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1.
Cardiol Young ; 33(7): 1043-1059, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37605816

RESUMO

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.


Assuntos
Cardiologia , Sistema Cardiovascular , Cirurgiões , Humanos , Criança , Qualidade de Vida , Assistência Centrada no Paciente
2.
Cardiol Young ; 32(12): 1966-1983, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35227341

RESUMO

BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). CONCLUSION: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.


Assuntos
Cardiologia , Humanos , Adulto , Criança , Cardiologia/educação , Certificação , Currículo , Bolsas de Estudo , Europa (Continente)
3.
Pediatr Cardiol ; 42(5): 1162-1169, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33837840

RESUMO

Arrhythmia is related to heart rate variability (HRV), which reflects the autonomic nervous regulation of the heart. We hypothesized that autonomic nervous ganglia, located at the junction of the superior vena cava's entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV. We aimed to investigate changes in heart rate and HRV in a cohort of children with univentricular heart defects, undergoing stepwise surgery towards total cavopulmonary connection (TCPC), and compare these results with healthy controls. Twenty four hours Holter-ECG recordings were obtained before BDG (n = 47), after BDG (n = 47), and after total cavopulmonary connection (TCPC) (n = 45) in patients and in 38 healthy controls. HRV was analyzed by spectral and Poincaré methods. Age-related z scores were calculated and compared using linear mixed effects modeling. Total HRV was significantly lower in patients before BDG when compared to healthy controls. The mean heart rate was significantly reduced in patients after BDG compared to before BDG. Compared to healthy controls, patients operated with BDG had significantly reduced heart rate and reduced total HRV. Patients with TCPC showed reduced heart rate and HRV compared with healthy controls. In patients after TCPC, total HRV was decreased compared to before TCPC. Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events.


Assuntos
Arritmias Cardíacas/etiologia , Técnica de Fontan/efeitos adversos , Frequência Cardíaca , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Técnica de Fontan/métodos , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Masculino , Período Pós-Operatório
4.
Cardiol Young ; 31(3): 344-351, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33407975

RESUMO

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making.


Assuntos
Antiarrítmicos/uso terapêutico , Anticoagulantes/uso terapêutico , Antivirais/uso terapêutico , Arritmias Cardíacas/tratamento farmacológico , Tratamento Farmacológico da COVID-19 , Cardiopatias Congênitas/terapia , Fatores Imunológicos/uso terapêutico , Monofosfato de Adenosina/análogos & derivados , Monofosfato de Adenosina/uso terapêutico , Alanina/análogos & derivados , Alanina/uso terapêutico , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Síndrome de Brugada/tratamento farmacológico , Síndrome de Brugada/epidemiologia , Síndrome de Brugada/fisiopatologia , COVID-19/epidemiologia , COVID-19/fisiopatologia , Procedimentos Cirúrgicos Cardíacos , Cardiologia , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/fisiopatologia , Transplante de Coração , Humanos , Transmissão Vertical de Doenças Infecciosas , Síndrome do QT Longo/tratamento farmacológico , Síndrome do QT Longo/epidemiologia , Síndrome do QT Longo/fisiopatologia , Miocardite/epidemiologia , Miocardite/fisiopatologia , Miocárdio , Pediatria , Medição de Risco , SARS-CoV-2 , Sociedades Médicas , Síndrome de Resposta Inflamatória Sistêmica/tratamento farmacológico , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologia , Taquicardia Ventricular/tratamento farmacológico , Taquicardia Ventricular/epidemiologia , Taquicardia Ventricular/fisiopatologia
5.
Cardiol Young ; 30(9): 1321-1327, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32727627

RESUMO

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.


Assuntos
Estenose da Valva Aórtica , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Reoperação , Suécia/epidemiologia , Resultado do Tratamento
6.
Cardiol Young ; 30(3): 353-360, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31920189

RESUMO

BACKGROUND: Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia. METHODS: Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993-2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016. RESULTS: The 1993-2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome. CONCLUSION: The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/epidemiologia , Procedimentos de Norwood/métodos , Sistema de Registros , Feminino , Seguimentos , Idade Gestacional , Transplante de Coração , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Gravidez , Diagnóstico Pré-Natal , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Suécia/epidemiologia
7.
Cardiol Young ; 30(4): 560-567, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32228736

RESUMO

Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be "the go-to online resource" for e-learning in CHD and paediatric-acquired heart disease. It is a carefully curated open access library of paedagogical material for all providers of care to children and adults with CHD or children with acquired heart disease, whether a trainee or a practising provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.


Assuntos
Cardiologia/educação , Educação a Distância , Educação Médica/organização & administração , Cardiopatias Congênitas/terapia , Pediatria/educação , Adulto , Criança , Currículo , Humanos
8.
Acta Paediatr ; 108(2): 266-270, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29978501

RESUMO

AIM: This study determined the use of standardised procedures for infant noninvasive blood pressure (NIBP) measurements in the Nordic countries and aimed to identify factors included in the standardisation and interpretation of NIBP measurements in infants. METHODS: A cross-sectional electronic questionnaire survey was sent to 84 physicians in all 23 university hospitals in Sweden, Norway, Denmark, Finland and Iceland and was completed from February to March 2017. The survey contained respondent characteristics, the presence and description of standardised procedures for NIBP measurements, daily practice of NIBP measurements and methodological considerations and interpretation of NIBP measurements in a healthy six-month-old child. RESULTS: We received responses from 55 of 84 physicians working in all 23 Nordic university hospitals, in paediatric cardiology (n = 22), general paediatrics (n = 16), paediatric nephrology (n = 14) and other fields (n = 3). Less than a quarter (23%) said their hospital issued specific NIBP procedures relating to infants and they referred to 19 different sources of information. The factors that were most commonly assessed for interpretation were age (100%), arousal state (78%) and cuff size (76%). CONCLUSION: Most of the university hospital units treating children lacked age-specific written procedures for measuring and interpreting infant NIBP, and there is a strong need for common Nordic guidelines.


Assuntos
Determinação da Pressão Arterial/normas , Pediatria/normas , Centros Médicos Acadêmicos/estatística & dados numéricos , Protocolos Clínicos , Estudos Transversais , Humanos , Lactente , Países Escandinavos e Nórdicos
9.
Pediatr Cardiol ; 40(4): 685-693, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30918992

RESUMO

Sinus node dysfunction (SND) causes significant morbidity in patients after Fontan surgery. Heart rate variability (HRV) reflects the autonomic regulation of the heart, and changes in HRV have been associated with SND in adults. We aimed to study whether changes in HRV could be detected in 24-h electrocardiographic (ECG) recordings in Fontan patients with SND. We compared HRV results from two patient groups; patients with Fontan circulation who later required a pacemaker due to severe SND (n = 12) and patients with Fontan circulation and SND, without indication for pacemaker treatment (n = 11), with two control groups; patients with Fontan circulation without SND (n = 90) and healthy controls (n = 66). The Poincaré plot index SD2 (representing changes in heart rate over 24-h) and the very low-frequency (VLF) HRV component were significantly higher in both SND groups, both compared with healthy controls and patients with Fontan circulation without SND. In SND patients with pacemakers, SD2 and VLF were slightly reduced compared to SND patients without pacemaker (p = 0.06). In conclusion, in Fontan patients with SND the HRV is significantly higher compared to healthy controls and Fontan patients without SND. However, in patients with severe SND requiring pacemaker, SD2 and VLF tended to be lower than in patients with SND without pacemaker, which could indicate a reduced diurnal HRV in addition to the severe bradycardia. This is a small study, but our results indicate that HRV analysis might be a useful method in the follow-up of Fontan patients regarding development of SND.


Assuntos
Arritmias Cardíacas/etiologia , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Frequência Cardíaca/fisiologia , Síndrome do Nó Sinusal/fisiopatologia , Adulto , Arritmias Cardíacas/terapia , Estudos de Casos e Controles , Eletrocardiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Marca-Passo Artificial , Complicações Pós-Operatórias
10.
J Adv Nurs ; 75(2): 380-387, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30209810

RESUMO

AIMS: To study parent's levels of uncertainty related to the transfer from pediatric to adult care in adolescents with congenital heart disease (CHD) and to identify potentially correlating factors. BACKGROUND: Parents acknowledge that during transition they struggle with finding ways of feeling secure in handing over the responsibility and letting go of control. Well-prepared and informed parents who feel secure are most likely better skilled to support their adolescent and to hand over the responsibility. DESIGN: A cross-sectional study. METHODS: Overall, 351 parents were included (35% response rate). Parental uncertainty was assessed using a Linear Analogue Scale (0-100). Data were collected between January - August 2016. Potential correlates were assessed using the readiness for transition questionnaire and sociodemographic data. RESULTS: The mean parental uncertainty score was 42.5. Twenty-four percent of the parents had a very low level of uncertainty (score 0-10) and 7% had a very high level (score 91-100). Overall, 26% of the mothers and 36% of the fathers indicated that they had not started thinking of the transfer yet. The level of uncertainty was negatively associated with the level of perceived overall readiness. Adolescents' age, sex, CHD complexity, and parental age were not related to uncertainty. CONCLUSION: A wide range in the levels of uncertainty was found. Parents who were less involved in the care, or perceived their adolescent as readier for the transition, felt less uncertain. Still, thirty percent of the parents had not started to think about the transfer to adult care.


Assuntos
Cardiopatias Congênitas/psicologia , Cardiopatias Congênitas/terapia , Pais/psicologia , Transição para Assistência do Adulto , Incerteza , Adolescente , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Pediatria , Inquéritos e Questionários , Adulto Jovem
11.
J Fam Nurs ; 25(3): 447-468, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31342815

RESUMO

Transfer to adult care for adolescents with chronic conditions ought to be determined by transition readiness. The aims of this study were (a) to describe the level of readiness for transition in adolescents with congenital heart disease, (b) to compare adolescents' assessment of transition readiness with their parents' assessments, and (c) to study potential correlates of transition readiness. A total of 157 triads of adolescents aged 14 to 18 years and their parents completed the Readiness for Transition Questionnaire. Adolescents scored higher on overall readiness than their parents. Multivariable analyses revealed that higher levels of adolescents' overall readiness were associated with a less threatening view of the illness, a higher level of empowerment, and with higher mothers' and fathers' overall readiness scores. Adolescents' responsibility scores were positively associated with age and parental adolescent responsibility scores. Parental involvement scores were negatively associated with adolescents' age and positively with the mothers' parental involvement scores. By using a triadic evaluation, the results of the present study significantly extend what is currently known about this population.


Assuntos
Comportamento do Adolescente/psicologia , Doença Crônica/psicologia , Doença Crônica/terapia , Cardiopatias Congênitas/psicologia , Cardiopatias Congênitas/terapia , Pais/psicologia , Transição para Assistência do Adulto , Adolescente , Adulto , Feminino , Humanos , Masculino , Inquéritos e Questionários
12.
Eur J Pediatr ; 176(7): 881-889, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28508990

RESUMO

The aim of the study was to explore what adolescents with congenital heart disease (CHD) view as important in the preparation for the transfer to adult care. We performed interviews in four focus groups with adolescents (14-18 years old) at four university hospitals in Sweden. Data was analysed using qualitative content analysis. The analysis revealed one main category; Becoming a manager of the condition and four subcategories; Sufficient knowledge about the health, Be a participant in the care, Parental support, and Communicate with others about the health. The adolescents' ages differentiated the discussion in the groups. The older adolescents seemed to have more interest in transition planning, information and transfer. The younger described more frustrations about communication and handling the disease. CONCLUSION: To become a manager of the CHD in daily life, the adolescents want disease specific knowledge, which should be communicated in a developmentally appropriate way. Adolescents want to participate and be involved in the transition process. They need support and guidance in how to communicate their CHD. Parental support is fundamental but it change over time. Moreover, peer-support is becoming more significant during the transition process. What is Known: • Transition during adolescence and transfer to adult care for adolescents with CHD is complex, and there is a shift in roles. • Adolescents often have poor knowledge and understanding about their heart condition and the consequences. What is New: • Adolescents call for disease specific information regarding health issues of importance for them in daily life. • Communicating the disease with other is a challenge- peer support from other adolescents with CHD could be a facilitator.


Assuntos
Atitude Frente a Saúde , Cardiopatias Congênitas/psicologia , Cardiopatias Congênitas/terapia , Transição para Assistência do Adulto , Adolescente , Adulto , Feminino , Grupos Focais , Humanos , Masculino , Relações Pais-Filho , Relações Profissional-Paciente , Pesquisa Qualitativa , Suécia
13.
Circulation ; 130(23): 2021-30, 2014 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-25446057

RESUMO

BACKGROUND: The Fontan procedure has improved survival in children with functionally univentricular hearts. With time, however, complications such as reduced exercise capacity are seen more frequently. Exercise intolerance is multifactorial, but pulmonary vascular resistance probably plays a crucial role. Elevated pulmonary vascular resistance has been associated with raised levels of endothelin-1, which are common both before and after Fontan operations. Treatment with endothelin-1 receptor antagonists could theoretically improve cardiopulmonary hemodynamics and exercise capacity. The aim of this study was therefore to examine the efficacy and safety of bosentan in Fontan patients. METHODS AND RESULTS: Seventy-five adolescents and adults were randomized 1:1 to 14 weeks of treatment with bosentan or placebo. Cardiopulmonary exercise test, functional class, blood samples, and quality-of-life questionnaires were evaluated at baseline and at the end of treatment. Sixty-nine patients (92%) completed the study. Peak oxygen consumption increased 2.0 mL·kg(-1)·min(-1) (from 28.7 to 30.7 mL·kg(-1)·min(-1)) in the bosentan group compared with 0.6 mL·kg(-1)·min(-1) (from 28.4 to 29.0 mL·kg(-1)·min(-1)) in the placebo group (P=0.02). Cardiopulmonary exercise test time increased by 0.48 minute (from 6.79 to 7.27 minutes) versus 0.08 minute (from 6.94 to 7.02 minutes; P=0.04). Nine bosentan-treated patients improved 1 functional class, whereas none improved in the placebo group (P=0.0085). Side effects were mild and occurred equally in both groups. No serious adverse effects were seen, and no patients had liver enzyme levels above the 3-fold upper limit. CONCLUSIONS: Bosentan improves exercise capacity, exercise time, and functional class in Fontan patients without serious adverse events or hepatotoxicity. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01292551.


Assuntos
Antagonistas dos Receptores de Endotelina/administração & dosagem , Tolerância ao Exercício/efeitos dos fármacos , Técnica de Fontan/efeitos adversos , Consumo de Oxigênio/efeitos dos fármacos , Complicações Pós-Operatórias/tratamento farmacológico , Sulfonamidas/administração & dosagem , Adolescente , Adulto , Bosentana , Criança , Pré-Escolar , Método Duplo-Cego , Antagonistas dos Receptores de Endotelina/efeitos adversos , Feminino , Hemodinâmica , Humanos , Lactente , Masculino , Placebos , Receptor de Endotelina A/sangue , Estatísticas não Paramétricas , Sulfonamidas/efeitos adversos , Resultado do Tratamento , Adulto Jovem
15.
Front Cardiovasc Med ; 11: 1349166, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38606378

RESUMO

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.

16.
Eur J Clin Pharmacol ; 69(6): 1275-83, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23307232

RESUMO

PURPOSE: Numerous studies have investigated causes of warfarin dose variability in adults, whereas studies in children are limited both in numbers and size. Mechanism-based population modelling provides an opportunity to condense and propagate prior knowledge from one population to another. The main objectives with this study were to evaluate the predictive performance of a theoretically bridged adult warfarin model in children, and to compare accuracy in dose prediction relative to published warfarin algorithms for children. METHOD: An adult population pharmacokinetic/pharmacodynamic (PK/PD) model for warfarin, with CYP2C9 and VKORC1 genotype, age and target international normalized ratio (INR) as dose predictors, was bridged to children using allometric scaling methods. Its predictive properties were evaluated in an external data set of children 0-18 years old, including comparison of dose prediction accuracy with three pharmacogenetics-based algorithms for children. RESULTS: Overall, the bridged model predicted INR response well in 64 warfarin-treated Swedish children (median age 4.3 years), but with a tendency to overpredict INR in children ≤2 years old. The bridged model predicted 20 of 49 children (41 %) within ± 20 % of actual maintenance dose (median age 7.2 years). In comparison, the published dosing algorithms predicted 33-41 % of the children within ±20 % of actual dose. Dose optimization with the bridged model based on up to three individual INR observations increased the proportion within ±20 % of actual dose to 70 %. CONCLUSION: A mechanism-based population model developed on adult data provides a promising first step towards more individualized warfarin therapy in children.


Assuntos
Algoritmos , Anticoagulantes/administração & dosagem , Anticoagulantes/farmacocinética , Coagulação Sanguínea/efeitos dos fármacos , Cálculos da Dosagem de Medicamento , Modelos Biológicos , Varfarina/administração & dosagem , Varfarina/farmacocinética , Adolescente , Adulto , Fatores Etários , Hidrocarboneto de Aril Hidroxilases/genética , Hidrocarboneto de Aril Hidroxilases/metabolismo , Criança , Pré-Escolar , Citocromo P-450 CYP2C9 , Monitoramento de Medicamentos/métodos , Feminino , Genótipo , Humanos , Lactente , Recém-Nascido , Coeficiente Internacional Normatizado , Masculino , Oxigenases de Função Mista/genética , Oxigenases de Função Mista/metabolismo , Farmacogenética , Fenótipo , Suécia , Vitamina K Epóxido Redutases
18.
Arch Dis Child ; 108(5): 398-404, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36657799

RESUMO

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.


Assuntos
Estenose da Valva Aórtica , Recém-Nascido , Humanos , Adolescente , Estudos Retrospectivos , Resultado do Tratamento , Constrição Patológica , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia
19.
Front Cardiovasc Med ; 10: 1285391, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38107261

RESUMO

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.

20.
Eur J Prev Cardiol ; 30(2): 169-176, 2023 01 24.
Artigo em Inglês | MEDLINE | ID: mdl-36198066

RESUMO

AIMS: Our objective was to assess changes in the birth prevalence of CHD over a half-century in a high-resource, nationwide setting, as well as changes in the prevalence of cardiac interventions in this population. METHODS AND RESULTS: The Swedish National Patient and Cause of Death registers were linked to estimate the annual rates of CHD and cardiac interventions among live-born infants from 1970 to 2017. Additionally, separate estimates were obtained by lesion complexity, from mild to the most complex forms of CHD. Overall, the numbers of live-born infants with a CHD identified varied from 624 to 2459 annual cases, with rates increasing steadily from 5.7 to an average of 20 per 1000 live births at the end of the study period, and with a more pronounced increase from 1996 to 2005. The largest increase over time was observed for mild CHD lesions. Overall, the proportion of cardiac interventions among patients with CHD declined from 40.7% in 1970 to below 15.0% after 2014. However, in the most complex CHD lesion groups, overall cardiac interventions increased from 57.1 to 76.8% in patients with conotruncal defects and from 32.8 to 39.5% in those with severe non-conotruncal defects. CONCLUSION: The live-birth prevalence of CHD in Sweden more than tripled during the past half-century, most likely resulting from more accurate diagnostic capabilities. The largest increase over time was observed among patients with simple defects. During the same period, overall cardiac interventions decreased whereas interventions for the most complex CHD groups increased.


Assuntos
Cardiopatias Congênitas , Lactente , Gravidez , Feminino , Humanos , Suécia/epidemiologia , Prevalência , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/terapia , Sistema de Registros , Nascido Vivo/epidemiologia
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