Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia

OBJECTIVE: This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation. METHODS: A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates. Competing risks analyses were constructed for outcomes after Norwood operation and after cavopulmonary shunt. Incremental risk factors for outcome events were sought. RESULTS: Overall survivals after the Norwood operation were 72%, 60%, and 54% at 1 month, 1 year, and 5 years, respectively. According to competing risks analysis, 97% of neonates reached a subsequent transition state by 18 months after Norwood operation, consisting of death (37%), cavopulmonary shunt (58%), or other state (2%, cardiac transplantation, biventricular repair, or Fontan operation). Risk factors for death occurring before subsequent transition included patient-specific variables (lower birth weight, smaller ascending aorta, older age at Norwood operation), institutional variables (institutions enrolling < or =10 neonates, two institutions enrolling >/=40 neonates), and procedural variables (shunt originating from aorta, longer circulatory arrest time, and management of the ascending aorta). Of neonates undergoing cavopulmonary shunt, 91% had reached a subsequent transition state by 6 years after cavopulmonary shunt, consisting of Fontan operation (79%), death (9%), or cardiac transplantation (3%). Risk factors for death occurring before subsequent transition included younger age at cavopulmonary shunt and need for right atrioventricular valve repair. CONCLUSIONS: Competing risks analysis defines the prevalence of the various outcomes after Norwood operation and predicts improved outcomes with successful modification of controllable risk factors.     

Surgical treatment of hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) was previously uniformally fatal within the first month of life. The development of the Norwood stage I operation has afforded new hope to the families of newborn infants with this defect. Recent, modification of the Norwood procedure has improved the surgical results. The one-year survival rate after the Norwood procedure is from 50% to 70% in major institutions. The results of second palliation (hemi-Fontan operation or bidirectional Glenn operation) are nearly acceptable. Recently, fetal echocardiography has allowed early diagnosis of HLHS, and after a prenatal diagnosis of HLHS, couples may be offered termination of the pregnancy. It is necessary to improve the results of the Norwood stage I operation, to save more fetuses and neonates with HLHS.     

Factors influencing early outcome of Norwood procedure for hypoplastic left heart syndrome.

OBJECTVIE: The operative outcome of the Norwood procedure for hypoplastic left heart syndrome is still not satisfactory. Conflicting reports concern factors associated with early Norwood procedure mortality and the reasons for the instability after surgery are not sufficiently understood. The purpose of this study was to determine some perioperative factors influencing early (30 days) outcome. METHODS: We retrospectively analyzed a group of 30 consecutive children with hypoplastic left heart syndrome (aged 5-39 days) who underwent Norwood procedure in 1997 and 1998. The following factors were considered and statistically analyzed: operative age, birth weight, operative weight, serum level of bilirubin, aminotransferases, creatinine, urea, arterial blood gasses, anatomic subgroups, ascending aorta and arch size, systemic to pulmonary modified right Blalock-Taussig shunt size, cardiopulmonary circulatory arrest time, cardiopulmonary bypass time, and delayed chest closure. Eighteen patients underwent hemi-Fontan procedure with one late death and the modified Fontan operation was performed in 16 of them (one late death). RESULTS: The early mortality was 37%. Seven deaths (64%) occurred during the first 24 h after operation. There was a significant difference between survivals and non-survivals in: birth weight (P=0.047), operative age (P=0.016), preoperative serum level of bilirubin (P=0.044), and cardiopulmonary circulatory arrest time (P=0.006). The other assessed factors were not found to be predictors of early mortality. All 16 survivals followed up are in New York Heart Association class I or II. CONCLUSIONS: Anatomic and functional status of the patient, as well as procedural factors are related to Norwood operation early mortality. High mortality in hypoplastic left heart syndrome after stage I surgery indicates the necessity of assessing all factors which may determine further improvement in the outcome.     

Comparison of hemodynamics between Norwood procedure and systemic-to-pulmonary artery shunt for single right ventricle patients

Objective: Despite that surgical outcomes of patients with hypoplastic left heart syndrome have improved, one of the problems remaining is the high interstage mortality after a stage I Norwood procedure. The purpose of this study was to determine the hemodynamic characteristics of hypoplastic left heart syndrome after a Norwood procedure. We examined the perioperative hemodynamic differences of the staged operation between the first stage of the Norwood procedure and systemic pulmonary shunt for single right ventricle patients. Methods: Data from 39 patients who underwent a Norwood procedure (right ventricle to pulmonary artery conduit: 19, Blalock–Taussig shunt, 20) were analyzed. There were nine early and seven interstage deaths. Bidirectional cavopulmonary shunt was performed in 15 patients and the Fontan procedure in 9 (group H). We defined the control group as 26 patients who underwent the first stage of a systemic pulmonary shunt for a single ventricle. Bidirectional cavopulmonary shunt was performed in 14 patients and the Fontan procedure in 8 (group C). We compared the perioperative hemodynamics of the staged operation between the two groups. Results: Cardiothoracic ratio and single ventricular diastolic dimension before bidirectional cavopulmonary shunt were acutely increased in group H (P=0.02, <0.001). There was no significant difference between the two different types of Norwood procedures. The pulmonary artery index for the right heart bypass operation was lower in group H than in group C (P<0.001). Oxygen saturation before bidirectional cavopulmonary shunt in group H decreased (P<0.001) and thus was lower than that in group C (P=0.003). Mortality and the postoperative clinical parameters of the right heart bypass operation were not different between the two groups. Conclusions: Patients with hypoplastic left heart syndrome showed hemodynamic instability of acutely increased cardiothoracic ratio, and single ventricular diastolic dimension despite decreased oxygen saturation interstage after stage I of a Norwood procedure. This suggests that this hemodynamic characteristics in hypoplastic left heart syndrome correlates with the higher mortality before second stage palliation than in found with single right ventricle patients.     

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

Objective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm²/m²; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity. Presented at the 59th Annual Scientific Meeting of the Japanese Association for Thoracic Surgery, held in Tokyo, Japan, October 1–4, 2006     

Single ventricle with systemic obstruction in early life: comparison of initial pulmonary artery banding versus the Norwood operation.

OBJECTIVES: The outcomes of initial pulmonary artery banding (PAB)+/-coarctation repair are compared with the Norwood operation in newborns with single ventricle (SV) and systemic obstruction (SO). METHODS: Between January 1987 and July 2000, 22 patients (median age, 12 days) with SV and aortic arch obstruction (AAO), subaortic stenosis (SAS), or both underwent surgery. Two initial surgical approaches were used: PAB+/-coarctation repair (group I, seven patients); Norwood type operation (group II, 15 patients). RESULTS: The overall mortality was 32% (seven of 22 patients). There was no late mortality. The mortality in group I was 43% versus 27% in group II. Recently, there has been no mortality following the Norwood operation in the last eight patients operated since 1995. Of the survivors, nine patients have undergone the Fontan operation and four patients have had the bidirectional Glenn (BDG) with no deaths. There was one repair of supravalvar aortic stenosis at the time of BDG in group II as opposed to eight reinterventions for SAS and/or AAO in four patients in group I (P=0.01). CONCLUSIONS: PAB+/-coarctation repair for SV and SO is associated with a high mortality and a high reoperation rate for SAS or recurrent AAO. Although the Norwood operation was also associated with a high mortality early on, it can now be performed with excellent outcome. This improvement, combined with a low reintervention rate for SAS or AAO, suggests that the Norwood operation is likely to emerge as the procedure of choice for SV and SO.     

False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome

The Norwood I operation, including placement of a shunt from the right ventricle to the pulmonary arteries, has been adopted by many surgeons for neonates with hypoplastic left heart syndrome. A three-year-old male who had undergone the Norwood I operation, and the Glenn operation, presented with a cervical pulsating tumor prior to the operation for total cavopulmonary connection. At the Glenn operation, the right ventricle to the pulmonary artery shunt was closed with a clip proximally, and the distal part was resected. Following the Glenn operation, the child had had recurrent deep sternal infections caused by Serratia marcescens. Cardiac catheterization showed a false aneurysm from the proximal shunt anastomosis. The bleeding after resternotomy was managed by initiating cardiopulmonary bypass via the groin vessels. Cerebral air embolies were prevented by systemic application of potassium, to achieve cardioplegic arrest during chest opening. The shunt was removed and the defect was closed. After the shunt was confirmed to be free from infection, a total cavopulmonary connection was performed after three days postoperatively. The case illustrates the management of retrosternal aneurysms during resternotomy in children.     

Early experience with a modified Norwood procedure using right ventricle to pulmonary artery conduit

BACKGROUND: A recent modification to the Norwood procedure involves the use of a right-ventricle (RV) to pulmonary artery (PA) conduit to provide pulmonary blood flow for patients with hypoplastic left heart syndrome (HLHS). This modification is thought to provide more stable hemodynamics by avoiding the diastolic "run-off" that occurs with a Blalock-Taussig shunt. METHODS: We reviewed our experience with the first 11 patients undergoing the RV-PA conduit modification of the Norwood operation and compared their outcomes with those of the preceding 22 patients who underwent a conventional Norwood procedure. RESULTS: Between July 1999 and March 2002, 33 patients with HLHS underwent the Norwood procedure at a median age of 5 days (range 1 to 31 days). Aortic atresia was present in 28 (85%). No significant difference was noted between the RV-PA (n = 11) and conventional Norwood (n = 22) groups with respect to measures of morbidity such as duration of mechanical ventilation or hospital stay. Patients who underwent the conventional Norwood procedure did have significantly lower diastolic blood pressure in the early postoperative period (38.4 +/- 4.4 mm Hg versus 49.5 +/- 4.3 mm Hg, p = 0.001). The operative and 1-year survival rates were 81% and 81%, respectively, for patients with the RV-PA modification, which was not significantly different from those of patients who underwent the conventional procedure, 81% and 73% (p = 1.00 and p = 0.36). Two patients developed a pseudoaneurysm of the RV infundibulum after placement of RV-PA conduit. Four sudden deaths occurred after hospital discharge, all occurring in the conventional Norwood group. CONCLUSIONS: The RV-PA conduit modification of the Norwood procedure results in excellent early survival. By avoiding low diastolic blood pressure this modification may provide superior perfusion to the coronary vascular bed and potentially reduce the risk of sudden unexpected death.     

Is a hybrid strategy a lower-risk alternative to stage 1 Norwood operation?

Background

For neonates with critical left ventricular outflow tract obstruction (LVOTO), hybrid procedures are an alternative to the Norwood stage 1 procedure. Despite perceived advantages, however, outcomes are not well defined. Therefore, we compared outcomes after stage 1 hybrid and Norwood procedures.

Staged Surgical Palliation in Hypoplastic Left Heart Syndrome and Its Variants

Abstract   Background: Surgical options for infants with hypoplastic left heart syndrome (HLHS) and/or its variants are cardiac transplantation or the heart-preserving staged palliation with Norwood operation, followed by a two-staged Fontan procedure. We describe our 17-year experience with staged palliation of HLHS and/or its variants. Methods: Between December 1989 and December 2006, 64 patients with HLHS and/or its variants underwent a Norwood procedure (mean age/weight, 11.8 ± 2.5 days/3.4 kg). Forty-four patients had classical HLHS. Twenty-eight percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 25 patients underwent a bidirectional Glenn procedure (stage II) and 11 patients a modified Fontan procedure (stage III). Others await stage II and/or stage III. The follow-up was 143.2 patient-years. Results: Including the learning curve, overall early mortality from 1989 to 1999 after the Norwood procedure was 39.06%. This decreased tremendously for the last seven years, and reduced to 12.8% in 2000 to 2003 until 0% in 2004 to 2006 (p < 0.005). The causes of mortality were sepsis, capillary leak, or heart failure. Three patients died between stages II and III. One patient underwent heart transplantation after the second stage because of heart failure. Among 34 Norwood survivors, four are slightly tachypneic from a mild pulmonary hyperperfusion; one presents symptoms of minimal brain disease. Conclusion: This report identified an outcome improvement after staged paliation of HLHS, attributed to an increase in experience and expertise gained over time. Lower operative weight, ascending aortic size, prolonged duration of cardiopulmonary bypass, and hypothermic circulatory arrest were identified to significantly influence early mortality after the Norwood procedure.     

Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome

OBJECTIVE: Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle-pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome. METHODS: Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle-pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery 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 5 patients and 5 mm in 14. RESULTS: All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle-pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%). CONCLUSION: Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle-pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.     

Simultaneous second-stage norwood operation and tracheoplasty

Tracheal stenosis is a rare complication that follows operations for congenital heart disease in the newborn period. We report the case of an infant with hypoplastic left heart syndrome who developed symptomatic tracheal stenosis 5 months after a first-stage Norwood operation. The combined surgical procedure of a bidirectional Glenn anastomosis and a tracheoplasty under cardiopulmonary bypass was undertaken.     

Risk assessment and early outcome following the Norwood procedure for hypoplastic left heart syndrome.

BACKGROUND: This study was undertaken to identify risk factors for early mortality following the Norwood procedure for hypoplastic left heart syndrome (HLHS) and develop a predictive risk model to monitor clinical performance. METHODS AND RESULTS: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure at a single institution. The early mortality was 29% (n=95). Estimated early mortality improved progressively and was 10% at the end of the series. Multivariable analysis identified that body surface area at operation, size of the ascending aorta, preoperative right ventricular function and source of pulmonary blood flow established at operation were risk factors for early mortality (P<0.05). These variables were included in a preoperative risk model. The duration of cardiopulmonary support was an independent risk factor, which was included in a separate operative risk model. The performance of the risk models was evaluated by goodness-of-fit analyses, using the Hosmer-Lemeshow test and receiver operating characteristic (ROC) curve. Both models were well calibrated across all deciles (P=0.64, P=0.77) and discriminated moderately well. The area under the ROC curve was 0.71 for Model 1 and 0.75 for Model 2. Risk adjustment broadly accounted for the variation in early mortality observed during this series. CONCLUSIONS: Patient-related and predetermined operative variables have a major influence on the early outcome following the Norwood procedure for HLHS. The identification of these risk factors allows the risk of early mortality to be calculated. This information could be applied as part of a risk-adjusted performance-monitoring system to enable early identification of meaningful changes in practice.     

Timing of reintervention influences survival and resource utilization following first-stage palliation of single ventricle heart disease

ObjectiveOutcomes after first-stage palliation of single-ventricle heart disease are influenced by many factors, including the presence of residual lesions requiring reintervention. However, there is a dearth of information regarding the optimal timing of reintervention. We assessed if earlier reintervention would be favorably associated with in-hospital outcomes among patients requiring unplanned reinterventions after the Norwood operation.MethodsThis was a single-center, retrospective review of all patients who underwent the Norwood procedure from January 1997 to November 2017 and required a predischarge unplanned surgical or transcatheter reintervention on 1 or more subcomponent areas repaired at the index operation. Outcomes of interest included in-hospital mortality or transplant, postoperative hospital length of stay, and inpatient cost. Associations between timing of reintervention and outcomes were assessed using logistic regression (mortality or transplant) or generalized linear models (postoperative hospital length of stay and cost), adjusting for baseline patient-related and procedural factors.ResultsOf 500 patients who underwent the Norwood operation, 92 (18.4%) required an unplanned reintervention. Median time to reintervention was 12 days (interquartile range, 5-35 days). There were 31 (33.7%) deaths or transplants, median postoperative hospital length of stay was 49 days (interquartile range, 32-87 days), and median cost was $328,000 (interquartile range, $204,000-$464,000). On multivariable analysis, each 5-day increase in time to reintervention increased the odds of mortality or transplant by 20% (odds ratio, 1.2; 95% confidence interval, 1.1-1.3; P = .004). Longer time to reintervention was also significantly associated with greater postoperative hospital length of stay (P < .001) and higher cost (P < .001).ConclusionsFor patients requiring predischarge unplanned reinterventions after the Norwood operation, earlier reintervention is associated with improved in-hospital transplant-free survival and resource use.     

The effect of surgical case volume on outcome after the Norwood procedure

OBJECTIVES: We analyze the effect of surgical case volume on the survival of infants with hypoplastic left heart syndrome who underwent stage I surgical palliation (the Norwood procedure). The purpose of our study was to understand more clearly the relative effects of institution and surgeon experience on patient outcome. METHODS: Using the Pediatric Health Information System database belonging to the pediatric hospital members of the Child Health Corporation of America, we identified newborn infants (< 30 days old on admission) from 1998 through 2001 admitted with the diagnosis of hypoplastic left heart syndrome. Stepwise multiple regression analysis was used to examine the association between both institutional and surgeon case volume with 28-day survival after the Norwood procedure. RESULTS: Twenty-nine hospitals and 87 surgeons performed 801 Norwood procedures during the study period. In the 4 of 29 institutions that averaged 1 or more Norwood procedures per month during the study period, survival averaged 78%. The remaining 25 institutions averaged 1 Norwood procedure every 9.6 weeks, with a survival of 59%. Data analysis revealed that higher institutional volume (P = .02) but not the number of cases performed by surgeons (P = .13) increased survival after the Norwood procedure. There was no such association with average length of stay in survivors or the time to mortality in nonsurvivors. CONCLUSION: Survival after the Norwood procedure is associated with institutional Norwood procedure volume but not with individual surgeon case volume, suggesting the need for improvements in institutional-based approaches to the care of children with hypoplastic left heart syndrome and the need for establishing regional referral centers for such high-risk procedures to improve patient survival.     

Norwood with right ventricle-to-pulmonary artery conduit is more effective than Norwood with Blalock–Taussig shunt for hypoplastic left heart syndrome: mathematic modeling of hemodynamics

Objective: The introduction of right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure for hypoplastic left heart syndrome resulted in a higher survival rate in many centers. A higher diastolic aortic pressure and a higher mean coronary perfusion pressure were suggested as the hemodynamic advantage of this source of pulmonary blood flow. The main objective of this study was the comparison of two models of Norwood physiology with different types of pulmonary blood flow sources and their hemodynamics. Method: Based on anatomic details obtained from echocardiographic assessment and angiographic studies, two three-dimensional computer models of post-Norwood physiology were developed. The finite-element method was applied for computational hemodynamic simulations. Norwood physiology with RV-PA 5-mm conduit and Blalock–Taussig shunt (BTS) 3.5-mm shunt were compared. Right ventricle work, wall stress, flow velocity, shear rate stress, energy loss and turbulence eddy dissipation were analyzed in both models. Results: The total work of the right ventricle after Norwood procedure with the 5-mm RV-PA conduit was lower in comparison to the 3.5-mm BTS while establishing an identical systemic blood flow. The Qp/Qs ratio was higher in the BTS group. Conclusions: Hemodynamic performance after Norwood with the RV-PA conduit is more effective than after Norwood with BTS. Computer simulations of complicated hemodynamics after the Norwood procedure could be helpful in establishing optimal post-Norwood physiology.     

Norwood术后心导管造影检查的应用及介入治疗

目的 探讨Norwood术后心导管造影检查结果以及介入治疗方法和疗效.方法 13例病婴均为Norwood术后而尚未行Glenn手术者,其中12例接受了介入治疗.结果 13例中6例出现新主动脉弓再狭窄,行主动脉成形术.1例左肺动脉狭窄,行左肺动脉球囊扩张术,3个月后左肺动脉置入支架.2例Sano-Shunt狭窄和1例BT-Shunt狭窄,均置入支架治疗,改善肺血流.4例合并体肺动脉侧支,其中3例采用Coil行侧支血管堵塞术,另1例于次日Glenn手术术中结扎.结论 Norwood术后血流动力学异常的发生率很高,术后造影和血流动力学的评估以及对新主动脉弓或肺动脉狭窄的介入治疗是必要的.

Abstract：

Objective To evaluate the outcome of the cardiac catheter angiograplasty for patients after Norwood procedure. Methods 13 patients, who had undergone Norwood procedure (prior to Glenn procedure)received cardiac catheterization and angiography. lnterventional therapy was performed in 12 patients as indicated. Results Angioplasty was carried out for re-coarctation in 6 of the13 patients. 1 patient with left pulmonary artery stenosis received balloon dilation and 3 months later a stent implantation. A stent was implanted to improve the pulmonary blood flow in 2 patients with Sano-Shunt stenosis and 1 patient with BT-Shunt stenosis. Transcatheter coil embolization was performed in 3 of the 4 patients with systemic to pulmonary collateral vessels. The other one was scheduled for Glenn procedure the next day. Conclusion The incidence of hemodynamic restrictions after Norwood procedure is high (12 of 13 patients). Postoperative angiography and hemodynamic assessment for diagnosis and interventional treatment for new pulmonary artery or aortic arch stenosis is necessary after Norwood procedure.     

Considerations in biventricular repair after the Norwood procedure.

OBJECTIVE: The Norwood procedure can be applicable as a first stage palliation in children who can eventually undergo a biventricular repair. Although usual management of these patients is a primary neonatal repair, in selected patients staged approach with a Norwood procedure in the neonatal period followed by a Rastelli procedure in the infancy for conversion to two-ventricle physiology has been used alternatively. METHODS: We report our experiences on two infants who underwent a previous palliation with the Norwood procedure for lesions other than hypoplastic left heart syndrome and converted to two-ventricle physiology by the use of a Rastelli-type procedure. This report examines considerations in biventricular repair after the Norwood procedure especially need for ventricular septal defect enlargement and approach to placement of the right ventricle to pulmonary artery conduit. RESULTS: Both of the infants who underwent staged approach with an initial Norwood procedure for lesions other than hypoplastic left heart syndrome survived the operations and were clinically well at mid-term follow-up. CONCLUSION: In selected patients, the staged approach is an alternative in management of malformations other than hypoplastic left heart syndrome which share the important physiologic features of aortic outlet obstruction and ductal dependency of systemic circulation. We recommend routine enlargement of ventricular septal defect and proper positioning of the conduit at the time of subsequent biventricular repair.     

Norwood Operation: Immediate vs Delayed Sternal Closure

BackgroundThe Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications.MethodsThis retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure).ResultsThe incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%).ConclusionsFor important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.     

Arch reconstruction without circulatory arrest: Scientific basis for continued use and application to patients with arch anomalies

Aortic arch hypoplasia is a common constituent of congenital heart disease. While repair of these lesions has been performed routinely during deep hypothermia and circulatory arrest, new approaches are emerging. One such approach, regional low-flow perfusion, will be described here. This technique exploits the anticipated modified Blalock-Taussig shunt as a perfusion conduit. With control of the brachiocephalic vessels and the descending thoracic aorta, circulatory support can be provided to the neonate with exposure identical to that obtained by circulatory arrest. While first applied to children undergoing the Norwood operation for hypoplastic left heart syndrome, this technique has recently been applied to children requiring complex arch surgery in the setting of biventricular repair. To date, 36 neonates requiring arch reconstruction (27 Norwood operations, 9 biventricular repairs) have been supported with regional low-flow perfusion. Thirty-day and hospital discharge survival has been 74% (20/27) for neonates undergoing Norwood operation, and 88% (8/9) for those undergoing biventricular repair. We will review the operative technique, methodologies, and clinical studies that led us to conclude that regional low-flow perfusion provides cerebral, as well as somatic, circulatory support to the neonate undergoing arch reconstruction. Copyright © 2002 by W.B. Saunders Company