Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants (original) (raw)
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Long-Term Results of Bilateral Pulmonary Artery Banding Versus Primary Norwood Procedure
Pediatric Cardiology, 2017
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included. The total number of patients diagnosed with HLHS was 334. Bilateral PABs were performed for 256 patients and primary Norwood procedures for 78 patients, as an initial procedure. Actuarial 5-year survival was 59.0%. The primary Norwood procedure group had better 5-year survival [75.5%; 95% confidence interval (CI) 63.2-84.1] than the bilateral PAB group (75.5 vs. 54.0%, log-rank p \ 0.001). However, the bilateral PAB group had more significant risk factors. When the risk-adjusted outcomes were evaluated, there was no significant difference between the primary Norwood group and the bilateral PAB group (76.4 vs. 78.1%, log-rank p = 0.87) in higher volume institutions. The primary Norwood group had better 5-year survival than the bilateral PAB group, but preoperative risk was higher in the bilateral PAB group. Because outcomes are comparable when performed at higher HLHS volume institutions, proper patient selection is important in achieving good long-term result.
The Journal of Thoracic and Cardiovascular Surgery, 2010
Objectives: In patients with high-risk hypoplastic left heart syndrome (HLHS), the Norwood operation (NW) in the neonatal period still results in high mortality compared with other cardiac surgery. Bilateral pulmonary artery banding (bPAB), a very effective initial procedure for HLHS, for which the specific evaluation is as yet unsatisfactory, was performed, and we report our findings in the present study. Methods: We have performed bPAB since 2006. A total of 17 patients with HLHS or a variant underwent bPAB before the NW. Echocardiography was performed between bPAB and the NW, and the flow acceleration just after bPAB and before NW was evaluated. Before the NW, a catheter examination was also performed. Results: bPAB was performed at 6.6 AE 0.6 days of age, and the NW at 130 AE 88 days. The patients' mean body weight (BW) was 2.5 AE 0.4 kg at bPAB and 4.0 AE 1.1 kg at the NW. The length of the tape for bPAB was 9.9 AE 0.6 mm in the right pulmonary artery (RPA) and 9.4 AE 0.6 mm in the left (LPA) because the RPA was usually wider than the LPA. The tape width was 2 mm in all cases. The catheter examination was performed at 95 AE 85 days after bPAB. The arterial oxygen saturation (SaO 2) was 71% AE 8.6%. Multivariate regression analysis revealed that SaO 2 was estimated well using 4 factors: the banding size of the RPA, BW at bPAB, BW at NW, and BW in the period between bPAB and catheter examination (R 2 ¼ 0.79). Echocardiography just after bPAB showed that the blood flow at the bPAB had accelerated to 3.0 AE 0.8 m/s in the RPA and 3.3 AE 0.8 m/s in the LPA (P ¼ .004). The estimated pressure gradient was 39.2 AE 17.6 mm Hg in the RPA and 46.1 AE 23.0 mm Hg in the LPA (P ¼ .006). The blood flow at bPAB was accelerated to 3.7 AE 0.7 m/s in the RPA and 4.0 AE 0.6 m/s in the LPA before NW (P ¼ .013). The estimated pressure gradient was 62.6 AE 27.6 mm Hg in the RPA and 56.1 AE 19.6 mm Hg in the LPA before NW (P ¼ .014). The catheter examination revealed mean wedge pressures of 18.0 AE 7.2 mm Hg for the RPA and 16.2 AE 4.3 mm Hg for the LPA. The operative mortality rate was 0%. One patient required a repeat operation to adjust the bPAB, and prolonged pleural effusion was observed in 1 case. Conclusions: The postoperative SaO 2 after bPAB correlated closely with the banding size and BW at bPAB, NW and during the period after bPAB. Because the mean PA pressure before NW was low enough for single ventricular circulation, the bPAB in this study was an effective option for high-risk patients undergoing HLHS or a variant. We believe the bPAB sizes used were suitable and were determined as follows: BW plus 7 mm for the LPA and BW plus 7.5 mm for the RPA.
Expectations and limitations after bilateral pulmonary artery banding
European Journal of Cardio-Thoracic Surgery, 2016
OBJECTIVES: Bilateral pulmonary artery banding (bil-PAB) has been developed as a part of a hybrid procedure for hypoplastic left heart syndrome (HLHS). We use this procedure for palliation of complex congenital heart disease, particularly in patients with arch anomaly. We reviewed our experience with bil-PAB. METHODS: Sixty-six consecutive cases between 2003 and 2014 were reviewed retrospectively. RESULTS: Fifty-one patients (77%) had single ventricle physiology (HLHS: 37, HLHS variant: 13, others: 1). Twelve patients had biventricular physiology [5 interrupted aortic arch with ventricular septal defect (VSD); 4 coarctation of the aorta with VSD; 2 truncus arteriosus; 1 other]. In 3 patients, it was difficult to determine if the physiology was single ventricle or biventricle due to borderline left ventricle size. Age and body weight at the time of operation were 4.3 ± 3.4 days and 2.7 ± 0.5 kg, respectively. Overall hospital mortality was 11% (7/66). Of the 42 patients from 2010, only 1 with multiple anomalies died between bil-PAB and the second-stage operation. Thirteen patients (20%) required reoperation of bil-PAB. The mean waiting time for the next operation was 44.3 ± 42.8 days. The body weight at the secondstage operation was 3.2 ± 0.8 kg. Seven (11%) patients developed patent ductus arteriosus obstruction during the waiting period. Twentyfour patients were 2.5 kg or less; 20 patients in this group progressed to the second-stage operation and of these 19 gained body weight. Forty-two patients were above 2.5 kg, but of these only 23 patients gained body weight. Ten patients underwent bil-PAB because of shock and 8 of these recovered from shock. In 2 borderline cases, the patients underwent the Norwood procedure and 1 patient had arch repair and VSD closure. CONCLUSIONS: Bil-PAB could be applied to a wide variety of complex diseases. Our mortality rates with bil-PAB improved significantly post-2010. Good indications for bil-PAB were shock and situations where it was unclear whether the physiology was single ventricle or biventricular. Body weight gain was difficult to predict, but patients weighing less than 2.5 kg could be expected to gain body weight after the procedure.
European Journal of Cardio-thoracic Surgery, 2003
Objective: Although significant progress has been made in the perioperative management of neonates with hypoplastic left heart syndrome (HLHS), early survival has plateaued. Moreover, low but important interstage mortality remains unsolved. With a systemic to pulmonary artery shunt, the combination of significant diastolic runoff into the pulmonary circulation, a large volume load on the single ventricle and precarious coronary perfusion result in a delicate physiologic state. In order to minimize these detrimental features, a right ventricle to pulmonary artery (RV to PA) conduit was used as the source of pulmonary blood flow in patients undergoing Stage I Norwood for HLHS. Methods: Prospective data collection in 15 consecutive patients who underwent Stage I Norwood with an RV to PA conduit. Results: Mean age at surgery was 2.5^2 days (range 1 -8), mean weight was 2.9^0.3 kg (range 2.2-3.6) and mean gestational age was 37 weeks (range 35 -40). Anatomic diagnosis was HLHS in all patients, aortic atresia was present in ten. Mean ascending aortic size was 2.9^0.9 mm (range 1.5 -5). Two patients had moderate atrioventricular valve regurgitation and a genetic syndrome and/or congenital anomaly was present in five patients. Thirteen patients received a 5-mm polytetrafluoroethylene RV to PA conduit, and a 4-mm conduit was used in two. Mean circulatory arrest time was 55^6 min. Postoperatively, mean diastolic blood pressure at 1, 8 and 24 h were 47^7, 46^3 and 43^6 mmHg, respectively. Median time to extubation was 23 h (range 9 -96) and was less than 24 h in ten patients. Median intensive care unit and hospital stay were 5 days (range 2 -19) and 10 days (6-22), respectively. Early mortality was 1/15 (6%). At a mean follow-up of 10.8^3.4 months, 12 patients underwent stage II, and three patient have completed the Fontan. Conclusion: RV to PA conduit eliminated diastolic runoff into the pulmonary vascular bed resulting in a higher diastolic blood pressure. This physiology appears to be associated with a more stable postoperative course and improved hospital survival. q
Circulation, 2002
Background-Outcome of patients with hypoplastic left heart (HLH) is mainly influenced by the successful first-step palliation according to the Norwood procedure. An alternative approach is heart transplantation (HTX). The feasibility of ductal stenting in newborns with duct-dependent systemic blood flow and bilateral pulmonary artery banding has been reported. But it remains to be elucidated whether this approach allows a new strategy for patients with HLH. Methods and Results-In patients with various forms of HLH (nϭ11) and prostaglandin E-1 administration, ductal stenting was performed with balloon expandable Jo stents or Saxx stents. Bilateral pulmonary artery banding was surgically accomplished 1 to 3 days after the transcatheter procedure. Unrestricted blood flow through the interatrial septum was secured by balloon dilatation atrial septotomy, as required. Interventional procedures were performed with no mortality. Stent and ductal patency were achieved for up to 331 days. Two patients underwent HTX, and 8 patients had a palliative 1-stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5 to 6 months. There were 2 deaths. One patient with preoperative right heart failure died after the reconstructive surgery, and 1 patient died 4 months after ductal stenting and bilateral banding awaiting HTX. Conclusions-The present study is the first clinical trial showing that stenting the duct followed by bilateral pulmonary artery banding in newborns with HLH allows the combination of neoaortic reconstruction, which is part of first-stage palliation of HLH, with the establishment of a bidirectional cavopulmonary connection. Additionally, it allows the chance for HTX after extended waiting periods.
The Annals of Thoracic Surgery, 2009
Background. The purpose of this study was to describe the experience with staged surgical reconstruction of the hypoplastic left heart syndrome (HLHS) with a right ventricle to pulmonary artery conduit and to identify the risk factors that influence late outcome. Methods. Between February 1998 and June 2007, 62 patients with HLHS underwent a Norwood procedure by using right ventricle to pulmonary artery conduit (median age, 9 days [range, 1 to 57]; median body weight 2.7 kg [range, 1.6 to 3.9 kg]). The subsequent 47 patients underwent a bidirectional Glenn procedure (stage 2). Thirty-two patients underwent a modified Fontan procedure (stage 3). Follow-up was complete (median, 32 months; range, 1 to 101). Results. Hospital mortality after the Norwood procedure was 8% (5 of 62 patients). Between stages, 9 patients died, 3 before stage 2 and 6 before stage 3. There was 1 late death after stage 3. Overall survival was 76% (47 of 62). The estimated 1-year and and 5-year survival rates were 80% and 73%, respectively. Using the any-mortality as the endpoint, prematurity (gestational age <37 weeks), body weight less than 2.5 kg at stage 1 operation, and tricuspid regurgitation 2؉ or more were associated with mortality. Using Cox regression analysis, body weight less than 2.5 kg and tricuspid regurgitation 2؉ or more were two independent factors associated with midterm survival. Conclusions. From 9 years of experience, despite good early survival after Norwood stage 1 palliation, low body weight and tricuspid valve regurgitation were still associated with worse outcome. More efforts should be made to improve the late results for patients with hypoplastic left heart syndrome.
Pulmonary Artery Banding Before the Damus–Kaye–Stansel Procedure
Pediatric Cardiology, 2006
Subaortic stenosis (SAS) in a single ventricle leads to myocardial hypertrophy and compromises Fontan results. Moreover, controversy exists concerning the optimal surgical strategy for relieving SAS. We have applied pulmonary artery banding (PAB) before the Damus-Kaye-Stansel procedure (DKS), and here we analyze factors that influence systemic ventricular compliance. Thirteen patients underwent PAB before DKS. Median PAB duration was 5.5 months (range, 20 days to 17.7 months). Procedures administered concomitantly with DKS were Blalock-Taussig shunt (n = 6), bidirectional cavopulmonary shunt (n = 5), and Fontan operation (n = 2). All survived and were doing well after a median follow-up 2.7 years. Cardiac catheterization before DKS showed that the mean pressure gradient across the systemic ventricular outflow tract and PAB were 20.6 ± 10.1 and 67.4 ± 10.2 mmHg, respectively. After DKS, systemic ventricular end diastolic pressure (SVEDP) was significantly correlated with PAB duration (r = 0.65, p = 0.022), but not with PAB or systemic ventricle outflow tract pressure gradients. After DKS, SVEDP decreased or fell to within the range in patients with PAB duration less than 7 months (p < 0.05). Seven patients had a successful Fontan operation, and 6 without risk factors are waiting operation. SVEDP was found to be correlated with PAB duration, and our findings indicate that short-term PAB can be considered a safe option in patients with a single ventricle and SAS.
Journal of Cardiac Surgery, 2013
Objective: Patients with double inlet left ventricle (DILV)/transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. The aim of this study is to evaluate the outcomes of initial pulmonary artery banding (PAB) compared with the Norwood-type reconstruction in neonates. Methods: A retrospective study of children with DILV and similar morphologies presenting between 1982 and 2012. The outcomes of initial PAB (n = 17) are compared with a Norwood-type reconstruction (n = 20). The two groups were similar with respect to age, gender, weight, noncardiac syndromes, and Fontan completion. Mean follow-up was longer in PAB patients (13.3 W 9.8 years) versus Norwood (4.5 W 3.0 years, p = 0.001). Results: Survival was 75% at eight years in the Norwood group versus 71% in the PAB group (p = 0.76). Mortality in the Norwood group was higher before 2002 (p = 0.06). The age of patients who underwent a bidirectional Glenn shunt was significantly higher for PAB group (PAB, 9.1 W 1.4 months vs. Norwood, 6.1 W 1.6 months; p < 0.001). Freedom from any type of reintervention (systemic outflow obstruction or coarctation) was similar (Norwood, 83% vs. PAB, 71%; p = 0.62). Freedom from heart block with a pacemaker insertion was significantly better for Norwood patients (Norwood, 89% vs. PAB, 41%; p = 0.001). Conclusions: The Norwood-type reconstruction provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining an unobstructed systemic outflow tract and avoiding the risk of heart block. Survival does not differ depending on the type of procedure. Patients with PAB show comparable satisfactory early and longterm results, with an increasing reoperation risk and heart block remaining a major concern.