, and 19 of them completed the Fontan operation at median age of 2.1 years. Overall survivals were 65% at 1 year and 63% at 2 years.
CONCLUSIONS: Improved survival for patients after first-stage palliation of hypoplastic left heart syndrome is reproducible for many centers by an application of the modified Norwood procedure with the right ventricletopulmonary artery shunt.
| Introduction |
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The Norwood procedure must provide unobstructed systemic blood flow from the right ventricle, relieve obstruction to pulmonary venous return, and limit pulmonary blood flow by a systemic-to-pulmonary shunt. Because the pulmonary circulation is perfused from the systemic circulation, a sudden shift in the resistance ratio between the two vascular beds can cause maldistribution of cardiac output. This phenomenon has been implicated as a major cause of early death in these infants [14]. To prevent hemodynamic instability associated with the systemic-to-pulmonary shunt, we constructed a nonvalved polytetrafluoroethylene shunt between the right ventricle and the pulmonary artery (RV-PA shunt) as an alternative pulmonary blood source. Our initial results with developing surgical techniques have been reported [15, 16]. To evaluate the impact of minor differences in surgical techniques on the overall outcome, we collected data obtained from patients undergoing the modified Norwood procedure with the RV-PA shunt at three Japanese institutions at which the average number of stage I palliation for HLHS per year was fewer than 10 during the study period.
| Patients and Methods |
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Surgical Technique
Detailed operative procedures have been previously reported [15, 16]. Briefly, we describe minor differences in surgical techniques among the institutions and key points at each step of the modified Norwood procedure with the RV-PA shunt.
Cardiopulmonary Bypass
To avoid use of total circulatory arrest as much as possible, cardiopulmonary bypass was established by dual arterial perfusion and single atrial or bicaval drainage cannulations. One perfusion cannula was inserted into a 3.0- or 3.5-mm polytetrafluoroethylene tube that was anastomosed to the innominate artery to keep continuous cerebral perfusion in all patients. In 34 patients, the other perfusion cannula was inserted into the descending aorta from the ductus arteriosus. During aortic reconstruction this cannula was removed, and circulation in the lower body was arrested at 20° to 22°C. In the remaining 39 patients, the descending aorta was directly cannulated in the lower posterior mediastinum, and the whole systemic perfusion was maintained at 28° to 31°C. Cardioplegic solution was administered through a cannula that was placed in the ascending aorta from an aortic arch incision or infused from a side port of the perfusion cannula during temporary total circulatory arrest. Modified ultrafiltration was routinely used after weaning from cardiopulmonary bypass.
Aortic Reconstruction and Atrial Septectomy
Because homograft is not available in Japan, aortic reconstruction without patch supplementation [8] was the procedure of choice. In all patients, ductus arteriosus tissue was completely excised, and a neoaorta was constructed by direct anastomoses of the descending aorta (Fig 1) and a proximal main pulmonary artery to an opened tiny native aorta (Fig 2). A small piece of pericardial patch was necessary in some patients. An atrial septectomy was performed with the heart beating in patients with bicaval cannulation. Otherwise the septum was excised during a period of total circulatory arrest for infusion of cardioplegic solution.
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Postoperative Management
The sternum was routinely left open, and patients underwent delayed sternal closure on postoperative day 1 to 11 (median, 3 days). Postoperative intensive care of patients with the RV-PA shunt was basically the same as that for neonates undergoing other types of operations. Thus, delicate manipulations to control pulmonary and systemic vascular resistance were not necessary. Ventilator settings were adjusted to keep arterial oxygen saturations between 75% and 85% and carbon dioxide levels from 40 to 50 mm Hg. It should be kept in mind that hypercarbia as is often used to increase pulmonary vascular resistance in patients with the systemic-pulmonary shunt often causes hypoxemia and subsequent hemodynamic deterioration in those with the RV-PA shunt. The inotropic drugs most used were dopamine or dobutamine 4 to 10 µg/kg per minute, epinephrine 0.05 to 0.1 µg/kg per minute; if necessary, calcium chloride 0.25 mmol/h was also used.
Statistical Methods
The medical records, operative reports, and echocardiographic results for all patients were reviewed. Follow-up was complete for all patients. Data were entered into a computerized database and analyzed with SPSS software (SPSS Inc, Chicago, IL). Hospital deaths were considered as any deaths during the hospital stay after the modified Norwood operation, regardless of duration. The potential risk factors for hospital mortality were analyzed by
2 test. Actuarial survival curve was computed by Kaplan-Meier methods. The Mann-Whitney rank sum test was used for comparison of continuous variables in the two groups. The data are presented as the mean ± standard deviation. The level of statistical significance was set at p = 0.05.
| Results |
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Three patients underwent primary Fontan operation at 5, 9, and 10 months of age. Forty-one patients underwent the bidirectional Glenn shunt (stage II palliation) after a mean interval of 6.9 months (median, 6.4 months; range, 2.6 to 14.6 months). There was no difference in the intraoperative interval between patients with a 5-mm shunt and those with 6-mm shunt (6.7 ± 2.0 versus 7.9 ± 3.2 months; p = 0.2). Twenty-six patients (63%) required concomitant procedure for pulmonary artery stenosis, including 9 instrumental manual dilation and 17 reconstructions. In addition, one of them underwent repair of total anomalous pulmonary connection associated with heterotaxy syndrome. There were 3 hospital deaths caused by hypoxemia, pneumonia, and sepsis. One late death occurred after takedown of the bidirectional Glenn shunt.
Nineteen patients underwent the Fontan operation after the stage II palliation at median age of 2.1 years (range, 0.9 to 3.2 years). One patient had tricuspid valve replacement at the time of the Fontan. Late reoperations were performed in 2 patients, including tricuspid valve replacement, and repair of coronary sinus orifice atresia [16]. The mean pulmonary artery index [17] measured by cineangiography before the Fontan operation in the 19 patients was 152 ± 45 (median, 151; range, 82 to 262). To date, the Fontan operation has been completed in a total of 22 patients, with one late death after the primary completion. The current status of all hospital survivors after stage I palliation is shown in Figure 4. Actuarial survival of the 73 patients undergoing the modified Norwood procedure with the RV-PA shunt was 65% (95% CI, 53% to 77%) at 1 year and 63% (95% CI, 51% to 65%) at 2 years (Fig 5). The 1-year and 2-year actuarial survivals for patients with each surgeon's first 10 cases were 53% (95% CI, 36% to 71%) at both times. Patients with each surgeon's recent cases had an actuarial survival at 1 and 2 years of 71% (95% CI, 57% to 86%) and 68% (95% CI, 52% to 83%), respectively (Fig 5).
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| Comment |
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An RV-PA valved or nonvalved conduit was first attempted by Norwood and associates [4] during the development of the surgical technique for HLHS. Kishimoto and coworkers [22] in Japan revived this technique using a valved xenopericardial roll and demonstrated stable postoperative hemodynamics with high diastolic arterial pressure. Since 1998 we have applied the RV-PA shunt, but with a small nonvalved polytetrafluoroethylene conduit in stage I palliation. Without delicate postoperative management the hospital survival for the 73 patients was 84%, and the actuarial survival at 2 years was 63%. Although the first 10 cases for each surgeon were identified as a risk factor for hospital mortality, the results of the present study suggested that improved survival for patients after stage I palliation for HLHS is reproducible for many centers by an application of the modified Norwood procedure with the RV-PA shunt.
A theoretical advantage of the RV-PA shunt over the systemic-to-pulmonary shunt is elimination of diastolic runoff into the pulmonary circulation with concurrent unloading of the systemic right ventricle [16]. Resultant high diastolic pressure, lower aortic saturation [15, 16, 22], and decreased volume work may improve myocardial perfusion and ventricular function. One drawback of the RV-PA shunt is volume load by reversal flow through the nonvalved conduit. We previously reported [16] that the most reversal flow occurs within 1 month after surgery and then decreases with time. Because HLHS hearts with a small RV-PA shunt are similar to univentricular hearts with pulmonary valve stenosis or after pulmonary artery banding, pulmonary blood flow is mainly limited by the diameter of the shunt. Our results indicate that an optimal size of the shunt is 5 mm for patients weighing between 2.0 kg and 2.5 kg, and 5 mm or 6 mm for heavier patients.
Because of the lack of suitable sized material, reported survival for patients weighing less than 2.5 kg after stage I palliation with the systemic-pulmonary shunt has been approximately 50% [23, 24]. Stable hemodynamics provided by the RV-PA shunt is particularly beneficial for this subgroup of patients. In the present series, 15 of 21 patients (71%) weighing less than 2.5 kg survived stage I palliation. As far as 6 patients weighing less than 2.0 kg are concerned, a 4-mm shunt was used in 3 patients and a 5-mm shunt in the other 3. Among them only one death occurred in the smallest 1.3-kg girl. Although she died of respiratory failure on postoperative day 7, her hemodynamics were quite stable with a 4-mm shunt.
Hypoxemia accounted for 35% (7 of 20 patients) of mortality before stage II palliation. Except for one hospital death in the 1.3-kg patient and two late deaths because of progressive shunt obstruction, the remaining 4 patients all received an additional 3-mm or 3.5-mm systemic-to-pulmonary shunt at stage I palliation. We suspect that the cause of lethal hypoxemia might not be failure of the RV-PA shunt but pulmonary vascular disease associated with HLHS [25].
Deep hypothermic total circulatory arrest has commonly been used during aortic reconstruction in stage I palliation. Even surgeons at institutions with high surgical volume required a mean duration of circulatory arrest of 44 to 57 minutes [8, 10, 26]. It is obvious that less-experienced surgeons need longer circulatory arrest times, predisposing critically ill infants to neurologic deficits and multiorgan failure. Continuous cerebral perfusion with or without descending aortic perfusion used in the present series minimized or avoided the use of total circulatory arrest and, at least in part, contributed to the improved outcome of stage I palliation for HLHS.
Poor development of the pulmonary artery before the Fontan operation is of great concern. Compression of the neoaorta constructed by a direct anastomosis of the main pulmonary artery on the central part of the branch pulmonary arteries was a possible cause of this problem. It is our impression that pulmonary artery growth in patients with the RV-PA is worse than in those with a systemic-to-pulmonary shunt and that the RV-PA shunt may provide less pulmonary blood flow than the systemic-to-pulmonary shunt. The present study demonstrated that resultant lower pulmonary vascular resistance made the Fontan operation possible in the majority of patients with a pulmonary artery index of less than 200.
In conclusion, improvement in survival of patients after stage I palliation for HLHS is possible for many surgeons by application of the modified Norwood procedure with the RV-PA shunt together with the use of cardiopulmonary bypass techniques to avoid total circulatory arrest. [18]
Discussion
DR CHRISTIAN PIZARRO (Wilmington, DE): Doctor Sano, have you been able to elucidate the real contribution of the right ventricletopulmonary artery (RV-PA) conduit to the improved results in a series in which the use of selective antegrade cerebral perfusion, low-flow cardiopulmonary bypass, and change in the technique of arch reconstruction with autologous material has also been introduced simultaneously?
Second, you had a higher mortality after the second-stage surgery. Do you have any cardiac catheterization data to demonstrate that these patients were appropriately palliated and prepared for that stage?
Finally, could you tell us a little more about those patients who had significant right ventricular (RV) failure? Were those patients who had significant preoperative tricuspid regurgitation, and if so did that change after the first stage? I enjoyed your presentation very much.
DR SANO: Thank you for your comments and questions, Dr Pizarro. The answer to the first question is that we do not know whether improvement of the result of first-stage palliation is really caused by RV-PA shunt or as a result of cerebral perfusion. Since 1995, we have adopted cerebral perfusion for patients with arch repair, and since 1998, we have changed the technique of first-stage palliation of hypoplastic left heart syndrome (HLHS). Although we cannot say anything statistically because the number of cases using RV-PA shunt Norwood is so small, I think we have not achieved any improvement until we adopted the RV-PA shunt.
I am looking forward to seeing your analysis, because you changed only one thing, from aorta-topulmonary artery shunt to RV-PA shunt.
The answer to the second question is that major causes of late deaths are hypoxemia and ventricular failure. In the beginning, we did not know that hypoxemia or shunt obstruction could progress so quickly. One of the late deaths occurred to the patient with severe tricuspid regurgitation preoperatively. Three patients died as a result of RV failure and 2 of them had massive tricuspid regurgitation preoperatively.
DR JOHN E. MAYER (Boston, MA): The question that keeps coming up and that I would be interested in your commenting on is, what is the effect of the incision in the right ventricle on late ventricular function? Have you seen either aneurysmlike formation or dyskinetic areas at the ventricular origin of the shunt? How might problems related to the ventriculotomy relate to the results in this series of patients, both in terms of deaths between stage 1 and stage 2 or the outcome after your bidirectional cavopulmonary shunts?
DR SANO: Thank you for your comments, Dr Mayer. That is my worry, too. I think ventriculotomy should be just underneath the pulmonary valve. Some patients, who received a 6-mm polytetrafluoroethylene (PTFE) graft, had a much bigger ventriculotomy and had developed RV failure. And also patients whose ventriculotomy was lower in the body of the right ventricle had dyskinesia around the ventriculotomy site and had much worse right ventricular function.
At the second-stage or third-stage repair, the ventriculotomy site became smaller and smaller and we could hardly see the ventriculotomy site. I personally have not experienced any aneurysm formation at the ventriculotomy site.
DR FRANK A. PIGULA (Pittsburgh, PA): My question is regarding the combination of the two techniques, that being the bringing up of the main pulmonary artery segment to the underside of the arch in combination with the RV-PA conduit. I have been struck by the observation that when you do that, there is really not a lot of room. And the geometry is a little bit cramped when you try to bring the RV-PA conduit behind that type of reconstruction. Has that presented any problems for you either with the reconstruction itself or with the left pulmonary artery?
DR SANO: I am sorry, I cannot understand.
DR PIGULA: When you combine the two techniques of the RV-PA conduit with the advancement of the main pulmonary artery segment to the underside of the arch, has that presented any problems for you?
DR SANO: Thank you, Dr Pigula. No, there was no problem because the distal part of the PA reconstruction was done before the neoaorta was reconstructed. The space between the arch and the RV was small after direct anastomosis of the aortic arch, descending aorta, and pulmonary artery; however, I had no experience of left pulmonary artery stenosis.
DR JAMES S. TWEDDELL (Milwaukee, WI): Just a question on the development of RV failure. Did you find any correlation between the development of RV failure and the size of the shunt you used? Would you propose some ideal shunt or conduit size?
DR SANO: The shunt size was different in each of the three centers. But in most of the patients, a shunt size was 5 mm.
In our initial series, we used a 4-mm PTFE shunt because I was worried about the high flow and the volume loaded to the RV. However, in these patients, the graft became obstructed very quickly. Two patients in our first series died suddenly because of hypoxemia.
The other centers where the 6-mm PTFE graft was used needed a much bigger ventriculotomy, and some of these patients demonstrated poor ventricular function and tricuspid regurgitation increased.
So we use a 4-mm PTFE graft in the patients weighing less than 2 kg, a 5-mm graft in the patients between 2.0 and 3.5 kg, and a 6-mm PTFE graft in the patients weighing more than 3.5 kg.
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