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AIM: This nationwide study evaluated the clinical impact that an early thymectomy, during congenital heart defect (CHD) surgery, had on the health of children and adolescents. METHODS: The subjects were patients aged 1-15 years who had undergone CHD surgery at the University Children's Hospital, Helsinki, where all CHD surgery in Finland is carried out, from 2006 to 2018. The parents or the cases and population-based controls, matched for sex, age and hospital district, completed electronic questionnaires. We excluded those with low birth weights or a known immunodeficiency. Adjusted odds ratios (aOR) and 95% confidence intervals (CI) were calculated for prespecified outcomes. RESULTS: We received responses relating to 260/450 (58%) cases and 1403/4500 (31%) controls and excluded 73 cases with persistent cardiac or respiratory complaints after surgery. The CHD group reported more recurrent hospitalisations due to infections (aOR 6.3, 95% CI 3.0-13) than the controls and more pneumonia episodes (aOR 3.5, 95% CI 2.1-5.6), asthma (aOR 2.5, 95% CI 1.5-4.1) and wheezing (aOR 2.1, 95% CI 1.5-2.9). CONCLUSION: Hospitalisation due to infections, pneumonia, wheezing and asthma was more common in children after a thymectomy due to open-heart surgery than population-based controls, underlining the importance of immunological follow-ups.
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Asma , Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Pneumonia , Sons Respiratórios , Timectomia , Humanos , Masculino , Asma/epidemiologia , Asma/etiologia , Feminino , Criança , Timectomia/efeitos adversos , Pré-Escolar , Adolescente , Lactente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Sons Respiratórios/etiologia , Cardiopatias Congênitas/cirurgia , Pneumonia/epidemiologia , Pneumonia/etiologia , Estudos de Casos e Controles , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Finlândia/epidemiologiaRESUMO
Background: Immune-mediated factors such as acute cellular rejections and donor-specific antibodies (DSAs) are risk factors for cardiac allograft vasculopathy (CAV). We studied a national cohort with a unified setting and thorough protocol endomyocardial biopsy (EMB) data for an association between cellular rejections, especially when mild and recurrent, and DSAs with CAV in pediatric heart transplant (HTx) patients. Methods: This is a retrospective, national cohort study of 94 pediatric HTxs performed between 1991 and 2019 and followed until December 31, 2020. Diagnosis of CAV was based on reevaluation of angiographies. Protocol and indication EMB findings with other patient data were collected from medical records. Associations between nonimmune and immune-mediated factors and CAV were analyzed with univariable and multivariable Cox regression analyses. Results: Angiographies performed on 76 patients revealed CAV in 23 patients (30%). Altogether 1138 EMBs (92% protocol biopsies) were performed on 78 patients (83%). During the first posttransplant year, grade 1 rejection (G1R) appeared in 45 patients (58%), and recurrent (≥2) G1R findings in 14 patients (18%). Pretransplant DSAs occurred in 13 patients (17%) and posttransplant DSAs in 37 patients (39%). In univariable analysis, pretransplant DSAs, appearance and recurrence of G1R findings, and total rejection score during the first posttransplant year, as well as recurrent G1R during follow-up, were all associated with CAV. In multivariable analysis, pretransplant DSAs and recurrent G1R during the first posttransplant year were found to be associated with CAV. Conclusions: Our results indicate that pretransplant DSA and recurrent G1R findings, especially during the first posttransplant year, are associated with CAV after pediatric HTx.
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Background. It has been unclear whether simple atrial septal defect (ASD) is an independent risk factor for infective endocarditis (IE). This study aimed to untangle the risk of endocarditis in a large nationwide cohort. Methods. We acquired data from the Finnish hospital discharge register on all individuals with ASD diagnosis from 1969 to 2019. Patients with complex congenital cardiac abnormalities were ruled out. Five individualized controls from the general population were matched to the ASD patient's birth year, sex, and residence at the index date. All the patients with ICD-8, -9, or -10 diagnosis codes for IE were gathered from the hospital discharge registry. Results. Altogether, 8322 patients with ASD and 39,237 individualized controls were enrolled in the study. Median follow-up was 21.6 years (IQR 11.8-36.9) from the first hospital contact. In total, 24 (16 male) cases of infective endocarditis among ASD patients and 10 (8 male) cases among controls were diagnosed during the follow-up. The incidence of endocarditis was 0.11 per 1000 person-years in the patients with ASD and 0.011 per 1000 person-years in the controls. The adjusted risk ratio for endocarditis was 13.51 (95% CI: 6.20-29.46) in patients with ASD compared to the control cohort. Patients with ASD and endocarditis had higher long-term mortality than individualized control patients (MRR 2.25, 95% CI: 1.23-4.11). Conclusions. The incidence of IE in patients with ASD was higher than in the general population. Mortality associated with IE was higher in patients with ASD compared to controls.
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Endocardite Bacteriana , Endocardite , Cardiopatias Congênitas , Comunicação Interatrial , Humanos , Masculino , Endocardite/diagnóstico , Endocardite/epidemiologia , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/epidemiologia , Comunicação Interatrial/complicações , Cardiopatias Congênitas/epidemiologia , Fatores de RiscoRESUMO
BACKGROUND: Coarctation of the aorta (CoA), a congenital narrowing of the proximal descending thoracic aorta, is a relatively common form of congenital heart disease. Untreated significant CoA has a major impact on morbidity and mortality. In the past 3 decades, transcatheter intervention (TCI) for CoA has evolved as an alternative to surgery. OBJECTIVES: The authors report on all TCIs for CoA performed from 2000 to 2016 in 4 countries covering 25 million inhabitants, with a mean follow-up duration of 6.9 years. METHODS: During the study period, 683 interventions were performed on 542 patients. RESULTS: The procedural success rate was 88%, with 9% considered partly successful. Complications at the intervention site occurred in 3.5% of interventions and at the access site in 3.5%. There was no in-hospital mortality. During follow-up, TCI for CoA reduced the presence of hypertension significantly from 73% to 34%, but despite this, many patients remained hypertensive and in need of continuous antihypertensive treatment. Moreover, 8% to 9% of patients needed aortic and/or aortic valve surgery during follow-up. CONCLUSIONS: TCI for CoA can be performed with a low risk for complications. Lifetime follow-up after TCI for CoA seems warranted.
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Coartação Aórtica , Hipertensão , Humanos , Seguimentos , Resultado do Tratamento , Aorta , Sistema de RegistrosRESUMO
OBJECTIVES: Patients with univentricular heart defects require lifelong imaging surveillance. Recent advances in non-invasive imaging have enabled replacing these patients' routine catheterisation. Our objective was to describe the safety and cost savings of transition of a tertiary care children's hospital from routine invasive to routine non-invasive imaging of low-risk patients with univentricular heart defects. METHODS: This single-centre cohort study consists of 1) a retrospective analysis of the transition from cardiac catheterisation (n = 21) to CT angiography (n = 20) before bidirectional Glenn operation and 2) a prospective study (n = 89) describing cardiac magnetic resonance before and after the total cavopulmonary connection in low-risk patients with univentricular heart defects. RESULTS: Pre-Glenn: The total length of CT angiography was markedly shorter compared to the catheterisation: 30 min (range: 20-60) and 125 min (range: 70-220), respectively (p < 0.001). Catheterisation used more iodine contrast agents than CT angiography, 19 ± 3.9 ml, and 10 ± 2.4 ml, respectively (p < 0.001). Controlled ventilation was used for all catheterised and 3 (15%) CT angiography patients (p < 0.001). No complications occurred during CT angiography, while they emerged in 19% (4/21) catheterisation cases (p < 0.001). CT angiography and catheterisation showed no significant difference in the radiation exposure. Pre-/post-total cavopulmonary connection: All cardiac magnetic resonance studies were successful, and no complications occurred. In 60% of the cardiac magnetic resonance (53/89), no sedation was performed, and peripheral venous pressure was measured in all cases. Cost analysis suggests that moving to non-invasive imaging yielded cost savings of at least 2500-4000 per patient. CONCLUSION: Transition from routine invasive to routine non-invasive pre-and post-operative imaging is safely achievable with cost savings.
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OBJECTIVES: The aim of this study was to assess predictors of BP and hypertension and relations between BP and LV mass in a population-based retrospective study of repaired isolated coarctation of aorta. METHODS: We collected follow-up data until 2018 of 284/304 (93%) patients with coarctation treated by surgery (n = 235) or balloon angioplasty/stent (n = 37/12) in our unit 2000-2012. Systolic hypertension was defined as systolic BP (SBP) z-score ≥+2 standard deviation (SD) or regular use of BP medication. LV hypertrophy was defined as LV mass z-score ≥+2 SD or LV mass index g/m2.7 ≥95th percentile. RESULTS: The median (25-75th percentiles) follow-up time and age at follow-up were 9.7 years (6.9-13.2) and 11.8 years (7.9-16.0), respectively. Age at first procedure (P = 0.011) and systolic arm-leg-gradient (P = 0.007) were positively and transverse arch (P = 0.007) and isthmus diameter (P = 0.001) z-scores at follow-up were negatively associated with SBP z-score adjusted for age at follow-up and need for reintervention for coarctation. Systolic hypertension was present in 53/284 (18.7%) and related with increasing age at first procedure (median 33.2 vs 0.6 months; P < 0.001) and arm-leg-gradient at follow-up (mean ± SD, -0.3 ± 14.6 vs -6.4 ± 11.6 mmHg; P = 0.047) adjusted for reintervention for coarctation and age at follow-up. LV hypertrophy was present in 20/227 (9.3%) and related with SBP z-score. CONCLUSIONS: Higher SBP and hypertension in repaired coarctation of aorta are related with increasing age at first procedure and arm-leg-gradient at follow-up. Transverse arch and isthmus diameters at follow-up are inversely related with SBP.
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Coartação Aórtica , Hipertensão , Coartação Aórtica/complicações , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgia , Pressão Sanguínea , Criança , Seguimentos , Humanos , Hipertensão/complicações , Hipertensão/epidemiologia , Hipertrofia/complicações , Estudos RetrospectivosRESUMO
BACKGROUND: Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence. OBJECTIVE: To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously. METHODS: Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes. RESULTS: Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms. CONCLUSION: Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.
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Permeabilidade do Canal Arterial , Traumatismos do Nervo Laríngeo Recorrente , Paralisia das Pregas Vocais , Humanos , Traumatismos do Nervo Laríngeo Recorrente/etiologia , Traumatismos do Nervo Laríngeo Recorrente/complicações , Paralisia das Pregas Vocais/epidemiologia , Paralisia das Pregas Vocais/etiologia , Permeabilidade do Canal Arterial/epidemiologia , Permeabilidade do Canal Arterial/cirurgia , Permeabilidade do Canal Arterial/complicações , Incidência , Estudos Retrospectivos , Cateterismo/efeitos adversosRESUMO
A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after completion of chemotherapy for osteosarcoma. Five months after completion of chemotherapy, at the age of 10 years, heart transplantation was performed. At 29 months since transplantation, the patient remains free of rejection and recurrence of osteosarcoma. (Level of Difficulty: Intermediate.).
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Transposition of the great arteries (TGA) is one of the most common cyanotic congenital heart diseases requiring neonatal surgical intervention. Parallel circulations that result in impaired cerebral oxygen delivery already in utero may lead to brain damage and long-term neurodevelopmental delay. Balloon atrial septostomy (BAS) is often employed to mix deoxygenated and oxygenated blood at the atrial level. However, BAS causes a sudden increase in arterial blood oxygenation and oxidative stress. We studied changes in oxygen saturation as well as metabolic profiles of plasma samples from nine newborn infants suffering from TGA before and until 48 h after undergoing BAS. The plasma metabolome clearly changed over time and alterations of four metabolic pathways, including the pentose phosphate pathway, were linked to changes in the cerebral tissue oxygen extraction. In contrast, no changes in levels of lipid peroxidation biomarkers over time were observed. These observations suggest that metabolic adaptations buffer the free radical burst triggered by re-oxygenation, thereby avoiding structural damage at the macromolecular level. This study enhances our understanding of the complex response of infants with TGA to changes in oxygenation induced by BAS.
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Myocardial dysfunction is a known risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS). Variants in some transcription factor and contractility genes, which are known to cause cardiomyopathy, have previously been associated with impaired right ventricular function in some HLHS patients. The care of HLHS patients is resource demanding. Identifying genetic variants associated with myocardial dysfunction would be helpful in tailoring the follow-up and therapeutic strategies. We tested whether a commercial cardiomyopathy gene panel could serve as a diagnostic tool in a Finnish cohort of HLHS patients with impaired right ventricular function to identify potentially pathogenic variants associated with poor prognosis. None of the patients had pathogenic or likely pathogenic variants in the studied cardiomyopathy-associated genes. Thus, our approach of performing a cardiomyopathy gene panel to identify pathogenic variants as directly causal or as modifiers for worse outcomes in hypoplastic left heart syndrome is not useful in clinical practice at the moment.
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INTRODUCTION: Newborn infants with transposition of the great arteries (d-TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11-year population-based cohort of d-TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care. MATERIAL AND METHODS: The national cohort consisted of all live-born infants with simple d-TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004-2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated. RESULTS: Preoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but the total mortality rate remained unchanged. All prenatally diagnosed infants (n = 27) survived. Lower gestational age (odds ratio 0.68, P = .012) and higher maternal age at birth (odds ratio 1.16, P = .036) were associated with increased mortality in multivariable analysis. Older infant age at time of operation (P = .002), longer aortic clamp time (P < .001), and higher maternal body mass index (P = .027) were associated with longer initial hospital stay. An extended need for hospital care during the first year of life was multi-factorial. CONCLUSIONS: In our cohort, none of the prenatally diagnosed d-TGA infants died. As a result of the limited prenatal detection rates, however, the sample size was insufficient to reach statistical significance. The d-TGA infants born with lower gestational age and to older mothers had increased mortality.
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Hospitalização/estatística & dados numéricos , Obesidade Materna , Transposição dos Grandes Vasos , Índice de Massa Corporal , Estudos de Coortes , Feminino , Finlândia/epidemiologia , Idade Gestacional , Humanos , Lactente , Mortalidade Infantil , Recém-Nascido , Idade Materna , Triagem Neonatal/métodos , Obesidade Materna/diagnóstico , Obesidade Materna/epidemiologia , Gravidez , Diagnóstico Pré-Natal/métodos , Diagnóstico Pré-Natal/estatística & dados numéricos , Fatores de Risco , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/mortalidade , Transposição dos Grandes Vasos/terapiaRESUMO
BACKGROUND The appearance of human leukocyte antigen (HLA) antibodies after solid organ transplantation predisposes recipients to graft dysfunction. In theory, vascular homografts, which are widely used in children with congenital heart defects, may cause allosensitization. MATERIAL AND METHODS In this single-center retrospective study, the presence of pre-existing HLA antibodies in pediatric heart transplant (HTx) recipients with a vascular homograft was evaluated in a cohort of 12 patients. HLA antibodies were screened before and after HTx and positive screening results were confirmed and identified using the Luminex® single antigen bead method. Endomyocardial biopsies (EMB) and coronary angiography studies were re-evaluated to assess the prevalence of acute rejections and coronary artery change in these patients. RESULTS At the time of HTx, 8 patients (67%) had HLA antibodies detected by the Luminex assay, none of which were heart donor specific (DSA). All patients had negative leukocyte crossmatch. One patient developed DSAs against homograft donor prior to HTx. After the HTx, 5 patients (42%) developed DSAs against the heart donor and 4 patients (40%) against the homograft donor. In 2 patients (17%), the antibodies were against both heart and homograft donors. The rejection rate or prevalence of coronary artery vasculopathy did not differ significantly between the homograft cohort and our historical controls. CONCLUSIONS Our results suggest that the prevalence of DSAs against homograft donor prior to HTx is relatively rare. However, almost half of the patients developed DSAs against homograft post-HTx. The clinical importance of these antibodies warrants further studies.
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Antígenos HLA/imunologia , Transplante de Coração/efeitos adversos , Isoanticorpos/imunologia , Adolescente , Criança , Pré-Escolar , Feminino , Rejeição de Enxerto/imunologia , Sobrevivência de Enxerto , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVES: The aim of the present study was to evaluate procedural risk factors, incidence and timing of reintervention because of recurrent aortic coarctation in children. METHODS: The study cohort consisted of 304 patients with isolated coarctation: 251 underwent surgery and 53 were treated percutaneously (40 balloon angioplasty, 13 stent) at the Helsinki Children's Hospital in 2000-2012. Characteristics, intervention and reintervention data were retrospectively collected from clinical records until 2014 (median follow-up 7.9 years). Age- and sex-matched comparisons between the treatment groups were performed in 86 patients (surgery n = 43, percutaneous n = 43). RESULTS: Forty of the 251 (16%) patients after surgery, 9/40 (23%) patients after balloon angioplasty and 4/13 (31%) patients after stent placement underwent a reintervention after a median time of 3.4, 11.7 and 19.5 months (P < 0.05), respectively. In the surgery group, all reinterventions occurred in children operated on ≤12 months of age and were related to lower body weight and smaller dimensions of the aorta. In the balloon angioplasty group, a higher post-procedure systolic arm-leg blood pressure gradient was associated with reintervention. After stent placement, three-fourths of the reinterventions were performed in a planned postinterventional catheterization. In the age- and sex-matched comparisons (median 5,7 years, range 0,5-17,6), post-procedure blood pressure gradients were higher (mean 10 vs 4 mmHg, P = 0.03), and reinterventions were more common (28%, 95% confidence interval 17-43 vs 2%, 95% confidence interval 0-12) in the percutaneous group compared to the surgery group. CONCLUSIONS: Reinterventions after surgery in neonates were relatively common. In older children, percutaneous treatment carried a higher risk of reinterventions, which were mainly related to residual coarctation after primary treatment.
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Coartação Aórtica/cirurgia , Adolescente , Coartação Aórtica/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Masculino , Recidiva , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , Fatores de TempoRESUMO
BACKGROUND: Childhood cardiomyopathies are progressive and often lethal disorders, forming the most common cause of heart failure in children. Despite severe outcomes, their genetic background is still poorly characterized. OBJECTIVES: The purpose of this study was to characterize the genetics of severe childhood cardiomyopathies in a countrywide cohort. METHODS: The authors collected a countrywide cohort, KidCMP, of 66 severe childhood cardiomyopathies from the sole center in Finland performing cardiac transplantation. For genetic diagnosis, next-generation sequencing and subsequent validation using genetic, cell biology, and computational approaches were used. RESULTS: The KidCMP cohort presents remarkable early-onset and severe disorders: the median age of diagnosis was 0.33 years, and 17 patients underwent cardiac transplantation. The authors identified the pathogenic variants in 39% of patients: 46% de novo, 34% recessive, and 20% dominantly-inherited. The authors report NRAP underlying childhood dilated cardiomyopathy, as well as novel phenotypes for known heart disease genes. Some genetic diagnoses have immediate implications for treatment: CALM1 with life-threatening arrhythmias, and TAZ with good cardiac prognosis. The disease genes converge on metabolic causes (PRKAG2, MRPL44, AARS2, HADHB, DNAJC19, PPA2, TAZ, BAG3), MAPK pathways (HRAS, PTPN11, RAF1, TAB2), development (NEK8 and TBX20), calcium signaling (JPH2, CALM1, CACNA1C), and the sarcomeric contraction cycle (TNNC1, TNNI3, ACTC1, MYH7, NRAP). CONCLUSIONS: Childhood cardiomyopathies are typically caused by rare, family-specific mutations, most commonly de novo, indicating that next-generation sequencing of trios is the approach of choice in their diagnosis. Genetic diagnoses may suggest intervention strategies and predict prognosis, offering valuable tools for prioritization of patients for transplantation versus conservative treatment.
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Cardiomiopatias/epidemiologia , Cardiomiopatias/genética , Testes Genéticos/métodos , Índice de Gravidade de Doença , Adolescente , Idade de Início , Cardiomiopatias/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Finlândia/epidemiologia , Humanos , Lactente , Recém-Nascido , Masculino , Linhagem , Estrutura Secundária de ProteínaRESUMO
OBJECTIVE: To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. DESIGN: Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000-2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR. RESULTS: Sixty-one patients underwent either BAV (n = 54) or surgical valvuloplasty (n = 7) for valvar AS at a median age of 29 days (range 6 hours to 16.9 years). The proportion of patients not requiring reintervention at 1, 5, and 10 years was 61%, 50%, and 29% in neonates and 83%, 73%, and 44% in older patients, respectively (p = .02); without difference between treatment groups. Larger proportion of patients remained free from valve surgery after optimal BAV result than after adequate or inadequate result (p = .01). The reason for the first reintervention was AS in 50%, AR in 36%, and combined aortic valve disease in 16% of cases. Early mortality (before hospital discharge) was 4.9%, and associated with critical AS in neonates. There was no late mortality during the follow-up. CONCLUSIONS: Although majority of congenital AS patients require more than one intervention during childhood, an optimal BAV result improves long-term outcome by increasing the proportion of patients remaining free from valve surgery. High long-term freedom from reintervention is attainable also in the neonatal population.
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Estenose da Valva Aórtica/terapia , Valva Aórtica/cirurgia , Valvuloplastia com Balão , Procedimentos Cirúrgicos Cardíacos , Adolescente , Valva Aórtica/fisiopatologia , Estenose da Valva Aórtica/congênito , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/fisiopatologia , Valvuloplastia com Balão/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Finlândia , Pesquisas sobre Atenção à Saúde , Hemodinâmica , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVES: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: A population-based cohort of 63 Finnish children with HLHS (BT n = 23, RV-PA n = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0.5-3 years after Stage 3. For comparison of systolic myocardial function, we evaluated the RV fractional area change (FAC), strain, strain rate and mechanical synchrony from the apical 4-chamber view by velocity vector imaging. RESULTS: There were no intergroup differences in demographics during the study period. At baseline, no intergroup differences were detected in RV systolic myocardial function. Before Stage 2, RV FAC was higher ( P = 0.03) in the RV-PA conduit group. At Stage 3, an increase in all systolic myocardial functional parameters was observed in the BT shunt group. After Stage 3, the BT shunt group had better RV systolic function. In multiple regression analysis, the shunt type and the stage of palliation had an impact on myocardial function. CONCLUSIONS: Although patients with HLHS initially palliated with a BT shunt demonstrate lower RV FAC after Stage I, RV FAC improves after Stage 2 with better systolic performance after Stage 3 compared with those initially palliated with an RV-PA conduit.
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Procedimento de Blalock-Taussig/estatística & dados numéricos , Coração/fisiopatologia , Síndrome do Coração Esquerdo Hipoplásico/epidemiologia , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/estatística & dados numéricos , Cateterismo Cardíaco , Pré-Escolar , Ecocardiografia , Feminino , Coração/diagnóstico por imagem , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Lactente , Recém-Nascido , Masculino , Procedimentos de Norwood/instrumentação , Cuidados Paliativos , Estudos RetrospectivosRESUMO
BACKGROUND: Quantitative echocardiographic assessment of right ventricular function is important in children with hypoplastic left heart syndrome (HLHS). The aim of this study was to examine the repeatability of different echocardiographic techniques, both manual and automated, to measure fractional area change (FAC) in patients with HLHS and to correlate these measurements with magnetic resonance imaging (MRI)-derived ejection fraction (EF). METHODS: Fifty-one children with HLHS underwent transthoracic echocardiography and cardiac MRI under the same general anesthetic as part of routine inter-stage assessment. FAC was measured from the apical four-chamber view using three different techniques: velocity vector imaging (VVI) (Syngo USWP 3.0; Siemens Healthineers), QLAB (Q-lab R 10.0; Philips Healthcare), and manual endocardial contour tracing (Xcelera, Philips Healthcare). Intra- and inter-observer variability was calculated using intra-class correlation coefficient (ICC). FAC was correlated with MRI EF calculated using a single standard method. RESULTS: Fractional area change had a good correlation with MRI-derived EF with an R value for VVI, QLAB, and manual methods of .7, .6, and .4, respectively. Intra- and inter-observer variability for FAC was good for automated echocardiographic methods (ICC>.85) but worse for manual method particularly inter-observer variability of FAC and end-systolic area. Both automated techniques tended to produce higher FAC values compared with manual measurements (P<.001). CONCLUSION: Automation improves the repeatability of FAC in HLHS. There are some differences between automated software in terms of correlation with MRI-derived EF. Measurement bias and wide limits of agreement mean that the same echocardiographic technique should be used during the follow-up of individual patients.
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Ecocardiografia/métodos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Processamento de Imagem Assistida por Computador/métodos , Disfunção Ventricular Direita/complicações , Disfunção Ventricular Direita/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Feminino , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Lactente , Imageamento por Ressonância Magnética , Masculino , Variações Dependentes do Observador , Reprodutibilidade dos Testes , Software , Disfunção Ventricular Direita/fisiopatologiaRESUMO
OBJECTIVES: We studied the long-term survival and rejection episodes of paediatric heart transplant recipients. METHODS: We included all paediatric patients (≤18 years) who underwent heart transplantation during 1991-2014 in Finland. Data were obtained retrospectively from a paediatric cardiac surgery database. Patient status was received from the Finnish population registry. All patients underwent yearly routine postoperative endomyocardial biopsies and coronary angiographies. RESULTS: Between 1991 and 2014, 68 heart transplantations were performed. The early mortality (<30 days after surgery) rate was 10% and follow-up coverage was 100%. The 10- and 15-year survival rates for all patients were 68% (95% confidence internal, CI, 56-80%) and 65% (95% CI 53-78%), respectively, including early mortality. The 1-year survival rate was 100% when excluding early operative mortality. Indications for heart transplantation were cardiomyopathy in 57% and cardiac malformations in 43% of patients, with similar long-term survival between the groups. During 23 years of follow-up, 43 patients (70%) had at least one rejection episode and 17 patients (29%) at least a grade 1 coronary artery vasculopathy finding. Patients with early rejection episodes (<3 months) had a higher incidence of late rejection episodes (P = 0.025). Older age at operation was a significant risk factor for the development of coronary artery vasculopathy (hazard ratio 1.1, 95% CI 1.0-1.3, P = 0.012). CONCLUSIONS: First-year survival was excellent. Asymptomatic rejection episodes were common among patients. Early rejection episodes are a risk factor for late rejection episodes and show a trend towards an increased risk of late death. Coronary artery vasculopathy remains a major challenge for late graft survival.
Assuntos
Rejeição de Enxerto/epidemiologia , Cardiopatias/cirurgia , Transplante de Coração , Adulto , Fatores Etários , Criança , Angiografia Coronária , Feminino , Finlândia , Sobrevivência de Enxerto , Cardiopatias/mortalidade , Cardiopatias/patologia , Humanos , Incidência , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
AIMS: Velocity vector imaging (VVI) is an echocardiographic technique based on speckle tracking, which has been validated for the left ventricle (LV). It has not been validated to assess the systemic right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS). The aim of this study was to evaluate whether VVI measurements reliably reflect RV systolic function in patients with HLHS when compared with RV ejection fraction (EF) calculated using magnetic resonance imaging (MRI). METHODS AND RESULTS: In this prospective study, 49 children with HLHS underwent transthoracic echocardiography and cardiac MRI under the same general anaesthetic as a part of routine assessment between the different stages of palliative surgery. Global RV fractional area change (FAC-VVI), strain (S), strain rate (SR), and peak systolic velocity (V) were analysed from the apical four-chamber view using the VVI technique. MRI-derived EF was calculated from a short-axis cine stack of images. Intra- and interobserver reproducibility was excellent for all VVI parameters (intraclass correlation coefficient >0.9). All VVI-derived parameters, except myocardial velocity, correlated with MRI-derived EF (FAC-VVI: R = 0.7, P < 0.001; S: R = -0.5, P < 0.001; SR: R = 0.5, P = 0.001, and V: R = 0.1, P = 0.4). CONCLUSIONS: All VVI-derived parameters, except V, correlate with MRI-derived EF, with FAC being the best predictor of it. Reproducibility of all VVI parameters is excellent. VVI provides a useful tool for the follow-up of RV function during the staged treatment protocol for HLHS.