Early postoperative outcomes in a series of infants with hypoplastic left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle to pulmonary artery conduit* (original) (raw)
Related papers
2010
Objective: Although the outcome of cardiac surgery in neonates with low birth weight (LBW) has improved, LBW remains a risk factor for surgical palliation. Few surgical series of LBW patients include those with hypoplastic left heart syndrome (HLHS). To identify variables associated with poor outcome in this group, we reviewed our experience with patients with HLHS and LBW who underwent Stage I Norwood palliation. Methods: Between January 1998 and December 2000, 20 consecutive LBW (,2500 g) neonates with HLHS ðn ¼ 13Þ or HLHS variant ðn ¼ 7Þ underwent surgical palliation. Retrospective review of all patient data and analysis to identify risk factors was performed. Results: Mean age at surgery was 5.1^4.6 days (range 1-17), mean weight was 1.98^0.44 kg (range 1.1-2.5), including nine patients under 2 kg. Ten patients were born at ,35 weeks gestation. Anatomic diagnosis included HLHS in 13 patients (10 with aortic atresia), unbalanced atrioventricular canal defect in two, double outlet right ventricle in two and other variants in three. Mean ascending aortic size was 4.0^1.8 mm (range 1.5-8). Associated cardiac defects were present in three patients, and a genetic syndrome and/or congenital anomaly was present in four of them. Mean circulatory arrest time was 60^10 min. Extracorporeal support was used perioperatively in 10 patients. Early mortality was 9/20 (45%). At a mean follow up at 22^10 months (range 8-38), six patients underwent stage II, and are awaiting stage III; four patients have completed their Fontan. Anatomic variant, ascending aortic size, prematurity, age at surgery, weight, duration of circulatory arrest, cardiopulmonary bypass time and associated non-cardiac anomalies were not risk factors for poor outcome whereas restrictive pulmonary venous drainage and coronary artery anomalies were associated with decreased survival. Conclusion: LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation. q
European Journal of Cardio-thoracic Surgery, 2002
Objective: Although the outcome of cardiac surgery in neonates with low birth weight (LBW) has improved, LBW remains a risk factor for surgical palliation. Few surgical series of LBW patients include those with hypoplastic left heart syndrome (HLHS). To identify variables associated with poor outcome in this group, we reviewed our experience with patients with HLHS and LBW who underwent Stage I Norwood palliation. Methods: Between January 1998 and December 2000, 20 consecutive LBW (,2500 g) neonates with HLHS ðn ¼ 13Þ or HLHS variant ðn ¼ 7Þ underwent surgical palliation. Retrospective review of all patient data and analysis to identify risk factors was performed. Results: Mean age at surgery was 5.1^4.6 days (range 1-17), mean weight was 1.98^0.44 kg (range 1.1-2.5), including nine patients under 2 kg. Ten patients were born at ,35 weeks gestation. Anatomic diagnosis included HLHS in 13 patients (10 with aortic atresia), unbalanced atrioventricular canal defect in two, double outlet right ventricle in two and other variants in three. Mean ascending aortic size was 4.0^1.8 mm (range 1.5-8). Associated cardiac defects were present in three patients, and a genetic syndrome and/or congenital anomaly was present in four of them. Mean circulatory arrest time was 60^10 min. Extracorporeal support was used perioperatively in 10 patients. Early mortality was 9/20 (45%). At a mean follow up at 22^10 months (range 8-38), six patients underwent stage II, and are awaiting stage III; four patients have completed their Fontan. Anatomic variant, ascending aortic size, prematurity, age at surgery, weight, duration of circulatory arrest, cardiopulmonary bypass time and associated non-cardiac anomalies were not risk factors for poor outcome whereas restrictive pulmonary venous drainage and coronary artery anomalies were associated with decreased survival. Conclusion: LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation. q
The Annals of Thoracic Surgery, 2004
Background. We report the development and implementation of a program designed to assign patients preoperatively to either transplant or Norwood procedure based on a score derived from known risk factors and to enhance postoperative care of infants undergoing the Norwood procedure. 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.
Variability in the Preoperative Management of Infants with Hypoplastic Left Heart Syndrome
Pediatric Cardiology, 2008
Infants with hypoplastic left heart syndrome (HLHS) commonly undergo initial surgical palliation during the first week of life. Few data exist on optimal preoperative management strategies; therefore, the management of these infants prior to surgery is anecdotal and variable. To more fully define this variability in preoperative care of infants with HLHS, a survey was designed to describe current preoperative management practices in the infant with HLHS. The questionnaire explored management styles as well as preoperative monitoring techniques and characteristics of the respondent's health care institution. The responses were compiled and are reported. A striking lack of consistency in preoperative management techniques for infants with HLHS is apparent. The impact of these preoperative strategies is unknown. Despite challenges in anatomic and hemodynamic variability at presentation, a prospective randomized controlled trial comparing ventilatory management techniques, enteral feeding strategies, and the utility of various monitoring tools on short-and long-term outcome is needed.
The American Journal of Cardiology, 2015
Newborns with hypoplastic left heart syndrome (HLHS) and other single right ventricular variants require substantial healthcare resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations, but has not been studied in single ventricle patients. Single Ventricle Reconstruction trial subjects were classified by whether or not they had a weekend admission and by day of the week of Norwood procedure. The primary outcome was hospital length of stay (LOS); secondary outcomes included transplant-free survival, intensive care unit (ICU) LOS and days of mechanical ventilation. Student's t-test with log transformation and the Wilcoxon rank sum test were used to analyze associations. Admission day was categorized for 533/549 subjects (13% weekend). The day of the Norwood was Thursday/ Friday in 39%. There was no difference in median hospital LOS, transplant-free survival, ICU LOS or days ventilated for weekend vs. non-weekend admissions. Day of Norwood procedure was not associated with a difference in hospital LOS, transplant-free survival, ICU LOS or days ventilated. Prenatally diagnosed infants born on the weekend had lower mean birth weight, younger gestational age, and were more likely to be intubated but did not have a difference in measured outcomes. In conclusion, in this cohort of single right ventricle patients, neither weekend admission, nor end-of-the-week Norwood procedure was associated with increased use of hospital resources or poorer outcomes. We speculate that the complex postoperative course following the Norwood procedure outweighs any impact that day of admission or operation may have on these outcomes.
The Journal of Thoracic and Cardiovascular Surgery, 2015
Objectives: The hybrid approach for the initial management of hypoplastic left heart syndrome shifts the risks of major open surgery from the vulnerable neonatal period to an older age. This study determined differences between the hybrid and the standard Norwood procedures in postoperative in-hospital mortality, renal failure, and survival to at least 2 years of age. Methods: Data from the Pediatric Health Information System, a detailed hospital discharge database of 43 freestanding children's hospitals, were analyzed. The Pediatric Health Information System includes demographic information, diagnosis, and procedure and clinical service data. Instrumental variable regression techniques were used to estimate the predicted probability of in-hospital mortality, renal failure, and survival to 24 months of age for infants with hypoplastic left heart syndrome who received a hybrid or Norwood procedure. The statistical models controlled for demographics and comorbid chromosomal anomalies. Results: A total of 3654 infants with hypoplastic left heart syndrome underwent intervention from 1998 to 2012. Of these, 242 underwent the hybrid approach and the remainder underwent the Norwood procedure. Instrumental variable models showed significantly reduced odds of patients who underwent the hybrid approach being diagnosed with renal failure (adjusted risk ratio [ARR], 0.48; 95% confidence interval [CI], 0.26-0.89); increased odds of surviving initial hospitalization (ARR, 1.28; 95% CI, 1.06-1.55); increased odds of survival, indicated by readmissions more than 6 months after initial hospitalization (ARR, 1.53; 95% CI, 1.05-2.22); and a decrease in length of stay by 20 days for the initial surgical hospitalization (95% CI, À27.4 to À13.9). Conclusions: The short term hospital-based outcomes and longer-term survival outcomes of the hybrid approach for hypoplastic left heart syndrome may be better than those of the Norwood procedure.
The Journal of Thoracic and Cardiovascular Surgery, 2015
Objective: Hybrid palliation for hypoplastic left heart syndrome has been developed as an alternative to neonatal Norwood surgery. At the second stage, a source of pulmonary blood flow has to be established. Options include an arterial modified Blalock-Taussig or a venous superior cavopulmonary shunt. Methods: We retrospectively reviewed patients who received second-stage palliation after the initial hybrid. Patients were stratified according to the source of pulmonary blood supply into the arterial shunt (n ¼ 17 patients) or venous shunt (n ¼ 26 patients). Results: Age and weight at second stage were lower in the arterial group (85 [45-268] days vs 152.5 [61-496] days, P ¼ .001 and 3.6 [2.7-9.4] kg vs 5.1 [2.97-9.4] kg, P ¼ .001, respectively). All recorded surgical times were shorter in the arterial group. Mechanical ventilation and intensive care stay were shorter in the venous group (5.
Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants
The Journal of Thoracic and Cardiovascular Surgery, 2013
Objectives: Preoperative comorbidities (PCMs) are known risk factors for Norwood stage I (NW1). We tested the hypothesis that short-term bilateral pulmonary arterial banding (bPAB) before NW1 could improve the prognosis of these high-risk patients. Methods: From January 2006 to October 2011, 17 high-risk patients with hypoplastic left heart syndrome (defined as having !4 of the following PCMs: prolonged mechanical ventilation; older age; sepsis; necrotizing enterocolitis; hepatic, renal, or heart failure; coagulopathy; pulmonary edema; high inotropic requirements; anasarca; weight <2.5 kg; and cardiac arrest) were identified. In addition to conventional treatment of PCMs, they underwent bPAB before NW1. bPAB was undertaken with Silastic slings and secured with ligaclips to a luminal diameter of approximately 3.5 to 4.0 mm. The patency of the ductus arteriosus was maintained with prostaglandin. NW1 was performed using a modified, right Blalock-Taussig shunt at a median interval of 8 days after bPAB. The data from these patients were retrospectively reviewed, and the 30-day mortality and 1-year survival were compared with the hypoplastic left heart syndrome population who underwent primary NW1 with <3 PCMs in the same period. Results: Of the bPAB patients, 5 (29.4%) died before NW1. All had !5 PCMs. Twelve patients (70.6%) survived to undergo NW1. One early death occurred after NW1 (8.3%). The 1-year survival rate for high-risk patients who underwent NW1 was 66.7%. The early mortality and 1-year survival for the 130 patients with <3 PCMs was 10% and 80%, respectively. Conclusions: Optimizing the balance between the pulmonary and systemic blood flow with a short period of bPAB and ductal patency can improve the perioperative conditions of high-risk patients before NW1. Those who survived bPAB and underwent NW1 had early mortality and 1-year survival comparable to the standard risk category, despite the severity of their initial condition. A rapid 2-stage NW1 strategy with bPAB and prostaglandin to maintain ductal patency can avoid the risks of suboptimal palliation and vascular injuries associated with hybrid procedures.
The Journal of Thoracic and Cardiovascular Surgery, 2006
Supplemental material is available online. Objective: This study was undertaken to determine the impact of specific intensive care procedures on preoperative hemodynamics, incidence of preoperative organ dysfunction, and in-hospital mortality among neonates with hypoplastic left heart syndrome with pulmonary overcirculation and to assess the influence of the change in preoperative management on early postoperative outcome. Methods: In this retrospective evaluation of 72 neonates with classic hypoplastic left heart syndrome and severe pulmonary overcirculation with different preoperative management strategies from 1992 to 1995 and from 1996 to 2000, univariate and multivariate analyses of risk factors were performed with stepwise logistic regression. Results: Among patients with ventilatory and inotropic support from admission until surgery, degree of metabolic acidosis (lowest recorded and prerepair pH values) was significantly higher than among patients who received systemic vasodilators without ventilation before surgery. Preoperative organ dysfunction occurred in 19 of 72 patients (26%), predominantly before 1996; the most significant was hepatic failure in 13 (68%). Lowest recorded and prerepair pH values did not predict the development of organ dysfunction, whereas inotropic medication, lack of afterload reduction, and especially ventilatory support correlated significantly with organ injury. In-hospital mortality decreased from 65% (13/20) to 13% (6/46) from the first to the second period. According to multivariate analysis, ventilatory support and organ dysfunction were significantly related to in-hospital mortality. Conclusion: In neonates with hypoplastic left heart syndrome, systemic afterload reduction can avoid preoperative artificial respiration, identified as a significant risk factor for the development of preoperative dysfunction of end organs and in-hospital mortality.