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| 2. |
- Fricke, Katrin, et al.
(författare)
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Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden
- 2022
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Ingår i: Journal of the American Heart Association. - : John Wiley & Sons. - 2047-9980. ; 11:7
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation. METHODS AND RESULTS: This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic-mitral atresia, aortic atresia-mitral stenosis (AA-MS), and aortic-mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob-LV), miniaturized and slit-like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA-MS (35%) was associated with both mortality (all other subtypes versus AA-MS: interstage-I: hazard ratio [HR], 2.7; P=0.006; overall: HR, 2.2; P=0.005) and MAEs (HR, 2.4; P=0.0009). Glob-LV (57%), noticed in all patients with AA-MS, 61% of patients with aortic stenosis-mitral stenosis, and 19% of patients with aortic atresia-mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob-LV: interstage-I: HR, 4.5; P=0.004; overall: HR, 3.4; P=0.0007) and MAEs (HR, 2.7; P=0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (P>0.15). Patients with AA-MS (35%) or Glob-LV (38%) palliated with a Blalock-Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA-MS: HR, 2.6; P=0.04; Glob-LV: HR, 2.1; P=0.03). CONCLUSIONS: Glob-LV and AA-MS are independent morphological risk factors for adverse short-and long-term outcome, especially if a Blalock-Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.
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| 4. |
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| 5. |
- Ambrosi, Aurelie, et al.
(författare)
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Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern
- 2012
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Ingår i: Annals of the Rheumatic Diseases. - London : BMJ Publishing Group. - 0003-4967 .- 1468-2060. ; 71:3, s. 334-340
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Tidskriftsartikel (refereegranskat)abstract
- Objective Congenital heart block may develop in the fetuses of Ro/SSA-positive and La/SSB-positive mothers. Recurrence rates of only 10-20% despite persisting maternal antibodies indicate that additional factors are critical for the establishment of heart block. The authors investigated the influence of other maternal and fetal factors on heart block development in a Swedish population-based cohort. less thanbrgreater than less thanbrgreater thanMethods The influence of fetal gender, maternal age, parity and time of birth on heart block development was analysed in 145 families, including Ro/La-positive (n=190) and Ro/La-negative (n=165) pregnancies. less thanbrgreater than less thanbrgreater thanResults There was a recurrence rate of 12.1% in Ro/La-positive women, and no recurrence in Ro/La-negative women. Fetal gender and parity did not influence the development of heart block in either group. Maternal age in Ro/La-positive pregnancies with a child affected by heart block was, however, significantly higher than in pregnancies resulting in babies without heart block (pandlt;0.05). Seasonal timing of pregnancy influenced the outcome. Gestational susceptibility weeks 18-24 occurring during January-March correlated with a higher proportion of children with heart block and lower vitamin D levels during the same period in a representative sample of Swedish women and a corresponding higher proportion of children with heart block born in the summer (pandlt;0.02). Maternal age or seasonal timing of pregnancy did not affect the outcome in Ro/La-negative pregnancies. less thanbrgreater than less thanbrgreater thanConclusion This study identifies maternal age and seasonal timing of pregnancy as novel risk factors for heart block development in children of Ro/La-positive women. These observations may be useful for counselling when pregnancy is considered.
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| 8. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
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Continuing pregnancy following a prenatal diagnosis of a cardiac defect: What support do parents need?
- 2015
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Ingår i: Cardiology 2015. 18th Annual Update on Pediatroc and Congenital Cardiovascular Disease. Challenges and Dilemmas. Feb 11-15, 2015. Scottsdale, Arizona, US..
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Konferensbidrag (övrigt vetenskapligt/konstnärligt)abstract
- Purpose To explore pregnant women´s/couples’ experiences of counseling and need for support during continued pregnancy following a prenatal diagnosis of congenital heart disease (CHD). Conceptual framework Couples choosing continued pregnancy need support from the time of prenatal diagnosis until delivery. Method Design: Qualitative study, using in-depth interviews 4-8 weeks after prenatal diagnosis. Setting: A tertiary center fetal cardiology unit in Sweden Sample: 12 pregnant women and their partners, consecutively recruited after a prenatal diagnosis of an isolated and significant cardiac defect in their fetus. Data analysis: Qualitative content analysis. Major findings The analysis resulted in four themes: Making the decision: Short waiting time for specialist evaluation together with clear, honest and straightforward information was essential. The importance of knowledge: Parents called for written information together with a high-quality regulated website with information about CHD. The importance of support: Continued and easy access, throughout pregnancy, to health care professionals, including a pediatric specialist nurse, was important. Other parents with similar experiences and social media were also valuable sources of support. Future and daily life: Practical and economical issues during the hospital stay and the initial period after the hospital stay were common concerns. Conclusion The results provided valuable knowledge of how to improve information and support during pregnancy. Short waiting time from first suspicion to definitive diagnosis and continued support throughout pregnancy emphasizing the role of the pediatric cardiology specialist nurse was important. Web-based information was warranted Clinical implications These results provide important information for a future intervention study of a structured follow-up program in collaboration between antenatal- and pediatric cardiac caregivers.
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| 9. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
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Parental reactions, distress, and sense of coherence after prenatal versus postnatal diagnosis of complex congenital heart disease
- 2019
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Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. ; 29:11, s. 1328-1334
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: A diagnosis of congenital heart disease (CHD) in offspring triggers psychological distress in parents. Results of previous studies have been inconsistent regarding the psychological impact of a prenatal versus a postnatal diagnosis. The aim of this study was to evaluate the influence of the time of diagnosis on levels of parental distress. Methods: Pregnant women and their partners with a fetus diagnosed with complex CHD, parents of children with postnatally diagnosed CHD, and pregnant women and their partners with uncomplicated pregnancies were invited to participate. Data were collected during pregnancy and 2–6 months after delivery using the Hospital Anxiety and Depression Scale, sense of coherence, life satisfaction, and Dyadic Adjustment Scale. Results: During pregnancy, the prenatal group scored lower sense of coherence compared to controls (p=0.044). Postnatally the prenatal group scored lower on sense of coherence compared to the postnatal group and controls (p=0.001; p=0.001). Postnatally, the prenatal and postnatal groups had higher levels of anxiety compared to controls (p=0.025; p=0.0003). Life satisfaction was lower in the prenatal group compared to that in the postnatal group and in controls (p=0.000; p=0.0004). Conclusion: Parents with a prenatal diagnosis of CHD in offspring report a low sense of coherence already during pregnancy which decreased further at follow-up. The same group reported a lower satisfaction with life compared to parents of a child with postnatal diagnosis of CHD and parents of a healthy child. This motivates further efforts to improve counselling and support during pregnancy and for parents after a prenatal diagnosis.
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| 10. |
- Bratt, Ewa-Lena, 1970, et al.
(författare)
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Parent’s experiences of counselling and their need for support following a prenatal diagnosis of congenital heart disease - a qualitative study in a Swedish context
- 2015
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Ingår i: BMC Pregnancy and Childbirth. - : Springer Science and Business Media LLC. - 1471-2393. ; 15
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Tidskriftsartikel (refereegranskat)abstract
- Background: Prenatal screening for foetal cardiac abnormalities has been increasingly practiced in Sweden during the last 25 years. A prenatal diagnosis may have medical benefits but may also cause sustained parental psychological distress. The aim of this study was to explore pregnant women’s, and their partner’s, experiences of counselling and need for support during continued pregnancy following a prenatal diagnosis of a cardiac defect. A second aim was to use this information to propose a structured follow-up programme for continued support after the first counselling. Method: Design: Qualitative study, using interviews performed 5–9 weeks after a prenatal diagnosis of congenital heart disease. Setting: A tertiary foetal cardiology unit in Sweden Sample: Six pregnant women and their 6 partners, consecutively recruited after a prenatal diagnosis of an isolated and significant cardiac defect. Data analysis: Qualitative content analysis. Results: The analysis resulted in three themes. 1/ Counselling and making a decision - the importance of knowledge and understanding: Short waiting time for specialist evaluation together with clear and straightforward information was essential. Parents called for written information together with a high-quality website with relevant information about congenital heart disease. 2/ Continued support during pregnancy: Continued and easy access to health care professionals, including a paediatric specialist nurse, throughout pregnancy, was important. Contact with couples with similar experiences and social media were also considered valuable sources of support. 3/ Next step – the near future: Practical and economical issues during the postnatal hospital stay and the initial period following the hospital stay were common concerns. Conclusions: The following aspects should be considered in a structured follow up program during pregnancy after a prenatal diagnosis of CHD; written information, access to a safe web-site with information of high quality in their native language, support from parents with similar experiences and continued contact with a specialist liaison nurse with experience of paediatric cardiology.
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| 11. |
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| 12. |
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| 13. |
- Clausen, Henning, et al.
(författare)
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Newborn Screening for High-Risk Congenital Heart Disease by Dried Blood Spot Biomarker Analysis.
- 2024
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Ingår i: JAMA Network Open. - 2574-3805. ; 7:6
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Tidskriftsartikel (refereegranskat)abstract
- Importance Congenital heart disease (CHD) is the most common human organ malformation, affecting approximately 1 of 125 newborns globally.Objectives Assessing the performance of 2 diagnostic tests using minimal amounts of dried blood spots (DBS) to identify high-risk CHD compared with controls in a Swedish cohort of neonates.Design, Setting, and Participants This diagnostic study took place in Sweden between 2019 and 2023 and enrolled full-term babies born between 2005 and 2023. All cases were identified through centralized pediatric cardiothoracic surgical services in Lund and Gothenburg, Sweden. Controls were followed up for 1 year to ensure no late presentations of high-risk CHD occurred. Cases were verified through surgical records and echocardiography.Exposure High-risk CHD, defined as cases requiring cardiac surgical management during infancy due to evolving signs of heart failure or types in which the postnatal circulation depends on patency of the arterial duct. Using 3-μL DBS samples, automated quantitative tests for NT-proBNP and interleukin 1 receptor-like 1 (IL-1 RL1; formerly known as soluble ST2) were compared against established CHD screening methods.Main Outcomes and Measures Performance of DBS tests to detect high-risk CHD using receiver operating characteristic curves; Bland-Altman and Pearson correlation analyses to compare IL-1 RL1 DBS with plasma blood levels.Results A total of 313 newborns were included (mean [SD] gestational age, 39.4 [1.3] weeks; 181 [57.8%] male). Mean (SD) birthweight was 3495 (483) grams. Analyzed DBS samples included 217 CHD cases and 96 controls. Among the CHD cases, 188 participants (89.3%) were high-risk types, of which 73 (38.8%) were suspected prenatally. Of the 188 high-risk cases, 94 (50.0%) passed pulse oximetry screening and 36 (19.1%) were initially discharged after birth without diagnoses. Combining NT-proBNP and IL-1 RL1 tests performed well in comparison with existing screening methods and enabled additional identification of asymptomatic babies with receiver operating characteristic area under the curve 0.95 (95% CI, 0.93-0.98).Conclusions and relevance In this diagnostic study, NT-proBNP and IL-1 RL1 DBS assays identified high-risk CHD in a timely manner, including in asymptomatic newborns, and improved overall screening performance in this cohort from Sweden. Prospective evaluation of this novel approach is warranted.
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| 14. |
- de-Wahl Granelli, Anne, 1970, et al.
(författare)
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Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns.
- 2009
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Ingår i: BMJ (Clinical research ed.). - 1468-5833. ; 338
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: To evaluate the use of pulse oximetry to screen for early detection of life threatening congenital heart disease. DESIGN: Prospective screening study with a new generation pulse oximeter before discharge from well baby nurseries in West Götaland. Cohort study comparing the detection rate of duct dependent circulation in West Götaland with that in other regions not using pulse oximetry screening. Deaths at home with undetected duct dependent circulation were included. SETTING: All 5 maternity units in West Götaland and the supraregional referral centre for neonatal cardiac surgery. PARTICIPANTS: 39,821 screened babies born between 1 July 2004 and 31 March 2007. Total duct dependent circulation cohorts: West Götaland n=60, other referring regions n=100. MAIN OUTCOME MEASURES: Sensitivity, specificity, positive and negative predictive values, and likelihood ratio for pulse oximetry screening and for neonatal physical examination alone. RESULTS: In West Götaland 29 babies in well baby nurseries had duct dependent circulation undetected before neonatal discharge examination. In 13 cases, pulse oximetry showed oxygen saturations
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| 15. |
- de-Wahl Granelli, Anne, 1970, et al.
(författare)
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Screening for duct-dependant congenital heart disease with pulse oximetry: a critical evaluation of strategies to maximize sensitivity
- 2005
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Ingår i: Acta Paediatr. - 0803-5253. ; 94:11, s. 1590-1596
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Tidskriftsartikel (refereegranskat)abstract
- AIM: To evaluate the feasibility of detecting duct-dependent congenital heart disease before hospital discharge by using pulse oximetry. DESIGN: Case-control study. SETTING: A supra-regional referral centre for paediatric cardiac surgery in Sweden. PATIENTS: 200 normal term newborns with echocardiographically normal hearts (median age 1.0 d) and 66 infants with critical congenital heart disease (CCHD; median age 3 d). METHODS: Pulse oximetry was performed in the right hand and one foot using a new-generation pulse oximeter (NGoxi) and a conventional-technology oximeter (CToxi). RESULTS: With the NGoxi, normal newborns showed a median postductal saturation of 99% (range 94-100%); intra-observer variability showed a mean difference of 0% (SD 1.3%), and inter-observer variability was 0% (SD 1.5%). The CToxi recorded a significantly greater proportion of postductal values below 95% (41% vs 1%) in the normal newborns compared with NGoxi (p<0.0001). The CCHD group showed a median postductal saturation of 90% (45-99%) with the NGoxi. Analysis of distributions suggested a screening cut-off of <95%; however, this still gave 7/66 false-negative patients, all with aortic arch obstruction. Best sensitivity was obtained by adding one further criterion: saturation of <95% in both hand and foot or a difference of >+/-3% between hand and foot. These combined criteria gave a sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%. CONCLUSION: Systematic screening for CCHD with high accuracy requires a new-generation oximeter, and comparison of saturation values from the right hand and one foot substantially improves the detection of CCHD.
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| 16. |
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| 17. |
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| 18. |
- Jashari, Haki, et al.
(författare)
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Coarctation repair normalizes left ventricular function and aorto-septal angle in neonates
- 2017
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Ingår i: Congenital Heart Disease. - : Computers, Materials and Continua (Tech Science Press). - 1747-079X .- 1747-0803. ; 12:2, s. 218-225
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Tidskriftsartikel (refereegranskat)abstract
- Background and aims: Patients with coarctation of the aorta (CoA) have increased left ventricular (LV) afterload that has been shown to impact the LV and ascending aortic function. We aimed to examine the effect of coarctation on LV function and aorto-septal angle (AoSA) before and after surgical repair.Methods: We retrospectively studied 21 patients with surgically repaired CoA at a median age of 9 (2-53) days at three time points: (1) just before intervention, (2) at short-term follow-up, and (3) at medium-term follow-up after intervention. AoSA was measured from the parasternal long axisview, at three time points during the cardiac cycle: (1) end diastole, (2) beginning of systole, and (3) at peak ejection in the descending aorta. In addition to conventional LV structure and function, global longitudinal strain, and strain rate were measured using STE technique and Tomtec soft-ware. Three groups of age matched healthy children served as controls at each time point.Results: AoSA was significantly wider before intervention, in particular at peak ejection in the descending aorta (1448 6 6.48 vs. 1368 6 4.18; P < .0001), and correlated with CoA pressure gradi-ent. After intervention, AoSA normalized and significantly correlated with the increase of LV cavity function and overall LV deformation parameters.Conclusions: AoSA is abnormally wide in neonates with CoA and is associated with severity ofobstruction, LV dysfunction and compromised LV global deformation.
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| 19. |
- Jashari, Haki, et al.
(författare)
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Persistent reduced myocardial deformation in neonates after CoA repair
- 2016
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Ingår i: International Journal of Cardiology. - : Elsevier. - 0167-5273 .- 1874-1754. ; 221, s. 886-891
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Tidskriftsartikel (refereegranskat)abstract
- INTRODUCTION: Surgical repair of coarctation of the aorta (CoA) is a safe procedure in children, however the condition is known for its potential recurrence and other related complications. The available evidence shows abnormal intrinsic properties of the aorta in CoA, thus suggesting additional effect, even after CoA repair, on left ventricular (LV) function. Accordingly, we sought to obtain a better understanding of LV myocardial mechanics in very early-corrected CoA using two-dimensional STE.METHODS AND RESULTS: We retrospectively studied 21 patients with corrected CoA at a median age of 9 (2-53) days at three time points: 1) just before intervention, 2) at short-term follow-up and 3) at medium-term follow-up after intervention and compared them with normal values. Speckle tracking analysis was conducted via vendor independent software, Tomtec. After intervention, LV function significantly improved (from -12.8±3.9 to -16.7±1.7; p<0.001), however normal values were not reached even at medium term follow-up (-18.3±1.7 vs. -20±1.6; p=0.002). Medium term longitudinal strain correlated with pre intervention EF (r=0.58, p=0.006). Moreover, medium term subnormal values were more frequently associated with bicuspid aortic valve (33.3% vs. 66.6%; p<0.05).CONCLUSION: LV myocardial function in neonates with CoA can be feasibly evaluated and followed up by speckle tracking echocardiography. LV subendocardial dysfunction however, remains in early infancy coarctation long after repair. Long-term follow-up through adulthood using myocardial deformation measurements should shed light on the natural history and consequences of this anomaly.
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| 20. |
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| 21. |
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| 22. |
- Lannering, Katarina, et al.
(författare)
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Low false-positive rate of perfusion index as a screening tool for neonatal aortic coarctation
- 2021
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Ingår i: Acta Paediatrica, International Journal of Paediatrics. - : Wiley. - 0803-5253 .- 1651-2227. ; 110:6, s. 1788-1794
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Tidskriftsartikel (refereegranskat)abstract
- © 2020 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica Aim: Adding perfusion index (PI) to pulse oximetry screening (POS) may increase neonatal detection of CoA (aortic coarctation). A cut-off <0.7% has been suggested but is associated with a high rate of false positives. We aimed to evaluate the specificity of PI when using repeated instead of single measurements. Methods: A pilot study was conducted in 50 neonates. PI was recorded in right hand and a foot by pulse oximeter. If PI was <0.7%, the measurement was immediately repeated up to 3 times. If all three measurements were <0.7% in hand and/or foot the screen was positive and echocardiography was performed. There were 3/50 false-positive screens. The protocol was therefore modified requiring 30min intervals between measurements. Results: An additional 463 neonates were included using the modified protocol at a median age of 18h. There were no false positives. The only neonate with CoA had a negative screen (PI hand 1.2% and foot 0.8%). The measurement required on average an extra 3min and 30s compared with POS only. Conclusion: The false-positive rate of PI was reduced by using repeated PI measurements. The sensitivity for CoA using this protocol should be evaluated in large-scale prospective studies.
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| 23. |
- Lannering, Katarina, et al.
(författare)
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Screening for Critical Congenital Heart Defects in Sweden
- 2023
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Ingår i: PEDIATRICS. - : American Academy of Pediatrics (AAP). - 0031-4005 .- 1098-4275. ; 152:4
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVES Early diagnosis of critical congenital heart defects (CCHD) improves survival. We evaluated the relative contributions of prenatal ultrasound, neonatal pulse oximetry screening (POS), and neonatal physical examination (NPE) to the early detection (before discharge) of CCHD in the context of increasing prenatal detection, and POS being a national standard since 2013.METHODS Retrospective, nationwide population-based study. All full-term live-born infants with CCHD in Sweden between 2014 and 2019 were included. CCHD was defined as a congenital heart defect requiring surgery or catheter-based intervention or resulting in death within 28 days of birth.RESULTS Of 630 infants, 89% were diagnosed before discharge or death, 42% prenatally, 11% from early symptoms, 23% by POS, and 14% from NPE after a negative POS. Four (0.6%) died undiagnosed before discharge and 64/630 (10%) were discharged undiagnosed, with 24/64 being readmitted with circulatory failure and causing 1 preoperative death. Coarctation was the most prevalent CCHD (N = 184), 25% of whom were detected prenatally (12% by POS and 29% by NPE). Two died undiagnosed before discharge and 30% were discharged undiagnosed. Transposition was the second most common defect (N = 150) and 43% were detected prenatally (33% by POS, 1 by NPE) and 2 died undiagnosed before POS. None was discharged undiagnosed.CONCLUSIONS POS and NPE remain important for the early detection of CCHD complementing prenatal ultrasound screening. Nevertheless, 1 in 10 with CCHD leaves the hospital without a diagnosis, with coarctation being the predominant lesion. Future research on CCHD screening should have a particular focus on this cardiac defect.
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| 24. |
- Lannering, Katarina, et al.
(författare)
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Screening for critical congenital heart defects in sweden
- 2024
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Ingår i: Obstetrical and Gynecological Survey. - : Lippincott Williams & Wilkins. - 0029-7828 .- 1533-9866. ; 79:4, s. 185-187
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Tidskriftsartikel (refereegranskat)abstract
- (Abstracted from Pediatrics 2023;152:e2023061949 Critical congenital heart defects (CCHDs) affect between 1 and 3 of every 1000 live-born infants and require intervention in the short term after birth. Early identification of affected infants contributes to significantly to better outcomes in both the short- and long-term.
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| 25. |
- Matsui, Hikoro, et al.
(författare)
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Morphological and physiological predictors of fetal aortic coarctation.
- 2008
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Ingår i: Circulation. - 1524-4539. ; 118:18, s. 1793-801
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Prenatal diagnosis of aortic coarctation suffers from high false-negative rates at screening and poor specificity. METHODS AND RESULTS: This retrospective study tested the applicability of published aortic arch and ductal Z scores (measured just before the descending aorta in the 3-vessel and tracheal view) and their ratio on 200 consecutive normal controls at a median of 22+/-0 gestational weeks (range, 15+/-4 to 38+/-4 weeks). Second, this study tested the ability of serial Z scores to distinguish fetuses with coarctation within a cohort with ventricular and/or great arterial disproportion detected at screening or fetal echocardiography. Third, it evaluated the diagnostic significance of associated cardiac lesions, coarctation shelf, and isthmal flow disturbance. We studied 44 fetuses with suspected coarctation at 24+/-0 weeks (range, 17+/-3 to 37+/-4 weeks). Receiver-operating characteristic curves were created. Logistic regression tested the association between z scores, additional cardiac diagnoses, and coarctation. Good separation was found of isthmal Z scores for cases requiring surgery from controls and false-positive cases, and receiver-operating characteristic curves showed an excellent area under the curve for isthmal Z score (0.963) and isthmal-to-ductal ratio (0.969). Serial isthmal Z scores improved to >-2 in suspected cases with normal outcomes; those requiring surveillance or surgery remained <-2. Minor lesions did not increase the diagnostic specificity of coarctation, but isthmal flow disturbance increased the odds ratio of true coarctation versus arch hypoplasia 16-fold. CONCLUSIONS: Isthmal Z scores and isthmal-to-ductal ratio are sensitive indicators of fetal coarctation. Serial measurements and abnormal isthmal flow patterns improve diagnostic specificity and may reduce false positives.
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| 26. |
- Mellander, Mats, 1947, et al.
(författare)
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Failure to diagnose critical heart malformations in newborns before discharge--an increasing problem?
- 2006
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Ingår i: Acta Paediatr. - : Wiley. - 0803-5253. ; 95:4, s. 407-13
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Tidskriftsartikel (refereegranskat)abstract
- AIM: To determine what proportion of newborns with critical heart defects are discharged undiagnosed from the maternity ward. METHODS: Data were retrieved retrospectively for infants born in our referral area from 1993 to 2001 and undergoing surgical or catheter-based intervention before 2 mo of age because of critical heart defects. RESULTS: 259 full-term infants had critical heart defects. Duct-dependent systemic circulation was present in 129, duct-dependent pulmonary circulation in 106, and 24 infants were not duct dependent but critically ill. In 51 infants (20%) the heart defect was not suspected before discharge from the maternity ward. Such late detection occurred more often in infants with duct-dependent systemic circulation (30%) and in children with defects that were not duct dependent (38%) than in children with duct-dependent pulmonary circulation (4%) (p<0.001). The proportion detected after discharge from the maternity ward increased from 13% in 1993-1995 and 21% in 1996-1998 to 26% in 1999-2001 (p<0.05). CONCLUSION: Many infants with critical congenital heart defects are not diagnosed before discharge from the neonatal maternity ward. The proportion discharged undiagnosed has increased. Neonatal screening fails mainly in children with duct-dependent systemic circulation.
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| 27. |
- Mellander, Mats, 1947, et al.
(författare)
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Mineralization defects in deciduous teeth of low birthweight infants.
- 1982
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Ingår i: Acta paediatrica Scandinavica. - 0001-656X. ; 71:5, s. 727-33
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Tidskriftsartikel (refereegranskat)abstract
- A group of 91 children with birthweights below 2000 g and 48 healthy full-term children, reference group, were examined for mineralization disturbances of the enamel of deciduous teeth. Perinatal data were collected retrospectively from obstetric and neonatal records. Within the low birthweight group, children with enamel hypoplasia had on the 5% level a significantly lower intake of breast milk during the first week of life than children without disturbances. The only perinatal complication associated with development of mineralization defects was IRDS. Infants with IRDS, however, had a significantly lower intake of breast milk during the first week of life than low birthweight without IRDS. A low frequency of enamel defects was found in the growth-retarded sub-group of the low birthweight infants. These infants had a significantly higher intake of breast milk during the first week compared to the AGA-group. A seasonal variation was found, with the highest prevalence of mineralization disturbances in infants born during winter months. It is concluded that the occurrence of mineralization defects of the deciduous teeth in LBW infants seems to be dependent upon the amount of breast milk given during the early neonatal period and also possibly in which season of the year they are born.
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| 28. |
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| 29. |
- Mellander, Mats, 1947
(författare)
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Perinatal management, counselling and outcome of fetuses with congenital heart disease
- 2005
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Ingår i: Semin Fetal Neonatal Med. - 1744-165X. ; 10:6, s. 586-93
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Tidskriftsartikel (refereegranskat)abstract
- Prenatal treatment options for fetal heart disease are still limited but pharmacological treatment of fetal tachyarrhythmias is usually effective. Prenatal catheter interventions are likely to be an option in selected fetal cardiac defects in the future. Delivery should be at a tertiary care centre if the need for immediate neonatal transport is anticipated. When a cardiac problem is diagnosed in a fetus, the parents should be counselled by a paediatric cardiologist specialized in fetal cardiology in close co-operation with the obstetric team. The rate of termination is influenced by gestational age at diagnosis, the severity of the heart defect and the presence of associated malformations. In fetuses with isolated cardiac malformations who are in sinus rhythm with good myocardial function and no or trivial atrioventricular valve regurgitation, the risk of spontaneous intra-uterine death is low. Prenatal echocardiography has the potential to improve postnatal survival in infants with critical heart defects, especially those with duct-dependent systemic or pulmonary circulations.
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| 30. |
- Mellander, Mats, 1947, et al.
(författare)
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Quality of life in children with hypoplastic left heart syndrome
- 2007
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Ingår i: Acta Paediatr. - : Wiley. - 0803-5253 .- 1651-2227. ; 96:1, s. 53-7
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Tidskriftsartikel (refereegranskat)abstract
- AIM: To measure quality of life in children with hypoplastic left heart syndrome and their families. METHODS: A questionnaire exploring socioeconomic status, structure and function of networks and psychological well-being was completed by the families of all 18 patients older than 2 years (age range 2.7-10.6). The results were compared with those of 180 healthy Swedish children matched for age and sex. RESULTS: There were no significant differences between the groups in any of the aspects of socioeconomic status. Study group parents had not more available time for their child (p < 0.05) and more separations/divorces (p < 0.01). The patients had lower self-esteem (p < 0.05), more psychosomatic symptoms (p < 0.01) [corrected] and lower peer acceptance (p < 0.01) than control children. CONCLUSION: With regard to psychological well-being, quality of life was significantly lower in children with hypoplastic left heart syndrome than in healthy controls.
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| 31. |
- Nilsson, Boris, et al.
(författare)
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Results of staged palliation for hypoplastic left heart syndrome: a complete population-based series.
- 2006
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Ingår i: Acta paediatrica (Oslo, Norway : 1992). - : Wiley. - 0803-5253. ; 95:12, s. 1594-600
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Tidskriftsartikel (refereegranskat)abstract
- AIM: To study the outcome of staged palliation for classic hypoplastic left heart syndrome. METHODS: Retrospective chart review. Risk factors for mortality were analysed using Cox's proportional hazard regression modelling. RESULTS: From 1993 to 2004, 55 infants underwent Norwood stage I procedure at a median age of 8 d (range 1-19 d). Hospital survival was 39/55 (71%), and there were six late deaths (before stage II). Birthweight, circulatory arrest time and cardiopulmonary bypass time were independent risk factors for stage I hospital mortality (p=0.029, p=0.001 and p=0.003, respectively). Poor right ventricular function prior to stage I was a significant predictor for interstage mortality (p=0.02). Thirty-two patients underwent bidirectional cavopulmonary anastomosis, at a median age of 6.5 mo (range 2.0-9.5 mo), with seven late deaths. Two patients had a heart transplant after stage II. Total cavopulmonary connection (TCPC) was performed in 13 patients, at a median age of 33 mo (range 21-45 mo), without mortality. Kaplan-Meier survival was 58%, 52% and 45% at 6, 12 and 48 mo, respectively. CONCLUSION: Low birthweight, long time on circulatory arrest and cardiopulmonary bypass were risk factors for stage I mortality. Poor right ventricular function was detrimental to intermediate outcome.
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| 32. |
- Nordenstedt, Helena, et al.
(författare)
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Global hälsa på läkarutbildningen igår, idag och imorgon
- 2021
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Ingår i: Socialmedicinsk Tidskrift. - : Stiftelsen socialmedicinsk tidskrift. - 0037-833X. ; 98:2, s. 232-243
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Undervisning i global hälsa förekommer i olika former vid samtliga läkarutbildningar i Sverige, och på vissa håll har kursmoment med fokus på internationell hälsa funnits i över trettio år. I denna artikel presenteras utvecklingenav global hälsa vid Sveriges läkarutbildningar – från dåtid till nutid och framtid. Textbidrag har inkommit från respektive lärosäte och sammanställts av Helena Nordenstedt och Hampus Holmer.
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| 33. |
- Nordenström, Kajsa, et al.
(författare)
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Low risk of necrotising enterocolitis in enterally fed neonates with critical heart disease: An observational study
- 2020
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Ingår i: Archives of Disease in Childhood: Fetal and Neonatal Edition. - : BMJ. - 1359-2998 .- 1468-2052. ; 105:6, s. 609-614
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Tidskriftsartikel (refereegranskat)abstract
- Objective: We aimed to investigate the frequency of necrotising enterocolitis (NEC) in infants with critical congenital heart disease (CCHD) hypothesising that preoperative enteral feeding does not increase the risk of NEC. Background: When NEC affects term infants, underlying risk factors such as asphyxia, sepsis or CCHD are often found. Due to fear of NEC development in infants with CCHD great caution is practised in many countries to defer preoperative enteral feeding, but in Sweden this is routinely provided. Design, setting and patients: An observational study of all infants born with CCHD who were admitted to Queen Silvia Children's Hospital in Gothenburg between 2010 and 2017. The International Classification of Diseases 10th Revision diagnosis code of NEC was used to identify NEC cases in this group. Infants described as fully fed' or who were fed at least 45 mL/kg/day before cardiac surgery were identified. Main outcome measures: NEC in infants with CCHD in relation to preoperative enteral feeding. Results: There were 458 infants with CCHD admitted during the study period. 408/458 were born at term and 361/458 required prostaglandin E1 before surgery. In total, 444/458 infants (97%) were fully fed or fed at least 45 mL/kg daily before cardiac surgery. Four of 458 infants developed NEC (0.9%). All four had other risk factors for NEC. Conclusions: This study showed a low risk of NEC in term infants fed enterally before cardiac surgery. We speculate that preoperative enteral feeding of neonates with CCHD does not increase the risk of NEC development. © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
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| 34. |
- Pasquini, L., et al.
(författare)
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Z-scores of the fetal aortic isthmus and duct: an aid to assessing arch hypoplasia
- 2007
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Ingår i: Ultrasound Obstet Gynecol. - 0960-7692. ; 29:6, s. 628-33
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVE: Prenatal diagnosis of isolated coarctation of the aorta suffers from high false positive and false negative rates. The aim of our study was to develop Z-scores for the aortic isthmus in normal fetuses as a reference for fetuses with suspected coarctation. METHODS: The aortic isthmal diameter, immediately proximal to the insertion of the arterial duct, was measured prospectively in the transverse (three vessel and trachea) and sagittal views in 221 normal fetuses at 18 to 37 weeks' gestation. The ductal diameter was measured immediately before it entered the descending aorta in the same view. All measurements were repeated three times by a single investigator and averaged. A second investigator re-measured the images of 50 cases to assess interobserver variability. Z-scores were created relating isthmal and ductal diameters to femur length and gestational age. The ratio between the isthmal and ductal diameters was calculated. RESULTS: The formula used to calculate Z-scores for the three diameters was: [ln(measured isthmal diameter) - (m ln(femur length or gestational age) + c)]/root MSE, where c is the intercept, m is a multiplier and MSE is the mean squared error. The ratio between isthmal and ductal diameters was close to a constant value of 1 (95% CI 0.97-1.01), regardless of the value of femur length or gestational age. CONCLUSION: We have defined Z-scores for the fetal aortic isthmus and arterial duct measured in the three vessels and trachea view and for the isthmus in the sagittal plane. In suspected coarctation, these Z-scores and the isthmal to ductal ratio may help in longitudinal assessment of the aortic arch and aid in the prenatal diagnosis of coarctation.
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| 35. |
- Schmiegelow, Kjeld, et al.
(författare)
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Methotrexate/6-mercaptopurine maintenance therapy influences the risk of a second malignant neoplasm after childhood acute lymphoblastic leukemia: results from the NOPHO ALL-92 study.
- 2009
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Ingår i: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 113:24, s. 6077-84
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Tidskriftsartikel (refereegranskat)abstract
- Among 1614 children with acute lymphoblastic leukemia (ALL) treated with the Nordic Society for Paediatric Haematology and Oncology (NOPHO) ALL-92 protocol, 20 patients developed a second malignant neoplasm (SMN) with a cumulative risk of 1.6% at 12 years from the diagnosis of ALL. Nine of the 16 acute myeloid leukemias or myelodysplastic syndromes had monosomy 7 (n = 7) or 7q deletions (n = 2). In Cox multivariate analysis, longer duration of oral 6-mercaptopurine (6MP)/methotrexate (MTX) maintenance therapy (P = .02; longest for standard-risk patients) and presence of high hyperdiploidy (P = .07) were related to increased risk of SMN. Thiopurine methyltransferase (TPMT) methylates 6MP and its metabolites, and thus reduces cellular levels of cytotoxic 6-thioguanine nucleotides. Of 524 patients who had erythrocyte TPMT activity measured, the median TPMT activity in 9 patients developing an SMN was significantly lower than in the 515 that did not develop an SMN (median, 12.1 vs 18.1 IU/mL; P = .02). Among 427 TPMT wild-type patients for whom the 6MP dose was registered, those who developed SMN received higher average 6MP doses than the remaining patients (69.7 vs 60.4 mg/m2; P = .03). This study indicates that the duration and intensity of 6MP/MTX maintenance therapy of childhood ALL may influence the risk of SMNs in childhood ALL.
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| 36. |
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| 37. |
- Söderström, Fanny, et al.
(författare)
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Active versus restrictive ligation strategy for patent ductus arteriosus : A retrospective two-center study of extremely preterm infants born between 22 + 0 and 25 + 6 weeks of gestational age
- 2024
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Ingår i: Early Human Development. - : Elsevier. - 0378-3782 .- 1872-6232. ; 191
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Tidskriftsartikel (refereegranskat)abstract
- BackgroundPatent ductus arteriosus (PDA) in premature infants is associated with adverse clinical outcomes. Mode and timing of treatment are still controversial. Data are limited in the most extremely premature infants <26 weeks of gestational age (GA), where clinical problems are most significant and patients are most vulnerable.AimsTo investigate whether different approaches to surgical closure of PDA in two large Swedish centers has an impact on clinical outcomes including mortality in extremely preterm infants born <26 weeks GA.Study designRetrospective, two-center, cohort study.SubjectsInfants born at 22+0–25+6 weeks GA between 2010 and 2016 at Uppsala University Children's Hospital (UUCH; n = 228) and Queen Silvia Children's Hospital Gothenburg (QSCHG; n = 220).Main outcome measuresSurvival, bronchopulmonary dysplasia (BPD), and retinopathy of prematurity (ROP).ResultsSurgical closure of PDA was more common and performed earlier at QSCHG (50 % vs 16 %; median age 11 vs 44 days; p < 0.01). Survival was similar in both centres. There was a higher incidence of severe BPD and longer duration of mechanical ventilation at UUCH (p < 0.01). There was a higher incidence of ROP, IVH and sepsis at QSCH (p < 0.05, p < 0.01 and p < 0.01). A sub-group analysis matching all surgically treated infants at QSCHG with infants at UUCH with the same GA showed similar results as the total cohort.ConclusionEarlier and higher rate of surgical PDA closure in this cohort of extremely preterms born <26 weeks GA did not impact mortality but was associated with lower rates of severe BPD and higher rates of severe ROP.
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| 38. |
- Söderström, Fanny, et al.
(författare)
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Active versus restrictive ligation strategy for patent ductus arteriosus - A retrospective two-center study of extremely preterm infants born between 22+0 and 25+6 weeks of gestational age
- 2024
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Ingår i: EARLY HUMAN DEVELOPMENT. - : Elsevier. - 0378-3782 .- 1872-6232. ; 191
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Tidskriftsartikel (refereegranskat)abstract
- Background: Patent ductus arteriosus (PDA) in premature infants is associated with adverse clinical outcomes. Mode and timing of treatment are still controversial. Data are limited in the most extremely premature infants <26 weeks of gestational age (GA), where clinical problems are most significant and patients are most vulnerable. Aims: To investigate whether different approaches to surgical closure of PDA in two large Swedish centers has an impact on clinical outcomes including mortality in extremely preterm infants born <26 weeks GA. Study design: Retrospective, two-center, cohort study. Subjects: Infants born at 22(+0)-25(+6) weeks GA between 2010 and 2016 at Uppsala University Children's Hospital (UUCH; n = 228) and Queen Silvia Children's Hospital Gothenburg (QSCHG; n = 220). Main outcome measures: Survival, bronchopulmonary dysplasia (BPD), and retinopathy of prematurity (ROP). Results: Surgical closure of PDA was more common and performed earlier at QSCHG (50 % vs 16 %; median age 11 vs 44 days; p < 0.01). Survival was similar in both centres. There was a higher incidence of severe BPD and longer duration of mechanical ventilation at UUCH (p < 0.01). There was a higher incidence of ROP, IVH and sepsis at QSCH (p < 0.05, p < 0.01 and p < 0.01). A sub -group analysis matching all surgically treated infants at QSCHG with infants at UUCH with the same GA showed similar results as the total cohort. Conclusion: Earlier and higher rate of surgical PDA closure in this cohort of extremely preterms born <26 weeks GA did not impact mortality but was associated with lower rates of severe BPD and higher rates of severe ROP.
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| 39. |
- Waern, M., et al.
(författare)
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Prenatal detection of congenital heart disease-results of a Swedish screening program 2013-2017
- 2021
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Ingår i: Bmc Pregnancy and Childbirth. - : Springer Science and Business Media LLC. - 1471-2393. ; 21:1
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Tidskriftsartikel (refereegranskat)abstract
- Background This report evaluates results of a screening program on prenatal detection of congenital heart defects in a geographical cohort of western Sweden between January 1st, 2013 and June 31st, 2017. During the study period 88,230 children were born in VGR. Methods Retrospective data on pregnant women from the Vastra Gotaland region that were referred to fetal cardiologists in Gothenburg were retrieved. To determine prenatal detection rate, all neonates who underwent surgery or catheter intervention for a critical congenital heart defect born between January 1st, 2014 and December 31st, 2016 were included. The four-chamber view was implemented into the routine scan in 2009 and implementation of the ISUOG guidelines, including the outflow tracts, started in the region in 2015. Results 113 fetuses received a prenatal diagnosis of a major congenital heart defect. 89% of these were referred because of a suspected cardiac malformation and 88% were diagnosed before 22 completed weeks. 59% of the patients diagnosed before 22 completed weeks opted for termination of pregnancy. During 2014-2016, 61 fetuses had a prenatal diagnosis of a critical congenital heart defect and a further 47 were diagnosed after birth, hence 56% were diagnosed prenatally, 82% for those which had a combination with an extracardiac abnormality and/or chromosomal aberration compared to 50% if an isolated critical congenital heart defect was diagnosed. For single ventricle cardiac defects such as hypoplastic left heart syndrome, double inlet left ventricle and tricuspid atresia, the detection rate was 100%. The detection rate for transposition of the great arteries and coarctation of the aorta was 9 and 18% respectively. Conclusions 56% of all fetuses with a critical congenital heart defect were diagnosed prenatally during 2014-2016 and approximately 53% of all major congenital heart defects 2013-2017 as compared to 13.8% in 2009 in the same region. An increased focus towards the fetal heart in the routine scan improved the prenatal detection rate of major congenital heart defects. The detection of congenital heart defects affecting the four-chamber view seems sufficient, but more training is needed to improve the quality of the examination of the outflow tracts.
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| 40. |
- Weismann, Constance G., et al.
(författare)
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Echocardiographic predictors of recoarctation following surgical repair – a Swedish national study
- 2021
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Ingår i: Annals of Thoracic Surgery. - : Elsevier BV. - 0003-4975. ; 111:4, s. 1380-1386
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Tidskriftsartikel (refereegranskat)abstract
- Background Following surgical repair of aortic coarctation (CoA) there is a risk for restenosis (reCoA), particularly in the first year of life. It was the aim of this study to identify reCoA risk factors by analyzing postoperative pre-discharge echocardiograms. Methods Retrospective analysis of echocardiograms of children born operated for CoA in Sweden 2011-2017. Results 253 children were included; median age at surgery 10 days; median follow-up 4.6 years. ReCoA occurred in 34 patients (13%; 74% by 6 months, 91% by 12 months). We generated two reCoA risk models applying a) aortic dimensions and b) the respective Z-scores combined with surgical and demographic factors. We defined reCoA risk categories as low (≤10%), moderate (11-29%), moderate-high (30-49%) and high (≥50%). Patients with a) isthmus ≤3.3mm (1- and 5-year event free survival 38 and 32%) or b) isthmus Z-score ≤-2.8 with a weight at surgery <4.4kg (1- and 5-year event free survival 21 and 16%) were at highest risk for reCoA. Conversely, patients at low risk had a) isthmus >3.7mm and distal aortic arch >3.5mm (1- and 5-year event free survival 97 and 97%), and b) isthmus and proximal aortic arch Z-score >-2.8 or operative weight >4.4kg with an isthmus Z-score <=-2.8 with (1- and 5-year event free survival 97 and 97%). Conclusion ReCoA risk can be predicted based on postoperative pre-discharge echocardiographic variables in combination with surgical and demographic factors. We suggest tailoring follow-up intervals individually according to the predicted reCoA risk.
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| 41. |
- Åmark, Kerstin, 1967, et al.
(författare)
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[Limited value of prenatal risk group screening for heart abnormalities. Time for a general screening?]
- 2004
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Ingår i: Lakartidningen. - 0023-7205. ; 101:48
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Tidskriftsartikel (refereegranskat)abstract
- 544 fetuses at increased risk of cardiac malformations were examined by echocardiography from gestational week 14 to 40 (median 21), during the period January 1989-March 2002. A heart defect was diagnosed in 70 fetuses. Three false positive diagnoses and eight false negative diagnoses occurred (sensitivity 89 % and specificity 99 %). Seven were terminated (three with trisomy, two with concomitant diaphragmatic hernia and two with single ventricles) and eight died in utero. 22 of 52 liveborn infants died during the neonatal period. Fetal arrhythmia was diagnosed in 30 cases of which eight died prenatally. This risk group screening identified only 3.3 % of all infants with heart defects born during the study period. The ambition to diagnose a greater proportion prenatally would require a general screening program. In order to guarantee a high quality of specialized fetal cardiology service, referrals should be centralized to only a few units in the country.
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| 42. |
- Öhman, Annika, et al.
(författare)
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Changing Epidemiology of Hypoplastic Left Heart Syndrome : Results of a National Swedish Cohort Study
- 2019
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Ingår i: Journal of the American Heart Association. - : John Wiley & Sons. - 2047-9980. ; 8:2
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Tidskriftsartikel (refereegranskat)abstract
- Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live-borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990-2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox-logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live-borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live-borns who underwent surgery increased between time periods.
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| 43. |
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| 44. |
- Öhman, Annika, et al.
(författare)
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Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia : A Swedish national cohort study
- 2020
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Ingår i: Cardiology in the Young. - : Cambridge University Press. - 1047-9511 .- 1467-1107. ; 30:3, s. 353-360
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Tidskriftsartikel (refereegranskat)abstract
- Background: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. Methods: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. Results: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. Conclusion: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
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| 45. |
- Öhman, Annika, et al.
(författare)
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Transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia: A Swedish national cohort study
- 2020
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Ingår i: Cardiology in the Young. - : Cambridge University Press (CUP). - 1047-9511 .- 1467-1107. ; 30:3
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Tidskriftsartikel (refereegranskat)abstract
- Background: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. Methods: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. Results: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. Conclusion: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
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