In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a ...modified Blalock-Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups.
Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial.
Transplant-free survival for the RVPAS versus modified Blalock-Taussig shunt groups did not differ at 6 years (64% versus 59%,
=0.25) or with all available follow-up of 7.1±1.6 years (log-rank
=0.13). The RVPAS versus modified Blalock-Taussig shunt treatment effect had nonproportional hazards (
=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48-0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86-2.17;
=0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33-1.74;
=0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year,
<0.001), primarily because of more interventions between the stage II and Fontan procedures (HR, 1.72; 95% confidence interval, 1.00-3.03). Complications did not differ by shunt type; by 6 years, 1 in 5 patients had had a thrombotic event, and 1 in 6 had had seizures.
By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock-Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications.
URL: https://www.clinicaltrials.gov. Unique identifier: NCT00115934.
Objective Despite increasing use of bilateral branch pulmonary artery banding (bPAB), both as a temporary stabilizing treatment and as part of comprehensive hybrid management of hypoplastic left ...heart syndrome, little is known about the long-term outcomes of the pulmonary arteries (PAs) in banded patients. Patients and Methods We conducted a retrospective review of all patients with ductal-dependent systemic circulation (2001-2013) undergoing bPAB placement at a single institution (bPAB, n = 50); patients who underwent a stage I Norwood procedure (Norwood, n = 53) were used for comparison. The need for PA interventions (surgical arterioplasty, balloon angioplasty, and stent implantation) and PA growth were assessed. Results Bands were in place for a median of 76 days. PA growth and size were similar between groups, but bPAB patients required more interventions (1.4 ± 2.9 vs 0.5 ± 1.2, P = .01). In competing risks analysis, only 20% of bPAB patients were alive and free from intervention at 5 years after bPAB removal. Multivariable Cox proportional hazards regression of operative interventions within the bPAB group demonstrated the following risk factors: subsequent 2-ventricle repairs (hazard ratio HR, 2.2; 95% confidence interval CI, 0.7-6.7), smallest band diameter (HR per additional millimeter, 0.059; 95% CI, 0.004-0.849), and duration of band placement more than 90 days (HR, 3.5; 95% CI, 1.0-12.6). Hemodynamics and Fontan candidacy did not differ between groups. Conclusions Patients with bPAB require additional interventions at earlier time points than Norwood patients. Patients with smaller bands and longer duration of banding are at high risk. Despite stenoses requiring additional interventions, Fontan candidacy is maintained.
We evaluated outcomes for common operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSDB) to provide contemporary benchmarks and examine variation between centers.
...Patients undergoing surgery from 2005 to 2009 were included. Centers with greater than 10% missing data were excluded. Discharge mortality and postoperative length of stay (PLOS) among patients discharged alive were calculated for 8 benchmark operations of varying complexity. Power for analyzing between-center variation in outcome was determined for each operation. Variation was evaluated using funnel plots and Bayesian hierarchical modeling.
Eighteen thousand three hundred seventy-five index operations at 74 centers were included in the analysis of 8 benchmark operations. Overall discharge mortality was: ventricular septal defect (VSD) repair = 0.6% (range, 0% to 5.1%), tetralogy of Fallot (TOF) repair = 1.1% (range, 0% to 16.7%), complete atrioventricular canal repair (AVC) = 2.2% (range, 0% to 20%), arterial switch operation (ASO) = 2.9% (range, 0% to 50%), ASO + VSD = 7.0% (range, 0% to 100%), Fontan operation = 1.3% (range, 0% to 9.1%), truncus arteriosus repair = 10.9% (0% to 100%), and Norwood procedure = 19.3% (range, 0% to 100%). Funnel plots revealed that the number of centers characterized as outliers were VSD = 0, TOF = 0, AVC = 1, ASO = 3, ASO + VSD = 1, Fontan operation = 0, truncus arteriosus repair = 4, and Norwood procedure = 11. Power calculations showed that statistically meaningful comparisons of mortality rates between centers could be made only for the Norwood procedure, for which the Bayesian-estimated range (95% probability interval) after risk-adjustment was 7.0% (3.7% to 10.3%) to 41.6% (30.6% to 57.2%). Between-center variation in PLOS was analyzed for all operations and was larger for more complex operations.
This analysis documents contemporary benchmarks for common pediatric cardiac surgical operations and the range of outcomes among centers. Variation was most prominent for the more complex operations. These data may aid in quality assessment and quality improvement initiatives.
The use of the Berlin Heart EXCOR in patients with functional single ventricle Weinstein, Samuel, MD, MBA; Bello, Ricardo, MD, PhD; Pizarro, Christian, MD ...
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
02/2014, Volume:
147, Issue:
2
Journal Article
Peer reviewed
Open access
Introduction The frequency and successful use of pediatric ventricular assist devices (VADs) as a bridge to cardiac transplantation have been steadily increasing since 2003, but the experience in ...patients with complex congenital heart disease has not been well described. Using a large prospectively collected dataset of children supported with the Berlin Heart EXCOR VAD, we have reviewed the experience in children with single ventricular anatomy or physiology (SV), and compared the results with those supported with biventricular circulation (BV) over the same time period. Methods The EXCOR Investigational Device Exemption study database was retrospectively reviewed. VAD implants under the primary cohort and compassionate use cohort between May 2007 and December 2011 were included in this review. Results Twenty-six of 281 patients supported with a VAD were SV. The most common diagnosis was hypoplastic left heart syndrome (15 of 26). Nine patients were supported after neonatal palliative surgery (Blalock-Taussig shunt or Sano), 12 after a superior cavopulmonary connection (SCPC), and 5 after total cavopulmonary connection (TCPC). Two patients received biventricular assist devices, 1 after stage I surgery and 1 after stage II. SV patients were supported for a median time of 10.5 days (range, 1-363 days) versus 39 days (range, 0-435 days) for BV ( P = .01). The ability to be bridged to transplant or recovery in SV patients is lower than for BV patients (11 of 26 42.3% vs 185 of 255 72.5%; P = .001). Three of 5 patients with TCPC were successfully bridged to transplant and were supported with 1 VAD. Seven of 12 patients with SCPC were bridged to transplant, and only 1 of 9 patients supported after a stage I procedure survived. Conclusions The EXCOR Pediatric VAD can provide a bridge to transplant for children with SV anatomy or physiology, albeit less successfully than in children with BV. In this small series, results are better in patients with SCPC and TCPC. VAD support for patients with shunted sources of pulmonary blood flow should be applied with caution.
Keeping the fundamentals in mind Pizarro, Christian, MD
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
04/2015, Volume:
149, Issue:
4
Journal Article
Commentary: Aortic valve intervention in children: The jury is still out Pizarro, Christian
Journal of thoracic and cardiovascular surgery/The Journal of thoracic and cardiovascular surgery/The journal of thoracic and cardiovascular surgery,
March 2022, 2022-03-00, 20220301, Volume:
163, Issue:
3
Journal Article
Neurodevelopmental disability is common after operations for congenital heart defects. We previously showed that patient and preoperative factors, center, and calendar year of birth explained less ...than 30% of the variance for the Psychomotor Development Index (PDI) and the Mental Development Index (MDI) of the Bayley Scales of Infant Development-Second Edition. Here we investigate how much additional variance in PDI and MDI is contributed by operative variables and postoperative events.
We analyzed neurodevelopmental outcomes after operations with cardiopulmonary bypass at age 9 months or younger between 1996 and 2009. We used linear regression to investigate the effect of operative factors (age, weight, and cardiopulmonary bypass variables) and postoperative events on neurodevelopmental outcomes, adjusting for center, type of congenital heart defect, year of birth, and preoperative factors.
We analyzed 1,770 children from 22 institutions with neurodevelopmental testing at age 13.3 months (range, 6 to 30 months). Among operative factors, longer total support time was associated with lower PDI and MDI (p < 0.05). When postoperative events were added, use of either extracorporeal membrane oxygenation or ventricular assist device support, and longer postoperative length of stay were associated with lower PDI and MDI (p < 0.05). Longer total support time was not a significant predictor in these models. After adjusting for patient, preoperative, intraoperative, and postoperative factors, measured intraoperative and postoperative factors accounted for 5% of the variances in PDI and MDI.
Operative factors may be less important than innate patient and preoperative factors and postoperative events in predicting early neurodevelopmental outcomes after cardiac operations in infants. Neurodevelopmental outcomes improved over calendar time when adjusted for patient and medical variables.
Objective The study objective was to expand on prior research examining intraoperative regional cerebral oxygen saturation (rSO2 ) measured by near-infrared spectroscopy (NIRS) by evaluating the ...predictive value of perioperative NIRS monitoring for neurodevelopmental outcomes after infant cardiac surgery. Methods Cross-sectional neurodevelopmental evaluation at 24 months of age with the Bayley Scales of Infant and Toddler Development, Third Edition was performed for patients who underwent cardiac surgery with perioperative NIRS monitoring between 2007 and 2010. Retrospective clinical data were extracted from the electronic medical record. Evaluation of selected NIRS measures, including preoperative rSO2 (baseline) as well as rSO2 nadir and percent decrease from baseline during the intraoperative and early postoperative periods, was undertaken. Results Perioperative NIRS and neurodevelopmental data were available for 31 patients without chromosomal anomalies who underwent cardiac surgery during infancy at a median age of 0.43 months. Optimal thresholds on NIRS measures identified through receiver operating characteristic analyses were intraoperative percent decrease of 52% for receptive communication delay and postoperative rSO2 nadir of 56% for cognitive delay and 49% for gross motor delay. When considered in conjunction with other clinical characteristics in stepwise linear regression analyses, intraoperative percent decrease of more than 52% entered into the final model for receptive communication outcome and postoperative rSO2 nadir of less than 56% entered into the final model for cognitive outcome. Conclusions Perioperative NIRS monitoring seems to enhance the ability to predict neurodevelopmental outcome. Specific NIRS measures associated with neurodevelopmental outcome, as well as optimal thresholds, seem to differ across the continuum of the perioperative period.
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