The management of cardiopulmonary bypass for pediatric cardiac surgery is more challenging than that in adults due to the smaller size, immaturity, and complexity of the anatomy in children. Despite ...major improvements in cardiopulmonary bypass, there remain many subjects of debate. This review article discusses the physiology of cardiopulmonary bypass for pediatric and congenital heart surgery, including topics related to hemodilution, hypothermia, acid–base strategies, inflammatory response, and myocardial protection.
The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model.
We ...analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A 5539 procedures performed in 90 hospitals: ≤50, group B 9322 procedures in 24 hospitals: 51-100, group C 13,331 procedures in 21 hospitals: 101-150, group D 18,972 procedures in 15 hospitals: ≥151).
The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0.
The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.
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Abstract
OBJECTIVES
Although primary repair in early infancy has for decades been the prevalent strategy for management of truncus arteriosus (TA), recent concerns about the levels of morbidity and ...mortality have led to consideration of a staged surgical approach. Our goal was to describe recent patterns of management, to characterize patients who underwent primary or staged repair and to evaluate risk factors associated with operative mortality in a contemporary multicentre cohort.
METHODS
In the Japanese Cardiovascular Surgery Database, we identified all cases of TA undergoing an initial surgical procedure from 2008 to 2018. Operative mortality was defined as death within 30 days of an operation or in-hospital death regardless of the length of hospital stay. The hospital volume was defined by the average volume of TA repairs per year.
RESULTS
The total number of patients undergoing initial surgery for TA was 286. Sixty-eight (24%, 68/286) underwent primary repair (primary repair group). The remaining 218 (76%, 218/286) underwent initial bilateral pulmonary artery banding as part of a planned staged approach (staged repair group). One hundred sixty-two patients out of 218 initially banded patients underwent the repair of TA during this study period. Concomitant diagnoses in the entire cohort included interrupted aortic arch repair in 36 patients and truncal valve regurgitation in 32. No centres handling an average of ≥2 truncus cases/year of the repair of TA were identified in this cohort. A total of 30% (85/286) of the cases were performed at centres that handled an average of ≥1 and <2 cases/year. The remaining 70% were at centres with <1 case/year. Overall, 37 patients (12.9%; 37/286) died. The operative mortality rates in the primary and staged repair groups were similar: that for the primary repair group was 16.2% (11/68) versus 11.9% for the staged repair group (26/218; P = 0.41). With multivariable logistic regression analysis, the factors most strongly associated with operative mortality were preoperative heart failure requiring catecholamine support (odds ratio, 4.18; 95% confidence interval 1.96–8.96) and the repeat bilateral pulmonary artery banding (odds ratio, 3.89; 95% confidence interval 1.08–14.07).
CONCLUSIONS
The staged repair of TA has emerged as the preferred option for surgical timing at most of the centres participating in the Japanese Cardiovascular Surgery Database. The management outcomes of the patients with TA were favourable, even for the patients at low-volume centres.
Surgical repair of truncus arteriosus (TA) remains challenging.
Background:Current surgical outcomes of congenital heart surgery for patients with Down syndrome are unclear.Methods and Results:Of 29,087 operations between 2008 and 2012 registered in the Japan ...Congenital Cardiovascular Surgery Database (JCCVSD), 2,651 were carried out for patients with Down syndrome (9%). Of those, 5 major biventricular repair procedures ventricular septal defect repair (n=752), atrioventricular septal defect repair (n=452), patent ductus arteriosus closure (n=184), atrial septal defect repair (n=167), tetralogy of Fallot (TOF) repair (n=108), as well as 2 major single ventricular palliations bidirectional Glenn (n=21) and Fontan operation (n=25) were selected and their outcomes were compared. The 90-day and in-hospital mortality rates for all 5 major biventricular repair procedures and bidirectional Glenn were similarly low in patients with Down syndrome compared with patients without Down syndrome. On the other hand, mortality after Fontan operation in patients with Down syndrome was significantly higher than in patients without Down syndrome (42/1,558=2.7% vs. 3/25=12.0%, P=0.005).Conclusions:Although intensive management of pulmonary hypertension is essential, analysis of the JCCVSD revealed favorable early prognostic outcomes after 5 major biventricular procedures and bidirectional Glenn in patients with Down syndrome. Indication of the Fontan operation for patients with Down syndrome should be carefully decided.
Concomitant heart failure is associated with poor clinical outcome in dialysis patients. The arteriovenous shunt, created as vascular access for hemodialysis, increases ventricular volume-overload, ...predisposing patients to developing cardiac dysfunction. The integral function of mitochondrial respiration is critically important for the heart to cope with hemodynamic overload. The involvement, however, of mitochondrial activity or reactive oxygen species (ROS) in the pathogenesis of ventricular-overload-induced heart failure has not been fully elucidated. We herein report that disorganization of mitochondrial respiration increases mitochondrial ROS production in the volume-overloaded heart, leading to ventricular dysfunction. We adopted the murine arteriovenous fistula (AVF) model, which replicates the cardinal features of volume-overload-induced ventricular dysfunction. Enzymatic assays of cardiac mitochondria revealed that the activities of citrate synthase and NADH-quinone reductase (complex Ⅰ) were preserved in the AVF heart. In contrast, the activity of NADH oxidase supercomplex was significantly compromised, resulting in elevated ROS production. Importantly, the antioxidant N-acetylcysteine prevented the development of ventricular dilatation and cardiac dysfunction, suggesting a pathogenic role for ROS in dialysis-related cardiomyopathy. A cardioprotective effect was also observed in metformin-treated mice, illuminating its potential use in the management of heart failure complicating diabetic patients on dialysis.
•This is the first national survey of fetal detection for congenital heart diseases (CHDs) in Japan•Overall prenatal detection rate is low in Japan compared to the European countries•Significant ...regional gaps of detection rates are recognized in Japan•Fetal diagnosis significantly reduces risks of emergency transfer for CHDs•Periodic official surveillance for prenatal cardiac diagnosis is required
Investigation into the detection rate (DR) of congenital heart diseases (CHDs) in fetuses is important for the assessment of fetal cardiac screening systems.
We highlight issues of fetal cardiac screening in Japan.
We performed an initial national survey of fetal diagnosis of CHDs from the data of the national registry for congenital heart surgery from 2013 to 2017. Subjects were neonates and infants with moderate or severe CHDs. We investigated DR in each prefecture in Japan and emergency transfer (ET) for neonates by analyzing distance and admission day of ET with or without fetal diagnoses.
: The overall average DR in Japan was 0.41 (0.02 increase every year). No regional significant relationship was found between DR and population in each prefecture. ET was performed in 12% of neonates with prenatal diagnosis and in 63% of neonates without resulting in significant risk for ET in fetuses without a fetal diagnosis OR 13.3 (11.6−15.3), p<0.001. The distance of ET was shorter and admission was earlier in the neonates with a prenatal diagnosis than in those without median 6.6 km (IQR: 4.1-25.7) vs 17.0 km (IQR: 7.4-35.3), median 0.0 day (IQR: 0.0-0.0) vs 0.0 day (IQR: 0.0-1.0), p<0.001, p<0.001, respectively
Prenatal cardiac diagnosis reduces geographic and chronological risks of ET for moderate to severe CHDs. DR is still developing and periodic official surveillance is required for improving prenatal cardiac diagnosis in Japan.
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In infants with ventricular septal defect (VSD) who undergo surgical intervention, body weight, along with age, is frequently thought to be the decisive predictor of morbidity and mortality after ...surgery; however, its information on quantitative risk assessment is limited.
All infants (<1 year old) with a fundamental diagnosis of VSD who underwent surgical VSD closure or pulmonary artery banding between 2012 and 2016 were identified from the Japan Cardiovascular Surgery Database Congenital Section. The outcome of interest was a composite end point of all-cause death and major complications within 30 days after surgery. We evaluated the association between body weight at surgery and composite end point using logistic regression models.
A total of 4947 cases were analyzed (median age, 125; interquartile range IQR, 79-193 days; median body weight, 4.94 IQR, 4.00-6.00 kg), including 4310 cases (87.1%) treated with surgical VSD closure and 637 (12.9%) treated with pulmonary artery banding. The surgical course was uncomplicated in 94.2% of cases, 23 (0.5%) died, and 283 (5.7%) experienced major complications. The risk of the composite end point was higher along with lower body weight (adjusted odds ratio, 1.56 for every −1 kg; 95% confidence interval, 1.30-1.88; P < .001) and plateaued at body weight of approximately >4.5 kg via smoothing spline curve. Importantly, cases with approximately <4.5 kg of body weight had higher predicted risk regardless of age.
Surgical intervention for infants with VSD was safely performed in contemporary practice; however, caution is warranted in lower body weight infants, particularly for infants with approximately <4.5 kg.
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