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1.
Article in English | MEDLINE | ID: mdl-38531618

ABSTRACT

BACKGROUND: This study focuses on biomarkers in infants after open heart surgery, and examines the association of high-sensitive troponin T (hs-cTnT), interleukin-6 (IL-6), and interleukin-8 (IL-8) with postoperative acute kidney injury (AKI), ventilatory support time and need of vasoactive drugs. METHODS: Secondary exploratory study from a double-blinded clinical randomized trial (Mile-1) on 70 infants undergoing open heart surgery with cardiopulmonary bypass (CPB). In this sub-study, the entire study population was examined without considering the study drugs. The biomarkers' peak concentration (highest concentration at 2 or 6 h post-CPB) were used for statistical analyses. RESULTS: Peak IL-8, hs-cTnT, and IL-6 occurred at 2 h post-CPB for 96%, 79%, and 63% of the patients, respectively. The odds ratio of developing AKI2-3 for IL-6 > 293 pg/mL was 23.4 (95% CI 5.3;104.0), for IL-8 > 100 pg/mL it was 11.5 (3.0;44.2), and for hs-cTnT >5597 pg/mL it was 6.1 (1.5; 24.5). In more than two third of the patients with the highest peak concentrations of IL-8, IL-6, and hs-cTnT, there was a need for ventilatory support for >24 h and use of vasoactive drugs at 24 h post-CPB, while in less than one third of the patients with the lowest peak concentrations of IL-8 and hs-cTnT such requirements were observed. CONCLUSIONS: The peak biomarker concentrations and CPB-time strongly predicted AKI2-3, with IL-6 and IL-8 emerging as strongest predictors. Furthermore, our findings suggest that measuring hs-cTnT and IL-8 just 2 h post-CPB-weaning may assist in identifying infants suitable for early extubation and highlight those at risk of prolonged ventilation.

2.
J Am Heart Assoc ; 13(6): e031722, 2024 Mar 19.
Article in English | MEDLINE | ID: mdl-38497454

ABSTRACT

BACKGROUND: Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. METHODS AND RESULTS: This 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. CONCLUSIONS: This 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. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.


Subject(s)
Fontan Procedure , Heart Defects, Congenital , Hypoplastic Left Heart Syndrome , Univentricular Heart , Child , Humans , Cohort Studies , Sweden/epidemiology , Hypoplastic Left Heart Syndrome/surgery , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Heart Ventricles/abnormalities , Treatment Outcome , Retrospective Studies
3.
Arch Dis Child ; 108(5): 398-404, 2023 05.
Article in English | MEDLINE | ID: mdl-36657799

ABSTRACT

OBJECTIVE: To compare long-term survival, reinterventions and risk factors using strict definitions of neonatal critical and non-critical valvular aortic stenosis (VAS). DESIGN: A nationwide retrospective study using data from patient files, echocardiograms and the Swedish National Population Registry. SETTING AND PATIENTS: All neonates in Sweden treated for isolated VAS 1994-2018. We applied the following criteria for critical aortic stenosis: valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function (fractional shortening ≤27%). Indication for treatment of non-critical VAS was Doppler mean gradient >50 mm Hg. MAIN OUTCOME MEASURES: Short-term and long-term survival, aortic valve reinterventions need of valve replacements, risk factors for reintervention and event-free survival. RESULTS: We identified 65 patients with critical VAS and 42 with non-critical VAS. The majority of the neonates were managed by surgical valvotomy. Median follow-up time was 13.5 years, with no patients lost to follow-up. There was no 30-day mortality. Long-term transplant-free survival was 91% in the critical stenosis group and 98% in the non-critical stenosis group (p=0.134). Event-free survival was 40% versus 67% (p=0.002) in the respective groups. Median time from the initial treatment to reintervention was 3.6 months versus 3.9 years, respectively (p=0.008). CONCLUSIONS: Critical VAS patients had significantly higher need for reintervention during the first year of life, lower event-free survival and lower freedom from aortic valve replacement at age ≥18 years, compared with neonates with non-critical stenosis.


Subject(s)
Aortic Valve Stenosis , Infant, Newborn , Humans , Adolescent , Retrospective Studies , Treatment Outcome , Constriction, Pathologic , Aortic Valve Stenosis/surgery , Aortic Valve/surgery
4.
Front Pediatr ; 10: 953770, 2022.
Article in English | MEDLINE | ID: mdl-36467479

ABSTRACT

Background: The present study presents a diagnostic course for the characterization of a congenital left ventricular outpouching (LVO) in a 4-year-old boy with severe neonatal heart failure, evaluating the added value of cardiac magnetic resonance (CMR) 4Dflow. Case presentation: A boy, born at full term, presented with heart failure immediately after birth. Echocardiography showed dilated left ventricle with poor function and LVO was initially interpreted as an aneurysm. No infection, inflammation, or other cause for heart failure was found. With intensive medical treatment, the heart function returned to normal, and eventually, all medication was terminated. At follow-up, surgical treatment of the LVO was discussed but after CMR 4Dflow, a thorough evaluation of the function of the left ventricle as well as the LVO was possible and the LVO was determined a double-chambered left ventricle with a good prognosis. Conclusions: The present case demonstrates the clinical usability of CMR 4Dflow for improved decision-making and risk assessment, revealing advanced hemodynamic flow patterns with no need for operation.

5.
J Am Heart Assoc ; 11(7): e022929, 2022 04 05.
Article in English | MEDLINE | ID: mdl-35348003

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.


Subject(s)
Hypoplastic Left Heart Syndrome , Mitral Valve Stenosis , Heart Ventricles/abnormalities , Heart Ventricles/diagnostic imaging , Humans , Hypoplastic Left Heart Syndrome/surgery , Palliative Care/methods , Retrospective Studies , Sweden/epidemiology , Treatment Outcome
6.
Scand Cardiovasc J ; 55(6): 345-353, 2021 12.
Article in English | MEDLINE | ID: mdl-34672849

ABSTRACT

Introduction. Congenital heart disease (CHD) is the most common type of birth defect today. The adult congenital heart disease (ACHD) population is constantly growing and becoming older and more patients require cardiac surgery. The objective of this study was to review the surgical outcome of the open heart procedures performed on ACHD patients in the last 10 years at Sahlgrenska University Hospital (SUH) through a retrospective descriptive cohort study. Methods. A retrospective data collection was performed for 421 patients who underwent a total of 439 surgical procedures between 2009 and 2018 at the Cardiothoracic department in SUH. The primary outcomes were early (<30 days) and late survival. Secondary outcomes were postoperative complications and independent risk factors for postoperative complications. Results. 30-day mortality was 1.9%. Long-term survival after 3, 5 and 10 years were 96% ± 1, 94.3% ± 1.3 and 92.4% ± 1.8. 82 major complications occurred after 46 procedures (11.6%). The most common major complication was re-exploration due to hemorrhage. Risk factors for major complications were acute surgery and prolonged extracorporeal circulation time. 173 minor complications occurred after 90 procedures (22.5%). The most common minor complication was prolonged intensive care unit stay (>48 h). Conclusion. This study presents satisfactory early and midterm survival. The survival and frequency of major postoperative complications are well in line with what other studies have presented. Patients undergoing resternotomies had no increased risk for mortality or postoperative complications.


Subject(s)
Cardiac Surgical Procedures , Heart Defects, Congenital , Adult , Cardiac Surgical Procedures/adverse effects , Cohort Studies , Heart Defects, Congenital/surgery , Hospital Mortality , Humans , Length of Stay , Postoperative Complications/etiology , Retrospective Studies , Risk Factors , Treatment Outcome
7.
Pediatr Crit Care Med ; 22(7): e402-e409, 2021 07 01.
Article in English | MEDLINE | ID: mdl-33739957

ABSTRACT

OBJECTIVES: We compared the effect of two inodilators, levosimendan and milrinone, on the plasma levels of myocardial injury biomarkers, that is, high-sensitivity troponin T and heart-type fatty acid binding protein, and on N-terminal prohormone of brain natriuretic peptide as a biomarker of ventricular function. We hypothesized that levosimendan could attenuate the degree of myocardial injury when compared with milrinone. DESIGN: A post hoc, nonprespecified exploratory secondary analysis of the Milrinone versus Levosimendan-1 trial (ClinicalTrials.gov Identifier: NCT02232399). SETTING: Two pediatric tertiary university hospitals. PATIENTS: Infants 1-12 months old, diagnosed with ventricular septal defect, complete atrioventricular septal defect, or Tetralogy of Fallot undergoing corrective surgery with cardiopulmonary bypass. INTERVENTIONS: Seventy patients received a loading dose of either levosimendan or milrinone at the start of cardiopulmonary bypass followed by an infusion of the respective drug, which continued for 26 hours. MEASUREMENTS AND MAIN RESULTS: Plasma levels of the three cardiac biomarkers were measured prior to the initiation of cardiopulmonary bypass and 2, 6, and 24 hours after weaning from cardiopulmonary bypass. In both groups, the levels of high-sensitivity troponin T and heart-type fatty acid binding protein were highest at 2 hours post cardiopulmonary bypass, whereas the highest level of N-terminal prohormone of brain natriuretic peptide occurred at 24 hours post cardiopulmonary bypass. There was no significant difference in the biomarkers' plasma levels between the study groups over time. Neither was there a significant difference in the postoperative peak plasma levels of the cardiac biomarkers. CONCLUSIONS: In this post hoc analysis of the MiLe-1 trial, there was no demonstrable difference in the postoperative cardiac biomarker profile of myocardial injury and ventricular function when comparing infants managed in the perioperative period with levosimendan versus milrinone.


Subject(s)
Cardiac Surgical Procedures , Milrinone , Simendan , Biomarkers , Cardiopulmonary Bypass , Cardiotonic Agents/adverse effects , Cardiotonic Agents/therapeutic use , Humans , Infant , Milrinone/adverse effects , Milrinone/therapeutic use , Simendan/adverse effects , Simendan/therapeutic use
8.
Acta Paediatr ; 110(5): 1610-1619, 2021 05.
Article in English | MEDLINE | ID: mdl-33351279

ABSTRACT

AIM: To study the long-term outcome after surgery for pulmonary atresia and ventricular septal defect (PA-VSD), and to determine association between the contribution of major aorto-pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death. METHODS: Patients who had undergone surgery for PA-VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross-checked against the Swedish National Population Register. RESULTS: Seventy patients were identified, giving an incidence of 5.3 newborns per 100 000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11-microdeletion syndrome (23%). Survival at follow-up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow-up time of 14.3 years (3.2-41.8 years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease. CONCLUSION: Long-term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome.


Subject(s)
Heart Septal Defects, Ventricular , Pulmonary Atresia , Collateral Circulation , Follow-Up Studies , Heart Septal Defects , Heart Septal Defects, Ventricular/surgery , Humans , Infant , Infant, Newborn , Pulmonary Artery , Pulmonary Atresia/surgery , Retrospective Studies
9.
J Am Heart Assoc ; 9(22): e017704, 2020 11 17.
Article in English | MEDLINE | ID: mdl-33153356

ABSTRACT

Background Despite advances in pediatric health care over recent decades, it is not clear whether survival in children with congenital heart disease (CHD) is still increasing. Methods and Results We identified all patients with CHD using nationwide Swedish health registries for 1980 to 2017. We examined the survival trends in children with CHD; we investigated the mortality risk in patients with CHD compared with matched controls without CHD from the general population using Cox proportional regression models and Kaplan-Meier survival analysis. Among 64 396 patients with CHD and 639 012 matched controls without CHD, 3845 (6.0%) and 2235 (0.3%) died, respectively. The mean study follow-up (SD) was 11.4 (6.3) years in patients with CHD. The mortality risk was 17.7 (95% CI, 16.8-18.6) times higher in children with CHD compared with controls. The highest mortality risk was found during the first 4 years of life in patients with CHD (hazard ratio [HR], 19.6; 95% CI, 18.5-20.7). When stratified by lesion group, patients with non-conotruncal defects had the highest risk (HR, 97.2; 95% CI, 80.4-117.4). Survival increased substantially according to birth decades, but with no improvement after the turn of the century where survivorship reached 97% in children with CHD born in 2010 to 2017. Conclusions Survival in children with CHD has increased substantially since the 1980s; however, no significant improvement has been observed this century. Currently, >97% of children with CHD can be expected to reach adulthood highlighting the need of life-time management.


Subject(s)
Heart Defects, Congenital/mortality , Adolescent , Case-Control Studies , Child , Child Mortality , Child, Preschool , Female , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/therapy , Humans , Infant , Kaplan-Meier Estimate , Male , Proportional Hazards Models , Registries , Survival Rate , Sweden
10.
Cardiol Young ; 30(9): 1321-1327, 2020 Sep.
Article in English | MEDLINE | ID: mdl-32727627

ABSTRACT

OBJECTIVE: This study describes short-term and long-term outcome after treatment of critical valvular aortic stenosis in neonates in a national cohort, with surgical valvotomy as first choice intervention. METHODS: All neonates in Sweden treated for critical aortic stenosis between 1994 and 2016 were included. Patient files were analysed and cross-checked against the Swedish National Population Registry as of December 2017, giving complete survival data. Diagnosis was confirmed by reviewing echo studies. Critical aortic stenosis was defined as valvular stenosis with duct-dependent systemic circulation or depressed left ventricular function. Primary outcome was all-cause mortality and secondary outcomes were reintervention and aortic valve replacement. RESULTS: Sixty-one patients were identified (50 boys, 11 girls). Primary treatment was surgical valvotomy in 52 neonates and balloon valvotomy in 6. Median age at initial treatment was 5 days (0-26), and median follow-up time was 10.8 years (0.14-22.6). There was no 30-day mortality but four late deaths. Freedom from reintervention was 66%, 61%, 54%, 49%, and 46% at 1, 5, 10, 15, and 20 years, respectively. Median time to reintervention was 3.4 months (4 days to 17.3 years). Valve replacement was performed in 23 patients (38%). CONCLUSIONS: Surgical valvotomy is a safe and reliable treatment in these critically ill neonates, with no 30-day mortality and long-term survival of 93% in this national study. At 10 years of age, reintervention was performed in 54% and at end of follow-up 38% had had an aortic valve replacement.


Subject(s)
Aortic Valve Stenosis , Aortic Valve/diagnostic imaging , Aortic Valve/surgery , Aortic Valve Stenosis/surgery , Female , Humans , Infant , Infant, Newborn , Male , Reoperation , Sweden/epidemiology , Treatment Outcome
11.
J Cardiothorac Vasc Anesth ; 34(8): 2072-2080, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32201198

ABSTRACT

OBJECTIVE: The present study aimed to determine the differential effects of intraoperative administration of milrinone versus levosimendan on myocardial function after pediatric cardiac surgery. Transthoracic echocardiography was used for myocardial function evaluation using biventricular longitudinal strain with 2-dimensional speckle tracking echocardiography in addition to conventional echocardiographic variables. DESIGN: A secondary analysis of a randomized, prospective, double-blinded clinical drug trial. SETTING: Two pediatric tertiary university hospitals. PARTICIPANTS: Infants between 1 and 12 months old diagnosed with ventricular septal defect, complete atrioventricular septal defect, or tetralogy of Fallot who were scheduled for corrective surgery with cardiopulmonary bypass. INTERVENTIONS: The patients were randomly assigned to receive an infusion of milrinone or levosimendan at the start of cardiopulmonary bypass and for 26 consecutive hours. MEASUREMENTS AND MAIN RESULTS: Biventricular longitudinal strain and conventional echocardiographic variables were measured preoperatively, on the first postoperative morning, and before hospital discharge. The association between perioperative parameters and postoperative myocardial function also was investigated. Images were analyzed for left ventricular (n = 67) and right ventricular (n = 44) function. The day after surgery, left ventricular longitudinal strain deteriorated in both the milrinone and levosimendan groups (33% and 39%, respectively). The difference was not significant. The corresponding deterioration in right ventricular longitudinal strain was 42% and 50% (nonsignificant difference). For both groups, biventricular longitudinal strain approached preoperative values at hospital discharge. Preoperative N-terminal pro-brain natriuretic peptide could predict the left ventricular strain on postoperative day 1 (p = 0.014). CONCLUSIONS: Levosimendan was comparable with milrinone for left and right ventricular inotropic support in pediatric cardiac surgery.


Subject(s)
Cardiac Surgical Procedures , Pyridazines , Cardiotonic Agents/therapeutic use , Child , Humans , Hydrazones , Infant , Milrinone , Prospective Studies , Simendan
12.
Pediatr Crit Care Med ; 20(10): 947-956, 2019 10.
Article in English | MEDLINE | ID: mdl-31274775

ABSTRACT

OBJECTIVES: It has been shown that, in contrast to other inotropic agents, levosimendan improves glomerular filtration rate after adult cardiac surgery. The aim of this study was to investigate the efficacy of levosimendan, compared with milrinone, in preventing acute kidney dysfunction in infants after open-heart surgery with cardiopulmonary bypass. DESIGN: Two-center, double-blinded, prospective, randomized clinical trial. SETTING: The study was performed in two tertiary pediatric centers, one in Sweden (Gothenburg) and one in Finland (Helsinki). PATIENTS: Infants between 1 and 12 months old, diagnosed with Tetralogy of Fallot, complete atrioventricular septal defect or nonrestrictive ventricular septal defect, undergoing total corrective cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: Seventy-two infants were randomized to receive a perioperative infusion of levosimendan (0.1 µg/kg/min) or milrinone (0.4 µg/kg/min). The infusion was initiated at the start of cardiopulmonary bypass and continued for 26 hours. MEASUREMENTS AND MAIN RESULTS: The primary outcome variable was the absolute value of serum creatinine data on postoperative day 1. Secondary outcomes included the following: 1) acute kidney injury according to the serum creatinine criteria of the Kidney Diseases: Improving Global Outcomes; 2) acute kidney injury with serum creatinine corrected for fluid balance; 3) plasma neutrophil gelatinase-associated lipocalin; 4) cystatin C; 5) urea; 6) lactate; 7) hemodynamic variables; 8) use of diuretics in the PICU; 9) need of dialysis; 10) length of ventilator therapy; and 11) length of PICU stays. There was no significant difference in postoperative serum creatinine between the treatment groups over time (p = 0.65). The occurrence rate of acute kidney injury within 48 hours was 46.9% in the levosimendan group and 39.5% in the milrinone group (p = 0.70). There were no significant differences in other secondary outcome variables between the groups. CONCLUSIONS: Levosimendan compared with milrinone did not reduce the occurrence rate of acute kidney injury in infants after total corrective heart surgery for atrioventricular septal defect, ventricular septal defect, or Tetralogy of Fallot.


Subject(s)
Acute Kidney Injury/prevention & control , Cardiopulmonary Bypass/methods , Cardiotonic Agents/administration & dosage , Heart Defects, Congenital/surgery , Milrinone/administration & dosage , Simendan/administration & dosage , Acute Kidney Injury/epidemiology , Cardiac Surgical Procedures/methods , Creatinine/blood , Double-Blind Method , Female , Finland , Heart Defects, Congenital/drug therapy , Heart Septal Defects/prevention & control , Heart Septal Defects, Ventricular/prevention & control , Humans , Infant , Male , Prospective Studies , Sweden , Tetralogy of Fallot/prevention & control
13.
Eur J Cardiothorac Surg ; 52(4): 789-797, 2017 Oct 01.
Article in English | MEDLINE | ID: mdl-29156017

ABSTRACT

OBJECTIVES: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS). METHODS: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events. RESULTS: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively). CONCLUSIONS: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present.


Subject(s)
Aortic Stenosis, Supravalvular/surgery , Cardiac Surgical Procedures/methods , Cardiology , Forecasting , Postoperative Complications/epidemiology , Societies, Medical , Adolescent , Aortic Stenosis, Supravalvular/mortality , Child , Child, Preschool , Europe/epidemiology , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Prevalence , Retrospective Studies , Survival Rate/trends
14.
Cardiol Young ; 27(9): 1823-1831, 2017 Nov.
Article in English | MEDLINE | ID: mdl-28780922

ABSTRACT

BACKGROUND: Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors. METHODS: This is a retrospective study of all children aged 0-18 years who underwent heart transplantation in Gothenburg from 1989 to 2014. RESULTS: A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0-17). The mean post-transplant follow-up time was 5.5 years (0.5-21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02-25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein-Barr virus infection - that is, a positive donor-negative recipient (p⩽0.0001) - and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028). Discussion This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.


Subject(s)
Heart Transplantation/adverse effects , Lymphoproliferative Disorders/epidemiology , Lymphoproliferative Disorders/etiology , Postoperative Complications/epidemiology , Sternotomy/adverse effects , Adolescent , Child , Child, Preschool , Female , Heart Defects, Congenital/surgery , Heart Ventricles/abnormalities , Humans , Immunosuppressive Agents/adverse effects , Infant , Infant, Newborn , Lymphoma/complications , Lymphoma/epidemiology , Male , Proportional Hazards Models , Retrospective Studies , Risk Factors , Sweden/epidemiology , Tacrolimus/adverse effects
15.
Int J Pediatr Otorhinolaryngol ; 79(2): 271-3, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25510988

ABSTRACT

Enlargement of mediastinal structures can cause compression of the trachea and/or bronchi. We describe two case reports in which stenosis of left main bronchus, secondary to compression from an enlarged mediastinal structure, were successfully managed with temporary placement of a metallic self-expanding stent while waiting for the compression to relieve.


Subject(s)
Airway Obstruction/therapy , Heart/anatomy & histology , Pulmonary Atelectasis/therapy , Stents , Airway Obstruction/etiology , Child , Female , Heart Transplantation , Humans , Organ Size , Pulmonary Atelectasis/etiology
16.
Paediatr Anaesth ; 23(3): 222-7, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23302040

ABSTRACT

OBJECTIVE: Earlier detection of coagulopathy in pediatric cardiac surgery patients. AIM: To determine whether thromboelastometry (TEM) analysis before weaning from cardiopulmonary bypass (CPB) and hemoconcentration is predictive of post-CPB results and whether analysis of clot firmness already after 10 min yields reliable results. BACKGROUND: Cardiac surgery with CPB induces a coagulopathy that may contribute to postoperative complications. Earlier detection increases the possibility of initiating countermeasures. METHODS/MATERIAL: Fifty-six pediatric cardiac surgery patients were included in a prospective observational study. HEPTEM and FIBTEM clotting time (CT), clot formation time (CFT), and clot firmness after 10 min (A10) and at maximum (MCF) were analyzed during CPB and after CPB and ultrafiltration with modified rotational thromboelastometry (ROTEM). The analyses were compared, and correlations and differences were calculated. RESULTS: Hemoconcentration with modified ultrafiltration increased hematocrit from 28 ± 3 to 37 ± 4% (P < 0.001). Correlation coefficients of the TEM variables during and after CPB ranged from 0.61 to 0.82 (all P < 0.001). HEPTEM-CT and HEPTEM-MCF differed significantly but the differences were marginal. Both HEPTEM and FIBTEM A10 measurements during CPB were significantly less than MCF (P < 0.001 for both), but the correlations were highly significant (HEPTEM: r = 0.95, P < 0.001; FIBTEM: r = 0.96, P < 0.001), and the differences were predictable, with narrow confidence intervals (HEPTEM: -8.2 mm (-8.9 to -7.5); FIBTEM: -0.5 mm (-0.7 to -0.3). CONCLUSION: The results suggest that intraoperative TEM analyses can be accelerated by analyzing HEPTEM/FIBTEM on CPB before hemoconcentration and by analyzing clot firmness already after 10 min.


Subject(s)
Blood Coagulation Disorders/diagnosis , Cardiac Surgical Procedures/methods , Thrombelastography/methods , Anesthesia, General , Anticoagulants/antagonists & inhibitors , Anticoagulants/therapeutic use , Blood Coagulation Disorders/blood , Cardiopulmonary Bypass , Child , Child, Preschool , Early Diagnosis , Female , Hematocrit , Hemoglobins/metabolism , Humans , Infant , Infant, Newborn , Intraoperative Complications/diagnosis , Intraoperative Complications/therapy , Male , Monitoring, Intraoperative , Platelet Count , Prospective Studies , Ultrafiltration
17.
Cardiol Young ; 23(2): 225-32, 2013 Apr.
Article in English | MEDLINE | ID: mdl-22691184

ABSTRACT

BACKGROUND: Shunt thrombosis after implantation of systemic-to-pulmonary shunts in paediatric patients is common. Acetyl salicylic acid is used for anti-thrombotic treatment; however, the effect is rarely monitored, although it is known that the response varies. The aim was to determine the effects of acetyl salicylic acid medication on platelet aggregation in children with systemic-to-pulmonary shunts. METHODS: A total of 14 children ­ median age 12 days; ranging from 3 to 100 days ­ were included in a prospective observational longitudinal study. All children were treated with oral acetyl salicylic acid (3­5 milligrams per kilogram once daily) after shunt implantation. Acetyl salicylic acid-dependent platelet aggregation in whole blood was analysed with impedance aggregometry (Multiplate®) after addition of arachidonic acid. Analyses were carried out before the primary operation, before and 5 and 24 hours after the first acetyl salicylic acid dose, and after 3­6 months of treatment. The therapeutic range for acetyl salicylic acid was defined as a test result less than 60 units. RESULTS: Acetyl salicylic acid reduced the arachidonic acid-induced platelet aggregation in all but one patient. Of the patients, 93% were in the therapeutic range 5 hours after acetyl salicylic acid intake, 86% were in the range after 24 hours, and 64% after 3­6 months. CONCLUSIONS: Acetyl salicylic acid reduces platelet aggregation after shunt implantation in paediatric patients, but a considerable percentage of the children are outside the therapeutic range. Monitoring of platelet aggregation has the potential to improve anti-platelet treatment after shunt implantation by identifying children with impaired acetyl salicylic acid response.


Subject(s)
Aspirin/pharmacology , Heart Defects, Congenital/surgery , Platelet Aggregation Inhibitors/pharmacology , Platelet Aggregation/drug effects , Thrombosis/prevention & control , Blalock-Taussig Procedure , Double Outlet Right Ventricle/complications , Double Outlet Right Ventricle/surgery , Electric Impedance , Female , Heart Defects, Congenital/complications , Heart Septal Defects/complications , Heart Septal Defects/surgery , Humans , Hypoplastic Left Heart Syndrome/complications , Hypoplastic Left Heart Syndrome/surgery , Infant , Infant, Newborn , Longitudinal Studies , Male , Norwood Procedures , Platelet Function Tests , Prospective Studies , Prosthesis Failure , Pulmonary Atresia/complications , Pulmonary Atresia/surgery , Thrombosis/complications
18.
Anesth Analg ; 112(1): 30-6, 2011 Jan.
Article in English | MEDLINE | ID: mdl-21048096

ABSTRACT

BACKGROUND: The majority of pediatric cardiac surgery patients receive blood transfusions. We hypothesized that the routine use of intraoperative thromboelastometry to guide transfusion decisions would reduce the overall proportion of patients receiving transfusions in pediatric cardiac surgery. METHODS: One hundred pediatric cardiac surgery patients were included in the study. Fifty patients (study group) were prospectively included and compared with 50 procedure- and age-matched control patients (control group). In the study group, thromboelastometry, performed during cardiopulmonary bypass, guided intraoperative transfusions. Intraoperative and postoperative transfusions of packed red blood cells, fresh frozen plasma, platelets, and fibrinogen concentrates, and postoperative blood loss and hemoglobin levels were compared between the 2 groups. RESULTS: The proportion of patients receiving any intraoperative or postoperative transfusion of packed red blood cells, fresh frozen plasma, platelets, or fibrinogen concentrates was significantly lower in the study group than in the control group (32 of 50 [64%] vs 46 of 50 [92%], respectively; P < 0.001). Significantly fewer patients in the study group received transfusions of packed red blood cells (58% vs 78%, P = 0.032) and plasma (14% vs 78%, P < 0.001), whereas more patients in the study group received transfusions of platelets (38% vs 12%, P = 0.002) and fibrinogen concentrates (16% vs 2%, P = 0.015). Neither postoperative blood loss nor postoperative hemoglobin levels differed significantly between the study group and the control group. CONCLUSIONS: The results suggest that routine use of intraoperative thromboelastometry in pediatric cardiac surgery to guide transfusions is associated with a reduced proportion of patients receiving transfusions and an altered transfusion pattern.


Subject(s)
Blood Transfusion/statistics & numerical data , Cardiac Surgical Procedures/adverse effects , Intraoperative Complications/epidemiology , Intraoperative Complications/prevention & control , Thrombelastography/methods , Adolescent , Blood Transfusion/trends , Cardiac Surgical Procedures/trends , Child , Child, Preschool , Female , Humans , Infant , Male , Prevalence , Prospective Studies
20.
Ann Thorac Surg ; 87(2): 584-7, 2009 Feb.
Article in English | MEDLINE | ID: mdl-19161783

ABSTRACT

BACKGROUND: Risk adjustment of outcomes in pediatric congenital heart surgery is challenging due to the great diversity in diagnoses and procedures. We have previously shown that variable life-adjusted display (VLAD) charts provide an effective graphic display of risk-adjusted outcomes in this specialty. A question arises as to whether the risk model used remains appropriate over time. METHODS: We used a recently developed graphic technique to evaluate the performance of an existing risk model among those patients at a single center during 2000 to 2003 originally used in model development. We then compared the distribution of predicted risk among these patients with that among patients in 2004 to 2006. Finally, we constructed a VLAD chart of risk-adjusted outcomes for the latter period. RESULTS: Among 1083 patients between April 2000 and March 2003, the risk model performed well at predicted risks above 3%, underestimated mortality at 2% to 3% predicted risk, and overestimated mortality below 2% predicted risk. There was little difference in the distribution of predicted risk among these patients and among 903 patients between June 2004 and October 2006. Outcomes for the more recent period were appreciably better than those expected according to the risk model. This finding cannot be explained by any apparent bias in the risk model combined with changes in case-mix. CONCLUSIONS: Risk models can, and hopefully do, become out of date. There is scope for complacency in the risk-adjusted audit if the risk model used is not regularly recalibrated to reflect changing standards and expectations.


Subject(s)
Cardiac Surgical Procedures/methods , Heart Defects, Congenital/mortality , Heart Defects, Congenital/surgery , Hospital Mortality/trends , Risk Adjustment , Cardiac Surgical Procedures/mortality , Child, Preschool , Cohort Studies , Female , Heart Defects, Congenital/diagnosis , Humans , Infant , Infant, Newborn , Male , Medical Audit , Monitoring, Physiologic , New Zealand , Postoperative Complications/mortality , Retrospective Studies , Survival Analysis , Time Factors , Total Quality Management , Treatment Outcome
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