Interstage Deaths Research Articles

Outcomes After Bidirectional Glenn Operation: Blalock-Taussig Shunt Versus Right Ventricle–to–Pulmonary Artery Conduit

There are distinct physiologic differences between patients with single-ventricle lesions who have undergone the Norwood procedure with a right ventricle-to-pulmonary artery conduit (NW-RVPA) compared with those patients who have undergone the Norwood operation with a Blalock-Taussig shunt (NW-BTS). We evaluated bidirectional Glenn operation outcomes and compared the two groups to assess whether the type of Norwood operation influenced outcomes. A retrospective chart review compared bidirectional Glenn operation outcomes for children undergoing the Norwood operation with NW-RVPA or NW-BTS at Children's Hospital Boston from January 1, 2002, to December 31, 2003. Of 80 patients undergoing the Norwood operation, 56 (NW-BTS, 27 versus NW-RVPA, 29) returned for the bidirectional Glenn operation at our institution. The NW-RVPA group had a lower median age at presentation for bidirectional Glenn (4.5 months versus 5.8 months; p = 0.01), but had better weight gain (20.6 g/day versus 16.5 g/day; p = 0.03) than the NW-BTS group. No interstage deaths occurred in the NW-RVPA group. There were no differences in morbidity or mortality after the BDG between the two groups. There were no differences in morbidity and mortality outcomes after the bidirectional Glenn operation between the NW-RVPA and NW-BTS groups. Despite younger age at presentation, the NW-RVPA patients had better growth rate, which may have contributed to the similar postoperative outcomes.

Fate of pulmonary arteries following Norwood Procedure

This study evaluated the requirement for surgical reoperation and catheter-based reintervention to central pulmonary arteries (CPAs) following Norwood Procedure (NP). We sought to identify the influence of various surgical techniques employed during NP on subsequent interventions. Between 1993 and 2004, 226 patients underwent Stage II following NP. Ninety-eight patients (43%) had completion of Fontan circulation (Stage III) and a further 107 (47%) are on course for Fontan completion with 21 (9%) inter-stage deaths. During NP, the aortic arch was reconstructed without additional material (n = 91, 40%) or with a pulmonary homograft patch (n = 135, 60%). Pulmonary blood flow was supplied by modified Blalock-Taussig shunt (n = 177, 78%) or right ventricle to pulmonary artery conduit (RV-PA; n = 49, 22%). The CPAs defect was closed directly (n = 69, 31%) or with a patch (n = 157, 69%). Complete resection of coarctation was performed in 126 patients (56%). Ninety-seven patients (43%) required surgical reoperation to CPAs during Stage II. Actuarial freedom from reoperation was 60+/-3%, 52+/-4% and 50+/-4% at 1, 5 and 10 years, respectively. On multivariable analysis, NP with RV-PA increased risk of reoperation (LR 8.3, 5.3-13.2; p < 0.001). Forty-one patients (18%) required catheter-based reintervention on CPAs. Actuarial freedom from reintervention was 98+/-1%, 72+/-4% and 58+/-6% at 1, 5 and 10 years, respectively. CPA problems were almost exclusively limited to the proximal Left pulmonary artery. On multivariable analysis, catheter-based reintervention became more common with time. Complete resection of coarctation increased risk of reintervention (LR 3.9, 1.6-9.6; p < 0.005). Arch reconstruction and CPAs repair techniques did not affect risk of reoperation or reintervention on CPAs. CPA stenoses and hypoplasia need surgical attention in approximately half of all patients undergoing the NP. The need for reoperation is increased when using the RV-PA conduit technique (although the majority of these are performed as part of the Stage II procedure). Catheter reinterventions are almost exclusively confined to the left CPA and are increased when the arch is shortened by resection of the coarctation tissue at time of NP.

Single-ventricle palliation for high-risk neonates: The emergence of an alternative hybrid stage I strategy

Survival after stage I palliation for hypoplastic left heart syndrome or related anomalies remains poor in high-risk neonates. We hypothesized that a less invasive hybrid approach would be beneficial in this patient population. The hybrid stage I procedure was performed in the catheterization laboratory. Via a median sternotomy, both branch pulmonary arteries were banded, and a ductal stent was delivered via a main pulmonary artery puncture and positioned under fluoroscopic guidance. Between October 2003 and June 2005, 14 high-risk neonates underwent a hybrid stage I procedure. Eleven of 14 had hypoplastic left heart syndrome. Two also underwent peratrial atrial septal stenting, and 5 required percutaneous atrial stenting later. Two neonates with an intact or highly restrictive atrial septum had emergency percutaneous atrial stent placement. Hospital survival was 11 (78.5%) of 14. One patient required extracorporeal membrane oxygenation support for intraoperative cardiac arrest. He underwent cardiac transplantation but died later of sepsis. One patient died of ductal stent embolization, and a third died of progressive cardiac dysfunction. The first 4 patients required pulmonary artery band revisions. There were none after we modified our technique and added branch pulmonary artery angiograms. There were 2 interstage deaths from atrial stent occlusion and from preductal retrograde coarctation. Eight patients underwent stage II procedures, consisting of aortic arch reconstruction, atrial septectomy, and cavopulmonary shunt. Two patients died after stage II. One patient is awaiting stage II. The hybrid stage I palliation is a valid option in high-risk neonates. As experience is accrued, it may become the preferred alternative. However, in aortic atresia, the development of preductal retrograde coarctation is a significant problem.

Lessons Learned from the Development of a New Hybrid Strategy for the Management of Hypoplastic Left Heart Syndrome

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.

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

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. 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. 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. 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.

Lessons Learned from the Development of a New Hybrid Strategy for the Management of Hypoplastic Left Heart Syndrome

Despite improvements in surgical techniques and perioperative management, the overall results of traditional staged palliation for hypoplastic left heart syndrome (HLHS) remain suboptimal. We report the evolution of a hybrid strategy in 34 patients coupling innovative surgical and transcatheter techniques that requires one open-heart procedure to reach a Fontan completion. Experience has led to modifications in technique, timing of procedures, and follow-up. Current initial palliation is by intraoperative, off-pump, placement of bilateral pulmonary artery bands and a PDA stent. Transcatheter creation of an unrestricted atrial septal defect is performed when necessary. Follow-up includes weekly cardiology assessment, including echocardiography with a surveillance catheterization performed at 6 weeks. At 3-6 months a comprehensive stage 2 procedure (elements of traditional stages 1-3) is performed. At 2 years, transcatheter Fontan completion with a covered stent is performed. Initial hybrid stage 1 was from August 2001 to December 2004 and included 29 newborns (1.8-4.2 kg). There were five hospital deaths and three interstage deaths. Procedural and interstage deaths were eliminated in the latter half of the experience. Comprehensive stage 2 included 18 patients. There were four deaths; two patients who died were brought urgently to operation. Transcatheter Fontan completion included 5 patients (not from the initial 29); there were no deaths and all returned home in 24 hours. With this strategy there is one exposure to cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest. There was a significant learning curve, which is highlighted. This initial experience warrants further investigation to determine whether this hybrid strategy will yield better short- and long-term outcomes.

Impact of right ventricle to pulmonary artery conduit on outcome of the modified norwood procedure

Background To determine and compare outcome of the modified Norwood procedure using either a systemic to pulmonary artery (SPA) shunt or right ventricle to pulmonary artery (RV-PA) conduit in a consecutive series of neonates at a single institution. Methods The medical records were retrospectively examined for preoperative demographic and echocardiographic data, operative variables, and postoperative clinical and hemodynamic data. From November 2001 to March 2003, 21 neonates had a modified Norwood procedure (SPA shunt, n = 8; RV-PA conduit, n = 13) at a median age of 5 days (range 1 to 18 days) and a median weight of 2.9 kg (range 1.7 to 4.1 kg). Of the 21 infants, 12 were considered high risk due to presence of low birth weight (n = 4), extracardiac or genetic anomalies (n = 5) or obstruction to pulmonary venous return (n = 5). Nine “high risk” infants were in the RV-PA conduit group. Results Overall Norwood operation survival was 90% (19/21) and did not differ between groups. There were 2/19 interstage deaths and Kaplan-Meier survival at 1 year is 79%. Neonates in the RV-PA conduit group had significantly higher diastolic blood pressures at 1, 6, and 24 hours postoperatively ( p < 0.05). Neonates in the SPA shunt group had significantly higher heart rates at 1 hour postoperatively ( p < 0.05) than those in the RV-PA group. There was a trend to higher number of ventilatory interventions to balance Qp:Qs in the SPA shunt group ( p = 0.06). Conclusions In a relatively high-risk group, neonates having an RV-PA conduit as part of the Norwood procedure have favorable postoperative hemodynamics and a good likelihood of stage I survival.