RESUMO
Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.
Assuntos
Cardiopatias Congênitas , Estudos Multicêntricos como Assunto , Valor Preditivo dos Testes , Humanos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Criança , Big Data , Imageamento por Ressonância Magnética , Projetos de Pesquisa , Fatores Etários , Adolescente , Pré-EscolarRESUMO
OBJECTIVES: Cardiac involvement in neonatal lupus erythematosis (NLE) can present as myocarditis/endocardial fibroelastosis (EFE). It is unknown whether high-sensitivity cardiac troponin T (hs-cTnT) is useful in identifying subclinical myocardial inflammation in infants exposed prenatally to anti-Ro antibodies. This study reports hs-cTnT levels in infants exposed to anti-Ro antibodies with/without cardiac NLE and reports cardiac MRI (CMR) findings in a subset of these children. METHODS: The study included 45 consecutive infants exposed prenatally to anti-Ro antibodies with (n = 7) or without (n = 38) cardiac NLE, who were seen at the SickKids NLE Clinic between 2012 and 2014. Hs-cTnT levels were measured at least once, and those infants with values of ≥30 ng/l were offered the opportunity to undergo CMR. Descriptive statistics were performed. RESULTS: Of 38 infants without cardiac NLE, 25 had a hs-cTnT level of ≥30 ng/l (including 1 of >113 ng/l); of these, 8 underwent CMR (all without myocarditis/EFE). All 7 infants with cardiac NLE had at least one hs-cTnT level of ≥30 ng/l, but only 2/7 had a level of >113 ng/l; 4/7 infants with cardiac NLE had CMR (all without myocarditis/EFE); 6/7 infants with cardiac NLE had their steroid treatment adjusted based on the trend in their hs-cTnT levels. CONCLUSION: Only 3/45 anti-Ro antibodies-exposed infants had hs-cTnT values outside the reference range reported in healthy infants. None of 12 infants who had CMR had subclinical myocarditis/EFE. Routine measurement of hs-cTnT in every anti-Ro antibody-exposed infant is not indicated. Further studies are needed to define the role of hs-cTnT as a biomarker for cardiac NLE.
Assuntos
Miocardite , Troponina T , Recém-Nascido , Criança , Humanos , Lactente , Coração , BiomarcadoresRESUMO
BACKGROUND: Neonatal Marfan syndrome is a rare disease with mortality in the first year of life reported as high as 95% predominantly due to progressive heart failure from valvar regurgitation and cardiomyopathy. Multisystem involvement and uncertain prognosis have historically precluded transplant candidacy, and current management options are of limited success. CASE REPORT: We present a baby girl with a postnatal diagnosis of neonatal Marfan syndrome who at 1 year of age underwent mitral valve and tricuspid valve repair with postoperative profound left ventricular and moderate right ventricular dysfunction necessitating biventricular assist device (BiVAD) support and subsequent heart transplant. A number of noncardiac issues persisted in our patient; however, she enjoyed a good quality of life for the initial 3 years posttransplant. Unfortunately, she subsequently developed rapidly progressive coronary allograft vasculopathy (CAV) with progressive deterioration in function and cardiac arrest. CONCLUSION: To our best knowledge, this is only the second case of neonatal Marfan syndrome to undergo heart transplant reported in the literature and the first with BiVAD support as a bridge to candidacy. This is also the first case of neonatal Marfan syndrome associated with intragenic duplication. This case though demonstrating that earlier listing, ventricular assist device (VAD) support and even primary transplant as treatment in neonatal Marfan syndrome should all be considered viable options but also portends a cautionary tale given the spectrum of comorbidities in this rare and severe disorder.
Assuntos
Cardiomiopatias , Transplante de Coração , Síndrome de Marfan , Lactente , Recém-Nascido , Feminino , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/diagnóstico , Qualidade de Vida , Cardiomiopatias/complicações , Valva TricúspideRESUMO
COVID vaccine-associated myocarditis was first identified in March 2021. There have been numerous case reports that detail the clinical course of paediatric patients older than age 12 with COVID vaccine-associated myocarditis. There are still very few reports of children between the ages of 5 and 11 with COVID vaccine-associated myocarditis. We present an 8 year- old with COVID vaccine-associated myocarditis after his second vaccination against SARS-CoV-2.
Assuntos
Vacinas contra COVID-19 , COVID-19 , Miocardite , Vacinas , Criança , Pré-Escolar , Humanos , Vacinas contra COVID-19/efeitos adversos , Miocardite/induzido quimicamente , Miocardite/diagnóstico , SARS-CoV-2RESUMO
Right ventricular (RV) pressure loading leads to RV and left ventricular (LV) dysfunction through RV hypertrophy, dilatation and fibrosis. Relief of RV pressure load improves RV function. However, the impact and mechanisms on biventricular reverse-remodelling and function are only partially characterized. We evaluated the impact of RV pressure overload relief on biventricular remodelling and function in a rabbit model of reversible pulmonary artery banding (PAB). Rabbits were randomized to three groups: (1) Sham-operated controls (n = 7); (2) PAB (NDef, n = 7); (3) PAB followed by band deflation (Def, n = 5). Sham and NDef animals were sacrificed at 6 weeks after PAB surgery. Def animals underwent PAB deflation at 6 weeks and sacrifice at 9 weeks. Biventricular geometry, function, haemodynamics, hypertrophy and fibrosis were compared between groups using echocardiography, magnetic resonance imaging, high-fidelity pressure-tipped catheters and histology. RV pressure loading caused RV dilatation, systolic dysfunction, myocyte hypertrophy and LV compression which improved after PAB deflation. RV end-diastolic pressure (RVEDP) decreased after PAB deflation, although remaining elevated vs. Sham. LV end-diastolic pressure (LVEDP) was unchanged following PAB deflation. RV and LV collagen volumes in the NDef and Def group were increased vs. Sham, whereas RV and LV collagen volumes were similar between NDef and Def groups. RV myocyte hypertrophy (r = 0.75, P < 0.001) but not collagen volume was related to RVEDP. LV myocyte hypertrophy (r = 0.58, P = 0.016) and collagen volume (r = 0.56, P = 0.031) correlated with LVEDP. In conclusion, relief of RV pressure overload improves RV and LV geometry, hypertrophy and function independent of fibrosis. The long-term implications of persistent fibrosis and increased biventricular filling pressures, even after pressure load relief, need further study. KEY POINTS: Right ventricular (RV) pressure loading in a pulmonary artery banding rabbit model is associated with RV dilatation, left ventricular (LV) compression; biventricular myocyte hypertrophy, fibrosis and dysfunction. The mechanisms and impact of RV pressure load relief on biventricular remodelling and function has not been extensively studied. Relief of RV pressure overload improves biventricular geometry in conjunction with improved RV myocyte hypertrophy and function independent of reduced fibrosis. These findings raise questions as to the importance of fibrosis as a therapeutic target.
Assuntos
Disfunção Ventricular Esquerda , Disfunção Ventricular Direita , Animais , Modelos Animais de Doenças , Fibrose , Ventrículos do Coração , Hipertrofia , Artéria Pulmonar , Coelhos , Disfunção Ventricular Esquerda/complicações , Função Ventricular Direita , Pressão VentricularRESUMO
Myocarditis remains a clinical challenge in pediatrics. Originally, it was recognized at autopsy before the application of endomyocardial biopsy, which led to a histopathology-based diagnosis such as in the Dallas criteria. Given the invasive and low-sensitivity nature of endomyocardial biopsy, its diagnostic focus shifted to a reliance on clinical suspicion. With the advances of cardiac magnetic resonance, an examination of the whole heart in vivo has gained acceptance in the pursuit of a diagnosis of myocarditis. The presentation may vary from minimal symptoms to heart failure, life-threatening arrhythmias, or cardiogenic shock. Outcomes span full resolution to chronic heart failure and the need for heart transplantation with inadequate clues to predict the disease trajectory. The American Heart Association commissioned this writing group to explore the current knowledge and management within the field of pediatric myocarditis. This statement highlights advances in our understanding of the immunopathogenesis, new and shifting dominant pathogeneses, modern laboratory testing, and use of mechanical circulatory support, with a special emphasis on innovations in cardiac magnetic resonance imaging. Despite these strides forward, we struggle without a universally accepted definition of myocarditis, which impedes progress in disease-targeted therapy.
Assuntos
Miocardite/diagnóstico , Miocardite/terapia , Animais , Biópsia , Criança , Tomada de Decisão Clínica , Terapia Combinada , Gerenciamento Clínico , Modelos Animais de Doenças , Suscetibilidade a Doenças/imunologia , Humanos , Imagem Multimodal , Miocardite/etiologia , Miocardite/mortalidade , Prognóstico , Avaliação de Sintomas , Resultado do TratamentoRESUMO
Most cardiac imaging conferences have adopted social media as a means of disseminating conference highlights to a global audience well beyond the confines of the conference location. A deliberate and thoughtful social media campaign has the potential to increase the reach of the conference and allow for augmented engagement. The coronavirus disease 2019 (COVID-19) pandemic triggered a radical transformation in not just the delivery of healthcare but also the dissemination of science within the medical community. In the past, in-person medical conferences were an integral annual tradition for most medical professionals to stay up to date with the latest in the field. Social distancing requirements of the COVID-19 pandemic resulted in either cancelling medical conferences or shifting to a virtual format. Following suit, for the first time in its history, the 2021 Society for Cardiovascular Magnetic Resonance (SCMR) annual meeting was an all-virtual event. This called for a modified social media strategy which aimed to re-create the sociability of an in-person conference whilst also promoting global dissemination of the science being presented. This paper describes the employment of social media as well as the evolution through the SCMR scientific sessions for 2020 and 2021 that serves as a model for future cardiovascular conferences.
Assuntos
COVID-19 , Mídias Sociais , Humanos , Espectroscopia de Ressonância Magnética , Pandemias , Valor Preditivo dos Testes , SARS-CoV-2RESUMO
BACKGROUND: The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study. METHODS: This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls. RESULTS: Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7-17 years) vs 13 years (range 4-14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m2 vs 93 ml/m2, p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS - 23.5% vs - 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (- 13.3% vs - 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m2 vs 120 ml/m2, p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (- 20.1% vs - 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = - 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = - 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = - 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m2 vs 118 ml/m2, p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (- 22% vs - 25%, p = 0.029). APC flow (HR 5.5 CI 1.9-16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30-1.69 p = 0.44) and T1 (HR1.006 CI 1.0-1.13, p = 0.07). CONCLUSIONS: Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes.
Assuntos
Ventrículos do Coração , Imagem Cinética por Ressonância Magnética , Adolescente , Criança , Fibrose , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/patologia , Ventrículos do Coração/cirurgia , Humanos , Valor Preditivo dos Testes , Estudos Retrospectivos , Função Ventricular EsquerdaRESUMO
OBJECTIVES: The objective of this study was to determine the evolution of fibrosis over time and its association with clinical status. METHODS: Children with repaired tetralogy of Fallot who had undergone at least two cardiac magnetic resonance examinations including T1 mapping at least 1 year apart were included. RESULTS: Thirty-seven patients (12.7 ± 2.6 years, 61% male) were included. Right ventricular free wall T1 increased (913 ± 208 versus 1023 ± 220 ms; p = 0.02). Baseline cardiac magnetic resonance parameters did not predict a change in imaging markers or exercise tolerance. The right ventricular free wall per cent change correlated with left ventricular T1% change (r = 0.51, p = 0.001) and right ventricular mass Z-score change (r = 0.51, p = 0.001). T1 in patients with late gadolinium enhancement did not differ from the rest. CONCLUSION: Increasing right ventricular free wall T1 indicates possible progressive fibrotic remodelling in the right ventricular outflow tract in this pilot study in children and adolescents with repaired tetralogy of Fallot. The value of T1 mapping both at baseline and during serial assessments will need to be investigated in larger cohorts with longer follow-up.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Fibrose , Tetralogia de Fallot , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Meios de Contraste , Feminino , Fibrose/etiologia , Gadolínio , Humanos , Masculino , Projetos Piloto , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgiaRESUMO
BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7⯱â¯6.4% vs. 48.5⯱â¯7.2%, Pâ¯=â¯.81) and exercise capacity (% predicted peak VO2:82.5⯱â¯17.7% vs. 75.6⯱â¯20.4%, Pâ¯=â¯.27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:râ¯=â¯-0.39, Pâ¯=â¯.04, rToF:râ¯=â¯-0.40, Pâ¯=â¯.03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:râ¯=â¯-0.77, Pâ¯<â¯.001, rToF:râ¯=â¯-0.69, Pâ¯<â¯.001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1⯱â¯3.9 vs.-25.7⯱â¯4.4, Pâ¯<â¯.001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.
Assuntos
Tolerância ao Exercício/fisiologia , Hipertrofia Ventricular Direita/fisiopatologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita/fisiologia , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia , Teste de Esforço , Feminino , Humanos , Hipertrofia Ventricular Direita/diagnóstico por imagem , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/patologia , Imageamento por Ressonância Magnética , Masculino , Análise por Pareamento , Consumo de Oxigênio , Complicações Pós-Operatórias/fisiopatologia , Atresia Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Volume Sistólico , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologiaRESUMO
BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion. METHODS: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls. RESULTS: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. CONCLUSION: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures. TRIAL REGISTRATION: Retrospectively registered data.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cirrose Hepática/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Estudos de Viabilidade , Feminino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Hemodinâmica , Humanos , Lactente , Cirrose Hepática/etiologia , Cirrose Hepática/fisiopatologia , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
BACKGROUND: Myocardial T1 relaxometry can be performed by contouring on individual T1-weighted source images (source method) or on a single T1 map (mapping method). OBJECTIVE: This study compares (a) agreement between native T1 and extracellular volume results of the two methods and (b) interobserver reproducibility of the two methods in children without heart disease and those with tetralogy of Fallot (TOF). MATERIALS AND METHODS: We retrospectively analyzed pediatric patients (controls and those with repaired TOF) with cardiac magnetic resonance examinations including extracellular volume quantification using the modified Look-Locker inversion recovery (MOLLI) sequence. We compared native T1 and extracellular volume of the entire left ventricle and interventricular septum derived using the source and the mapping approaches. RESULTS: In the control group (n=25, median age 14.0 years, interquartile range [IQR] 11.5-16.5 years), the mapping method produced lower native T1 values than the source method in the interventricular septum (mean difference ± standard deviation [SD] = 12±15 ms, P<0.001). In the TOF group (n=50, median age 13.3 years, IQR 9.9-15.0 years), the mapping method produced lower values for native T1 and extracellular volume in the interventricular septum (mean difference 9±14 ms and 0.6±1.1%, P<0.001). In 6-12% of the children, differences were >3 standard deviations from the mean difference. Interobserver reproducibility between the two methods by intraclass correlation coefficients were clinically equivalent. CONCLUSION: T1 and extracellular volume values generated by the source and mapping methods show systematic differences and can vary significantly in an individual child, and thus cannot be used interchangeably in clinical practice. The source method might allow for easier detection and, in some cases, mitigation of artifacts that are not infrequent in children and can be difficult to appreciate on the T1 map.
Assuntos
Imageamento por Ressonância Magnética/métodos , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Adolescente , Estudos de Casos e Controles , Criança , Meios de Contraste , Feminino , Humanos , Interpretação de Imagem Assistida por Computador , Masculino , Compostos Organometálicos , Reprodutibilidade dos Testes , Estudos RetrospectivosRESUMO
This technical innovation paper describes a technique for performing cardiac-gated, respiratory-navigated cardiovascular magnetic resonance angiography using an extracellular gadolinium-based contrast agent at 1.5 Tesla (T) with a dual phase bolus injection and slow infusion technique.
Assuntos
Técnicas de Imagem de Sincronização Cardíaca , Meios de Contraste/administração & dosagem , Cardiopatias Congênitas/diagnóstico por imagem , Angiografia por Ressonância Magnética/métodos , Compostos Organometálicos/administração & dosagem , Técnicas de Imagem de Sincronização Respiratória , Adolescente , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Imageamento Tridimensional , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/diagnóstico por imagemRESUMO
BACKGROUND/OBJECTIVE: Takayasu arteritis (TA) is characterized by extensive aortic, large and midsize arterial wall inflammation. The aim of this study was to assess the morphological and elastic properties of the aorta and large arteries and the impact on left ventricular (LV) mechanics in children with TA. METHODS: Seven pediatric TA patients (6 female patients, 13.8 ± 3.2 years) were assessed with magnetic resonance imaging, vascular ultrasound, applanation tonometry, and echocardiography from February 2015 until July 2017 and compared with 7 age- and sex-matched controls. Takayasu arteritis disease activity was assessed clinically by the Pediatric Vasculitis Activity Score (PVAS). RESULTS: Pediatric TA patients showed increased carotid-to-radial artery pulse wave velocity (8.1 ± 1.8 vs. 6.4 ± 0.6 m/s, p = 0.03) and increased carotid-to-femoral artery pulse wave velocity (8.3 ± 1.9 vs. 5.1 ± 0.8 m/s, p < 0.01) when compared with controls. Patients demonstrated increased LV mass index (74.3 ± 18.8 vs. 56.3 ± 10.9 g/m, p = 0.04), altered myocardial deformation with increased basal rotation (-9.8 ± 4.5 vs. -4.0 ± 2.0 degrees, p = 0.01) and torsion (19.9 ± 8.1 vs. 9.1 ± 3.1 degrees, p = 0.01), and impaired LV diastolic function with decreased mitral valve E/A ratio (1.45 ± 0.17 vs. 2.40 ± 0.84, p = 0.01), increased mitral valve E/E' ratio (6.8 ± 1.4 vs. 4.9 ± 0.7, p < 0.01), and increased pulmonary vein A-wave velocity (26.7 ± 5.7 vs. 16.8 ± 3.3 cm/s, p = 0.03). Carotid-to-radial artery pulse wave velocity was associated with systolic (R = 0.94, p < 0.01), diastolic (R = 0.85, p = 0.02), and mean blood pressure (R = 0.91, p < 0.01), as well as disease activity by PVAS (R = 0.75, p = 0.05). The PVAS was associated with carotid-to-radial artery pulse wave velocity (R = 0.75, p = 0.05), as well as systolic (R = 0.84, p = 0.02), diastolic (R = 0.82, p = 0.03), and mean blood pressure (R = 0.84, p = 0.02). CONCLUSIONS: Increased arterial stiffness is present in pediatric TA patients and associated with increased blood pressure and TA disease activity. Pediatric TA patients demonstrate altered LV mechanics, LV hypertrophy, and impaired diastolic function.
Assuntos
Aorta , Artérias , Arterite de Takayasu , Rigidez Vascular/fisiologia , Disfunção Ventricular Esquerda , Adolescente , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Artérias/diagnóstico por imagem , Artérias/fisiopatologia , Canadá , Criança , Correlação de Dados , Ecocardiografia/métodos , Técnicas de Imagem por Elasticidade/métodos , Feminino , Humanos , Masculino , Manometria/métodos , Gravidade do Paciente , Análise de Onda de Pulso/métodos , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/fisiopatologia , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/patologia , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
OBJECTIVES: The objective of this study was to investigate the effect of location and number of anomalously connected pulmonary veins and any associated atrial septal defect (ASD) on the magnitude of left-to-right shunting in patients with partial anomalous pulmonary venous connection (PAPVC), and how that influences right ventricular volume loading. METHODS AND RESULTS: The cardiac magnetic resonance (CMR) and echocardiography examinations of 26 paediatric patients (mean age, 11.2 ± 5.1 years) with unrepaired PAPVC were analysed. Fourteen patients had right-sided, 11 left-sided and 1 patient bilateral PAPVC. An ASD was present in 11 patients, of which none had a Qp/Qs < 1.5 and 8 had a Qp/Qs≥ 2.0. No patient with isolated left upper PAPVC experienced a Qp/Qs ≥ 2.0 compared to 9/12 patients with right upper PAPVC. Qp/Qs correlated with indexed right ventricle (RV) end-diastolic volume (RVEDVi, r = 0.59, p = 0.002) by CMR and with echocardiographic right ventricular end-diastolic dimension (RVED) z-score (r = 0.68, p = 0.003). A RVEDVi >124 ml/m2 by CMR and a RVED z-score >2.2 by echocardiography identified patients with a Qp/Qs ≥1.5 with good sensitivity and specificity. CONCLUSIONS: An asymptomatic patient with a single anomalously connected left upper pulmonary vein and without an ASD is unlikely to have a significant left-to-right shunt. On the other hand, right-sided PAPVC is frequently associated with a significant left-to-right shunt, especially when an ASD is present. KEY POINTS: ⢠Patients with PAPVC and ASD routinely have a significant left-to-right shunt. ⢠Patients with right PAPVC are likely to have a significant left-to-right shunt. ⢠Patients with left PAPVC are unlikely to have a significant left-to-right shunt. ⢠CMR is helpful in decision-making for patients with PAPVC.
Assuntos
Comunicação Interatrial/diagnóstico , Ventrículos do Coração/diagnóstico por imagem , Hemodinâmica/fisiologia , Imagem Cinética por Ressonância Magnética/métodos , Veias Pulmonares/anormalidades , Adolescente , Criança , Pré-Escolar , Ecocardiografia , Feminino , Comunicação Interatrial/fisiopatologia , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Masculino , Veias Pulmonares/diagnóstico por imagemRESUMO
CORRECTION TO: J CARDIOVASC MAGN RESON (2017) 19: 75. DOI: 10.1186/S12968-017-0389-8: In the original publication of this article [1] the "Competing interests" section was incorrect. The original publication stated the following competing interests.
RESUMO
Cardiovascular thrombosis is rare in children and usually occurs in the presence of predisposing conditions, such as indwelling vascular catheters, tumors, aneurysms, ventricular dysfunction, or after surgery. Clots can occur in the cardiac chambers, arteries or veins, or inside conduits. Detection of thrombi is feasible with a variety of magnetic resonance imaging (MRI) techniques, including unenhanced methods but also contrast-enhanced MR angiography. In this essay we illustrate the MRI appearance of cardiovascular thrombosis in children and suggest an imaging protocol based on our clinical experience.