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There are several choices for the correction of complex transposition of the great arteries and double outlet right ventricle not amenable to the Rastelli-type surgery, but outcome data are limited to small series. This study aims to report results after the aortic root translocation and en bloc rotation of the outflow tract procedures.This is a retrospective, multicentric, observational study. Clinical, anatomy, procedural, and detailed follow-up data (median, 4.43 years) were collected.A total of 70 patients (62.9% male; median age, 1 year; range 4 days to 12.4 years) were included: n = 43 in the aortic root translocation group and n = 27 in the en bloc rotation group. Those in the aortic root translocation group were older (P = .01) and more likely to have had previous procedures (P .0001), but cardiac anatomy was similar in both groups. Aortic root translocation and en bloc rotation early mortality (30 days) was similar (4.7% vs 3.7%, P = .8). Late survival and freedom from any cardiac reintervention were 92.7% and 16.9% at 15 years overall, respectively. Freedom from right ventricular outflow tract/conduit reintervention was better in the en bloc rotation group than in the aortic root translocation group (100% vs 24.5%, P = .0003), but more patients in the en bloc rotation group had moderate (or worse) aortic valve regurgitation during follow-up (16% vs 2.6%, P = .07).Both aortic root translocation and en bloc rotation are valuable surgical options for the treatment of complex transposition of the great arteries and double outlet right ventricle. In the en bloc rotation group, there was better freedom from right ventricular outflow tract reinterventions, but a higher probability of aortic valve regurgitation. Identifying the main driving forces for these observed differences requires further study of these procedures.

Objectives The en-bloc rotation of the outflow tracts (EBR) enables an anatomic correction of transposition of the great arteries with VSD and left ventricular outflow tract obstruction. The anatomical condition or previous palliative procedures may allow choosing an elective date for the anatomic correction. The aim of this study was to evaluate the optimal age for performing the EBR based on the largest series published so far. Methods At the Children’s Heart Center Linz the EBR was performed in 33 patients between 2003-2021. Median age at operation was 74 (IQR 17-627) days. 12 patients were newborns (≤28 days), 9 older than 369 days. These two groups were compared to the remaining patients each regarding peri- and postoperative data, complications, reinterventions and mortality. The median follow-up period was 5.4 (IQR 0.99-11.74) years. Results In-hospital mortality was 6.1%. All-cause mortality was lower in patients younger than 369 days at time of EBR (4.2% vs 44.4% in patients >369 days old, p = 0.013). In newborns ICU stay (median 18.5 days vs 8 days, p = 0.008) and in hospital stay (median 29.5 days vs 15 days, p = 0.026) were significantly longer, the risk for postoperative AV block was higher (33.3 vs 0%, p = 0.012) than in patients who had been corrected anatomically after the neonatal period. Conclusions The results of this study suggest the deferral of the EBR to the post-newborn period. A significantly higher mortality rate in patients of older age at operation seems to recommend the anatomic correction during the first year of life.

Objective To systematically review and perform a meta-analysis of radiation associated risks of cardiovascular disease in all groups exposed to radiation with individual radiation dose estimates. Design Systematic review and meta-analysis. Main outcome measures Excess relative risk per unit dose (Gy), estimated by restricted maximum likelihood methods. Data sources PubMed and Medline, Embase, Scopus, Web of Science Core collection databases. Eligibility criteria for selecting studies Databases were searched on 6 October 2022, with no limits on date of publication or language. Animal studies and studies without an abstract were excluded. Results The meta-analysis yielded 93 relevant studies. Relative risk per Gy increased for all cardiovascular disease (excess relative risk per Gy of 0.11 (95% confidence interval 0.08 to 0.14)) and for the four major subtypes of cardiovascular disease (ischaemic heart disease, other heart disease, cerebrovascular disease, all other cardiovascular disease). However, interstudy heterogeneity was noted (P Conclusions Results provide evidence supporting a causal association between radiation exposure and cardiovascular disease at high dose, and to a lesser extent at low dose, with some indications of differences in risk between acute and chronic exposures, which require further investigation. The observed heterogeneity complicates a causal interpretation of these findings, although this heterogeneity is much reduced if only higher quality studies or those at moderate doses or low dose rates are considered. Studies are needed to assess in more detail modifications of radiation effect by lifestyle and medical risk factors. Systematic review registration PROSPERO CRD42020202036

ObjectivesRadon is a ubiquitous occupational and environmental lung carcinogen. We aim to quantify the association between radon progeny and lung cancer mortality in the largest and most up-to-date pooled study of uranium miners.MethodsThe pooled uranium miners analysis combines 7 cohorts of male uranium miners with 7754 lung cancer deaths and 4.3 million person-years of follow-up. Vital status and lung cancer deaths were ascertained between 1946 and 2014. The association between cumulative radon exposure in working level months (WLM) and lung cancer was modelled as the excess relative rate (ERR) per 100 WLM using Poisson regression; variation in the association by temporal and exposure factors was examined. We also examined analyses restricted to miners first hired before 1960 and with ResultsIn a model that allows for variation by attained age, time since exposure and annual exposure rate, the ERR/100 WLM was 4.68 (95% CI 2.88 to 6.96) among miners who were less than 55 years of age and were exposed in the prior 5 to ConclusionsThis new large, pooled study confirms and supports a linear exposure–response relationship between cumulative radon exposure and lung cancer mortality which is jointly modified by temporal and exposure factors.

OBJECTIVES Percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits are at risk for infective endocarditis (IE). In children and adults with a congenital heart disease, a pulmonary valve implant is frequently necessary. Prosthetic valve endocarditis is a conservatively barely manageable, serious life-threatening condition. We investigated the results of surgical pulmonary valve replacements in patients with IE. METHODS A total of 20 patients with congenital heart disease with the definite diagnosis of IE between March 2013 and July 2020 were included in this single institutional, retrospective review. Infected conduits were 11 Melody, 5 Contegra, 3 homografts and 1 Matrix P Plus. All of the infected prosthetic material was removed from the right ventricular outflow tract up to the pulmonary bifurcation. Pulmonary homografts were implanted after pulmonary root resection as right ventricle-to-pulmonary artery conduits. RESULTS All patients survived and were discharged infection-free. The mean time from the conduit implant to the operation for IE was 4.9 years [95% confidence interval (CI), 3.0–6.9]. The median intensive care unit stay was 3.0 days (95% CI, 2.0–4.7), and the median hospital time was 25.0 days (95% CI, 19.2–42.0). Median follow-up time was 204.5 days (range 30 days to 5 years) without death or recurrent endocarditis. CONCLUSIONS The surgical treatment of IE of percutaneous pulmonary valve prostheses and right ventricle-to-pulmonary artery conduits is a safe and effective therapeutic concept. Early surgical referral of patients with suspicion of IE should be pursued to avoid sequelae such as right ventricular failure, septic emboli, intracardiac expansion and antibiotic resistance.

BackgroundDespite reductions in exposure for workers and the general public, radon remains a leading cause of lung cancer. Prior studies of underground miners depended heavily upon information on deaths among miners employed in the early years of mine operations when exposures were high and tended to be poorly estimated.ObjectivesTo strengthen the basis for radiation protection, we report on the follow-up of workers employed in the later periods of mine operations for whom we have more accurate exposure information and for whom exposures tended to be accrued at intensities that are more comparable to contemporary settings.MethodsWe conducted a pooled analysis of cohort studies of lung cancer mortality among 57,873 male uranium miners in Canada, Czech Republic, France, Germany, and the United States, who were first employed in 1960 or later (thereby excluding miners employed during the periods of highest exposure and focusing on miners who tend to have higher quality assessments of radon progeny exposures). We derived estimates of excess relative rate per 100 working level months (ERR/100 WLM) for mortality from lung cancer.ResultsThe analysis included 1.9 million person-years of observation and 1,217 deaths due to lung cancer. The relative rate of lung cancer increased in a linear fashion with cumulative exposure to radon progeny (ERR/100 WLM=1.33; 95% CI: 0.89, 1.88). The association was modified by attained age, age at exposure, and annual exposure rate; for attained ages

OBJECTIVES Fontan patients are at lifelong risk for developing complications, which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long-term survival of extracardiac Fontan patients in the modern era was investigated. The objective of this study was to investigate if surgical and interventional procedures in patients with protein-losing enteropathy (PLE) and/or plastic bronchitis (PB) and a failing Fontan circulation can postpone or avoid HTX. METHODS Retrospective data collection of all children who underwent a Fontan procedure between January 1999 and July 2021 at our centre was performed. Patients were surveyed regarding the occurrence of PLE or PB and their outcome was reported descriptively. HTX-free survival of patients who underwent a rescue procedure due to PLE/PB was evaluated. RESULTS Three hundred and seventy [94.1% (95% confidence interval, 91.4–96.3)] Fontan patients were free of HTX or death at last follow-up after a median follow-up time of 6.7 years. PB/PLE was diagnosed in 34 patients during the observation period. A rescue procedure was undertaken in 16 pts. at a median time of 6.5 months (range: 1 day to 9.4 years) since the initial diagnosis of PLE/PB. In these patients, HTX-free survival was 75% (95% confidence interval, 47.6–92.7) at a median follow-up time of 4.0 years after the procedure. Range: 3.5 months to 13.9 years. CONCLUSIONS Extracardiac Fontan patients in the modern era expect reasonable HTX-free survival rates. Surgical and/or interventional rescue strategies for Fontan failure can postpone HTX for a sustained period of time.

Introduction and Objectives Radon is an established risk factor for lung cancer. Less clear is whether radon causes other diseases than lung cancer. To further investigate such risks, updated mortality data from the German uranium miner cohort study (1946–2013) were analyzed. Methods The cohort includes 58,974 men who were employed for at least 6 months between 1946 and 1989 at the former Wismut uranium mining company in Eastern Germany. Considered endpoints were mainly cancers other than lung cancer, circulatory diseases and non-malignant respiratory diseases. Exposure to radon and its progeny and external gamma radiation was retrospectively estimated through a comprehensive job-exposure matrix. Statistical modelling was performed by internal Poisson regression for grouped data with baseline stratification by age and calendar year. Excess relative risks (ERR) per 100 WLM (Working Level Month) and 95% confidence intervals (CI) were estimated for cumulative 5-year lagged exposure to radon (mean=280, median=33, max=3,224 WLM). Results Within the follow-up period 1946–2013, a total of 5,122 cancer deaths other than lung cancer occurred. In this group, a statistically significant association was found (ERR/100 WLM=0.014; 95% CI: 0.006, 0.021). Regarding individual cancer sites, the majority of risk estimates were positive (14 out of 18), and two of them reached borderline significance (colon and liver cancer). This holds true even after adjustment for external gamma radiation. No increased ERR/100 WLM was found for the groups of deaths from circulatory diseases (n=10,665) and from non-malignant respiratory diseases excluding silicosis/other pneumoconiosis (n=1,674). Conclusion Our findings provide some evidence of an increased mortality risk for cancers other than lung cancer associated with radon. Chance, confounding by unconsidered risk factors and uncertainty in exposure assessment cannot be completely ruled out. If present at all, the radon-related risk for diseases and cancers other than lung cancer is substantially lower than that for lung cancer.

A neoaortic aneurysm after a Norwood type reconstruction of the aorta can develop due to systemic pressure on the former pulmonary artery wall. A complex valve sparing procedure can preserve native valves and avoid a valve replacement with requirement for anticoagulation. This type of operation was carried out in 3 patients, 2 of them after a Fontan palliation, 1 after a Norwood–Rastelli repair. The reconstruction was done using Dacron prostheses for the replacement of the dilated wall, similar to a Yacoub modification in 2 cases and to a David’s modification in 1 patient. The postoperative course was uneventful and postoperative valve function was good in all cases.

Background The Pooled Uranium Miners Analysis (PUMA) study draws together information from cohorts of uranium miners from Canada, the Czech Republic, France, Germany and the USA. Methods Vital status and cause of death were ascertained and compared with expectations based upon national mortality rates by computing standardized mortality ratios (SMRs) overall and by categories of time since first hire, calendar period of first employment and duration of employment as a miner. Results There were 51 787 deaths observed among 118 329 male miners [SMR = 1.05; 95% confidence interval (CI): 1.04, 1.06]. The SMR was elevated for all cancers (n = 16 633, SMR = 1.23; 95% CI: 1.21, 1.25), due primarily to excess mortality from cancers of the lung (n = 7756, SMR = 1.90; 95% CI: 1.86, 1.94), liver and gallbladder (n = 549, SMR = 1.15; 95% CI: 1.06, 1.25), larynx (n = 229, SMR = 1.10; 95% CI: 0.97, 1.26), stomach (n = 1058, SMR = 1.08; 95% CI: 1.02, 1.15) and pleura (n = 39, SMR = 1.06; 95% CI: 0.75, 1.44). Lung-cancer SMRs increased with duration of employment, decreased with calendar period and persisted with time since first hire. Among non-malignant causes, the SMR was elevated for external causes (n = 3362, SMR = 1.41; 95% CI: 1.36, 1.46) and respiratory diseases (n = 4508, SMR = 1.32; 95% CI: 1.28, 1.36), most notably silicosis (n = 814, SMR = 13.56; 95% CI: 12.64, 14.52), but not chronic obstructive pulmonary disease (n = 1729, SMR = 0.98; 95% CI: 0.93, 1.02). Conclusions Whereas there are important obstacles to the ability to detect adverse effects of occupational exposures via SMR analyses, PUMA provides evidence of excess mortality among uranium miners due to a range of categories of cause of death. The persistent elevation of SMRs with time since first hire as a uranium miner underscores the importance of long-term follow-up of these workers.

OBJECTIVES The aortic arch enlargement in the Norwood procedure is classically carried out using a curved homograft patch on the inner curvature of the neoaortic arch. The study investigates the outcome of a newly used artificial patch from a vascular prosthesis as an alternative to a homograft patch. METHODS Since April 2007, we used curved polytetrafluorethylene (PTFE) patches cut out of a prosthesis as an alternative to homograft patches for the aortic arch reconstruction. The decision for either patch material was made due to anatomic reasons, preferring PTFE patches in larger aortas. In this study, 224 Norwood patients, operated between April 2007 and April 2018, were analysed. A total of 104 patients received a PTFE patch (group PTFE), and 120 patients got a pulmonary homograft patch (group homograft). A single-centre retrospective analysis was carried out concerning postoperative course and long-term follow-up regarding aortic arch interventions and reoperations and comparing the 2 material groups. RESULTS There were no material associated operative or postoperative complications. In-hospital mortality was 13% in group PTFE. Six children died late during follow-up (6%). One aortic isthmus dilatation (1%) was carried out 12 months after the Norwood procedure in this group, no arch reoperation was necessary during the complete follow-up. CONCLUSIONS The curved PTFE patch showed good qualities in operative technical demands and excellent long-term results. In selected cases of hypoplastic left heart syndrome, it can be well used as alternative to the pulmonary homograft.

Background From 1946 to 1990, more than 400 000 people were employed by Wismut AG, a Soviet/Soviet-German corporation (German abbreviation: SAG/ SDAG), in the East German states of Saxony and Thuringia. In the early years in particular, employees were exposed to large amounts of radon and respirable crystalline silica. Methods In a cohort of 35 204 former underground employees of Wismut AG, mortality was analyzed in comparison to the general male population of East Germany, and the pertaining standardized mortality ratios (SMRs) were calculated. Results 18 510 persons in the study cohort died in the follow-up period 1960-2013. Mortality from lung cancer was 2.36 higher in the study cohort than in the general population (95% confidence interval, [2.28; 2.45]); the associated SMRs rose markedly with increasing radon exposure. Mortality from silicosis and other types of pneumoconiosis was elevated by a factor of 22.62 [21.20; 24.11], and the associated SMRs rose exponentially with increasing exposure to respirable crystalline silica. Mortality from both of these causes was still markedly elevated more than 20 years after Wismut AG had ceased its activities. Mortality from a wide range of other diseases was elevated as well, with the following SMRs: stomach cancer, 1.28 [1.17; 1.40]; liver cancer, 1.34 [1.15; 1.55]; all tumors other than lung cancer, 1.06 [1.02; 1.09]; infections, 1.18 [1.01; 1.38]; cerebrovascular diseases, 1.33 [1.26; 1.41]; and influenza/pneumonia, 1.13 [1.01; 1.27]. Mortality from a small number of other causes was found to be markedly lowered in the study cohort (mental illness, renal diseases, and nervous system diseases). The role of occupational risk factors, lifestyle differences and other reasons for the latter results is unclear. Conclusion Underground miners employed by Wismut AG displayed marked excess mortality due to silicosis/other pneumoconiosis and lung cancer. The contribution of individual occupational risk factors for these and other causes of death with increased SMR are being further investigated in analyses within the study cohort.

BACKGROUND: From 1946 to 1990, more than 400 000 people were employed by Wismut AG, a Soviet/Soviet-German corporation (German abbreviation: SAG/SDAG), in the East German states of Saxony and Thuringia. In the early years in particular, employees were exposed to large amounts of radon and respirable crystalline silica. METHODS: In a cohort of 35 204 former underground employees of Wismut AG, mortality was analyzed in comparison to the general male population of East Germany, and the pertaining standardized mortality ratios (SMRs) were calculated. RESULTS: 18 510 persons in the study cohort died in the follow-up period 1960–2013. Mortality from lung cancer was 2.36 higher in the study cohort than in the general population (95% confidence interval, [2.28; 2.45]); the associated SMRs rose markedly with increasing radon exposure. Mortality from silicosis and other types of pneumoconiosis was elevated by a factor of 22.62 [21.20; 24.11], and the associated SMRs rose exponentially with increasing exposure to respirable crystalline silica. Mortality from both of these causes was still markedly elevated more than 20 years after Wismut AG had ceased its activities. Mortality from a wide range of other diseases was elevated as well, with the following SMRs: stomach cancer, 1.28 [1.17; 1.40]; liver cancer, 1.34 [1.15; 1.55]; all tumors other than lung cancer, 1.06 [1.02; 1.09]; infections, 1.18 [1.01; 1.38]; cerebrovascular diseases, 1.33 [1.26; 1.41]; and influenza/pneumonia, 1.13 [1.01; 1.27]. Mortality from a small number of other causes was found to be markedly lowered in the study cohort (mental illness, renal diseases, and nervous system diseases). The role of occupational risk factors, lifestyle differences and other reasons for the latter results is unclear. CONCLUSION: Underground miners employed by Wismut AG displayed marked excess mortality due to silicosis/other pneumoconiosis and lung cancer. The contribution of individual occupational risk factors for these and other causes of death with increased SMR are being further investigated in analyses within the study cohort.

Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3–16.9 years) under compulsory education requirements, who were treated at the Children’s Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1–100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents’ assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6–16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect.

ObjectivesEpidemiological studies of underground miners have provided clear evidence that inhalation of radon decay products causes lung cancer. Moreover, these studies have served as a quantitative basis for estimation of radon-associated excess lung cancer risk. However, questions remain regarding the effects of exposure to the low levels of radon decay products typically encountered in contemporary occupational and environmental settings on the risk of lung cancer and other diseases, and on the modifiers of these associations. These issues are of central importance for estimation of risks associated with residential and occupational radon exposures.MethodsThe Pooled Uranium Miner Analysis (PUMA) assembles information on cohorts of uranium miners in North America and Europe. Data available include individual annual estimates of exposure to radon decay products, demographic and employment history information on each worker and information on vital status, date of death and cause of death. Some, but not all, cohorts also have individual information on cigarette smoking, external gamma radiation exposure and non-radiological occupational exposures.ResultsThe PUMA study represents the largest study of uranium miners conducted to date, encompassing 124 507 miners, 4.51 million person-years at risk and 54 462 deaths, including 7825 deaths due to lung cancer. Planned research topics include analyses of associations between radon exposure and mortality due to lung cancer, cancers other than lung, non-malignant disease, modifiers of these associations and characterisation of overall relative mortality excesses and lifetime risks.ConclusionPUMA provides opportunities to evaluate new research questions and to conduct analyses to assess potential health risks associated with uranium mining that have greater statistical power than can be achieved with any single cohort.

The interaction between the sequence of feeding of hay and concentrate and the hydrothermal processing of barley in alleviating concentrate effects on intake, and hindgut fermentation in horses was tested. Six Arabian mares (4-10 years of age, 410 ± 35 kg body weight) were used to evaluate the effects of feeding sequence (FS) and type of barley (TB) on intake, and faecal volatile fatty acids (VFA), activities of α-amylase (AA: EC 3.2.1.1), carboxymethyl cellulase (CMCase: EC 3.2.1.4), microcrystalline cellulase (MCCase: EC 3.2.1.91) and general filter paper degrading activity (FPD). Mares were offered a ration of air-dried alfalfa and concentrate (70:30 as-fed) in four subsequent periods of 14 days including 8 days of adaptation and 6 days of sampling. In each period and each meal, mares received concentrate either 30 min after (HC) or 30 min before (CH) alfalfa hay. Barley was either milled or boiled in water. Rectal samples were grabbed directly from rectum once per period. Mares subjected to CH had higher dry matter intakes than mares under HC regime. The acetate:propionate ratio (A:P ratio) in rectal content was higher with CH than HC. The AA activity was higher under CH than under HC. Mares fed boiled barley had lower rectal concentrations of VFA and propionate and a higher A:P ratio than mares fed milled barley. Furthermore, the rectal content showed a higher MCCase activity but a lower AA activity when mares were fed boiled compared with milled barley. Interactions between FS and TB were observed with respect to CMCase activity, and concentrations of propionate and valerate. In conclusion, the present results suggest that both, feeding concentrate before hay and boiling the barley, might improve the hindgut environment in Arabian mares, and that the two measures were mostly additive and sometimes even synergistic.

Background Venoarterial extracorporeal membrane oxygenation (VA-ECMO) may be life-saving in several clinical situations, but it is also one of the most invasive therapeutic procedures, with significant potential for life-threatening complications. Pulse pressure waves are typically very small or even absent at the onset of ECMO therapy, and will reappear with the improvement of cardiac function. A low pulse pressure may indicate low cardiac output due to heart failure during sustained ECMO support. A sudden loss of pulse pressure during ECMO therapy, however, may reveal complications like pericardial tamponade, hemothorax or pneumothorax. Near infrared spectroscopy (NIRS) has been shown to be useful in detecting cerebral and lower limb ischemic events during ECMO therapy and could furthermore improve differential diagnosis in the event pulsatility of the arterial pressure trace is lost. Methods We are reporting on 3 different complications of ECMO and their impact on arterial pulse pressure, arterial oxygen tension and regional tissue oxygenation measured by NIRS. Results Pericardial hematoma, overinflation of the lung, and tension pneumothorax may impede cardiac output during VA-ECMO and cause a loss of pulse pressure. Monitoring of regional tissue oxygenation using NIRS, in addition to arterial and mixed venous oxygen tension, may allow early recognition and treatment of ECMO complications. Conclusions Together with the appearance of a flat, non pulsatile arterial pressure trace as well as a reduction in mixed venous oxygen saturation the improvement of upper body rSO2 measured by NIRS enables timely recognition of complications that interfere with natural cardiac output during VA-ECMO.

OBJECTIVES Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients. Patients were followed from birth until the end of 2015. Cardiac procedures taking place at the time of the bidirectional Glenn or Fontan procedure or heart transplantation were not included. RESULTS Sixty-five of the Norwood patients (20.5%) had at least one additional surgical cardiac procedure. Nine patients (2.8%) needed open procedures prior to the Norwood operations, 11.0% had procedures in the interstage I, 3.5% in the interstage II and 9.1% of the Fontan patients had cardiac reoperations afterwards. Main indications for unplanned surgery were insufficient pulmonary perfusion and tricuspid regurgitation. Eighty-one patients (25.6%) had at least one interstage catheter intervention during follow-up mainly addressing stenosis of the pulmonary arteries, aortic arch stenosis or aortopulmonary collaterals. CONCLUSIONS The number of unplanned reoperations and interventions during staged palliation is remarkably high showing surgical peaks in the interstage I and after the Fontan procedure and an interventional peak in the interstage II. Thorough early information of the parents about possibly anticipated additional procedures is necessary.

Background The en bloc rotation of the outflow tracts is a surgical option for anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction with preserved growth potential in all tubular structures and the option for keeping the native pulmonary valve. The aim of this study was to analyze our 15-year experience with this operation. Methods A retrospective single-center study including 27 consecutive patients, who underwent the en bloc rotation between 2003 and 2019, was performed. Median age at operation was 103 days (interquartile range [IQR], 17-117 days), and median body weight was 5.2 kg (IQR, 3.66-8.4 kg). Six patients had undergone 1 to 4 previous operations. In 18 patients (66.7%), the pulmonary valve could be preserved, and in 9 patients (33.3%) a transannular patch was performed. Median follow-up was 5.7 years (IQR, 1.6-9.0 years). Results One patient died of chronic left ventricular failure during the hospital stay. Two more died after discharge: 1 of aspiration after a cerebral hemorrhage and 1 of sudden death. Five other patients required reoperations or had reinterventions: 1 ventricular septal defect closure, 1 aortic valve repair, 1 patch plasty, 2 dilatations of supravalvular pulmonary stenosis, and 1 stent into a left pulmonary artery. During the follow-up period, no gradient in the left ventricular outflow tract was found, and postoperative growth of the pulmonary valve was shown. Conclusions The en bloc rotation allows an anatomic correction of the complex transposition with growth potential in all tubular structures. Reoperations or reinterventions on the pulmonary valve or on the left ventricular outflow tract have not been necessary to date.

International audience; Background: The Pooled Uranium Miners Analysis (PUMA) study draws together information about workers employed in uranium mining in Canada, the Czech Republic, France, Germany, and the United States. We compared mortality in PUMA to expectations based upon national mortality rates.Methods: The cohort includes over 120,000 uranium miners and over 50,000 deaths. Vital status and cause of death were ascertained and standardized mortality ratios (SMRs) were computed; SMRs were tabulated by categories of attained age, calendar period of follow-up, duration of employment as a miner, and calendar period of first employment as a miner. Results: Overall, the miners in PUMA experienced all cause mortality rates near those for the general population (SMR=1.0; 95%CI: 1.0, 1.0). There were more deaths than expected due to cancer of the lung (SMR=1.8; 95%CI: 1.8, 1.9), larynx (SMR=1.1; 95%CI: 0.9, 1.3), and liver (SMR=1.1; 95%CI: 0.9, 1.2). Non-malignant respiratory disease mortality also was in excess in PUMA (SMR=1.1; 95%CI: 1.0, 1.2). The relative excesses of mortality due to lung cancer and non-malignant respiratory disease persisted into more recent decades of follow-up, tended to increase with duration of employment as a uranium miner, and tended to decrease with more recent year of first employment as a uranium miner. While lung cancer was elevated in all of the PUMA cohorts, the relative excess of lung cancer was greatest among US Colorado Plateau (SMR=4.7; 95%CI: 4.3, 5.1) and Czech miners (SMR=2.9; 95%CI: 2.8, 3.1), intermediate for US New Mexico (SMR=1.9; 95%CI: 1.6, 2.1) and German Wismut (SMR=1.9; 95%CI: 1.8, 1.9) miners, and smallest among French (SMR=1.3; 95%CI:1.2, 1.5), Canadian Ontario (SMR=1.3; 95%CI: 1.3, 1.4), and Canadian Eldorado (SMR=1.3; 95%CI: 1.2, 1.4) miners. Conclusions: PUMA provides evidence of excess mortality among uranium miners and underscores the importance of long term follow-up to identify the effects of occupational exposures.

Aortic arch reconstruction is a highly demanding procedure in congenital heart surgery. The 2 conventional options for cardiopulmonary bypass - deep hypothermic circulatory arrest or antegrade cerebral perfusion - both result in lack of perfusion of the entire or lower body. In 2003, the Children's Heart Center Linz started to use a double-arterial cannulation technique for aortic arch reconstructions in order to provide whole body perfusion during the entire operation. Our technique, demonstrated in this video tutorial, is as follows: After inserting an arterial cannula into the innominate artery, followed by venous cannulation, the cardiopulmonary bypass is started. The left pleural cavity is opened directly above the diaphragm. The inferior pulmonary ligament is transected and the descending aorta is then visualized and can be cannulated directly. Both arterial cannulas are linked via a Y-connector. The surgeons in our center have used this method in more than 450 uni- and biventricular newborns and children. There has not been a lethal incident associated with this cannulation technique; one intraoperative lesion of the descending aorta was repaired immediately. Our double-arterial cannulation technique is a simple and safe method for perfusing the brain and the lower parts of the body during aortic arch reconstruction.

Objectives To examine the risk of death from leukaemia in relation to occupational chronic low-level external and internal radiation exposure in a cohort of 58 972 former German uranium miners with mortality follow-up from 1946 to 2013. Methods The red bone marrow (RBM) dose from low-linear energy transfer (LET) (mainly external γ-radiation) and high-LET (mainly radon gas) radiation was estimated based on a job-exposure matrix and biokinetic/dosimetric models. Linear excess relative risks (ERR) and 95% CIs were estimated via Poisson regression for chronic lymphatic leukaemia (CLL) and non-CLL. Results The mean cumulative low-LET and high-LET RBM doses among the 86% radiation-exposed workers were 48 and 9 mGy, respectively. There was a positive non-significant dose-response for mortality from non-CLL (n=120) in relation to low-LET (ERR/Gy=2.18; 95% CI −0.41 to 6.37) and high-LET radiation (ERR/Gy=16.65; 95% −1.13 to 46.75). A statistically significant excess was found for the subgroup chronic myeloid leukaemia (n=31) in relation to low-LET radiation (ERR/Gy=7.20; 95% CI 0.48 to 24.54) and the subgroup myeloid leukaemia (n=99) (ERR/Gy=26.02; 95% CI 2.55 to 68.99) for high-LET radiation. The ERR/Gy tended to be about five to ten times higher for high-LET versus low-LET radiation; however, the CIs largely overlapped. Results indicate no association of death from CLL (n=70) with either type of radiation. Conclusions Our findings indicate an increased risk of death for specific subtypes from non-CLL in relation to chronic low-LET and high-LET radiation, but no such relation for CLL.

Author(s): Rage, Estelle; Do, Minh; Demers, Paul A; Fenske, Nora; Kreuzer, Michaela; Laurier, Dominique; Samet, Jon; Schubauer-Berigan, Mary; Sobotzki, Christina; Tomasek, Ladislav; Zablotska, Lydia; Richardson, David

Background Neo-aortic root dilatation accounts for the majority of reoperations needed after the Ross procedure with implantation of the pulmonary autograft as complete root replacement. This study evaluates early results of external prosthetic reinforcement of the autograft. Methods From July 2015 to October 2017, 16 adolescent and adult patients received a Ross procedure at our department by this technique. A congenital-dysplastic valve was present in 13 patients, including 9 patients with a bicuspid aortic valve. Clinical and echocardiographic follow-up is complete with a mean duration of 19.7 ± 5.8 months. Results The mean age at operation was 27.1 ± 16.1 years. Mean aortic cross-clamping time was 102 ± 39 minutes. No bleeding complication occurred. The median stay on the intensive care unit was 2 days. In-hospital mortality was 0%. All patients were discharged with no or trivial aortic regurgitation. In one patient both the autograft and homograft were replaced because of endocarditis 3 months after the primary operation, leading to 93.8% freedom from reoperation at 2 years. There were no late deaths during the study period. The latest echocardiography confirmed absence of aortic regurgitation grade >I in all patients. Neo-aortic root diameters remained stable during follow-up. Conclusion The presented modification of the Ross procedure does not prolong ischemia time, and can be performed with a low operative morbidity and mortality and an excellent early valve function.

International audience; MELODI (Multidisciplinary European Low Dose Initiative) is a European radiation protection research platform with focus on research on health risks after exposure to low-dose ionising radiation. It was founded in 2010 and currently includes 44 members from 18 countries. A major activity of MELODI is the continuous development of a long-term European Strategic Research Agenda (SRA) on low-dose risk for radiation protection. The SRA is intended to identify priorities for national and European radiation protection research programs as a basis for the preparation of competitive calls at the European level. Among those key priorities is the improvement of health risk estimates for exposures close to the dose limits for workers and to reference levels for the population in emergency situations. Another activity of MELODI is to ensure the availability of European key infrastructures for research activities, and the long-term maintenance of competences in radiation research via an integrated European approach for training and education. The MELODI SRA identifies three key research topics in low dose or low dose-rate radiation risk research (1) dose and dose rate dependence of cancer risk, (2) radiation-induced non-cancer effects and (3) individual radiation sensitivity. The research required to improve the evidence base for each of the three key topics relates to three research lines (1) research to improve understanding of the mechanisms contributing to radiogenic diseases, (2) epidemiological research to improve health risk evaluation of radiation exposure and (3) research to address the effects and risks associated with internal exposures, differing radiation qualities and inhomogeneous exposures. The full SRA and associated documents can be downloaded from the MELODI website (http//www.melodi-online.eu/sra.html). © 2017, The Author(s).

Epidemiological miner cohort data used to estimate lung cancer risks related to occupational radon exposure often lack cohort-wide information on exposure to tobacco smoke, a potential confounder and important effect modifier. We have developed a method to project data on smoking habits from a case-control study onto an entire cohort by means of a Monte Carlo resampling technique. As a proof of principle, this method is tested on a subcohort of 35,084 former uranium miners employed at the WISMUT company (Germany), with 461 lung cancer deaths in the follow-up period 1955-1998. After applying the proposed imputation technique, a biologically-based carcinogenesis model is employed to analyze the cohort's lung cancer mortality data. A sensitivity analysis based on a set of 200 independent projections with subsequent model analyses yields narrow distributions of the free model parameters, indicating that parameter values are relatively stable and independent of individual projections. This technique thus offers a possibility to account for unknown smoking habits, enabling us to unravel risks related to radon, to smoking, and to the combination of both.

Purpose Long-term health risks of occupational exposures to uranium processing were examined to better understand potential differences with uranium underground miners and nuclear reactor workers. Methods A cohort study of mortality of workers from Port Hope, Canada (1950–1999) and Wismut, Germany (1946–2008) employed in uranium milling, refining, and processing was conducted. Poisson regression was used to evaluate the association between cumulative exposures to radon decay products (RDP) and gamma-rays and causes of death potentially related to uranium processing. Results The pooled cohort included 7431 workers (270,201 person-years of follow-up). Mean RDP exposures were lower than in miners while gamma-ray doses were higher than in reactor workers. Both exposures were highly correlated (weighted rho = 0.81). Radiation risks of lung cancer and cardiovascular diseases (CVD) in males were increased but not statistically significant and compatible with risks estimated for miners and reactor workers, respectively. Higher RDP-associated CVD risks were observed for exposures 5–14 years prior to diagnosis compared to later exposures and among those employed

OBJECTIVES Double-arterial cannulation enables cerebral perfusion and lower body perfusion during aortic arch reconstruction. The aim of this study was to analyse and report our experience of using this cannulation and perfusion technique on paediatric patients. METHODS A retrospective single-centre study was carried out on 407 consecutive paediatric patients who underwent an aortic arch reconstruction under double-arterial cannulation between 2003 and 2015. The median age of the patients at surgery was 8 (range 2-5570) days, and body weight was 3.3 (range 1.8-60) kg. All operations were performed through standard median sternotomy. One arterial cannula was inserted into the innominate artery and the second one into the supradiaphragmatic descending aorta. Primary end points were 30-day mortality, acute renal failure requiring dialysis and time until lactate level decreased to ≤2 mmol/l postoperatively. RESULTS We found an in-hospital mortality of 8.6%. Lethal incident was not associated with the cannulation method, and 1 intraoperative lesion of the descending aorta could be repaired immediately. The median lactate level of the patients on arrival at the intensive care unit was 3.5 mmol/l [quartile (Q)1: 2.3-Q3: 4.7] and creatinine was 0.48 mg/100 ml (Q1: 0.40-Q3: 0.58). The longest duration until the lactate level decreased to ≤ 2 mmol/l was found in the group of 264 univentricular patients (median 11 h, Q1: 6-Q3: 24). Seven (1.7%) patients of the whole cohort required peritoneal dialysis postoperatively. CONCLUSIONS Double-arterial cannulation is a simple and safe method for perfusing the brain and the lower parts of the body during aortic arch reconstruction. Perioperative survival and freedom from procedure-related complications in this demanding patient population are encouraging.

It is still not fully understood whether and how factors such as time, age and smoking modify the relationship between lung cancer and radon at low exposures and exposure rates. Improved knowledge is necessary for the dose conversion of radon in working level month (WLM) into effective dose, as currently discussed by the International Commission on Radiological Protection (ICRP). An update of the German uranium miner cohort study (n = 58,974 men) with a 10-year extension of mortality follow-up (1946-2013) was used to further examine this issue. Internal Poisson regression was applied to estimate the excess relative risk (ERR) for lung cancer mortality per unit of cumulative radon exposure in WLM with exponential time-related effect modifiers. In the full cohort restricted to100 WLM the estimated overall ERR/WLM was 0.006 [95% confidence interval (CI): 0.003; 0.010] based on 1,254 lung cancer deaths and 1,620,190 person-years at risk. Both age at and time since exposure turned out to be important modifiers of the ERR/WLM and were included in the final model. Here, the ERR/WLM centered on age at exposure of 30 years, and 20 years since exposure was 0.016 (95% CI: 0.008; 0.028). This value decreased statistically significantly by approximately 40% and 60% for each 10-year increase in age at exposure and time since exposure, respectively. The joint effect of smoking and radon exposure was investigated in the sub-cohort of miners hired in 1960 or later, which includes data on smoking status. The centered ERR/WLM was slightly higher for non/light smokers compared to moderate/heavy smokers (0.022 versus 0.013). The current findings provide evidence for an increased lung cancer risk at low radon exposures or exposure rates that is modified by age and time. The observed risk is lower, but statistically compatible to those of other miner studies at low exposures or exposure rates. These findings reject an additive- and support a sub- to (supra-) multiplicative interaction between smoking and radon.

Objectives Patients with severe left ventricular outflow tract obstruction often suffer from impaired left ventricular function, endocardial fibroelastosis and borderline-sized structures. The early Ross-Konno operation can offer complete repair due to outflow tract stenosis and enable the functional recovery and growth of small structures. Methods Between 2008 and March 2017, 44 early Ross-Konno procedures were performed at our centre. Thirty-five patients were neonates, and 9 were infants less than 3 months of age. A retrospective single-centre investigation was carried out analysing early and late deaths, postoperative complications and reoperations during the follow-up period. Potential prognostic influence factors as previous fetal intervention, associated lesions as presence of a VSD or hypoplastic aortic arch or severe endocardial fibroelastosis were examined. Results The in-hospital mortality rate was 7% (3 of 44), and the late mortality rate was 2%. There were no deaths in the group with ventricular septal defects (9 cases), and there was 1 death in the group with critical aortic stenosis without arch repair (1 of 24; 4%). The most deaths occurred in patients with critical aortic stenosis and aortic arch repair (3 of 11; 27%) (P = 0.012). Fifteen patients with foetal intervention had a mid-term survival rate of 87%. Reoperations were necessary in 19 of 40 surviving patients during a mean follow-up period of 5.9 years. Conclusions The early Ross-Konno procedure can enable biventricular repair even in borderline left ventricles with good long-term outcome. Thus far, there were no reoperations at the level of the autograft or the left ventricular outflow tract in our cohort. The highest operative risk was observed in patients with critical aortic stenosis and aortic arch hypoplasia.

Background A causal association between exposure to radon and its progeny and lung cancer is well established. However, a better knowledge on effect modification by time, age and exposure rate and on risk at low exposures or exposure rates is of high importance. Method We analysed 58 974 male uranium miners of the German Wismut cohort with a mortality follow-up from 1946 to 2013 and a sub-cohort of 26 765 miners hired after 1960 characterised by radon values at low exposure-rates. Internal Poisson regression was applied to estimate the Excess Relative Risk (ERR) per unit of cumulative radon exposure in Working Level Months (WLM) with exponential time-related effect modifiers. Results In the full cohort the crude ERR/WLM was 0.0019 (95% confidence interval (CI): 0.0017;0.0022) based on 3.947 lung cancer deaths. Age at median exposure, time since median exposure, and exposure-rate were strong effect modifiers. Taking them into account led to an estimate of 0.0067 (95%CI: 0.0052;0.0087) for an age at median exposure of 30 years, a time since median exposure of 20 years, and an exposure-rate of 3 WL (strong inverse exposure-rate effect). In the 1960+ sub-cohort, the crude ERR/WLM was 0.0111 (95%CI: 0.0064;0.0173) based on 495 lung cancer deaths. In this relatively young sub-cohort, time since median exposure was also a strong modifier, leading to an ERR/WLM of 0.0165 (95%CI: 0.0083;0.0247) at time since median exposure of 20 years. Conclusion The present findings provide evidence for an increased lung cancer risk also at low exposures or exposure rates among miners.

Purpose Long-term health risks of occupational exposures to uranium processing were examined to better understand potential differences with uranium underground miners and nuclear reactor workers. Methods Two cohort studies of workers from Port Hope, Canada (1950–1999) and Wismut, Germany (1946–2008) employed in uranium milling, refining, and processing were pooled. Poisson regression was used to evaluate the association between cumulative exposures to radon decay products (RDP) and gamma-rays and causes of death potentially related to uranium processing. Results The pooled cohort included 7431 workers (2 70 201 person-years of follow-up). Mean RDP exposures were lower than in miners while gamma-ray doses were higher than in reactor workers. Both exposures were highly correlated (weighted r=0.89). Risks of lung cancer and cardiovascular diseases (CVD) in males were increased but not significant and compatible with risks estimated for miners and reactor workers, respectively. Higher RDP-associated CVD risks were observed for exposures 5–14 years prior to diagnosis compared to later exposures and among those employed Conclusions In the largest study of uranium processing workers to systematically examine radiation risks of multiple outcomes from RDP exposures and gamma-rays, estimated radiation risks were compatible with risks reported for uranium miners and nuclear reactor workers. Continued follow-up and pooling with other cohorts of uranium processing workers are necessary for future comparisons with other workers of the nuclear fuel cycle.

Epidemiological studies of uranium miners helped to establish radon as a human carcinogen. However, radon remains a leading occupational cause of cancer mortality, and many workers are exposed to radon at levels at which excess risk of lung cancer has been observed in occupational cohort studies. Prior pooled studies of underground miners provided important quantitative estimates of radon-associated lung cancer risk. Recently, efforts have been undertaken to strengthen uranium miner studies to address contemporary occupational safety concerns. New cohorts of underground miners have been enumerated, existing cohorts have been expanded, and follow-up of the major cohorts of miners has been extended substantially. An international collaborative study has been undertaken to combine information from many of the world’s most informative cohort studies of uranium miners; the combined study cohort encompasses more than 1 00 000 miners. This talk will describe the major themes of this project, the goals of the collaborators and the challenges that we have encountered to-date. We will describe similarities and differences between findings from these key cohorts and identify some major gaps in current knowledge about radon’s effects on human health. Finally, we will discuss how international collaborative studies can strengthen our understanding of risks associated with occupational and environmental radon exposures.

Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6.3–16.9 years) with hypoplastic left heart syndrome, who were treated at the Children’s heart Center Linz between 1997 and 2009, (n = 74), were surveyed in reference to cognitive achievements. 43 patients were examined prospectively by psychologists using the Wechsler intelligence scale for children IV in order to determine the respective total intelligence quotient index for each child’s developmental stage. The mean index was 84.5 (percentile rank 26.4). The statistical spread and standard deviation ranged from a minimum of 40 to a maximum of 134 ± 20.8. The results for verbal comprehension, perceptual reasoning, and processing speed corresponded with total index results and were thus lower than the mean value of the normative values. The assessment of working memory showed results in the average. Prenatal diagnosis, type of lung perfusion, anatomy, and various cerebral perfusion techniques did not significantly affect the cognitive results of the patients. The results show that hypoplastic left heart syndrome patients can be successfully tutored formally as well as personally in cognitive areas, although when compared to healthy children, they showed lower results for intellectual area parameters.

The Wismut cohort is currently the largest single study on the health risks associated with occupational exposures to ionising radiation and dust accrued during activities related to uranium mining. The cohort has ∼59 000 male workers, first employed between 1946 and 1989, at the Wismut Company in Germany. The main effect is a statistically significant increase in mortality from lung cancer with both increasing cumulative radon exposure and silica dust exposure. Risks for cancers of the extrathoracic airways, all extra-pulmonary cancers and cardiovascular diseases associated with radiation exposures have been evaluated. Cohort mortality rates for some other cancer sites, stomach and liver, are statistically significantly increased in relation to the general population, but not statistically significantly related to occupational exposures. No associations between leukaemia mortality and occupational doses of ionising radiation were found.

Objective To quantify the relationship between death from non-malignant respiratory diseases (NMRD) and exposure to silica dust or radon in a cohort of 58 690 former German uranium miners. Methods In the follow-up period from 1946 to 2008, a total of 2336 underlying deaths from NMRDs occurred, including 715 deaths from chronic obstructive pulmonary diseases (COPD) and 975 deaths from silicosis or other pneumoconiosis. Exposure to respirable crystalline silica and radon was individually assessed by means of a comprehensive job-exposure matrix. Risk analyses were based on a linear Poisson regression model with the baseline stratified by age, calendar year and duration of employment. Results There was no increase in risk of death from COPDs or any other NMRDs in relation to cumulative exposure to silica (mean=5.9, max=56 mg/m 3 -years), except in the group of deaths from silicosis or other pneumoconiosis. Here, a strong non-linear increase in risk was observed. Cumulative radon exposure (mean=280; max=3224 Working Level Months) was not related to death from COPDs or any other NMRDs. Conclusions The present findings do not indicate a relationship between mortality from COPD with silica dust or radon. However, validity of cause of death and lack of control for smoking remain potential sources of bias.

Recent epidemiology studies highlighted the detrimental health effects of exposure to low dose and low dose rate ionizing radiation (IR): nuclear industry workers studies have shown increased leukaemia and solid tumour risks following cumulative doses of