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Ann Thorac Surg 2004;77:477-483
© 2004 The Society of Thoracic Surgeons
a Department of Critical Care Medicine, Loma Linda University Children's Hospital, Loma Linda, California, USA
b Department of Cardiology, Loma Linda University Children's Hospital, Loma Linda, California, USA
d Department of Cardiothoracic Surgery, Departments of Pediatrics and Surgery, Loma Linda University Children's Hospital, Loma Linda, California, USA
c School of Allied Health Professionals, Loma Linda University, Loma Linda, California, USA
Accepted for publication August 6, 2003.
* Address reprint requests to Dr Checchia, Washington University School of Medicine, St. Louis Children's Hospital, Campus Box 8116, One Children's Place, Suite 5S20, St. Louis, MO 63110, USA
e-mail: checchia_p{at}kids.wustl.edu
| Abstract |
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METHODS: A weighted score for each of six variables comprised the scoring system: ventricular function, tricuspid regurgitation, ascending aortic diameter, atrial septal defect blood flow characteristics, blood type, and age. The scoring system was used to prospectively assign mortality risk and lead to recommendation of either Norwood procedure or transplantation.
RESULTS: Survival following the Norwood procedure significantly improved after the management program was implemented (88% versus 40% at 48 hours, 57% versus 10% at 30 days, and 50% versus 10% at 1 year, p < 0.0001 at each time point). The survival of the group that received a score of 7 or less (high risk) who underwent the Norwood procedure was 78% at 48 hours, 44% at 30 days, and 33% at 1 year; survival rates among patients considered lower risk (greater than 7) were 100% at 48 hours and 80% at 30 days and 1 year. Transplant outcomes remained unchanged.
CONCLUSIONS: We report improved survival following the Norwood procedure after the implementation of an institutional management approach aimed at improving the outcome of infants with hypoplastic left heart syndrome and may help neutralize historical biases toward Norwood procedure or transplantation.
| Introduction |
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Loma Linda University Children's Hospital (LLUCH) has favored transplantation to manage HLHS [10]; however, diminished availability of organs and reports of improved survival after surgical palliation [11, 12] led to a reexamination of this institutional transplantation bias. We evaluated our approach to HLHS with the primary goal of improving the outcomes of transplantation and palliative reconstruction. This evaluation led to the development of a program, the goal of which was to assign patients preoperatively to either transplant or surgical palliation based on known risk factors, and to enhance postoperative care of infants having stage I reconstruction. We anticipated that the protocol would limit the total number of infants with HLHS awaiting transplantation to only infants in whom findings suggested significantly increased risks if they were to be managed by palliative reconstruction. We also hoped the protocol would ensure commitment to care and outcomes that would be independent of the surgical strategy used. The purpose of the current study was twofold: to examine the effect of the protocol on improving outcome of patients with HLHS and to identify the specific factors responsible for that outcome.
| Material and methods |
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Study population
We included all patients with the diagnosis of HLHS, which was defined as concordant atrioventricular and ventriculoarterial connections and either aortic valve atresia or stenosis. Patients were excluded only if surgical intervention was performed at another institution before referral.
Scoring system
A weighted score for each of six factors comprised the scoring system. Factors included ventricular function, tricuspid regurgitation, ascending aortic diameter, atrial septal defect blood flow characteristics, blood type, and age. The scoring system was weighted to reflect the relative importance of each factor based on group consensus interpretation of available data. The echocardiogram immediately preceding surgery was used for evaluation and scoring.
The scoring system is outlined in Table 1. Ventricular dysfunction was determined by a qualitative assessment of right ventricular ejection fraction. Function was evaluated as good if the ejection fraction was higher than 40%, marginal if 30% to 40%, and poor if less than 30%. Tricuspid regurgitation was assigned to be either moderate or severe. Moderate tricuspid regurgitation was defined as extension of the regurgitant jet to the back of the right atrium by color Doppler, or more than 25% of right atrial area in the plane in which the color Doppler tricuspid regurgitation jet was the greatest. Severe tricuspid regurgitation was defined as extension of the regurgitant jet to the back of the right atrium and more than 50% of right atrial area in the plane in which the color Doppler tricuspid regurgitation jet was the greatest. The ascending aortic size was measured just distal to the valve annulus. The atrial septal defect was deemed restrictive if the mean flow velocity across the defect was measured at more than 1.5 m/s with lack of variability in flow velocity. Blood type was considered because of its effect on organ availability and wait list time. Age at surgery was considered a factor to control for the practice of offering the Norwood procedure only after perceived excessive transplant wait times.
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Surgical management
No significant change was made in the well-established surgical and perioperative protocols for infants who received cardiac transplantation. The technique of orthotopic cardiac transplantation for HLHS has been described previously [13].
The modified Norwood reconstruction consisted of mobilization of the descending aorta, excision of all ductal tissue, and reconstruction of the neoaorta using native hypoplastic ascending aorta and arch augmented by pulmonary artery trunk and immobilized ascending aortic tissue. The right and left pulmonary artery continuity was achieved with autologous pericardial patch.
Infants in whom hemodynamic stability was maintained during the manipulation and dissection of the innominate artery had the construction of the shunt-innominate artery and anastomosis done first. The shunt was then used as the site for arterial systemic perfusion. Otherwise, the arterial cannula was placed into the patent ductus arteriosus and the shunt was constructed later during cardiopulmonary bypass.
Cardiopulmonary bypass was established using arterial cannulation of the ductus arteriosus through the main pulmonary artery and a single venous cannula in the right atrium. The pump circuit was primed with crystalloid solution that resulted in hemodilution (hematocrit of approximately 8% to 10%) during the cooling phase and period of reconstruction. Patients were cooled to a rectal temperature of 20°C. Once on bypass, the ductus was snared around the cannula, directing all blood to the aorta, and the main pulmonary artery was transected obliquely just proximal to its bifurcation. The confluence of the right and left pulmonary arteries was augmented with untreated autologous pericardium. The arterial cannula was then inverted, the distal right and left pulmonary arteries snared, and arterial flow was delivered retrogradely through the Blalock-Taussig shunt into the innominate artery. The ductus was ligated and excised from the isthmus of the aorta. The left carotid and subclavian vessels were occluded with snares and the descending aorta was clamped. A single dose of blood cardioplegia was delivered if the ascending aorta was used in reconstruction; otherwise, the reverse blood flow through the shunt was maintained, perfusing the innominate artery and ascending aorta.
The undersurface of the aortic arch was opened and the arch was reconstructed using the mobilized descending aorta and main pulmonary artery. Atrial septectomy was performed under low-flow sucker-bypass. The neoaorta was cannulated for arterial inflow, and full cardiopulmonary bypass was reinstituted, followed by rewarming, ultrafiltration, and hemoconcentration. Ultrafiltration and hemoconcentration were performed during the rewarming and reperfusion phase. Infants were weaned off cardiopulmonary bypass with a hematocrit of 30% to 36%. The oximetric catheter was placed in the superior vena cava and directed cephalad to the jugular vein. All patients left the operating room with an open sternotomy, covered with Betadine-impregnated plastic sheeting.
Postoperative monitoring
Each patient was cared for and monitored using routine intensive care practices for the LLUCH pediatric cardiac intensive care unit. These included central venous catheter placement, central venous pressure monitoring, and arterial blood pressure monitoring. In addition, a 4F oximetric catheter (4F OxyCath; Abbott Laboratories, North Chicago, IL) was placed in the superior vena cava to allow continuous monitoring of estimated mixed venous oxygen saturation (SvO2). The oximetric catheter was placed intraoperatively and calibrated in the operating room. If the surgeon was unable to place oximetric catheter during surgery or if the catheter failed to function properly during the initial 48 hours after surgery, simultaneous arterial and central venous blood gas measurements were obtained hourly for the first 24 hours and every 2 hours for the next 24 hours.
Postoperative protocol
The postoperative management protocol included delayed sternal closure, continuous mixed venous saturation measurements, and calculation of the ratio of pulmonary to systemic blood flow (Qp/Qs), AVO2 difference, and oxygen extraction ratio computed by the bedside nurse (see Appendix). Postoperative management centered on maximizing SvO2, and circulatory balance was estimated using the Fick equation. Interventions were recommended based on the protocol and the protocol extended throughout the first 48 hours after operation (Table 2).
Final clinical decisions were made by either the attending surgeon or cardiac intensivist caring for the patient.
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Statistical analysis
Descriptive statistics were used to summarize the data. A Z-test was conducted to compare the survival rate between the historical control group and the management program group. Conditional logistic regression analysis was done to evaluate the influence of individual factors on survival. All analyses were conducted with the use of statistical software SAS version 8 (SAS Institute, Cary, NC).
| Results |
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To determine the effect of the management program on short-, intermediate-, and long-term survival, comparisons of postoperative mortality were made at 48 hours, 30 days, and 1 year after initial treatment. Survival among HLHS patients who underwent the Norwood procedure significantly improved after the implementation of the management program when compared with the historical control group (Fig 1). This improvement was noted at the three time points measured (88% versus 40% at 48 hours, 57% versus 10% at 30 days, and 50% versus 10% at 1 year, p < 0.0001 at each time point).
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The management program group consisted of 18 patients; each was assigned a preoperative score from which an operative pathway was recommended. Twelve patients received a score of 7 or less and were considered to be at higher complication risk for the Norwood procedure, leading to the recommendation for transplant. Despite the institutional recommendation, only 3 families opted for transplantation and the remaining 9 underwent the Norwood procedure. Survival rates among the 3 transplanted patients from the high-risk Norwood group was 100% at 48 hours and 66% at 30 days and 1 year. Survival among the 9 patients who received a score of 7 or less who underwent the Norwood procedure was 78% at 48 hours, 44% at 30 days, and 33% at 1 year. Six patients received a A-vo2 difference, and score of more than 7 and were considered lower risk candidates for the Norwood procedure. Five of these 6 patients (the low-risk Norwood group) underwent the Norwood procedure and survival rates were 100% at 48 hours and 80% at 30 days and 1 year (Fig 2). The sixth patient had transplantation and was alive after 1 year of follow-up. Statistical comparisons did not yield significant results due to the limited population size.
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Logistic regression analysis was performed on each group as well as the population as a whole to evaluate the effect of individual factors on the increased survival. The use of the postoperative protocol significantly increased survival at 48 hours (odds ratio [OR], 8.99; confidence interval [CI], 1.27 to 63.88; p = 0.03) and 30 days (OR, 12.0; CI, 1.18 to 122.27; p = 0.03). Logistic analysis of individual factors of the scoring system did not reveal any single factor that predicted survival.
| Comment |
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Several investigators have attempted to identify patients at highest risk for death after the Norwood procedure [3, 7, 8, 1418]. They sought to improve outcomes by implementing postoperative treatment protocols. These protocols have demonstrated improved survival utilizing SvO2 monitoring [19, 20], inotropic support [21], or a combination of approaches [2]. Our approach is unique, as preoperative decisions did not depend on an individual variable. Instead, we have combined elements from each strategy to develop a selection process to provide for the highest chance of success with a protocol that optimizes postoperative treatment.
Selection of appropriate candidates for the Norwood procedure is multifactorial. Indeed, our analysis suggests that no single variable was more important than another in the preoperative decision algorithm. The variables chosen were based on published reports available at the time of initiation of our protocol, as well as institutional experience. Our group sought to incorporate anatomic, physiologic, and logistic factors into a single, weighted scoring system. One of the stronger associations with increased mortality after the Norwood procedure is the diameter of the ascending aortic arch [8, 12, 18, 22]. Thus, the scoring system was weighted more heavily for this factor. It has been the experience in our institution and among others, that greater postnatal age is associated with higher Norwood procedure mortality [2325]. Finally, to account for organ availability, the longer waiting time for patients with blood type O was accounted for in the scoring system.
Preoperative ventricular dysfunction has been associated with increased incidence of postoperative complications in children who have undergone the Norwood procedure [14]. While ventricular dysfunction has a detrimental effect on survival following Norwood palliation, the condition does not appear to jeopardize survival during the wait for heart transplantation. Consequently, the presence of preoperative ventricular dysfunction shifts our management recommendation toward transplant. Similarly, the presence of a restrictive atrial septal defect [26] and clinically relevant tricuspid regurgitation has been reported as risk factors for mortality after Norwood reconstruction [6, 22]. Hence, presence of these variables tends to shift the decision score toward transplantation.
The postoperative protocol incorporates many of the strategies supported by several recent investigations. A goal-directed approach using constant SvO2 monitoring [2, 1921], milrinone [21], and delayed sternal closure [27] was used. The relative contribution of each of these components is unknown in our population. However, Tweddell and associates [2] have recently reported their experience with the additive effect of each of the various postoperative components. We believe that the use of SvO2 monitoring and bedside nurse calculations of AVO2 differences allowed earlier anticipation of deterioration of clinical status. Furthermore, the use of milrinone may be beneficial in reducing the severity of low cardiac output syndrome during the first 48 hours after the operation [28]. One other possibility is that because our data showed that more than 96% of nursing assessments were completed on time, the management program may have had an unexpected benefit in improving nursing diligence by providing the bedside nurse with a template of physiologic variables unique to the Norwood patient.
The inability to correlate survival improvement to individual components of the management approach, both preoperatively and postoperatively, represents a limitation of the study. However, we speculate that the individual factors of the preoperative scoring system and the postoperative protocol were less important to our success than the cumulative effects of the entire program.
Our institutional commitment to succeed includes focused, intense nursing education; the addition of a full-time pediatric cardiac intensivist; and scheduling that allowed a single surgeon to perform the vast majority of the procedures. This commitment allowed for greater standardization of care delivered to the patients. We speculate that the implementation of this program at other institutions would have equal impact because our success occurred at an institution that has historically favored transplantation and has previously had limited experience and success with the Norwood procedure.
In conclusion, we report the implementation of an institutional management approach aimed at improving the outcome of infants with HLHS. Survival improved in patients undergoing surgical palliation with the Norwood procedure at an institution historically favoring transplantation. This accomplishment represented a significant paradigm shift for the institution. The reproducibility of our success using this management program at institutions already committed to staged surgical palliation remains unknown.
| Acknowledgments |
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| Appendix |
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AVO2 = arteriovenous oxygen saturation; OER = oxygen extraction ratio; Qp/Qs = ratio of pulmonary to systemic blood flow; SaO2 = arterial oxygen saturation; SvO2 = mixed venous oxygen saturation.
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