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1.	Aburawi, Elhadi, et al. (författare) A transthoracic Doppler echocardiography study of C-reactive protein and coronary microcirculation in children after open heart surgery. 2007 Ingår i: Cardiology in the Young. - 1467-1107. ; 17:5, s. 472-477 Tidskriftsartikel (refereegranskat)abstract Background Systemic inflammation has been suggested to underlie in part the elevated risk of arrhythmias and myocardial dysfunction during the first weeks after cardiac surgery. Recent transthoracic Doppler studies from our centre indicated increased basal coronary arterial flow in children 5 days after cardiopulmonary bypass surgery. In these children, we investigated whether the inflammatory mediator, C-reactive protein, could influence this association. Methods The peak flow velocity, velocity time-integral in diastole and systole, and basal blood flow in the proximal part of the left anterior descending artery, were assessed by transthoracic Doppler echocardiography 1 day before, and 5 days after, cardiac surgery in 17 children with ventricular and atrioventricular septal defects whose mean age at surgery was 6 months. Levels of C-reactive protein in the plasma were measured at both time-points. Results Prior to surgery, all children had levels of C-reactive protein under the limit for detection, that is less than 0.8 milligrams per litre. The levels of the protein had increased significantly by the second day, when the median value was 25, and the range from 20 to 142 milligrams per litre. They remained elevated on the fifth day after surgery, when the median was 11, and the range from 3 to 20 milligrams per litre. On the fifth day, the percentage increase in velocity time integral corrected for left ventricular mass was significantly lower in those patients with C-reactive protein greater than or equal to 10 milligrams per litre than in the remaining patients. Also, both the velocity time integral and the velocity of diastolic peak flow correlated inversely with log C-reactive protein, r being equal to −0.54 and p less than 0.02 and r equal to −0.74 and p less than 0.01, respectively, particularly among those patients in whom clamping of the aorta lasted for more than 1 hour, r for this statistic being equal to −0.8. Conclusion The postsurgical increase in the velocity of coronary arterial flow in children is inversely associated with rising levels of C-reactive protein. The duration of the aortic cross-clamping during surgery strengthens the association between levels of C-reactive protein and the microcirculatory changes.
2.	Aburawi, Elhadi, et al. (författare) Acute respiratory viral infections aggravate arterial endothelial dysfunction in children with type 1 diabetes. 2004 Ingår i: Diabetes Care. - : American Diabetes Association. - 1935-5548 .- 0149-5992. ; 27:11, s. 2733-2735 Tidskriftsartikel (refereegranskat)abstract Despite improvements in therapy for children with type 1 diabetes, the prevalence of cardiovascular morbidity in adulthood due to accelerated atherosclerosis remains significant (1). Similar to other cardiovascular risk factors, the diabetic state facilitates arterial endothelial injury, a primary event in the pathogenesis of atherosclerosis (2). Although several pediatric studies have reported an association of diabetes with arterial endothelial dysfunction (3,4), pathogenic animal studies have suggested that even though this disease predisposes to endothelial dysfunction and atherosclerosis, it might not be sufficient to cause them (5). Notably, type 1 diabetes increases the propensity for both chronic and acute infections in part by weakening the immune mechanisms (6). The risk is particularly increased for respiratory tract infections, but other infections have also been associated with diabetes (7). Furthermore, diabetic patients are at greater risk for infection-related mortality (8), and the excess risk appears to be linked to cardiovascular diseases (9). In the present study, we investigated whether viral respiratory tract infections in children with type 1 diabetes might impose an additional burden on the arterial endothelial function.
3.	Aburawi, Elhadi, et al. (författare) Coronary Blood Flow by Transthoracic Echocardiography in Children with Endomyocardial Fibrosis. 2009 Ingår i: Pediatric Cardiology. - : Springer Science and Business Media LLC. - 0172-0643 .- 1432-1971. ; 30, s. 371-373 Tidskriftsartikel (refereegranskat)abstract We report herein the coronary flow (CF) pattern determined by transthoracic Doppler echocardiography (TTDE) in two children with biventricular endomyocardial fibrosis (EMF). Endomyocardial biopsy showed various degrees of cardiac fibrosis in both patients as well as signs of chronic inflammation in one of the patients. TTDE showed a significant increase in CF in both the left anterior descending coronary artery and the posterior descending coronary artery compared with age-matched healthy controls. The diastolic flow in patients with EMF appeared to terminate earlier than in controls. The mechanisms and the potential significance of these novel findings are discussed.
4.	Aburawi, Elhadi, et al. (författare) Early changes in myocardial repolarization and coronary perfusion after cardiopulmonary bypass surgery for ASD repair in children 2013 Ingår i: BMC Cardiovascular Disorders. - : Springer Science and Business Media LLC. - 1471-2261. ; 13 Tidskriftsartikel (refereegranskat)abstract Background: In adults, impaired myocardial repolarization and increased risk of arrhythmia are known consequences of open heart surgery. Little is known, however, about post-operative consequences of cardiopulmonary bypass surgery in children. The aim of this study was to assess ventricular repolarization and coronary perfusion after bypass surgery for atrial septal defect (ASD) repair in children. Methods: Twelve patients with ASD were assessed one day before and 5-6 days after ASD repair. Myocardial repolarization (corrected QT interval, QTc, QT dispersion, QTd, and PQ interval) was determined on 12-lead electrocardiograms. Coronary flow in proximal left anterior descending artery (peak flow velocity in diastole, PFVd) was assessed by transthoracic Doppler echocardiography. Results: Ten of the 12 (83%) children had normal myocardial repolarization before and after surgery. After surgery, QTc increased 1-9% in 5 (42%) patients, decreased 2-11% in 5 (42%) patients and did not change in 2 (16%) patients. Post-op QTc positively correlated with bypass time (R=0.686, p=0.014) and changes in PFVd (R=0.741, p=0.006). After surgery, QTd increased 33-67% in 4 (33%) patients, decreased 25-50% in 6 patients (50%) and did not change in 2 (16%) patients. After surgery, PQ interval increased 5-30% in 4 (33%) patients, decreased 4-29% in 6 (50%) patients and did not change in 1 (8%) patient. Post-op PQ positively correlated with bypass time (R=0.636, p=0.027). As previously reported, PFVd significantly increased after surgery (p<0.001). Conclusions: Changes in QTc, PQ and PFVd are common in young children undergoing surgery for ASD repair. Post-op QTc significantly correlates with bypass time, suggesting prolonged cardiopulmonary bypass may impair ventricular repolarization. Post-op QTc significantly correlates with PFVd changes, suggesting increased coronary flow may also impair ventricular repolarization. The clinical significance and reversibility of these alternations require further investigations.
5.	Aburawi, Elhadi, et al. (författare) Effects of cardio-pulmonary bypass surgery on coronary flow in children assessed with transthoracic Doppler echocardiography. 2007 Ingår i: American Journal of Physiology: Heart and Circulatory Physiology. - : American Physiological Society. - 1522-1539 .- 0363-6135. ; 293:2, s. 1138-1143 Tidskriftsartikel (refereegranskat)abstract Perturbation of coronary blood flow (CF) is an important contributor to myocardium-related complications. The study was primarily designed to assess the impact of cardiopulmonary bypass (CPB) surgery on CF by aid of transthoracic Doppler echocardiography. Changes in CF after off-pump coarctation surgery were also studied. All ultrasounds were performed before and 5 ± 1 days after surgery. Eighteen children underwent CPB surgery of ventricular left-to-right shunts at the mean age of 6 mo, while off-pump surgery (aortic coarctectomy) was undertaken at the mean age of 10 days in 12 children. After CPB surgery, both left anterior descending coronary artery mean diameter and basal CF increased from 1.7 ± 0.3 to 2.1 ± 0.4 mm (P = 0.001) and 27 ± 10 to 47 ± 15 ml/min (P = 0.0001), respectively. These two coronary variables decreased after off-pump coarctectomy: left anterior descending coronary artery mean diameter from 1.8 ± 0.1 to 1.7 ± 0.1 mm (P = 0.06), and CF from 44 ± 12 to 25 ± 8 ml/min (P = 0.001). The findings are in keeping with the hypothesis that the previously reported impairment of coronary flow reserve after CPB surgery could be due to increase in basal coronary flow after CPB. Off-pump coarctectomy seems to have little impact on CF, as the postsurgical decline in flow in these patients seems to relate to the reduction in cardiac pressure afterload.
6.	Aburawi, Elhadi H., et al. (författare) Effects of N-3 Polyunsaturated Fatty Acids on Left Ventricular Function and Coronary Flow in Children with Type 1 Diabetes Mellitus 2011 Ingår i: Pediatric Research. - : Springer Science and Business Media LLC. - 1530-0447 .- 0031-3998. ; 70:227 Konferensbidrag (refereegranskat)abstract Purposes: Dietary supplementation with N-3 Polyunsaturated Fatty Acids (n-3 PUFAs) could have beneficial effects on cardiovascular system in patients with type 1 diabetes mellitus (DM1). Methods: In a double-blind placebo controlled crossover study, 33 children with DM1 duration of more than one year were randomly and equally assigned to either n-3 PUFAs (2 gm/day, Nycoplus® Omega-3, 1000 mg) or placebo treatment for 8 weeks. Following a 4-week period recovery, the groups were crossovered with above treatments for another 8 weeks. Transthoracic Doppler echocardiography (TTDE) study was done on pre and post treatment visits, and after one month's treatment free recovery for left ventricular function and flow in the left anterior descending coronary artery (LAD). Results: Of recruited children 28 (85%) completed the study. n-3 PUFAs treatment was associated with increase in mean cardiac index (CI; from 2.7±0.4 to 3.7±0.8 l/min/m2, p< 0.0001) and left ventricular fractional shortening (FS; from 31±2.5 to 39±3%, p< 0.0001). The treatment decreased both LAD peak flow velocity (PFVd) from 96±17 to 68±12 cm/s, p< 0.0001 and LAD CF from 105±31 to 66±15 ml/min, p< 0.0001). One month after stopping the treatment CI decreased from 3.7±0.8 to 2.6±0.5 l/min/m2, p< 0.0001 and mean FS from 39±3 to 32±2, p< 0.0001. Mean PFVd increased from 68±12 to 90±12 cm/s, p< 0.0001 and CF from 66±15 to 108±30 ml/min, p< 0.0001. Conclusions: In patients with DM1 n-3 PUFA therapy increased cardiac index and LV systolic function. The basal coronary flow decrease improving the circumstances for better coronary flow reserve.
7.	Aburawi, Elhadi, et al. (författare) QTc and QTd Changes after Cardiopulmonary Bypass Surgery in Children 2014 Ingår i: European Journal of Cardiovascular Medicine. - : Healthcare Bulletin. - 2042-4884. ; 3:1 Tidskriftsartikel (refereegranskat)abstract The aim of the study was to assess the impact of cardiopulmonary bypass surgery on corrected QT (QTc) and QT dispersion (QTd) intervals. The possible role of inflammation on these variables was investigated.
8.	Ahlström, Love, et al. (författare) Preoperative Coronary Anatomy Assessment with Echocardiography and Morbidity After Arterial Switch Operation of Transposition of the Great Arteries 2018 Ingår i: Pediatric Cardiology. - : Springer Science and Business Media LLC. - 0172-0643 .- 1432-1971. ; 39:8, s. 1620-1626 Tidskriftsartikel (refereegranskat)abstract In transposition of the great arteries (TGA), certain coronary patterns have been associated with major adverse events early after the arterial switch operation (ASO). We sought to determine the impact of preoperative echocardiographic (ECHO) diagnosis on the intra and postoperative morbidity. All patients with TGA born between June 2001 and June 2017 and who underwent ASO were reviewed. Data on presumed coronary anatomy (CA) preoperatively were obtained from the preoperative ECHO report. Intraoperative CA was categorized according to Yacoub classification. Major postoperative morbidity included at least one of the following: delayed sternal closure (DSC), prolonged (> 72 h) mechanical ventilation, reintubation, peritoneal dialysis (PD), ECMO, reoperation, and readmission within 30 days after surgery. 240 patients with median age of 5 days (range 1–614) and mean weight at surgery was 3.6 kg (1.8–8.4) were included. Preoperative ECHO assessment of CA was available in 228 patients. Intraoperatively, 181 patients (75%) were found to have type A, 25 patients had type B or C or intramural (B–C–IM; 10%), and 34 patients had type D or E (D–E; 14%). Patients with types B, C, and intramural coronary (B–C–IM) had increased risk for delayed sternum closure (9/25 vs. 20/181 in type A and 8/34 in type D–E; p = 0.04), peritoneal dialysis (4/25 vs. 8/181 and 1/34; p = 0.04), and ECMO (2/25 vs. 1/131 and 1/34; p = 0.02). Within the B–C–IM group, preoperative ECHO raised suspicion of type A in 13 patients (i.e., incorrect diagnosis, ID; 52%), whereas non-A CA was suspected in 12 patients (i.e., correct diagnosis, CD; 48%). With the exception of reoperation, which was seen only in the ID subgroup (4/12 vs. 0/10 in the CD subgroup; p = 0.04), the intraoperative (cardiopulmonary bypass time and cross-clamp time) and postoperative morbidity indices were comparable in both ID and CD subgroups (p > 0.1). Although there is a significant risk for early postoperative morbidity in TGA patients with single, interarterial, and intramural CA, there seems to be relatively limited influence of preoperative ECHO assessment of coronary anatomy on this morbidity burden.
9.	Ahlström, Love, et al. (författare) Surgical Age and Morbidity After Arterial Switch for Transposition of the Great Arteries 2019 Ingår i: Annals of Thoracic Surgery. - : Elsevier BV. - 1552-6259 .- 0003-4975. ; 108:4, s. 1242-1247 Tidskriftsartikel (refereegranskat)abstract Background: Transposition of the great arteries (TGA) is a complex congenital heart disease that requires early diagnosis as well as advanced surgical repair and postoperative support. We sought herein to study the impact of surgical timing on early postoperative morbidity.Methods: We reviewed all patients with TGA corrected at our institution via arterial switch operation (ASO) between June 2001 and June 2016. Major postoperative morbidity (MPM) and death within 30 days after ASO were documented. Patients with double outlet right ventricle, chromosome abnormalities and non-cardiac diseases were excluded. MPM was defined as presence of at least 1 of the following: delayed sternum closure, reoperation, prolonged mechanical ventilation, noninvasive ventilation after extubation, peritoneal dialysis, ECMO and readmission. Results: 241 patients were included, with medians for birth weight, gestational week and age at surgery of 3.5 kg, 39 weeks, and 5 days, respectively. MPM was encountered in 32.3% of patients. Prematurity (p=0.001) and need for aortic arch repair at the time of ASO (p=0.04) were associated with significant increase in MPM. Non-A coronary anatomy, associated ventricular septal defect requiring surgical closure and fetal diagnosis of TGA had no significant impact on MPM (p=0.35, 0.08 and 0.21, respectively). There was no significant difference in MPM between the surgical groups (p=0.49).Conclusions: Early complications after ASO do occur and are mostly associated with prematurity and need for aortic arch repair. Timing of surgical repair does not seem to influence the rate of these complications.
10.	Avdikos, Vasileios, et al. (författare) Outcomes following surgical repair of absent pulmonary valve syndrome : 30 years of experience from a Swedish tertiary referral centre 2022 Ingår i: Interactive Cardiovascular and Thoracic Surgery. - : Oxford University Press (OUP). - 1569-9293 .- 1569-9285. ; 35:2 Tidskriftsartikel (refereegranskat)abstract Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.
11.	Bhat, Misha, et al. (författare) Fetal iGRASP cine CMR assisting in prenatal diagnosis of complicated cardiac malformation with impact on delivery planning 2019 Ingår i: Clinical Physiology and Functional Imaging. - : Wiley. - 1475-0961 .- 1475-097X. ; 39:4, s. 231-235 Tidskriftsartikel (refereegranskat)abstract Limited visualisation of the fetal heart and vessels by fetal ultrasound due to suboptimal fetal position, patient habitus and skeletal calcification may lead to missed diagnosis, overdiagnosis and parental uncertainty. Counseling and delivery planning may in those cases also be tentative. The recent fetal cardiac magnetic resonance (CMR) reconstruction method utilising tiny golden angle iGRASP (iterative Golden-angle RAdial Sparse Parallel MRI) allows for cine imaging of the fetal heart for use in clinical practice. This case describes an unbalanced common atrioventricular canal where limited ultrasound image quality and visibility of the aortic arch precluded confirming or ruling out presence of a ventricular septal defect. Need of prostaglandins or neonatal intervention was thus uncertain. Cardiovascular magnetic resonance imaging confirmed ultrasound findings and added value by ruling out a significant ventricular septal defect and diagnosing arch hypoplasia. This confirmed the need of patient relocation for delivery at a paediatric cardiothoracic surgery centre and prostaglandins could be initiated before the standard postnatal ultrasound. The applied CMR method can thus improve diagnosis of complicated fetal cardiac malformation and has direct clinical impact. This article is protected by copyright. All rights reserved.
12.	Bhat, Misha, et al. (författare) Longitudinal ECG changes in tetralogy of Fallot and association with surgical repair 2024 Ingår i: Frontiers in Cardiovascular Medicine. - 2297-055X. ; 11 Tidskriftsartikel (refereegranskat)abstract Background: ECG abnormalities have been linked to adverse changes in right ventricular (RV) morphology and poor clinical outcomes in repaired Tetralogy of Fallot (rTOF). Our aim was to describe how ECG changes progress in early and intermediate follow-up and whether types of surgical strategy at the time of primary repair affected these changes. Methods: We studied patients with rTOF born 2000–2018 operated at our institution. Seven time points in relation to primary repair, follow-up, and pulmonary valve replacement (PVR) were identified. Patients correct with valve sparing repair (VSR), trans-annular patch (TAP) including with a monocusp valve (TAP + M) and with at least 3 ECGs were included. PQ interval, QRS duration, dispersion, and fragmentation, QTc duration and dispersion, JTc as well as presence of a right bundle branch block (RBBB) were analyzed. Medical records were reviewed for demographic and surgical data. Results: Two hundred nineteen patients with 882 ECGs were analyzed with a median follow-up time of 12.3 years (8.4, 17) with 41 (19%) needing PVR during the study period. QRS duration increased at time of primary repair to discharge from 66 msec (IQR 12) to 129 msec (IQR 27) (p < 0.0001) and at 1- and 6- year follow-up but showed only a modest and temporary decrease after PVR. QTc increased at the time of primary repair as well as prior to PVR. PQ interval showed a small increase at the time of primary repair, was at its highest prior to PVR and decreased with PVR. Type of surgical repair affected mainly QTc and JTc and was consistently longer in the TAP + M group until PVR. In VSR, QTc and JTc were prolonged initially compared to TAP but were similar after 1 year. After PVR, there were no differences in adverse ECG changes between surgical groups. Conclusions: PQ interval and QRS duration best correspond to the assumed volume load whereas the relationship with QTc and JTc is more complex, suggesting that these represent more complex remodeling of the myocardium. Before PVR, QTc and JTc are longer in the TAP + M group which may be due to a longer surgical incision.
13.	Birck, Malene M., et al. (författare) Infection-induced coronary dysfunction and systemic inflammation in piglets are dampened in hypercholesterolemic milieu 2011 Ingår i: American Journal of Physiology: Heart and Circulatory Physiology. - : American Physiological Society. - 1522-1539 .- 0363-6135. ; 300:5, s. 1595-1601 Tidskriftsartikel (refereegranskat)abstract Birck MM, Pesonen E, Odermarsky M, Hansen AK, Persson K, Frikke-Schmidt H, Heegaard PM, Liuba P. Infection-induced coronary dysfunction and systemic inflammation in piglets are dampened in hypercholesterolemic milieu. Am J Physiol Heart Circ Physiol 300: H1595-H1601, 2011. First published February 25, 2011; doi:10.1152/ajpheart.01253.2010.-The synergism of infection with conventional cardiovascular risk factors in atherosclerosis is much debated. We hypothesized that coronary arterial injury correlates with infection recurrence and pathogen burden and is further aggravated by hypercholesterolemia. Forty-two Gottingen minipigs were assigned to repeated intratracheal inoculation of PBS, Chlamydia pneumoniae (Cpn), or both Cpn and influenza virus at 8, 11, and 14 wk of age. Animals were fed either standard or 2% cholesterol diet (chol-diet.). At 19 wk of age coronary vasomotor responses to acetylcholine (ACh) and adenosine were assessed in vivo and blood and tissue samples were collected. Nonparametric tests were used to compare the groups. In cholesterol-fed animals, total cholesterol/HDL was significantly increased in infected animals compared with noninfected animals [3.13 (2.17-3.38) vs. 2.03 (1.53-2.41), respectively; P = 0.01]. C-reactive protein (CRP) rose in infected animals [10.60 (4.96-18.00) vs. 2.47 (1.44-3.01) mu g/ml in noninfected; P < 0.01] without significant difference between the mono- and coinfected groups. Among coinfected animals, both CRP and haptoglobin were lower in those fed chol-diet than in those fed standard diet (P < 0.05). The vasoconstricting response to ACh was most prominent in coinfected animals (769.3 (594-1,129) cm; P = 0.03 vs. noninfected [342 (309-455) cm] and P = 0.07 vs. monoinfected [415 (252.5-9711.8) cm]}. Among monoinfected animals, similar to CRP, a trend for less vasoconstriction was observed in those fed chol-diet (P = 0.08). Coinfection of piglets appears to be associated with more pronounced coronary muscarinic vasomotor dysfunction. In monoinfected animals, use of chol-diet seems to dampen both coronary dysfunction and systemic inflammation induced by infection.
14.	Björk Werner, Josefin, et al. (författare) Factors Influencing Need for Late ASD Closure after Neonatal Repair of Severe Pulmonary Valve Obstruction and Intact Ventricular Septum 2018 Ingår i: Cardiology in the Young. - 1467-1107. ; 28:S1, s. 151-151 Konferensbidrag (refereegranskat)abstract Background: In neonates with critical pulmonary stenosis (CPS) or pulmonary atresia with intact ventricular septum (PAIVS), a nonrestrictive atrial septal defect (ASD) has been speculated to improve the initial clinical course after pulmonary valvotomy (PV) but some concerns exist in terms of its potentially longterm adverse effect on the right ventricle`s (RV) growth and persistent desaturation due to right-to-left (R-L) shunt. Objective: to assess the relationship between the size of ASD and the need for post-valvotomy reinterventions. Method: Patients with PAIVS and CPS treated at our center during 2001- 2015 were reviewed. Exclusion criteria were associated cardiac malformations and hypoplastic RV deemed unsuitable for biventricular circulation. Clinical and echocardiographic data were retrieved from the hospital ́s databases. Results: In total, 48 patients (18 with PAIVS and 30 CPS) were included. The median follow-up was 5 and 8 years, respectively. One patient with PAIVS died on day 3 after surgical valvotomy and Blalock- Taussig shunt (BTs). The majority (89%) of patients with PAIVS had surgical valvotomy whereas transcatheter valvotomy was used in the majority (87%) of patients with CPS. Palliation with BTs or PDA stenting was used in 13 (72%) patients with PAIVS and in 4 (13%) patients with CPS. Reintervention within 1 month after initial repair was needed in 4 (22%) patients with PAIVS and in 4 (13%) patients with CPS. Later reinterventions were performed in in 11(61%) patients with PAIVS and in 10(33%) patients with CPS. Of these, 7 (39%) patients with PAIVS and 5 (17%) with CPS underwent ASD closure due to persistent resting desaturation. The latter did not correlate with ASD size after valvotomy (p>0.1). Initial palliation with BTs was the only variable associated with ASD device closure (p=0.04). No patient required univentricular conversion. Conclusion: Neonatal biventricular repair for severe pulmonary valve obstruction has low mortality but significant need for late reinterventions, mostly consisting of ASD closure due to clinically significant desaturation secondary to R-L shunt. The use of aorto-pulmonary shunt, probably illustrating a more severe form of RV hypoplasia, but not the size of the ASD, predicts the need for later ASD closure due to desaturation.
15.	Bonamy, Anna-Karin Edstedt, et al. (författare) Blood Pressure in 6-Year-Old Children Born Extremely Preterm 2017 Ingår i: Journal of the American Heart Association. - : WILEY. - 2047-9980. ; 6:8 Tidskriftsartikel (refereegranskat)abstract Background-Advances in perinatal medicine have increased infant survival after very preterm birth. Although this progress is welcome, there is increasing concern that preterm birth is an emerging risk factor for hypertension at young age, with implications for the lifetime risk of cardiovascular disease. Methods and Results-We measured casual blood pressures (BPs) in a population-based cohort of 6-year-old survivors of extremely preterm birth (< 27 gestational weeks; n=171) and in age-and sex-matched controls born at term (n=172). Measured BP did not differ, but sex, age-, and height-adjusted median z scores were 0.14 SD higher (P=0.02) for systolic BP and 0.10 SD higher (P=0.01) for diastolic BP in children born extremely preterm than in controls. Among children born extremely preterm, shorter gestation, higher body mass index, and higher heart rate at follow-up were all independently associated with higher BP at 6 years of age, whereas preeclampsia, smoking in pregnancy, neonatal morbidity, and perinatal corticosteroid therapy were not. In multivariate regression analyses, systolic BP decreased by 0.10 SD (P=0.08) and diastolic BP by 0.09 SD (P=0.02) for each week-longer gestation. Conclusions-Six-year-old children born extremely preterm have normal but slightly higher BP than their peers born at term. Although this finding is reassuring for children born preterm and their families, follow-up at older age is warranted.
16.	Broberg, Olof, et al. (författare) Characterization of Cardiac, Vascular, and Metabolic Changes in Young Childhood Cancer Survivors 2021 Ingår i: Frontiers in Pediatrics. - : Frontiers Media SA. - 2296-2360. ; 9 Tidskriftsartikel (refereegranskat)abstract Background: Childhood cancer survivors (CCS) are at an increased risk for cardiovascular diseases (CVD). It was the primary aim of this study to determine different measures of cardiac, carotid, lipid, and apolipoprotein status in young adult CCS and in healthy controls. Methods: Cardiac and common carotid artery (CCA) structure and function were measured by ultrasonography. Lipids and apolipoproteins were measured in the blood. Peripheral arterial endothelial vasomotor function was assessed by measuring digital reactive hyperemia index (PAT-RHI) using the Endo-PAT 2000. Results: Fifty-three CCS (20-30 years, 35 men) and 53 sex-matched controls were studied. The CCS cohort was divided by the median dose of anthracyclines into a low anthracycline dose (LAD) group (50-197 mg/m2, n = 26) and a high anthracycline dose (HAD) group (200-486 mg/m2, n = 27). Carotid distensibility index (DI) and endothelial function determined by PAT-RHI were both lower in the CCS groups compared with controls (p < 0.05 and p = 0.02). There was no difference in carotid intima media thickness. Atherogenic apolipoprotein-B (Apo-B) and the ratio between Apo-B and Apoliprotein-A1 (Apo-A1) were higher in the HAD group compared with controls (p < 0.01). Apo-B/Apo-A1-ratio was over reference limit in 29.6% of the HAD group, in 15.4% of LAD group, and in 7.5% of controls (p = 0.03). Measured lipid markers (low density lipoprotein and total cholesterol and triglycerides) were higher in both CCS groups compared with controls (p < 0.05). Systolic and diastolic function were measurably decreased in the HAD group, as evidenced by lower EF (p < 0.001) and lower é-wave (p < 0.005) compared with controls. CCA DI correlated with Apo-B/Apo-A1-ratio and Apo-A1. Follow-up time after treatment correlated with decreased left ventricular ejection fraction (p = 0.001). Conclusion: Young asymptomatic CCS exhibit cardiac, vascular, lipid, and apolipoprotein changes that could account for increased risk for CVD later in life. These findings emphasize the importance of cardiometabolic monitoring even in young CCS.
17.	Broberg, Olof, et al. (författare) Childhood Cancer Survivors Have Impaired Strain-Derived Myocardial Contractile Reserve by Dobutamine Stress Echocardiography 2023 Ingår i: Journal of Clinical Medicine. - 2077-0383. ; 12:8, s. 1-14 Tidskriftsartikel (refereegranskat)abstract Abnormal left ventricular contractile reserve (LVCR) is associated with adverse cardiac outcomes in different patient cohorts and might be useful in the detection of cardiomyopathy in childhood cancer survivors (CCS) after cardiotoxic treatment. The aim of this study was to evaluate LVCR by dobutamine stress echocardiography (DSE) combined with measures of myocardial strain in CCS previously treated with anthracyclines (AC). Fifty-three CCS (age 25.34 ± 2.44 years, 35 male) and 53 healthy controls (age 24.40 ± 2.40 years, 32 male) were included. Subjects were examined with echocardiography at rest, at low-dose (5 micrograms/kg/min), and at high-dose (40 micrograms/kg/min) dobutamine infusion. Left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS), strain rate (GSR), and early diastolic strain rate (GEDSR) at different DSE phases were used as measures of LVCR. The mean follow-up time among CCS was 15.8 ± 5.8 years. GLS, GSR, and LVEF were lower at rest in CCS compared to controls (p ≤ 0.03). LVEF was within the normal range in CCS. ΔGLS, ΔGSR, and ΔGEDSR but not ΔLVEF were lower in CCS compared to controls after both low- (p ≤ 0.048) and high-dose dobutamine infusion (p ≤ 0.023). We conclude that strain measures during low-dose DSE detect impaired myocardial contractile reserve in young CCS treated with AC at 15-year follow-up. Thus, DSE may help identify asymptomatic CCS at risk for heart failure and allows for tailored follow-up accordingly.
18.	Broberg, Olof, et al. (författare) Circulating leptin is associated with adverse vascular changes in young adult survivors of childhood cancer 2024 Ingår i: Cardiology in the Young. - 1047-9511 .- 1467-1107. Tidskriftsartikel (refereegranskat)abstract INTRODUCTION: Proteomics may help discover novel biomarkers and underlying mechanisms for cardiovascular disease. This could be useful for childhood cancer survivors as they show an increased risk of cardiovascular disease. The aim of this study was to investigate circulating cardiovascular proteins in young adult survivors of childhood cancer and their relationship to previously reported subclinical cardiovascular disease.METHODS: Ninety-two cardiovascular proteins were measured in 57 childhood cancer survivors and in 52 controls. For proteins that were significantly different between childhood cancer survivors and controls, we performed correlations between protein levels and measures of peripheral arterial stiffness (carotid distensibility and stiffness index, and augmentation index) and endothelial dysfunction (reactive hyperemia index).RESULTS: Leptin was significantly higher in childhood cancer survivors compared to controls (normalized protein expression units: childhood cancer survivors 6.4 (1.5) versus 5.1 (1.7), p < 0.0000001) after taking multiple tests into account. Kidney injury molecule-1, MER proto-oncogene tyrosine kinase, selectin P ligand, decorin, alpha-1-microglobulin/bikunin precursor protein, and pentraxin 3 showed a trend towards group differences (p < 0.05). Among childhood cancer survivors, leptin was associated with anthracycline treatment after adjustment for age, sex, and body mass index (p < 0.0001). Higher leptin correlated with lower carotid distensibility after adjustment for age, sex, body mass index, and treatments with radiotherapy and anthracyclines (p = 0.005).CONCLUSION: This proteomics approach identified that leptin is higher in young asymptomatic adult survivors of childhood cancer than in healthy controls and is associated with adverse vascular changes. This could indicate a role for leptin in driving the cardiovascular disease burden in this population.
19.	Christierson, Lea, et al. (författare) Multi-Modal in Vitro Experiments Mimicking the Flow Through a Mitral Heart Valve Phantom Ingår i: Cardiovascular Engineering and Technology. - 1869-408X. Tidskriftsartikel (refereegranskat)abstract Purpose: Fluid-structure interaction (FSI) models are more commonly applied in medical research as computational power is increasing. However, understanding the accuracy of FSI models is crucial, especially in the context of heart valve disease in patient-specific models. Therefore, this study aimed to create a multi-modal benchmarking data set for cardiac-inspired FSI models, based on clinically important parameters, such as the pressure, velocity, and valve opening, with an in vitro phantom setup. Method: An in vitro setup was developed with a 3D-printed phantom mimicking the left heart, including a deforming mitral valve. A range of pulsatile flows were created with a computer-controlled motor-and-pump setup. Catheter pressure measurements, magnetic resonance imaging (MRI), and echocardiography (Echo) imaging were used to measure pressure and velocity in the domain. Furthermore, the valve opening was quantified based on cine MRI and Echo images. Result: The experimental setup, with 0.5% cycle-to-cycle variation, was successfully built and six different flow cases were investigated. Higher velocity through the mitral valve was observed for increased cardiac output. The pressure difference across the valve also followed this trend. The flow in the phantom was qualitatively assessed by the velocity profile in the ventricle and by streamlines obtained from 4D phase-contrast MRI. Conclusion: A multi-modal set of data for validation of FSI models has been created, based on parameters relevant for diagnosis of heart valve disease. All data is publicly available for future development of computational heart valve models.
20.	Clausen, Henning, et al. (författare) Evaluation of Circulating Cardiovascular Biomarker Levels for Early Detection of Congenital Heart Disease in Newborns in Sweden 2020 Ingår i: JAMA Network Open. - : American Medical Association (AMA). - 2574-3805. ; 3:12 Tidskriftsartikel (refereegranskat)abstract Importance: Congenital heart disease (CHD) is the most common congenital malformation in humans worldwide. Circulating cardiovascular biomarkers could potentially improve the early detection of CHD, even in asymptomatic newborns. Objectives: To assess the performance of a dried blood spot (DBS) test to measure the cardiovascular biomarker amino terminal fragment of the prohormone brain-type natriuretic peptide (NT-proBNP) levels in newborns and to compare DBS with standard EDTA analysis in control newborns during the first week of life. Design, Setting, and Participants: This diagnostic study was conducted in a single regional pediatric service in southern Sweden. Healthy, term neonates born between July 1, 2018, and May 31, 2019, were prospectively enrolled and compared against retrospectively identified newborns with CHD born between September 1, 2003, and September 30, 2019. Neonates who required inpatient treatment beyond the standard postnatal care were excluded. Exposure: New DBS test for NT-proBNP quantification in newborns that used 3 μL of blood vs the current screening standard. Main Outcomes and Measures: Performance of the new test and when combined with pulse oximetry screening was measured by receiver operating characteristic curve analysis. Performance of the new test and EDTA screening was compared using Pearson linear correlation analysis. Results: The DBS samples of 115 neonates (81 control newborns and 34 newborns with CHD, of whom 63 were boys [55%] and the mean [SD] gestational age was 39.6 [1.4] weeks) were analyzed. The new NT-proBNP test alone identified 71% (n = 24 of 34) of all CHD cases and 68% (n = 13 of 19) of critical CHD cases as soon as 2 days after birth. Detection of any CHD type improved to 82% (n = 28 of 34 newborns) and detection of critical CHD improved to 89% (n = 17 of 19 newborns) when combined pulse oximetry screening and NT-proBNP test results were used. Performance of the NT-proBNP test was excellent when control newborns were matched to newborns with CHD born between July 1, 2018, and May 31, 2019 (area under the curve, 0.96; SE, 0.027; 95% CI, 0.908-1.0; asymptotic P < .05). Conclusions and Relevance: This study found that NT-proBNP assay using minimal DBS samples appears to be timely and accurate in detecting CHD in newborns and to discriminate well between healthy newborns and newborns with various types of CHD. This finding warrants further studies in larger cohorts and highlights the potential of NT-proBNP to improve neonatal CHD screening.
21.	Clausen, Henning, et al. (författare) Newborn Screening for High-Risk Congenital Heart Disease by Dried Blood Spot Biomarker Analysis. 2024 Ingår i: JAMA Network Open. - 2574-3805. ; 7:6 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.
22.	Clausen, Henning, et al. (författare) Single-centre experience of balloon valvuloplasty for severe aortic stenosis in childhood: could horizontal aortic root angulation predict outcomes? 2022 Ingår i: Cardiology in the Young. - 1467-1107. ; 32:S2, s. 197-198 Konferensbidrag (refereegranskat)
23.	Dalén, Magnus, et al. (författare) Long‐Term Survival After Single‐Ventricle Palliation : A Swedish Nationwide Cohort Study 2024 Ingår i: Journal of the American Heart Association. - 2047-9980. ; 13:6 Tidskriftsartikel (refereegranskat)abstract BackgroundLong‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported.Methods and ResultsThis nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow‐up was 98.7% complete (10 patients emigrated). Mean follow‐up was 11.3 years (maximum, 26.7 years). Long‐term survival was significantly higher in patients with left ventricular compared with RV dominance (10‐year survival: 91.0% [95% CI, 87.3%–93.6%] versus 71.1% [95% CI, 66.4%–75.2%]). RV dominance had a significant impact on outcomes after first‐stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology.ConclusionsThis study provides clinically relevant knowledge about the long‐term prognosis in patients with different underlying cardiac anomalies undergoing single‐ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation.RegistrationURL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.
24.	Englund, Emma, et al. (författare) Adverse Lipid and Inflammatory Changes in Young Nondiabetic First-Degree Relatives of Type 1-Diabetic Patients 2018 Ingår i: Cardiology in the Young. - 1467-1107. ; 28:S1, s. 43-44 Konferensbidrag (refereegranskat)abstract Background: Previous studies from our and other centers on young patients with type 1 diabetes (T1D) have indicated pro-atherosclerotic changes in the carotid arteries and in the lipid and systemic inflammatory profiles without a clear relationship between these changes and the hyperglycemic control. We have also earlier found in these patients a certain HLA-related genetic susceptibility to adverse vascular changes.Objective: To assess whether pro-atherosclerotic changes are present in apparently healthy first- degree relatives of patients with T1D.Methods: Plasma lipids, C-peptide (index of insulin secretion), C-reactive protein (CRP), and the carotid artery intima-media thickness (CA-IMT), compliance (CAC) and stiffness index (SI) were assessed in up to 116 non-diabetic first-degree relatives (FDR; mean age: 12.6 years; 56 female) of patients with T1D and in up to 43 age-matched control individuals (mean age: 13.3 years; 23 female) without heredity for cardiovascular risk factors. The number and type of clinically manifest acute infections during the past year were obtained via a questionnaire validated in our previous studies.Results: There was no difference in age, gender, body mass index, arterial blood pressure and C- peptide levels between the FDR and control groups (p>0.2). Also, there was no significant difference in SI (p=0.2), CAC (p=0.1) and CA-IMT (p=0.9) between the groups. In the FDR group, plasma CRP and LDL- cholesterol (LDL-C) were increased (p<0.05 for both) and HDL-C was decreased (p<0.0001) as compared to the control group. The changes in LDL-C and HDL-C were most marked in FDR individuals with ≥ 4 acute respiratory infections during the past year (Figure).Conclusion: Adverse lipidemic and inflammatory changes are present in healthy non-diabetic first- degree relatives of T1D patients, probably related to their genetic susceptibility to this disease. Further analyses of additional indices of peripheral arterial function in these groups are under way.
25.	Englund, Emma, et al. (författare) Circulating Levels of Endostatin Are Increased in Young Nondiabetic First-Degree Relatives of Patients With Type 1 Diabetes 2019 Ingår i: Cardiology in the Young. - 1467-1107. ; 29:S1 Konferensbidrag (refereegranskat)
26.	Fernlund, Eva I., et al. (författare) Serum biomarkers of early stages of hypertrophic cardiomyopathy in a young population 2015 Ingår i: Journal of the American College of Cardiology. - 0735-1097. ; 65:10S, s. 787-787 Konferensbidrag (refereegranskat)abstract Background: Hypertrophic cardiomyopathy (HCM) is the most common monogenic cardiac disorder and the leading cause of sudden cardiac death in the young. Although in a majority of HCM cases there are gene mutations coding for sarcomere proteins, the onset for the clinical consequences of these mutations are difficult to predict, as these mutations do not show any clear relationship to the degree of myocardial hypertrophy. Hence identification of early markers for this disease is important. The aim of this study was to investigate novel serum biomarkers reflecting myocardial remodeling, microfibrosis and coronary endotheliopathy in young presymtomatic HCM patients and in individuals at risk for developing HCM. Methods: Eighty-nine participants (18 HCM patients, 14 HCM-risk individuals, and 57 healthy controls) with median age of 15 (range 0-30) years underwent assessment with echocardiography and serum analysis for myostatin, cathepsin S, endostatin, type I collagen degradation marker (ICTP), matrix metalloproteinase (MMP) 9, vascular (VCAM) and intercellular adhesion molecules (ICAM). In some individuals, myocardial perfusion was measured both at rest and after adenosine via magnetic resonance. Results: Both cathepsin S and endostatin were increased in the HCM group (p0.3) and diastolic function, expressed as E/e' (p0.3). In the HCM-risk group, myostatin was decreased (p0.1). Conclusion: To the best of our knowledge, this is the first study to suggest early onset changes in biomarkers of myoblast regulation, endothelial function and matrix remodeling in young presymptomatic HCM patients and in HCM-risk individuals.
27.	Fernlund, Eva, et al. (författare) Peripheral microvascular function is altered in young individuals at risk for hypertrophic cardiomyopathy and correlates with myocardial diastolic function 2015 Ingår i: American Journal of Physiology. Heart and Circulatory Physiology. - : American Physiological Society. - 0363-6135 .- 1522-1539. ; 308:11, s. H1351-H1358 Tidskriftsartikel (refereegranskat)abstract Hypertrophic cardiomyopathy (HCM) is a major cause of sudden cardiac death in the young. Based on previous reports of functional abnormalities in not only coronary but also peripheral vessels in adults with HCM, we aimed to assess both peripheral vascular and myocardial diastolic function in young individuals with an early stage of HCM and in individuals at risk for HCM. Children, adolescents, and young adults (mean age: 12 yr) with a family history of HCM who either had (HCM group; n = 36) or did not have (HCM-risk group; n = 30) echocardiography-documented left ventricular (LV) hypertrophy as well as healthy matched controls (n = 85) and healthy young athletes (n = 12) were included in the study. All underwent assessment with 12-lead electrocardiography, two-dimensional echocardiography, tissue Doppler imaging and laser Doppler with transdermal iontophoresis of ACh and sodium nitroprusside. LV thickness and mass were increased in HCM and athlete groups compared with control and HCM-risk groups. The mitral E-to-e ratio, measured via tissue Doppler, was increased in HCM (P less than 0.0001) and HCM-risk (P less than 0.01) groups compared with control and athlete groups, as were microvascular responses to ACh (HCM group: P less than 0.045 and HCM- risk group: P less than 0.02). Responses to ACh correlated with the E-to-e ratio (r = 0.5, P = 0.001). Microvascular responses to sodium nitroprusside were similar in all groups (P = 0.2). HCM-causing mutations or its familial history are associated with changes in cardiac diastolic function and peripheral microvascular function even before the onset of myocardial hypertrophy. Tissue Doppler can be used to differentiate HCM from physiological LV hypertrophy in young athletes.
28.	Fricke, Katrin, et al. (författare) Fetal cardiac magnetic resonance imaging of the descending aorta in suspected left-sided cardiac obstructions 2023 Ingår i: Frontiers in Cardiovascular Medicine. - 2297-055X. ; 10, s. 01-10 Tidskriftsartikel (refereegranskat)abstract Background: Severe left-sided cardiac obstructions are associated with high morbidity and mortality if not detected in time. The correct prenatal diagnosis of coarctation of the aorta (CoA) is difficult. Fetal cardiac magnetic resonance imaging (CMR) may improve the prenatal diagnosis of complex congenital heart defects. Flow measurements in the ascending aorta could aid in predicting postnatal CoA, but its accurate visualization is challenging.Objectives: To compare the flow in the descending aorta (DAo) and umbilical vein (UV) in fetuses with suspected left-sided cardiac obstructions with and without the need for postnatal intervention and healthy controls by fetal phase-contrast CMR flow. A second objective was to determine if adding fetal CMR to echocardiography (echo) improves the fetal CoA diagnosis.Methods: Prospective fetal CMR phase-contrast flow in the DAo and UV and echo studies were conducted between 2017 and 2022.Results: A total of 46 fetuses with suspected left-sided cardiac obstructions [11 hypoplastic left heart syndrome (HLHS), five critical aortic stenosis (cAS), and 30 CoA] and five controls were included. Neonatal interventions for left-sided cardiac obstructions (n = 23) or comfort care (n = 1 with HLHS) were pursued in all 16 fetuses with suspected HLHS or cAS and in eight (27%) fetuses with true CoA. DAo or UV flow was not different in fetuses with and without need of intervention. However, DAo and UV flows were lower in fetuses with either retrograde isthmic systolic flow [DAo flow 253 (72) vs. 261 (97) ml/kg/min, p = 0.035; UV flow 113 (75) vs. 161 (81) ml/kg/min, p = 0.04] or with suspected CoA and restrictive atrial septum [DAo flow 200 (71) vs. 268 (94) ml/kg/min, p = 0.04; UV flow 89 vs. 159 (76) ml/kg/min, p = 0.04] as well as in those without these changes. Adding fetal CMR to fetal echo predictors for postnatal CoA did not improve the diagnosis of CoA.Conclusion: Fetal CMR-derived DAo and UV flow measurements do not improve the prenatal diagnosis of left-sided cardiac obstructions, but they could be important in identifying fetuses with a more severe decrease in blood flow across the left side of the heart. The physiological explanation may be a markedly decreased left ventricular cardiac output with subsequent retrograde systolic isthmic flow and decreased total DAo flow.
29.	Fricke, Katrin, et al. (författare) Fetal Echocardiographic Dimension Indices: Important Predictors of Postnatal Coarctation 2021 Ingår i: Pediatric Cardiology. - : Springer Science and Business Media LLC. - 0172-0643 .- 1432-1971. ; 42:3, s. 517-525 Tidskriftsartikel (refereegranskat)abstract The aim of the study is to identify reliable quantitative fetal echocardiographic predictors for postnatal development of coarctation (CoA). In this retrospective study, we included 65 fetuses with a prenatally suspected, isolated CoA, born 2010–2018. Dimensions of the cardiac structures, aortic, and ductal arches expressed as ratios and Z-scores were analyzed in relation to outcome. Fetuses that developed CoA postnatally (34%) exhibited significantly smaller Z-scores of left cardiac structures from the mitral valve to the aortic isthmus. The most sensitive and specific predictors were a carotid-subclavian artery index (CSAI) of < 0.78 (92.3% sensitivity, 96.8% specificity) or a product of isthmus-to-duct ratio in the three-vessel trachea view (3VT) and the mitral-to-tricuspid valve ratio (I/D3VTxMV/TV) of < 0.37 (100% sensitivity, 94.6% specificity). When comparing different Z-score datasets, we observed large and highly significant differences. Postnatal CoA can be predicted with high accuracy during fetal life using CSAI or I/D3VTxMV/TV. The latter may be particularly useful if adequate sagittal aortic arch images cannot be obtained. As significant and clinically unacceptable differences in Z-scores were observed for the same measurements, this calls for a large multi-center collaboration to generate reliable fetal echocardiographic Z-scores.
30.	Fricke, Katrin, et al. (författare) 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 Ingår i: Journal of the American Heart Association. - : John Wiley & Sons. - 2047-9980. ; 11:7 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.
31.	Frieberg, Petter, et al. (författare) Computational Fluid Dynamics Support for Fontan Planning in Minutes, Not Hours : The Next Step in Clinical Pre-Interventional Simulations 2022 Ingår i: Journal of Cardiovascular Translational Research. - : Springer Science and Business Media LLC. - 1937-5395 .- 1937-5387. ; 15:4, s. 708-720 Tidskriftsartikel (refereegranskat)abstract Computational fluid dynamics (CFD) modeling may aid in planning of invasive interventions in Fontan patients. Clinical application of current CFD techniques is however limited by complexity and long computation times. Therefore, we validated a "lean" CFD method to magnetic resonance imaging (MRI) and an "established" CFD method, ultimately aiming to reduce complexity to enable predictive CFD during ongoing interventions. Fifteen Fontan patients underwent MRI for CFD modeling. The differences between lean and established approach, in hepatic and total flow percentage to the left pulmonary artery (%LPA), power loss and relative wall shear stress area were 1.5 ± 4.0%, -0.17 ± 1.1%, -0.055 ± 0.092 mW and 1.1 ± 1.4%. Compared with MRI, the lean and established method showed a bias in %LPA of -1.9 ± 3.4% and -1.8 ± 3.1%. Computation time was for the lean and established approach 3.0 ± 2.0 min and 7.0 ± 3.4 h, respectively. We conclude that the proposed lean method provides fast and reliable results for future CFD support during interventions.
32.	Frieberg, Petter, et al. (författare) Improvement of computational fluid dynamics simulations of flow in patients with total cavo pulmonary connection and predicting interventional outcomes 2016 Ingår i: Journal of Cardiovascular Magnetic Resonance. - 1097-6647. ; 18, s. 1-3 Tidskriftsartikel (refereegranskat)
33.	Frieberg, Petter, et al. (författare) In vivo hepatic flow distribution by computational fluid dynamics can predict pulmonary flow distribution in patients with Fontan circulation 2023 Ingår i: Scientific Reports. - 2045-2322. ; 13:1 Tidskriftsartikel (refereegranskat)abstract In Fontan patients, a lung deprived of hepatic blood may develop pulmonary arterio-venous malformations (PAVMs) resulting in shunting, reduced pulmonary vascular resistance (PVR) and decreased oxygenation. To provide guidance for corrective invasive interventions, we aimed to non-invasively determine how the hepatic to pulmonary blood flow balance correlates with pulmonary flow, PVR, and with oxygen saturation. Magnetic resonance imaging (MRI) data from eighteen Fontan patients (eight females, age 3–14 years) was used to construct patient-specific computational fluid dynamics (CFD) models to calculate the hepatic to pulmonary blood flow. This was correlated with pulmonary vein flow, simulated PVR and oxygen saturation. Clinical applicability of the findings was demonstrated with an interventional patient case. The hepatic to pulmonary blood flow balance correlated with right/left pulmonary vein flow (R2 = 0.50), left/right simulated PVR (R2 = 0.47), and oxygen saturation at rest (R2 = 0.56). In the interventional patient, CFD predictions agreed with post-interventional MRI measurements and with regressions in the cohort. The balance of hepatic blood to the lungs has a continuous effect on PVR and oxygen saturation, even without PAVM diagnosis. MRI combined with CFD may help in planning of surgical and interventional designs affecting the hepatic to pulmonary blood flow balance in Fontan patients.
34.	Frieberg, Petter, et al. (författare) Simulation of aortopulmonary collateral flow in Fontan patients for use in prediction of interventional outcomes 2018 Ingår i: Clinical Physiology and Functional Imaging. - : Wiley. - 1475-097X .- 1475-0961. ; 38:4, s. 622-629 Tidskriftsartikel (refereegranskat)abstract Purpose: Patients with complex congenital heart disease may need to be converted to a Fontan circulation with systemic venous return surgically connected to the pulmonary circulation. These patients frequently form aortopulmonary collaterals (APC), that is arterial inflows to the pulmonary artery vascular tree. The aim of this study was to develop a method to calculate the effect of APC on the pulmonary flow distribution based on magnetic resonance imaging (MRI) measurements and computational fluid dynamics simulations in order to enable prediction of interventional outcomes in Fontan patients.Methods: Patient-specific models of 11 patients were constructed in a 3D-design software based on MRI segmentations. APC flow was quantified as the difference between pulmonary venous flow and pulmonary artery flow, measured by MRI. A method was developed to include the modulating effect of the APC flow by calculating the patient-specific relative pulmonary vascular resistance. Simulations, including interventions with a Y-graft replacement and a stent dilatation, were validated against MRI results.Results: The bias between simulated and MRI-measured fraction of blood to the left lung was 29 53%. Including the effects of the APC flow in the simulation (n = 6) reduced simulation error from 98 70% to 52 63%. Preliminary findings in two patients show that the effect of surgical and catheter interventions could be predicted using the demonstrated methods.Conclusions: The work demonstrates a novel method to include APC flow in predictive simulations of Fontan hemodynamics. APC flow was found to have a significant contribution to the pulmonary flow distribution in Fontan patients.
35.	Gyllenhammar, Tom, et al. (författare) Young patients with hypertrophic cardiomyopathy, but not subjects at risk, show decreased myocardial perfusion reserve quantified with CMR. 2014 Ingår i: European Heart Journal-Cardiovascular Imaging. - : Oxford University Press (OUP). - 2047-2412 .- 2047-2404. ; 15:12, s. 1350-1357 Tidskriftsartikel (refereegranskat)abstract To determine if myocardial perfusion (MP) during hyperaemia is decreased in young patients with hypertrophic cardiomyopathy (HCM). Also, to determine if an MP decrease is associated with diastolic dysfunction, and to investigate if young subjects at risk of HCM show differences in MP compared with controls.
36.	Hallböök, Tove, et al. (författare) Pediatric neurovascular disease. 2006 Ingår i: Acta Pædiatrica. - : Wiley. - 1651-2227 .- 0803-5253. ; 95:6, s. 766-766 Tidskriftsartikel (refereegranskat)
37.	Hidestrand, Pip, et al. (författare) Impact of Underweight, Overweight, and Obesity on Health-Related Quality of Life in Children with Tetralogy of Fallot Variants Ingår i: Pediatric Cardiology. - 0172-0643. Tidskriftsartikel (refereegranskat)abstract Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for cardiovascular morbidity and mortality. The aim of this study was to evaluate whether body mass index (BMI) is associated with health-related quality of life (HRQoL) in patients with variants of Tetralogy of Fallot (TOF). Patients and parents of children with variants of TOF–CHD were asked to fill out the PedsQL 4.0 questionnaire and provide weight and length. Patients were categorized into low, normal, and high BMI percentiles. Other demographic data were obtained from the Swedish national registry for congenital heart disease (SWEDCON). Statistical analyses included non-parametric Mann–Whitney U test, Fisher exact, and Chi-square tests. Eighty-five patients were included. Twelve were overweight or obese, 57 had a normal BMI, and 16 were underweight. There was a significant difference in age and gender between the groups. Comparing overweight/obese children to those with normal BMI, physical and social functioning were impaired, while emotional and school function were comparable between the groups. This applied to both child and parental assessment. When comparing underweight to normal weight children, school functioning assessed by the parent was the only domain significantly different from patients with a normal BMI. Children with variants of TOF and overweight/obesity have lower HRQoL, particularly in physical and social functioning, while underweight children may have impaired school functioning. We suggest that preventive measures aimed at maintaining a normal weight should be taken early in life to reduce long-term cardiovascular risk in the CHD population.
38.	Holmquist, Peter, et al. (författare) Urine pi-Glutathion S-transferase but not Tamm-Horsfall protein correlates with Carotid artery intima media thickness in childhood type 1 diabetes 2014 Ingår i: BMC Cardiovascular Disorders. - : Springer Science and Business Media LLC. - 1471-2261. ; 14 Tidskriftsartikel (refereegranskat)abstract Background: Renal disease remains a serious threat in patients with insulin-dependent (type1) diabetes. Hence its detection early in the life of patients with type1 diabetes is crucial. Several lines of evidence suggest similar mechanisms for the development of both renal and arterial disease. We sought to investigate in young patients with type1 diabetes whether pi-Glutathione S-transferase to creatinine (pi-GST:crea) and Tamm-Horsfall protein to creatinine (THP:crea) ratios, markers of distal tubular renal function, relate to subclinical markers of arterial disease, which appear to onset early and develop rapidly in type1 diabetes. Methods: Seventy-one children and adolescents (median age and diabetes duration 14 and 6 years, respectively) with type1 diabetes for at least 6 months were assessed for timed urine levels of pi-GST, THP, HbA1c, albumin, and plasma C-reactive protein (CRP). Carotid artery intima-media thickness (IMT), brachial artery flow-mediated dilatation (FMD), and cutaneous microvascular function were assessed by high-resolution ultrasound and laser Doppler, respectively. Results: Two patients had microalbuminuria (> 20 mu g/min), and were therefore removed from the study population. p-GST: crea ratio and THP: crea showed no relationship to the demographic, diabetes, or inflammatory indices. Lower p-GST: crea ratio was associated with greater IMT (p = 0.01, r = -0.29), particularly in female patients (p = 0.004, r =0.49). The association of p-GST: crea ratio with IMT was stronger in patients with passive smoke exposure (p = 0.002, r = -0.43). Among post-pubertal patients, lower pi-GST: crea ratio was also associated with lower microvascular response to Ach (acetylcholine; p = 0.03, r = 0.49). Conclusions: In young patients with type1 diabetes, proximal tubular dysfunction as suggested by lower levels of p-GST: crea ratio seems to be paralleled by changes in arterial structure and microvascular function.
39.	Holmquist, Peter, et al. (författare) Urine α-Glutathione S-Transferase, systemic inflammation and arterial function in juvenile type 1 diabetes. 2012 Ingår i: Journal of Diabetes and its Complications. - : Elsevier BV. - 1873-460X .- 1056-8727. ; 26:3, s. 199-204 Tidskriftsartikel (refereegranskat)abstract BACKGROUND: Despite marked improvement in therapy and monitoring of patients with insulin-dependent (type 1) diabetes, diabetic nephropathy remains a serious complication, with subsequent end-stage renal disease in about 20% of cases. OBJECTIVE: To investigate in young patients with type 1 diabetes whether urine α-Glutathione S-transferase to creatinine ratio (α-GST:crea) relates to markers of systemic inflammation and subclinical vasculopathy. DESIGN: Children and adolescents (median age and diabetes duration 14 and 6years, respectively) with type 1 diabetes screened in a previous study for proximal tubular (urine α-GST:crea ratio) and renal (plasma creatinine, cystatin C glomerular filtration rate (GFR), and timed urine albumin excretion rate (AER)) function were, within the same timeframe, also investigated for vascular (blood pressure, carotid artery intima-media thickness (IMT) and compliance (CAC), brachial artery flow-mediated dilatation (FMD) and plasma cyclic guanosine monophosphate (cGMP) and inflammatory (C-reactive protein (CRP), and tumor necrosis factor-alpha (TNF-α)) profiles. Exposure to environmental tobacco smoke (ETS) was assessed through questionnaire (n=67 respondents). RESULTS: None of the patients (n=69) had overt renal insufficiency. AER correlated with age (p=0.01, r=0.3), diabetes duration (p=0.02, r=0.3), FMD (p=0.04, r=-0.3, n=52), CAC (p=0.03, r=-0.3, n=62) and cGMP (p=0.01, r=-0.3, n=59). α-GST:crea was lower (p=0.03) in patients than in controls. α-GST:crea appeared to be particularly lower in older patients (p=0.004, r=-0.34 vs age), in those with worse diabetic control (p=0.03, r=-0.26 vs HbA1c), and in those with lower carotid artery elasticity (p=0.017, r=0.3 vs CAC). Although ETS had no direct significant impact on α-GST:crea, α-GST:crea correlated with FMD only in patients with ETS (r=0.5, p=0.009, n=13). α-GST:crea showed positive association with TNF-α (p=0.01, r=0.3). CONCLUSION: In children and adolescents with type 1 diabetes, lower levels of urine excretion of α-GST:crea appear to be associated with decreasing elasticity and endothelial vasomotor function of peripheral arteries, especially in patients with ETS. In contrast, higher levels of α-GST:crea are more common in patients with elevated markers of systemic inflammation. Large scale prospective studies are needed to clarify the meaning and mechanisms of this association.
40.	I. Fernlund, Eva, et al. (författare) Early repolarization in children with unexplained syncope 2011 Ingår i: Journal of Electrocardiology. - : Elsevier BV. - 1532-8430 .- 0022-0736. ; 44:2, s. 10-10 Konferensbidrag (refereegranskat)abstract Introduction: It has traditionally been believed that early repolarization (ER) is benign. Significant association between ER and sudden cardiac arrest due to idiopathic ventricular fibrillation was recently found in a large cohort of adult survivors of sudden cardiac arrest. In some prior studies, unexplained syncope has been linked to risk of sudden death, but the mechanisms remain speculative.We assessed herein the prevalence of ER in children referred to our center for unexplained syncope. Methods: We evaluated retrospectively electrocardiograms from such children (n = 29; mean age, 12.1 years; range, 7-18 years) for presence of ER, which was defined as an elevation of the QRS-ST junction (J-point) in at least 2 leads of at least 1 mm (0.1 mV) above the baseline level. The anterior precordial leads (V1-V3) were excluded from the analysis to avoid inclusion of patients with right ventricular dysplasia or Brugada syndrome. Agematched children (n = 33; mean age, 12.3 years; range, 7-16 years) with noncardiac chest pain were included as controls. Results: Early repolarization was detected in 45% (13/29) of children with unexplained syncope vs 24% (8/33) in the chest pain group. Among children with syncope, ER was far more frequent in males than in females (8/12 vs 5/ 17, respectively). Echocardiography showed normal functional and structural findings in all children. Conclusion: In this relatively small-scale retrospective study of children with unexplained syncope with otherwise normal cardiac findings, we found particularly among those of male gender a greater prevalence of ER than in controls (noncardiac chest pain).With view to earlier findings of Haisaguerre et al (NEJM 2008), this intriguing association warrants further prospective studies addressing its precise clinical implication and underlying mechanisms.
41.	Jablonowski, Robert, et al. (författare) Regional adenosine-induced hypoperfusion without hyperenhancement on LGE-MRI in young HCM patients : Comparison to subjects at risk of HCM and healthy volunteers 2015 Ingår i: Journal of Cardiovascular Magnetic Resonance. - 1097-6647. ; 17:Suppl 1, s. 1-2 Konferensbidrag (refereegranskat)
42.	Jablonowski, Robert, et al. (författare) Regional Stress-Induced Ischemia in Non-fibrotic Hypertrophied Myocardium in Young HCM Patients. 2015 Ingår i: Pediatric Cardiology. - : Springer Science and Business Media LLC. - 0172-0643 .- 1432-1971. ; 36:8, s. 1662-1669 Tidskriftsartikel (refereegranskat)abstract The relationship between hypertrophy, perfusion abnormalities and fibrosis is unknown in young patients with hypertrophic cardiomyopathy (HCM). Since mounting evidence suggests causal relationship between myocardial ischemia and major adverse cardiac events, we sought to investigate whether (1) regional myocardial perfusion is decreased in young HCM patients and in individuals at risk of HCM, and (2) hypoperfused areas are larger than areas with fibrosis. HCM patients (n = 12), HCM-risk subjects (n = 15) and controls (n = 9) were imaged on a 1.5 T MRI scanner. Myocardial hypertrophy was assessed on cine images. Perfusion images were acquired during adenosine hyperemia and at rest. Maximum upslope ratios of perfusion (stress/rest) were used for semiquantitative analysis. Fibrosis was assessed by late gadolinium enhancement (LGE). Results are presented as median and range. Perfusion in HCM-risk subjects and in non-hypertrophied segments in HCM patients showed no difference compared to controls (P = ns). Hypertrophic segments in HCM patients without LGE showed decreased perfusion compared to segments without hypertrophy [1.5 (1.1-2.3) vs. 2.0 (1.8-2.6), P < 0.001], and hypertrophic segments with LGE showed even lower perfusion using a segmental analysis [0.9 (0.6-1.8), P < 0.05]. The extent of hypoperfused myocardium in HCM patients during adenosine exceeded the extent of fibrosis on LGE [20 (0-48) vs. 4 (0-7) % slice area, P < 0.05] and hypoperfused areas at rest (P < 0.001). Regional perfusion is decreased in hypertrophied compared to non-hypertrophied myocardium and is lowest in fibrotic myocardium in young HCM patients but does not discriminate HCM-risk subjects from controls. The stress-induced hypoperfused regions exceed regions with LGE, indicating that hypoperfusion precedes fibrosis and may be a more sensitive marker of diseased myocardium in HCM.
43.	Jacobs, Jeffrey P., et al. (författare) Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database : The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC) 2023 Ingår i: World Journal for Pediatric and Congenital Heart Surgery. - 2150-1351. ; 14:4, s. 464-473 Tidskriftsartikel (refereegranskat)abstract The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.
44.	Jacobs, Jeffrey P., et al. (författare) Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database : The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC) 2023 Ingår i: Cardiology in the Young. - 1047-9511. ; 33:7, s. 1043-1059 Tidskriftsartikel (refereegranskat)abstract The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.
45.	Lannering, Katarina, et al. (författare) Screening for Critical Congenital Heart Defects in Sweden 2023 Ingår i: PEDIATRICS. - : American Academy of Pediatrics (AAP). - 0031-4005 .- 1098-4275. ; 152:4 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.
46.	Lannering, Katarina, et al. (författare) Screening for critical congenital heart defects in sweden 2024 Ingår i: Obstetrical and Gynecological Survey. - : Lippincott Williams & Wilkins. - 0029-7828 .- 1533-9866. ; 79:4, s. 185-187 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.
47.	Lewis, Michael J., et al. (författare) Long-Term Follow-Up of Right Ventricle to Pulmonary Artery Biologic Valved Conduits Used in Pediatric Congenital Heart Surgery 2023 Ingår i: Pediatric Cardiology. - : Springer Science and Business Media LLC. - 0172-0643 .- 1432-1971. ; 44:1, s. 102-115 Tidskriftsartikel (refereegranskat)abstract Valved conduit reconstruction between the right ventricle (RV) and the pulmonary circulation is often necessary in the surgical treatment of complex congenital heart defects. The aim of this study is to evaluate the long-term performance of the three types of conduits we have used and assess risk factors for conduit failure. Retrospective, single-center review of 455 consecutive pediatric patients with 625 conduits from 1990 to 2019 undergoing RV-to-pulmonary artery (PA) reconstruction with a valved conduit. The three conduit types investigated were pulmonary homograft, aorta homograft, and bovine jugular vein (BJV) graft. Overall patient survival was 91.4%, freedom from conduit replacement (FCR) was 47.4%, and freedom from reintervention (FFR) was 37.8% with a median follow-up of 8.7 years (interquartile range 4.3–13.3 years). For pulmonary homografts, 10-, 20-, and 28-year FCR was 79.6%, 68.6%, and 66.0%, respectively. For aortic homografts, 10-, 20-, and 30-year FCR was 49.8%, 31.5%, and 23.0%, respectively. For BJV grafts, 10- and 19-year FCR was 68.1% and 46.0%, respectively. When controlling for baseline variables, FCR was similar for pulmonary homografts and BJV grafts. Overall patient survival was excellent. Risk factors for conduit failure in patients operated with reconstruction of the RV-PA outflow tract included low age, low weight, small conduit size, and certain cardiac diagnoses. There was no evidence for a shorter life span of the second graft. Pulmonary homografts and BJV grafts performed similarly but the risk of endocarditis was greater in the BJV group.
48.	Liuba, Petru, et al. (författare) Acute Chlamydia pneumoniae infection causes coronary endothelial dysfunction in pigs. 2003 Ingår i: Atherosclerosis. - 1879-1484. ; 167:2, s. 215-222 Tidskriftsartikel (refereegranskat)abstract Background: Coronary endothelial dysfunction contributes to the pathogenesis of acute coronary syndromes (ACSs). Acute Chlamydia pneumoniae infection has been epidemiologically associated with ACS. In this study, we investigated whether acute C. pneumoniae infection could alter the endothelial vasomotor function of porcine coronary vessels. Methods and results: Twenty pigs, 7–9 kg in weight, were inoculated intratracheally with C. pneumoniae (n=12) or saline (n=8), and investigated at 3 days (five infected/four non-infected) and 2 weeks (5+2 infected/four non-infected) after inoculation. The endothelium-dependent reactivity of coronary microcirculation was assessed at both time points by measuring peak coronary flow velocity (CFV) in response to bradykinin, before and after infusions with glutathione, an antioxidant, and Image-arginine, a substrate for nitric oxide synthase (NOS). CFV after bradykinin was significantly decreased in infected animals at both time points. At 2 weeks, both glutathione and Image-arginine significantly improved CFV after bradykinin. CFV after sodium nitroprusside (SNP) was similar in both groups. At 3 days, the relaxation responses of bradykinin-induced pre-contracted left anterior descending (LAD) coronary rings to bradykinin were significantly less in infected animals. NG-nitro-Image-arginine-methyl-ester, an NOS inhibitor, had significantly greater inhibitory effect on bradykinin-induced relaxation in infected animals. Plasma nitrate–nitrite and fibrinogen, and NOS activity from LAD coronary samples were significantly increased in infected animals. Conclusion: Acute C. pneumoniae infection causes endothelial dysfunction of both resistance and epicardial coronary vessels, and favours a pro-coagulant status. These effects could in part account for the epidemiologically suggested association between acute infection and ACS.
49.	Liuba, Petru, et al. (författare) Acute infections in children are accompanied by oxidative modification of LDL and decrease of HDL cholesterol, and are followed by thickening of carotid intima-media. 2003 Ingår i: European Heart Journal. - 1522-9645. ; 24:6, s. 517-523 Tidskriftsartikel (refereegranskat)abstract Background Atherosclerosis begins early in life. Infections might contribute to the pathogenesis of atherosclerosis. In this study, we investigated whether acute infections in children could alter the carotid wall morphology and the tipid profile. Methods Mean carotid intima-media thickness (IMT) was measured by high-resolution ultrasound in 28 hospitatised children (mean age: 5 2 years), who fulfilled the diagnostic criteria of acute infections (body temperature, >38 degreesC; C-reactive protein, >15 mg/ml, and clinical), and in 20 age- and gender-matched controls. Antibodies against oxidised tow-density lipoprotein (anti-oxLDL antibodies), as well as total and high-density lipoprotein cholesterol (HDL-C) were analysed in all children. The infection group was investigated both during the acute illness and 3 months after clinical recovery (post-infection). Results During the acute illness, the infection group had elevated anti-oxLDL antibodies and decreased HDL-C, as compared to those obtained at 3 months and in controls (p<0.05). These changes in the infection group were followed, at 3 months, by thickening of carotid intima-media. Those who received antibiotics during their acute illness had less carotid thickening than those who were not treated with antibiotics (p<0.05). Conclusion Acute infections in children seem to be accompanied by enhanced oxidative modification of LDL and by decrease in HDL-C. These lipid changes may be followed by thickening of carotid artery intima-media. These findings suggest that, in childhood, acute infections could be-associated with increased risk of atherosclerosis, and warrant further studies on this topic. (C) 2003 The European Society of Cardiology.
50.	Liuba, Petru, et al. (författare) Acute respiratory viral infections aggravate arterial endothelial dysfunction in children with type 1 diabetes mellitus. 2004 Ingår i: Circulation. - 1524-4539. ; 109:7, s. 118-118 Konferensbidrag (refereegranskat)

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