Practice trends over time in the care of infants with hypoplastic left heart syndrome: A report from the National Pediatric Cardiology Quality Improvement Collaborative

2017 ◽  
Vol 12 (3) ◽  
pp. 315-321 ◽  
Author(s):  
Waldemar F. Carlo ◽  
James F. Cnota ◽  
Robert J. Dabal ◽  
Jeffrey B. Anderson
2016 ◽  
Vol 27 (4) ◽  
pp. 731-738 ◽  
Author(s):  
Preeti Ramachandran ◽  
Eileen King ◽  
Ashley Nebbia ◽  
Robert H. Beekman ◽  
Jeffrey B. Anderson

AbstractPurposePatients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry.MethodsWe carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites. Antithrombotic medications used at Stage I and Stage II discharge were evaluated. Variability of antithrombotics use at the individual patient level and intersite variability, incidence of shunt thrombosis, and other adverse events such as cardiac arrest, seizure, stroke, and need for cardiac catheterisation intervention in the interstage period were identified. Antithrombotic strategies for hybrid Stage I patients were evaluated but they were excluded from the variability and outcomes analysis.ResultsA total of 932 Stage I and 923 Stage II patients were included in the study: 93.8% of Stage I patients were discharged on aspirin and 4% were discharged on no antithrombotics, and 77% of Stage II patients were discharged on aspirin and 17.5% were discharged on no antithrombotics. Only three patients (0.2%) presented with interstage shunt thrombosis. The majority of patients who died during interstage or required shunt dilation and/or stenting were discharged home on aspirin.ConclusionAspirin is the most commonly used antithrombotic following Stage I and Stage II palliation. There is more variability in the choice of antithrombotics following Stage II compared with Stage I. The incidence of interstage shunt thrombosis and associated adverse events was rare.


2013 ◽  
Vol 24 (2) ◽  
pp. 253-262 ◽  
Author(s):  
Russell R. Cross ◽  
Ashraf S. Harahsheh ◽  
Robert McCarter ◽  
Gerard R. Martin ◽  

AbstractIntroductionDespite improvements in care following Stage 1 palliation, interstage mortality remains substantial. The National Pediatric Cardiology-Quality Improvement Collaborative captures clinical process and outcome data on infants discharged into the interstage period after Stage 1. We sought to identify risk factors for interstage mortality using these data.Materials and methodsPatients who reached Stage 2 palliation or died in the interstage were included. The analysis was considered exploratory and hypothesis generating. Kaplan–Meier survival analysis was used to screen for univariate predictors, and Cox multiple regression modelling was used to identify potential independent risk factors.ResultsData on 247 patients who met the criteria between June, 2008 and June, 2011 were collected from 33 surgical centres. There were 23 interstage mortalities (9%). The identified independent risk factors of interstage mortality with associated relative risk were: hypoplastic left heart syndrome with aortic stenosis and mitral atresia (relative risk = 13), anti-seizure medications at discharge (relative risk = 12.5), earlier gestational age (relative risk = 11.1), nasogastric or nasojejunal feeding (relative risk = 5.5), unscheduled readmissions (relative risk = 5.3), hypoplastic left heart syndrome with aortic atresia and mitral stenosis (relative risk = 5.2), fewer clinic visits with primary cardiologist identified (relative risk = 3.1), and fewer post-operative vasoactive medications (relative risk = 2.2).ConclusionInterstage mortality remains substantial, and there are multiple potential risk factors. Future efforts should focus on further exploration of each risk factor, with potential integration of the factors into surveillance schemes and clinical practice strategies.


2004 ◽  
Vol 14 (S1) ◽  
pp. 27-33 ◽  
Author(s):  
Richard Martinez

Hypoplastic left heart syndrome has provided the pediatric cardiology community one of its largest challenges. Until the mid-1980s, the mortality for this lesion was close to 100%. Through the pioneering work of Bill Norwood,1 Len Bailey,2 and the continued work of many pediatric cardiologists and surgeons, the current five-year survival for staged Norwood palliation and cardiac transplantation is now approximately 75%.3,4


2019 ◽  
Vol 29 (4) ◽  
pp. 481-487
Author(s):  
Pilar Anton-Martin ◽  
Rhucha Joshi ◽  
Mounica Rao ◽  
Sindhu Pandurangi ◽  
Chasity Wellnitz ◽  
...  

AbstractPurpose:(1) To characterise changes in dead space fraction during the first 120 post-operative hours in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome, including hybrid procedure; (2) to document whether dead space fraction varied by shunt type (Blalock–Taussig shunt and Sano) and hybrid procedure; and (3) to determine the association between dead space fraction and outcomes.Methods:Retrospective chart review in neonates undergoing stage 1 palliation for hypoplastic left heart syndrome in a cardiac intensive care unit over a consecutive 30-month period. A linear mixed model was used to determine the differences in dead space over time. Multivariable linear regression and a multivariable linear mixed model were used to assess the association between dead space and outcomes at different time points and over time, respectively.Results:Thirty-four neonates received either a Blalock–Taussig shunt (20.5%), Sano shunt (59%), or hybrid procedure (20.5%). Hospital mortality was 8.8%. Dead space fractions in patients undergoing the hybrid procedure were significantly lower on day 1 (p = 0.01) and day 2 (p = 0.02) and increased over time. A dead space fraction >0.6 on post-operative days 3–5 was significantly associated with decreased duration of mechanical ventilation in all surgical groups (p < 0.001).Conclusions:Dead space fraction >0.6 on post-operative days 3–5 was associated with lower duration of mechanical ventilation in all surgical groups. A more comprehensive, prospective assessment of dead space in this delicate patient population would likely be beneficial in improving outcomes.


2021 ◽  
pp. 107484072098730
Author(s):  
Kathleen A. Mussatto ◽  
Maria I. Van Rompay ◽  
Felicia L. Trachtenberg ◽  
Victoria Pemberton ◽  
Lisa Young-Borkowski ◽  
...  

Survival for hypoplastic left heart syndrome (HLHS) has improved dramatically. Little is known about early family function, quality of life (QOL), or well-being/adjustment for parents of infants with HLHS. Parent/family outcomes over time, predictors, and differences in 143 mothers and 72 fathers were examined. Parents reported better family function compared with published norms, but 26% experienced family dysfunction. QOL and well-being were significantly lower than adult norms. QOL scores generally declined over time, whereas self-reported well-being improved. Responses from mothers and fathers showed different trends, with mothers having worse scores on most measures and at most time points. Being a single parent was a risk factor for poorer family function, but not for lower individual QOL or well-being. Family characteristics, stress, and coping skills were predictive of outcomes. Parents’ psychosocial responses to the challenges of life with infants with HLHS change over time. Individually tailored psychosocial support is needed.


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