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1.
Am Heart J ; 224: 85-97, 2020 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-32353587

RESUMO

BACKGROUND: Children with congenital heart disease are at risk for growth failure due to inadequate nutrient intake and increased metabolic demands. We examined the relationship between anthropometric indices of nutrition (height-for-age z-score [HAZ], weight-for-age z-score [WAZ], weight-for-height z-score [WHZ]) and outcomes in a large sample of children undergoing surgery for congenital heart disease. METHODS: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having index cardiac surgery at age 1 month to 10 years were included. Indices were calculated by comparing patients' weight and height to population norms from the World Health Organization and Centers for Disease Control and Prevention. Outcomes included operative mortality, composite mortality or major complication, major postoperative infection, and postoperative length of stay. For each outcome and index, the adjusted odds ratio (aOR) (for mortality, composite outcome, and infection) and adjusted relative change in median (for postoperative length of stay) for a 1-unit decrease in index were estimated using mixed-effects logistic and log-linear regression models. RESULTS: Every unit decrease in HAZ was associated with 1.40 aOR of mortality (95% CI 1.32-1.48), and every unit decrease in WAZ was associated with 1.33 aOR for mortality (95% CI 1.25-1.41). The relationship between WHZ and outcome was nonlinear, with aOR of mortality of 0.84 (95% CI 0.76-0.93) for 1-unit decrease when WHZ ≥ 0 and a nonsignificant association for WHZ < 0. Trends for other outcomes were similar. Overall, the incidence of low nutritional indices was similar for 1-ventricle and 2-ventricle patients. Children between the age of 1 month and 1 year and those with lesions associated with pulmonary overcirculation had the highest incidence of low nutritional indices. CONCLUSIONS: Lower HAZ and WAZ, suggestive of malnutrition, are associated with increased mortality and other adverse outcomes after cardiac surgery in infants and young children. Higher WHZ over zero, suggestive of obesity, is also associated with adverse outcomes.

2.
Cardiol Young ; 30(4): 560-567, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32228736

RESUMO

Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be "the go-to online resource" for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.

3.
ASAIO J ; 2020 Apr 17.
Artigo em Inglês | MEDLINE | ID: mdl-32317557

RESUMO

As COVID-19 cases surge worldwide, an urgent need exists to enhance our understanding of the role of extracorporeal membrane oxygenation (ECMO) in the management of severely ill patients with COVID-19 who develop acute respiratory and cardiac compromise refractory to conventional therapy. The purpose of this manuscript is to review our initial clinical experience in 32 patients with confirmed COVID-19 treated with ECMO.A multi-institutional registry and database was created and utilized to assess all patients who were supported with ECMO provided by SpecialtyCare. Data captured included patient characteristics, pre-COVID-19 risk factors and comorbidities, confirmation of COVID-19 diagnosis, features of ECMO support, specific medications utilized to treat COVID-19, and short-term outcomes through hospital discharge. This analysis includes all of our patients with COVID-19 supported with ECMO, with an analytic window starting March 17, 2020 when our first COVID-19 patient was placed on ECMO, and ending April 9, 2020.During the 24 days of this study, 32 consecutive patients with COVID-19 were placed on ECMO at 9 different hospitals. As of the time of analysis, 17 remain on ECMO, 10 died prior to or shortly after decannulation, and 5 are alive and extubated after removal from ECMO, with one of these 5 discharged from the hospital. Adjunctive medication in the surviving patients while on ECMO was as follows: 4 of 5 survivors received intravenous steroids, 3 of 5 survivors received antiviral medications (Remdesivir), 2 of 5 survivors were treated with anti-interleukin-6-receptor monoclonal antibodies (Tocilizumab or Sarilumab), and 1 of 5 survivors received hydroxychloroquine.An analysis of 32 COVID-19 patients with severe pulmonary compromise supported with ECMO suggests that ECMO may play a useful role in salvaging select critically ill patients with COVID-19. Additional patient experience and associated clinical and laboratory data must be obtained to further define the optimal role of ECMO in patients with COVID-19 and ARDS. These initial data may provide useful information to help define the best strategies to care for these challenging patients, and may also provide a framework for much-needed future research about the use of ECMO to treat patients with COVID-19.

4.
Ann Thorac Surg ; 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-32246937

RESUMO

BACKGROUND: Approximately 10% to 20% of children are readmitted after congenital heart surgery. Very little is known about biomarkers as predictors of risk of unplanned readmission following pediatric congenital heart surgery. Novel cardiac biomarker ST2 may be associated with risk of unplanned readmission. ST2 concentrations are believed to reflect cardiovascular stress and fibrosis. Our objective was to explore the relationship between pre- and postoperative ST2 biomarker levels and risk of readmission within one year after congenital heart surgery. METHODS: We prospectively enrolled pediatric patients <18 years of age who underwent at least one congenital heart operation at Johns Hopkins Hospital from 2010 - 2014. Plasma samples were collected immediately prior to surgery and at the end of bypass. We used Kaplan-Meier survival analysis and multivariable Cox regression models adjusting for variables used in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database mortality risk model. RESULTS: In our cohort (N=145), there were 39 children with readmissions within 365 days. The median time to unplanned readmission was 54 days (IQR: 10 - 153). Kaplan-Meier analysis demonstrated a significant difference across terciles of pre- and post-operative ST2 biomarker levels. After adjustment, elevated serum levels of ST2 measured preoperatively and postoperatively were associated with increased risk of readmission (hazard ratio range 2.5 to 3.7, all p<0.05). CONCLUSIONS: Elevated levels of ST2 are significantly associated with increased risk of unplanned readmission within one-year after pediatric congenital heart surgery. Novel serum biomarker ST2 can be used for risk stratification or estimating postsurgical prognosis.

7.
Ann Thorac Surg ; 2020 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-32119855

RESUMO

BACKGROUND: The oncologic efficacy of segmentectomy is controversial. We compared long- term survival in clinical stage IA (T1N0) patients undergoing lobectomy and segmentectomy in Medicare patients in the STS database. METHODS: The Society of Thoracic Surgeons General Thoracic Surgery Database (STS- GTSD) was linked to Medicare data in 14,286 lung cancer patients who underwent segmentectomy (n=1654) or lobectomy (n=12,632) for clinical stage IA disease from 2002-15. Cox regression was used to create a long-term survival model. Patients were then propensity matched on demographic and clinical variables to derive matched pairs. RESULTS: In Cox modeling, segmentectomy is associated with survival similar to lobectomy in the entire cohort [HR 1.04, 95%CI (0.89,1.20), P=0.64] and in the matched subcohort. A subanalysis restricted to the 2009-15 population (n=11,811), when T1a tumors were specified and PET scan results and mediastinal staging procedures were accurately recorded in the database, also showed that segmentectomy and lobectomy continue to have similar survival [HR 1.00, 95% CI (0.87,1.16)]. Subanalysis of the pathologic N0 patients demonstrated the same results. CONCLUSIONS: Lobectomy and segmentectomy for early stage lung cancer are equally effective treatments with similar survival. STS surgeons appear to be selecting patients appropriately for sublobar procedures.

8.
Ann Thorac Surg ; 2020 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-32105714

RESUMO

BACKGROUND: Optimal methods to assess resource utilization in congenital heart surgery remain unclear. We compared traditional cost-to-charge ratio methods with newer standardized cost methods that aim to more directly assess resources consumed. METHODS: Clinical data from The Society of Thoracic Surgeons Database were linked with resource use data from the Pediatric Health Information Systems Database (2010 to 2015). Standardized cost methods specific to the congenital heart surgery population were developed and compared with cost-to-charge ratio methods. Resource use in the overall population and variability across hospitals were described using hierarchical mixed effect models adjusting for case-mix. RESULTS: Overall, 43 hospitals (65,331 patients) were included. There were minimal population-level differences in the distribution of resource use as estimated by the two methods. At the hospital level, there was less apparent variability in resource use across centers with the standardized cost vs cost-to-charge ratio method, overall (coefficient of variation 20% vs 25%) and across complexity (The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery [STAT]) categories. When hospitals were categorized into tertiles by resource use, 33% changed classification depending on which resource use method was used (26% by one tertile and 7% by two tertiles). CONCLUSIONS: In this first evaluation of standardized cost methodology in the congenital heart population, we found minimal differences vs traditional methods at the population level. At the hospital level, the magnitude of variation in resource use was less with standardized cost methods, and approximately one third of centers changed resource use categories depending on the methodology used. Because of these differences, care should be taken in future studies and in benchmarking and reporting efforts in selecting optimal methodology.

10.
Ann Thorac Surg ; 109(1): 164-170, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31323208

RESUMO

BACKGROUND: The purpose of this study was to evaluate the association between preoperative biomarker levels and 365-day readmission or mortality after pediatric congenital heart surgery. METHODS: Children aged 18 years or younger undergoing congenital heart surgery (n = 145) at Johns Hopkins Hospital from 2010 to 2014 were enrolled in the prospective cohort. Novel biomarkers suppression of tumorgenicity 2, galectin-3, N-terminal prohormone brain natriuretic peptide, and glial fibrillary acidic protein were measured. The composite study endpoint was unplanned readmission within 365 days after discharge or mortality either in hospital during the surgical admission or within 365 days after discharge. A clinical model based on covariates used in The Society of Thoracic Surgeons Congenital Heart Surgery Database mortality risk model and an augmented model using the clinical model in conjunction with a novel biomarker panel were evaluated. RESULTS: Readmission or mortality within 365 days of surgery occurred among 39 pediatric patients (27%). The clinical model alone resulted in a c-statistic of 0.719 (95% confidence interval, 0.63 to 0.81). The clinical model in conjunction with the log-transformed biomarkers improved the c-statistic to 0.805 (95% confidence interval, 0.73 to 0.88). The addition of biomarkers resulted in a significant improvement to the clinical model alone (P value = 0.035). CONCLUSIONS: Novel biomarkers may add predictive value when assessing the likelihood of 365-day readmission or mortality after pediatric congenital heart surgery. After adjusting for clinical and novel biomarkers, preoperative and postoperative suppression of tumorgenicity 2 remained associated with 365-day readmission or mortality. Currently, The Society of Thoracic Surgeons clinical congenital mortality risk model can be applied to identify children with increased risk of repeat hospitalizations and postdischarge mortality and may inform preventative care interventions that aim to reduce these adverse events.

11.
Cardiol Young ; 30(1): 74-81, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31806066

RESUMO

BACKGROUND: Neonates are at high risk of bleeding after open-heart surgery. We sought to determine pre-operative and intra-operative risk factors for increased bleeding after neonatal open-heart surgery with cardiopulmonary bypass. METHODS: We conducted a retrospective cohort study of neonates (0-30 days old) who underwent open-heart surgery with cardiopulmonary bypass from January, 2009, to March, 2013. Cardiac diagnosis; demographic and surgical data; and blood products, haemostatic agents, and anti-thrombotic agents administered before, during, and within 24 hours after surgery were abstracted from the electronic health record and anaesthesia records. The outcome of interest was chest tube output (in ml/kg body weight) within 24 hours. Relationships between chest tube output and putative associated factors were evaluated by unadjusted and adjusted linear regression. RESULTS: The cohort consisted of 107 neonates, of whom 79% had a Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Mortality Category of 4 or 5. Median chest tube output was 37 ml/kg (range 9-655 ml/kg). Age, African-American race, and longer durations of surgery and cardiopulmonary bypass each had statistically significant associations with increased chest tube output in unadjusted analyses. In multivariable analysis, African-American race retained an independent, statistically significant association with increased chest tube output; the geometric mean of chest tube output among African-American neonates was 71% higher than that of Caucasians (95% confidence interval, 29-125%; p = 0.001). CONCLUSION: Among neonates with CHD undergoing open-heart surgery with cardiopulmonary bypass, African-American race is independently associated with greater chest tube output over the first 24 hours post-operatively.

12.
Circulation ; 141(8): 641-651, 2020 Feb 25.
Artigo em Inglês | MEDLINE | ID: mdl-31736357

RESUMO

BACKGROUND: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency associated with a decline in exercise performance. Rigorous clinical trials aimed at improving physiology and guiding pharmacotherapy are lacking. METHODS: The FUEL trial (Fontan Udenafil Exercise Longitudinal) was a phase III clinical trial conducted at 30 centers. Participants were randomly assigned udenafil, 87.5 mg twice daily, or placebo in a 1:1 ratio. The primary outcome was the between-group difference in change in oxygen consumption at peak exercise. Secondary outcomes included between-group differences in changes in submaximal exercise at the ventilatory anaerobic threshold, the myocardial performance index, the natural log of the reactive hyperemia index, and serum brain-type natriuretic peptide. RESULTS: Between 2017 and 2019, 30 clinical sites in North America and the Republic of Korea randomly assigned 400 participants with Fontan physiology. The mean age at randomization was 15.5±2 years; 60% of participants were male, and 81% were white. All 400 participants were included in the primary analysis with imputation of the 26-week end point for 21 participants with missing data (11 randomly assigned to udenafil and 10 to placebo). Among randomly assigned participants, peak oxygen consumption increased by 44±245 mL/min (2.8%) in the udenafil group and declined by 3.7±228 mL/min (-0.2%) in the placebo group (P=0.071). Analysis at ventilatory anaerobic threshold demonstrated improvements in the udenafil group versus the placebo group in oxygen consumption (+33±185 [3.2%] versus -9±193 [-0.9%] mL/min, P=0.012), ventilatory equivalents of carbon dioxide (-0.8 versus -0.06, P=0.014), and work rate (+3.8 versus +0.34 W, P=0.021). There was no difference in change of myocardial performance index, the natural log of the reactive hyperemia index, or serum brain-type natriuretic peptide level. CONCLUSIONS: In the FUEL trial, treatment with udenafil (87.5 mg twice daily) was not associated with an improvement in oxygen consumption at peak exercise but was associated with improvements in multiple measures of exercise performance at the ventilatory anaerobic threshold. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02741115.

13.
Ann Thorac Surg ; 109(3): 848-855, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31689407

RESUMO

BACKGROUND: The Society of Thoracic Surgeons (STS) General Thoracic Surgery Database (GTSD) has developed composite quality measures for lobectomy and esophagectomy. This study sought to develop a composite measure including all resections for lung cancer. METHODS: The STS lung cancer composite score is based on 2 outcomes: risk-adjusted mortality and morbidity. GTSD data were included from January 2015 to December 2017. "Star ratings" were created for centers with 30 or more cases by using 95% Bayesian credible intervals. The Bayesian model was performed with and without inclusion of the minimally invasive approach to assess the impact of approach on the composite measure. RESULTS: The study population included 38,461 patients from 256 centers. Overall operative mortality was 1.3% (495 of 38,461). The major complication rate was 7.9% (3045 of 38,461). The median number of nodes examined was 10 (interquartile range, 5 to 16); the median number of nodal stations sampled was 4 (interquartile range, 3 to 5). Positive resection margins were identified in 3.7% (1420 of 38,461). A total of 214 centers with 30 or more cases were assigned star ratings. There were 7 1-star, 194 2-star, and 13 3-star programs; 70.6% of resections were performed through a minimally invasive approach. Inclusion of minimally invasive approach, which was adjusted for in previous models, altered the star ratings for 3% (6 of 214) of the programs. CONCLUSIONS: Participants in the STS GTSD perform lung cancer resection with low morbidity and mortality. Lymph node data suggest that participants are meeting contemporary staging standards. There is wide variability among participants in application of minimally invasive approaches. The study found that risk adjustment for approach altered ratings in 3% of participants.

14.
Am Heart J ; 220: 192-202, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31855716

RESUMO

For decades, physicians have administered corticosteroids in the perioperative period to infants undergoing heart surgery with cardiopulmonary bypass (CPB) to reduce the postoperative systemic inflammatory response to CPB. Some question this practice because steroid efficacy has not been conclusively demonstrated and because some studies indicate that steroids could have harmful effects. STRESS is a randomized, placebo-controlled, double-blind, multicenter trial designed to evaluate safety and efficacy of perioperative steroids in infants (age < 1 year) undergoing heart surgery with CPB. Participants (planned enrollment = 1,200) are randomized 1:1 to methylprednisolone (30 mg/kg) administered into the CPB pump prime versus placebo. The trial is nested within the existing infrastructure of the Society of Thoracic Surgeons Congenital Heart Surgery Database. The primary outcome is a global rank score of mortality, major morbidities, and hospital length of stay with components ranked commensurate with their clinical severity. Secondary outcomes include several measures of major postoperative morbidity, postoperative hospital length of stay, and steroid-related safety outcomes including prevalence of hyperglycemia and postoperative infectious complications. STRESS will be one of the largest trials ever conducted in children with heart disease and will answer a decades-old question related to safety and efficacy of perioperative steroids in infants undergoing heart surgery with CPB. The pragmatic "trial within a registry" design may provide a mechanism for conducting low-cost, high-efficiency trials in a heretofore-understudied patient population.


Assuntos
Anti-Inflamatórios/uso terapêutico , Ponte Cardiopulmonar/efeitos adversos , Cardiopatias Congênitas/cirurgia , Metilprednisolona/uso terapêutico , Complicações Pós-Operatórias/prevenção & controle , Síndrome de Resposta Inflamatória Sistêmica/prevenção & controle , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/efeitos adversos , Método Duplo-Cego , Humanos , Hiperglicemia/epidemiologia , Lactente , Recém-Nascido , Infecções/epidemiologia , Tempo de Internação , Metilprednisolona/administração & dosagem , Metilprednisolona/efeitos adversos , Avaliação de Resultados em Cuidados de Saúde , Placebos/uso terapêutico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/mortalidade , Sistema de Registros , Projetos de Pesquisa , Síndrome de Resposta Inflamatória Sistêmica/mortalidade , Estados Unidos
15.
World J Pediatr Congenit Heart Surg ; 11(1): 14-21, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31835986

RESUMO

Multi-institutional databases and registries have proliferated over the last decade in all specialties of medicine. They may be especially helpful in low-frequency/high-acuity fields such as pediatric and congenital heart diseases. The Society of Thoracic Surgeon's Congenital Heart Surgery Database (STSCHSD) is the largest single data set for the congenital heart disease population and includes contemporaneous data from over 120 programs in the United States (and several outside of the United States), capturing greater than 98% of the congenital cardiac surgical procedures in the United States. In 2010, the Congenital Cardiac Anesthesia Society partnered with the STSCHSD to incorporate anesthesia-related elements into the data set. Voluntary site participation in the anesthesia data has grown steadily. Currently, over 60 sites performing more than 60% of cardiac bypass procedures in the STSCHSD are submitting anesthesia data annually into the STSCHSD. Anesthesia data include perioperative medication usage, modalities for hemodynamic and neurologic monitoring, blood product, antifibrinolytic and procoagulant use, and anesthesia-related adverse events. This special article provides a descriptive summary of relevant findings to date, reflecting the wide variety in anesthesia practice patterns present among institutions and illustrates the functionality of a multisite registry in pediatric cardiac anesthesia which can be utilized both locally and nationally.

16.
Eur J Cardiothorac Surg ; 57(1): 63-71, 2020 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-31006006

RESUMO

OBJECTIVES: Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II). We compared BSID-II and Bayley-III scores in infants undergoing cardiac surgery. METHODS: We evaluated 2198 infants who underwent operations with cardiopulmonary bypass between 1996 and 2009 at 26 institutions. We used propensity score matching to limit confounding by indication in a subset of patients (n = 705). RESULTS: Overall, unadjusted Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores (90.7 ± 17.2 vs 77.6 ± 18.8, P < 0.001), and unadjusted Bayley-III composite cognitive and language scores were higher than BSID-II Mental Development Index scores (92.0 ± 15.4 vs 88.2 ± 16.7, P < 0.001). In the propensity-matched analysis, Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores [absolute difference 14.1, 95% confidence interval (CI) 11.7-17.6; P < 0.001] and the Bayley-III classified fewer children as having severe [odds ratio (OR) 0.24; 95% CI 0.14-0.42] or mild-to-moderate impairment (OR 0.21; 95% CI 0.14-0.32). The composite of Bayley-III cognitive and language scores was higher than BSID-II Mental Development Index scores (absolute difference 4.0, 95% CI 1.4-6.7; P = 0.003), but there was no difference between Bayley editions in the proportion of children classified as having severe cognitive and language impairment. CONCLUSIONS: The Bayley-III yielded higher scores than the BSID-II and classified fewer children as severely impaired. The systematic bias towards higher scores with the Bayley-III precludes valid comparisons between early and contemporary cardiac surgery cohorts.

17.
J Am Coll Cardiol ; 74(22): 2786-2795, 2019 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-31779793

RESUMO

BACKGROUND: Patients undergoing complex pediatric cardiac surgery remain at considerable risk of mortality and morbidity, and variation in outcomes exists across hospitals. The Pediatric Cardiac Critical Care Consortium (PC4) was formed to improve the quality of care for these patients through transparent data sharing and collaborative learning between participants. OBJECTIVES: The purpose of this study was to determine whether outcomes improved over time within PC4. METHODS: The study analyzed 19,600 hospitalizations (18 hospitals) in the PC4 clinical registry that included cardiovascular surgery from August 2014 to June 2018. The primary exposure was 2 years of PC4 participation; this provided adequate time for hospitals to accrue data and engage in collaborative learning. Aggregate case mix-adjusted outcomes were compared between the first 2 years of participation (baseline) and all months post-exposure. We also evaluated outcomes from the same era in a cohort of similar, non-PC4 hospitals. RESULTS: During the baseline period, there was no evidence of improvement. We observed significant improvement in the post-exposure period versus baseline for post-operative intensive care unit mortality (2.1% vs. 2.7%; 22% relative reduction [RR]; p = 0.001), in-hospital mortality (2.5% vs. 3.3%; 24% RR; p = 0.001), major complications (10.1% vs. 11.5%; 12% RR; p < 0.001), intensive care unit length of stay (7.3 days vs. 7.7 days; 5% RR; p < 0.001), and duration of ventilation (61.3 h vs. 70.6 h; 13% RR; p = 0.01). Non-PC4 hospitals showed no significant improvement in mortality, complications, or hospital length of stay. CONCLUSIONS: This analysis demonstrates improving cardiac surgical outcomes at children's hospitals participating in PC4. This change appears unrelated to secular improvement trends, and likely reflects PC4's commitment to transparency and collaboration.

18.
Cardiol Young ; 29(12): 1510-1516, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31767043

RESUMO

BACKGROUND: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. METHODS: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days). RESULTS: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98). CONCLUSIONS: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

19.
Artigo em Inglês | MEDLINE | ID: mdl-31669019

RESUMO

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

20.
Artigo em Inglês | MEDLINE | ID: mdl-31740116

RESUMO

OBJECTIVE: The study objective was to describe the surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life. METHODS: From 1994 to 2000, 565 neonates with aortic atresia were admitted to 26 Congenital Heart Surgeons' Society hospitals and followed annually for vital status. Initial management included surgical palliation (n = 453) and primary cardiac transplantation (n = 68). PedsQL health-related quality of life questionnaires were sent cross-sectionally to a subgroup of 198 patients alive at previous follow-up, with 80 responses. RESULTS: Risk of death was initially high for both treatment strategies. However, compared with initial surgical palliation, survival with primary transplantation, including wait-list mortality, was greater and persisted long-term (65% vs 40% at 15 years; P = .002). Survival after secondary transplantation (48% at 9 years) was lower than after primary transplantation (74%). Health-related quality of life total score was lower overall than that of the general adolescent population (71 ± 16 vs 84 ± 13; P = .0001; normal = 100), but similar to that of adolescents with chronic diseases. It was similar in the surgical palliation and primary transplantation groups (70 ± 16 vs 75 ± 15; P = .3). Patients who received surgical palliation reported more symptoms (76 ± 15 vs 63 ± 18; P = .02). CONCLUSIONS: Patients receiving primary heart transplantation for aortic atresia in 1994 to 2000 experienced better survival, fewer symptoms, and equivalent quality of life compared with those undergoing initial surgical palliation. Notwithstanding the limited availability of neonatal and infant donor hearts, primary transplantation may be considered for those neonates with risk factors predictive of exceptionally poor survival after surgical palliation.

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