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Atrial septal defects (ASDs) are common in neonates. Although past studies suggest ASDs ≥ 3 mm in term neonates (TNs) are less likely to close, there is paucity of data regarding the natural history in preterm neonates (PNs), information that would inform surveillance. We sought to compare spontaneous closure rates and need for intervention for ASDs in TNs/near term (≥ 36 weeks) versus PNs (< 36 weeks). We included all TNs and PNs who underwent echocardiography at ≤ 1 month between 2010 and 2018 in our institution with an ASD ≥ 3 mm, without major congenital heart disease, and with repeat echocardiogram(s). Spontaneous resolution was defined as size diminution to < 3 mm or closure. We included 156 TNs (mean gestational age at birth 38.6 ± 1.4 weeks) and 156 PNs (29.6 ± 3.7 weeks) with a mean age at follow-up of 16 ± 19 and 15 ± 21 months, respectively (p = 0.76). Based on maximum color Doppler diameter, in TNs, ASD resolution occurred in 95% of small (3-5 mm), 87% of moderate (5.1-8 mm), and 60% of large (> 8 mm) defects; whereas, in PNs, resolution occurred in 79% of small, 76% of moderate, and 33% of large ASDs. There was a significant association between size and ASD resolution in TNs (p = 0.003), but not PNs (p = 0.17). Overall, ASD resolution rate was higher in TNs (89%) versus PNs (78%) (p = 0.009), and fewer TNs (1%) compared to PNs (7%) required ASD intervention (p = 0.02). Most ASDs identified in TNs and PNs spontaneously resolve. PNs, however, demonstrate lower ASD resolution and higher intervention rates within all size groups. These data should inform follow-up of affected neonates.
Assuntos
Comunicação Interatrial , Recém-Nascido , Humanos , Comunicação Interatrial/diagnóstico por imagem , Ecocardiografia , Ultrassonografia Doppler em Cores , Resultado do Tratamento , Cateterismo CardíacoRESUMO
BACKGROUND: Aortopathy in Turner syndrome is associated with aortic dilation, and the risk of dissection is increased when the aortic size index is ≥ 2-2.5 cm/m2. We evaluated the aortic biophysical properties in paediatric Turner syndrome using cardiac MRI to determine their relationship to aortic size index. METHODS: Turner syndrome patients underwent cardiac MRI to evaluate ventricular function, aortic dimensions, and biophysical properties (aortic stiffness index, compliance, distensibility, pulse wave velocity, and aortic and left ventricular elastance). Spearman correlation examined correlations between these properties and aortic size index. Data was compared to 10 controls. RESULTS: Of 25 Turner syndrome patients, median age 14.7 years (interquartile range: 11.0-16.8), height z score -2.7 (interquartile range: -2.92 - -1.54), 24% had a bicuspid aortic valve. Turner syndrome had increased diastolic blood pressure (p < 0.001) and decreased left ventricular end-diastolic (p < 0.001) and end-systolic (p = 0.002) volumes compared to controls. Median aortic size index was 1.81 cm/m2 (interquartile range: 1.45-2.1) and 7 had an aortic size index > 2 cm/m2. Aortic and left ventricular elastance were greater in Turner syndrome compared to controls (both p < 0.001). Increased aortic size index correlated with increased aortic elastance (r = 0.5, p = 0.01) and left ventricular elastance (r = 0.59, p = 0.002) but not aortic compliance. Higher ascending aortic areas were associated with increased aortic compliance (r = 0.44, p = 0.03) and left ventricular elastance (r = 0.49, p = 0.01). CONCLUSION: Paediatric Turner syndrome with similar aortic size index to controls showed MRI evidence of abnormal aortic biophysical properties. These findings point to an underlying aortopathy and provide additional parameters that may aid in determining risk factors for aortic dissection.
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BACKGROUND: Fontan associated liver disease (FALD) is an increasingly recognized complication of the single ventricle circulation characterized by hepatic venous congestion leading to hepatic fibrosis. Within the Fontan myocardium, fibrotic myocardial remodeling may occur and lead to ventricular dysfunction. Magnetic resonance imaging (MRI) T1 mapping can characterize both myocardial and liver properties. OBJECTIVE: The aim of this study was to compare myocardial and liver T1 between single ventricle patients with and without a Fontan and biventricular controls. MATERIALS AND METHODS: A retrospective study of 3 groups of patients: 16 single ventricle patients before Fontan (SVpre 2 newborns, 9 pre-Glenn, 5 pre-Fontan, 31% single right ventricle [SRV]), 16 Fontans (56% SRV) and 10 repaired d-transposition of the great arteries (TGA). Native modified Look-Locker inversion T1 times were measured in the myocardium and liver. Cardiac MRI parameters, myocardial and liver T1 values were compared in the three groups. Correlations were assessed between liver T1 and cardiac parameters. RESULTS: Myocardial T1 was higher in SVpre (1,056 ± 48 ms) and Fontans (1,047 ± 41 ms) compared to TGA (1,012 ± 48 ms, P < 0.05). Increased liver T1 was found in both SVpre (683 ± 82 ms) and Fontan (727 ± 49 ms) patients compared to TGA patients (587 ± 58 ms, P < 0.001). There was no difference between single left ventricle (SLV) versus SRV myocardial or liver T1. Liver T1 showed moderate correlations with myocardial T1 (r = 0.48, confidence interval [CI] 0.26-0.72) and ejection fraction (r = -0.36, CI -0.66-0.95) but not with other volumetric parameters. CONCLUSION: Increased liver T1 at both pre- and post-Fontan stages suggests there are intrinsic liver abnormalities early in the course of single ventricle palliation. Increased myocardial T1 and its relationship to liver T1 suggest a combination of edema from passive venous congestion and/or myocardial fibrosis occurring in this population. Liver T1 may provide an earlier marker of liver disease warranting further study.
Assuntos
Hiperemia , Transposição dos Grandes Vasos , Recém-Nascido , Humanos , Estudos Retrospectivos , Hiperemia/patologia , Miocárdio/patologia , Fibrose , Fígado/diagnóstico por imagem , Fígado/patologia , Imagem Cinética por Ressonância Magnética , Valor Preditivo dos TestesRESUMO
PURPOSE: To develop and validate a three-parameter model for improved precision multiparametric SAturation-recovery single-SHot Acquisition (mSASHA) cardiac T1 and T2 mapping with high accuracy in a single breath-hold. METHODS: The mSASHA acquisition consists of nine images of variable saturation recovery and T2 preparation in 11 heartbeats with T1 and T2 values calculated using a three-parameter model. It was validated in simulations and phantoms at 3 T with comparison to a four-parameter joint T1 -T2 technique. The mSASHA acquisition was compared with MOLLI, SASHA, and T2 -prepared balanced SSFP in 10 volunteers. RESULTS: The mSASHA technique had high accuracy in phantoms compared to spin echo, with -0.2 ± 0.3% T1 error and -2.4 ± 1.3% T2 error. The mSASHA coefficient of variation in phantoms for T1 was similar to MOLLI (0.7 ± 0.2% for both) and T2 -prepared balanced SSFP for T2 (1.3 ± 0.7% vs 1.4 ± 0.3%, adjusted p > .05 for both). In simulations, three-parameter mSASHA had higher precision than four-parameter joint T1 -T2 for both T1 and T2 (46% and 11% reductions in T1 and T2 interquartile range for native myocardium). In vivo myocardial mSASHA T1 was similar to SASHA (1523 ± 18 ms vs 1520 ± 18 ms) with similar coefficient of variation to both MOLLI and SASHA (3.3 ± 0.6% vs 3.1 ± 0.6% and 3.3 ± 0.5% respectively, adjusted p > .05 for all). Myocardial mSASHA T2 was 37.1 ± 1.1 ms with similar precision to T2 -prepared balanced SSFP (6.7 ± 1.7% vs 6.0 ± 1.6%, adjusted p > .05). CONCLUSION: Three-parameter mSASHA provides high-accuracy cardiac T1 and T2 quantification in a single breath-hold with similar precision to MOLLI and T2 -prepared balanced SSFP. Further study is required to both establish normative values and demonstrate clinical utility in patient populations.
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Imageamento por Ressonância Magnética , Miocárdio , Coração/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética/métodos , Imagens de Fantasmas , Reprodutibilidade dos TestesRESUMO
PURPOSE: To describe and validate a simultaneous proton density fat-fraction (PDFF) imaging and water-specific T1 mapping (T1(Water) ) approach for the liver (PROFIT1 ) with R2∗ mapping and low sensitivity to B1+ calibration or inhomogeneity. METHODS: A multiecho gradient-echo sequence, with and without saturation preparation, was designed for simultaneous imaging of liver PDFF, R2∗ , and T1(Water) (three slices in ~13 seconds). Chemical-shift-encoded MRI processing yielded fat-water separated images and R2∗ maps. T1(Water) calculation utilized saturation and nonsaturation-recovery water-separated images. Several variable flip angle schemes across k-space (increasing flip angles in sequential RF pulses) were evaluated for minimization of T1 weighting, to reduce the B1+ dependence of T1(Water) and PDFF (reduced flip angle dependence). T1(Water) accuracy was validated in mixed fat-water phantoms, with various PDFF and T1 values (3T). In vivo application was illustrated in five volunteers and five patients with nonalcoholic fatty liver disease (PDFF, T1(Water) , R2∗ ). RESULTS: A sin3 (θ) flip angle pattern (0 < θ < π/2 over k-space) yielded the largest PROFIT1 signal yield with negligible B1+ dependence for both T1(Water) and PDFF. Mixed fat-water phantom experiments illustrated excellent agreement between PROFIT1 and gold-standard spectroscopic evaluation of PDFF and T1(Water) (<1% T1 error). In vivo PDFF, T1(Water) , and R2∗ maps illustrated independence of the PROFIT1 values from B1+ inhomogeneity and significant differences between volunteers and patients with nonalcoholic fatty liver disease for T1(Water) (927 ± 56 ms vs. 1033 ± 23 ms; P < .05) and PDFF (2.0% ± 0.8% vs. 13.4% ± 5.0%, P < .05). R2∗ was similar between groups. CONCLUSION: The PROFIT1 pulse sequence provides fast simultaneous quantification of PDFF, T1(Water) , and R2∗ with minimal sensitivity to B1+ miscalibration or inhomogeneity.
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Fígado , Hepatopatia Gordurosa não Alcoólica , Prótons , Tecido Adiposo , Humanos , Fígado/diagnóstico por imagem , Imageamento por Ressonância Magnética , Hepatopatia Gordurosa não Alcoólica/diagnóstico por imagem , Reprodutibilidade dos Testes , ÁguaRESUMO
BACKGROUND: Global longitudinal strain (GLS), most commonly measured at the endocardium, has been shown to be superior to left ventricular (LV) ejection fraction (LVEF) for the identification of systolic dysfunction and prediction of outcomes in heart failure (HF). We hypothesized that strains measured at different myocardial layers (endocardium = ENDO, epicardium = EPI, average = AVE) will have distinct diagnostic and predictive performance for patients with HF. METHODS: Layer-specific GLS, layer-specific global circumferential strain (GCS) and global radial strain (GRS) were evaluated by cardiovascular magnetic resonance imaging (CMR) feature tracking in the Alberta HEART study. A total of 453 subjects consisted of healthy controls (controls, n = 77), at-risk for HF (at-risk, n = 143), HF with preserved ejection fraction (HFpEF, n = 87), HF with mid-range ejection fraction (HFmrEF, n = 88) and HF with reduced ejection fraction (HFrEF, n = 58). For outcomes analysis, CMR-derived imaging parameters were adjusted with a base model that included age and N-terminal prohormone of b-type natriuretic peptide (NT-proBNP) to test their independent association with 5-year all-cause mortality. RESULTS: GLS_EPI distinguished all groups with preserved LVEF (controls - 16.5 ± 2.4% vs. at-risk - 15.5 ± 2.7% vs. HFpEF - 14.1 ± 3.0%, p < 0.001) while GLS_ENDO and all GCS (all layers) were similar among these groups. GRS was reduced in HFpEF (41.1 ± 13.8% versus 48.9 ± 10.7% in controls, p < 0.001) and the difference between GLS_EPI and GLS_ENDO were significantly larger in HFpEF as compared to controls. Within the preserved LVEF groups, reduced GRS and GLS_EPI were significantly associated with increased LV mass (LVM) and LVM/LV end-diastolic volume EDV (concentricity). In multivariable analysis, only GLS_AVE and GRS predicted 5-year all-cause mortality (all ps < 0.05), with the strongest association with 5-year all-cause mortality by Akaike Information Criterion analysis and significant incremental value for outcomes prediction beyond LVEF or GLS_ENDO by the likelihood ratio test. CONCLUSION: Global strains measured on endocardium, epicardium or averaged across the wall thickness are not equivalent for the identification of systolic dysfunction or outcomes prediction in HF. The endocardium-specific strains were shown to have poorest all-around performance. GLS_AVE and GRS were the only CMR parameters to be significantly associated with 5-year all-cause mortality in multivariable analysis. GLS_EPI and GRS, as well as the difference in endocardial and epicardial strains, were sensitive to systolic dysfunction among HF patients with normal LVEF (> 55%), in whom lower strains were associated with increased concentricity.
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Insuficiência Cardíaca/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Idoso , Alberta , Fenômenos Biomecânicos , Estudos de Casos e Controles , Feminino , Fatores de Risco de Doenças Cardíacas , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Medição de Risco , Disfunção Ventricular Esquerda/mortalidade , Disfunção Ventricular Esquerda/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.
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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: Pulmonary edema is a cardinal feature of heart failure but no quantitative tests are available in clinical practice. The goals of this study were to develop a simple cardiovascular magnetic resonance (CMR) approach for lung water quantification, to correlate CMR derived lung water with intra-cardiac pressures and to determine its prognostic significance. METHODS: Lung water density (LWD, %) was measured using a widely available single-shot fast spin-echo acquisition in two study cohorts. Validation Cohort: LWD was compared to left ventricular end-diastolic pressure or pulmonary capillary wedge pressure in 19 patients with heart failure undergoing cardiac catheterization. Prospective Cohort: LWD was measured in 256 subjects, including 121 with heart failure, 82 at-risk for heart failure and 53 healthy controls. Clinical outcomes were evaluated up to 1 year. RESULTS: Within the validation cohort, CMR LWD correlated to invasively measured left-sided filling pressures (R = 0.8, p < 0.05). In the prospective cohort, mean LWD was 16.6 ± 2.1% in controls, 17.9 ± 3.0% in patients at-risk and 19.3 ± 5.4% in patients with heart failure, p < 0.001. In patients with or at-risk for heart failure, LWD > 20.8% (mean + 2 standard deviations of healthy controls) was an independent predictor of death, hospitalization or emergency department visit within 1 year, hazard ratio 2.4 (1.1-5.1, p = 0.03). CONCLUSIONS: In patients with heart failure, increased CMR-derived lung water is associated with increased intra-cardiac filling pressures, and predicts 1 year outcomes. LWD could be incorporated in standard CMR scans.
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Água Extravascular Pulmonar/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Edema Pulmonar/diagnóstico por imagem , Adulto , Idoso , Estudos de Casos e Controles , Causas de Morte , Progressão da Doença , Serviço Hospitalar de Emergência , Água Extravascular Pulmonar/metabolismo , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/terapia , Humanos , Pulmão/metabolismo , Masculino , Pessoa de Meia-Idade , Admissão do Paciente , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Edema Pulmonar/etiologia , Edema Pulmonar/mortalidade , Edema Pulmonar/terapia , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Fatores de TempoRESUMO
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.
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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.
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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: Understanding cardiac MR T1 mapping values might require examination of the effects of age, gender, and heart failure risk factors. PURPOSE/HYPOTHESIS: To evaluate the effects of gender, age, and presence of heart failure risk factors on myocardial native T1 and extracellular volume fraction (ECV). STUDY TYPE: Retrospective, cross-sectional, observational study. POPULATION: Secondary analysis of cardiac MR data, separated by gender and health status, based on the presence of at least one heart failure risk factor. FIELD STRENGTH/SEQUENCE: Cardiac MR imaging at 1.5T, including T1 mapping using the SAturation recovery single-SHot Acquisition (SASHA) sequence. ASSESSMENT: Interventricular septal region-of-interest analysis for assessment of native T1 and ECV. STATISTICAL TESTS: Group comparisons performed using Student t-test, or nonparametric equivalent. Linear regression was used to assess relationships between age and T1 measurements. RESULTS: Native T1 and ECV were available in 187 and 143 subjects, respectively. T1 and ECV were independent of age in all groups (Native T1 : healthy women P = 0.655; healthy men P = 0.906; at-risk women P = 0.487; at-risk men P = 0.683; ECV: healthy women P = 0.685; healthy men P = 0.199; at-risk women P = 0.152; at-risk men P = 0.747). T1 and ECV were higher in healthy women versus men (1202 ± 30 ms versus 1167 ± 36 ms, P = 0.0000 and 22 ± 2% versus 20 ± 2%, P = 0.0089), while values were similar in women and men with risk factors (1197 ± 55 ms versus 1193 ± 45 ms, P = 0.6556, 21 ± 2% versus 21 ± 3%, P = 0.5039). No differences existed in native T1 or ECV between women with or without risk factors (P = 0.6344 and P = 0.1026), whereas men with risk factors showed higher native T1 values (P = 0.0070). DATA CONCLUSION: Native T1 and ECV measured with SASHA do not vary with age, regardless of gender or the presence of factors for heart failure. Native T1 and ECV are higher in healthy women than men, but do not differ in the presence of risk factors, suggesting a different myocardial response to risk factors between genders. LEVEL OF EVIDENCE: 3 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:1307-1317.
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Insuficiência Cardíaca/diagnóstico por imagem , Coração/diagnóstico por imagem , Processamento de Imagem Assistida por Computador/métodos , Imageamento por Ressonância Magnética , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Adulto JovemRESUMO
PURPOSE: Conventional calculation of myocardial strain requires tissue-tracking. A surrogate for strain called global fractional shortening (GFS) is proposed based on changes in dimensions of endocardial and epicardial surfaces without tissue-tracking. METHODS: Three-dimensional endocardial and epicardial left ventricular surfaces traced at end-diastole and end-systole using conventional steady-state free precession cine images were used to calculate GFScc (circumferential), GFSll (longitudinal), and GFSrr (radial) using fractional length changes in each direction over the heart surface. GFS values were validated using finite element models (FEM) and in vivo using tagging-derived strains (εcc ,εll ,εrr ) in patients with a wide range of ejection fraction (EF) and diagnosis (n=32). GFS was also measured in 31 patients with Fabry disease and matched healthy controls. RESULTS: GFS values were within 3% of average FEM-derived Lagrangian strains and had good agreement in vivo (GFScc =-14 ± 4%, εcc =-14 ± 4%, R(2) =0.85; GFSll =-12 ± 4%, εll =-12 ± 4%, R(2) =0.72; GFSrr =46 ± 21%). εrr could not be measured reliably from tagging. Compared with healthy controls with matched EF, patients with Fabry disease had significantly increased GFScc (Endo) (-28 ± 3% versus -25 ± 2%), decreased GFScc(Epi) (-10 ± 2% versus -11 ± 2%) and decreased GFSll for all components. CONCLUSION: GFS yields similar values to conventionally measured strains without requiring tissue-tracking. Compared with controls, patients with Fabry disease have significant differences in several GFS components.
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Técnicas de Imagem por Elasticidade/métodos , Doença de Fabry/diagnóstico , Doença de Fabry/fisiopatologia , Imagem Cinética por Ressonância Magnética/métodos , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/fisiopatologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Algoritmos , Anisotropia , Módulo de Elasticidade , Doença de Fabry/complicações , Feminino , Humanos , Interpretação de Imagem Assistida por Computador/métodos , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Disfunção Ventricular Esquerda/etiologiaRESUMO
PURPOSE: To validate a new saturation recovery single-shot acquisition (SASHA) pulse sequence for T1 mapping and to compare SASHA T1 values in heart failure patients and healthy controls. THEORY: The SASHA sequence consists of 10 electrocardiogram-triggered single-shot balanced steady-state free precession images in a breath-hold. The first image is acquired without magnetization preparation and the remaining nine images follow saturation pulses with variable saturation recovery times. METHODS: SASHA was validated through Bloch equation simulations, Monte Carlo simulations, and phantom experiments. Pre- and postcontrast myocardial and blood T1 values were measured in 29 healthy volunteers and 7 patients with heart failure. RESULTS: SASHA T1 values had excellent agreement (bias, 5 ± 5 ms) with spin echo experiments in phantoms with a wide range of physiologic T1 and T2 values and its accuracy was independent of flip angle, absolute T1 , T2 , and heart rate. The average baseline myocardial T1 in heart failure patients was higher than in healthy controls (1200 ± 32 vs. 1170 ± 9 ms, P < 0.05) at 1.5T, as was the calculated blood-tissue partition coefficient, λ, (0.42 ± 0.04 vs. 0.38 ± 0.02, P < 0.05), consistent with diffuse myocardial fibrosis. CONCLUSIONS: The SASHA sequence is a simple and fast approach to in vivo T1 mapping with good accuracy in simulations and phantom experiments.
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Técnicas de Imagem de Sincronização Cardíaca , Insuficiência Cardíaca/patologia , Aumento da Imagem/métodos , Interpretação de Imagem Assistida por Computador/métodos , Imagem Cinética por Ressonância Magnética/métodos , Meios de Contraste , Feminino , Gadolínio DTPA , Humanos , Masculino , Miocárdio/patologia , Compostos Organometálicos , Imagens de Fantasmas , Reprodutibilidade dos TestesRESUMO
BACKGROUND: The late cardiotoxic effects of anthracycline chemotherapy influence morbidity and mortality in the growing population of childhood cancer survivors. Even with lower anthracycline doses, evidence of adverse cardiac remodeling and reduced exercise capacity exist. We aim to examine the relationship between cardiac structure, function and cardiovascular magnetic resonance (CMR) tissue characteristics with chemotherapy dose and exercise capacity in childhood cancer survivors. METHODS: Thirty patients (15 ± 3 years), at least 2 years following anthracycline treatment, underwent CMR, echocardiography, and cardiopulmonary exercise testing (peak VO(2)). CMR measured ventricular function, mass, T(1) and T(2) values, and myocardial extracellular volume fraction, ECV, a measure of diffuse fibrosis based on changes in myocardial T1 values pre- and post-gadolinium. Cardiac function was also assessed with conventional and speckle tracking echocardiography. RESULTS: Patients had normal LVEF (59 ± 7%) but peak VO(2) was 17% lower than age-predicted normal values and were correlated with anthracycline dose (r = -0.49). Increased ECV correlated with decreased mass/volume ratio (r = -0.64), decreased LV wall thickness/height ratio (r = -0.72), lower peak VO(2)(r = -0.52), and higher cumulative dose (r = 0.40). Echocardiographic measures of systolic and diastolic function were reduced compared to normal values (p < 0.01), but had no relation to ECV, peak VO(2) or cumulative dose. CONCLUSIONS: Myocardial T1 and ECV were found to be early tissue markers of ventricular remodeling that may represent diffuse fibrosis in children with normal ejection fraction post anthracycline therapy, and are related to cumulative dose, exercise capacity and myocardial wall thinning.
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Antraciclinas/efeitos adversos , Fibrose Endomiocárdica/induzido quimicamente , Fibrose Endomiocárdica/diagnóstico , Fibrose Endomiocárdica/fisiopatologia , Imageamento por Ressonância Magnética/métodos , Resistência Física/fisiologia , Adolescente , Criança , Meios de Contraste , Estudos Transversais , Ecocardiografia , Teste de Esforço , Feminino , Gadolínio DTPA , Humanos , Modelos Lineares , Masculino , Neoplasias/tratamento farmacológico , Oxigênio/sangue , Fatores de Risco , Sobreviventes , Remodelação Ventricular/efeitos dos fármacosRESUMO
Background: Prenatal alcohol exposure (PAE) has teratogenic effects on numerous body systems including the heart. However, research magnetic resonance imaging (MRI) studies in humans with PAE have thus far been limited to the brain. This study aims to use MRI to examine heart structure and function, brain volumes, and body composition in children and adolescents with PAE. Methods: Heart, brain, and abdominal 3T MRI of 17 children, adolescents, and young adults with PAE and 53 unexposed controls was acquired to measure: (1) left ventricular ejection fraction, end-diastolic volume, end-systolic volume, stroke volume, cardiac output, longitudinal strain, circumferential strain, and heart mass; (2) total brain, cerebellum, white matter, grey matter, caudate, thalamus, putamen, and globus pallidus volumes; and (3) subcutaneous fat, visceral fat, muscle fat, and muscle (body composition). Results: Cardiac MRI revealed no abnormalities in the PAE group on evaluation by a paediatric cardiologist and by statistical comparison with a control group. Cardiac parameters in both groups were in line with previous reports, including expected sex- and age-related differences. Cerebellum, caudate, and globus pallidus volumes were all smaller. Body mass index and subcutaneous fat percent were higher in females with PAE relative to control females, but lower in males with PAE relative to control males. Conclusions: Children with PAE did not have abnormalities in MRI-derived measures of cardiac structure or function despite smaller brain volumes and sex-specific differences in body composition relative to healthy controls.
Contexte: L'exposition prénatale à l'alcool (EPA) engendre des effets tératogènes dans de nombreux systèmes et organes du corps humain, notamment le cÅur. Cependant, la recherche à l'aide de l'imagerie par résonance magnétique (IRM) chez des humains ayant des antécédents d'EPA s'est limitée aux effets sur le cerveau jusqu'à maintenant. Cette étude vise à utiliser l'IRM pour examiner la fonction et la structure du cÅur, le volume de diverses parties du cerveau ainsi que la composition corporelle chez des enfants et des adolescents ayant des antécédents d'EPA. Méthodologie: Chez 17 enfants, adolescents et jeunes adultes ayant été exposés à l'alcool au stade prénatal et chez 53 personnes n'ayant pas d'antécédents d'EPA, des images du cÅur, du cerveau et de l'abdomen ont été acquises par la technique d'IRM 3T afin de mesurer : i) la fraction d'éjection du ventricule gauche, le volume télédiastolique, le volume télésystolique, le volume de sang éjecté, le débit cardiaque, la déformation longitudinale, la déformation circonférentielle et la masse cardiaque; ii) les volumes du cerveau en entier, du cervelet, de la substance blanche, de la substance grise, du noyau caudé, du thalamus, du putamen et du globus pallidus; et iii) le pourcentage de tissu adipeux contenu sous la peau, dans les viscères et dans les muscles ainsi que le pourcentage de muscles (composition corporelle). Résultats: Les images obtenues par l'IRM cardiaque n'ont pas révélé d'anomalies chez le groupe ayant des antécédents d'EPA après évaluation par un cardiologue pédiatrique et comparaison statistique avec le groupe témoin. Les paramètres cardiaques mesurés chez les deux groupes reflétaient les données ayant été précédemment rapportées, y compris les attentes liées aux différences quant au sexe et à l'âge. Les volumes du cervelet, du noyau caudé et du globus pallidus étaient diminués chez les personnes ayant des antécédents d'EPA. Alors que l'indice de masse corporelle et le pourcentage de tissu adipeux sous-cutané étaient plus élevés chez les personnes de sexe féminin ayant des antécédents d'EPA que chez les personnes de sexe féminin appartenant au groupe témoin, ces mêmes paramètres se trouvaient diminués chez les personnes de sexe masculin ayant des antécédents d'EPA comparativement aux personnes de sexe masculin appartenant au groupe témoin. Conclusions: Chez les enfants ayant des antécédents d'EPA, les mesures de la fonction et de la structure cardiaques dérivées des données de l'IRM ne présentaient pas d'anomalies, bien qu'une diminution du volume de certaines parties du cerveau et des différences dans la composition corporelle propres au sexe aient été observées dans ce groupe comparativement aux personnes en santé appartenant au groupe témoin.
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Background: Fontan-associated liver disease (FALD) is characterized by hepatic congestion and progressive hepatic fibrosis in patients with the Fontan operation. This condition is generally clinically silent until late, necessitating techniques for early detection. Liver T1 mapping has been used to screen for FALD, but without consideration of regional variations in T1 values. Methods: Liver T1 measured with a liver-specific T1 mapping sequence (PROFIT1) in Fontan patients was compared with cohorts of patients with biventricular congenital heart disease (BiV-CHD) and controls with normal cardiac function and anatomy. Results: Liver T1 was significantly elevated in the Fontan cohort (n = 20) compared with patients with BiV-CHD (n = 12) and controls (n = 9) (781, 678, and 675 milliseconds, respectively; P < 0.001), with a consistent pattern of significantly elevated T1 values in the peripheral compared with central liver regions (ΔT1 = 54, 2, and 11 milliseconds; P < 0.001). PROFIT1 also yielded simultaneous T2∗ maps and fat fraction values that were similar in all groups. Fontan liver T1 values were also significantly elevated as compared with BiV-CHD and controls as measured with the cardiac (modified Look-Locker inversion) acquisitions (728, 583, and 583 milliseconds, respectively; P < 0.001) and values correlated with PROFIT1 liver T1 (R = 0.87, P < 0.001). Conclusions: Fontan patients have globally increased liver T1 values and consistent spatial variations, with higher values in the peripheral liver regions as compared with spatially uniform values in BiV-CHD and controls. The spatial patterns may provide insight into the progression of FALD. Liver T1 mapping studies should include uniform spatial coverage to avoid bias based on slice locations in this population.
Contexte: L'hépatopathie associée à une intervention de Fontan (FALD, pour Fontan-associated liver disease) se caractérise par une congestion hépatique et une fibrose hépatique évolutive chez les patients qui ont subi une intervention de Fontan. Il s'agit d'un état pathologique silencieux en début de progression, pour lequel des techniques de détection précoce sont requises. La cartographie T1 du foie est utilisée pour le dépistage de la FALD, mais sans que les variations locales des valeurs obtenues soient prises en compte. Méthodologie: Des valeurs T1 hépatiques ont été mesurées avec une séquence cartographique conçue pour le foie (PROFIT1) chez des patients qui ont subi une intervention de Fontan. Ces valeurs ont été comparées à celles d'une cohorte de patients atteints de cardiopathie congénitale biventriculaire (CC-BiV) et à celles de témoins dont l'anatomie et la fonction cardiaques étaient normales. Résultats: Les valeurs T1 hépatiques étaient significativement plus élevées chez les patients ayant subi une intervention de Fontan (n = 20) que chez les patients atteints de CC-BiV (n = 12) et chez les témoins (n = 9) (781 ms, 678 ms, 675 ms, p < 0,001), et ces valeurs tendaient à être plus élevées dans les régions périphériques que dans les régions centrales du foie (ΔT1 = 54 ms, 2 ms, 11 ms, p < 0,001). La séquence PROFIT1 a aussi permis l'obtention des valeurs de cartographie T2∗ et de teneur en matières grasses dans le foie, et ces valeurs étaient comparables pour tous les groupes. L'utilisation d'une séquence cardiaque (MOLLI, pour modified Lock-Locker inversion) a également engendré des valeurs T1 hépatiques significativement plus élevées chez les patients ayant subi l'intervention de Fontan que chez les patients atteints de CC-BiV et les témoins (728 ms, 583 ms, 583 ms, p < 0,001). Ces valeurs étaient par ailleurs corrélées avec les valeurs T1 hépatiques obtenues par la séquence PROFIT1 (R = 0,87, p < 0,001). Conclusions: Dans l'ensemble, les patients ayant subi l'intervention de Fontan présentaient des valeurs T1 hépatiques élevées accompagnées de variations spatiales. Les valeurs périphériques étaient systématiquement plus élevées, tandis que celles obtenues chez les patients atteints de CC-BiV et chez les témoins étaient uniformes. Les tendances qui sous-tendent ces variations spatiales pourraient fournir des pistes pour mieux comprendre la progression de la FALD. Enfin, les études de cartographie T1 hépatiques dans cette population devraient couvrir uniformément le foie pour éviter les biais liés à la coupe.
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Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C. Design, Setting, and Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries). Patients with MIS-C (defined by Centers for Disease Control and Prevention criteria) and patients with KD (defined by American Heart Association criteria) were included. Patients with KD who had evidence of a recent COVID-19 infection or missing or unknown COVID-19 status were excluded. Main Outcomes and Measures: Patient demographic characteristics, clinical features, disease course, and outcome variables were collected from the IKDR data set. Using body mass index (BMI)/weight z score percentile equivalents, patient weight was categorized as normal weight (BMI <85th percentile), overweight (BMI ≥85th to <95th percentile), and obese (BMI ≥95th percentile). The association between adiposity category and clinical features and outcomes was determined separately for KD and MIS-C patient groups. Results: Of 1767 children, 338 with KD (median age, 2.5 [IQR, 1.2-5.0] years; 60.4% male) and 1429 with MIS-C (median age, 8.7 [IQR, 5.3-12.4] years; 61.4% male) were contemporaneously included in the study. For patients with MIS-C vs KD, the prevalence of overweight (17.1% vs 11.5%) and obesity (23.7% vs 11.5%) was significantly higher (P < .001), with significantly higher adiposity z scores, even after adjustment for age, sex, and race and ethnicity. For patients with KD, apart from intensive care unit admission rate, adiposity category was not associated with laboratory test features or outcomes. For patients with MIS-C, higher adiposity category was associated with worse laboratory test values and outcomes, including a greater likelihood of shock, intensive care unit admission and inotrope requirement, and increased inflammatory markers, creatinine levels, and alanine aminotransferase levels. Adiposity category was not associated with coronary artery abnormalities for either MIS-C or KD. Conclusions and Relevance: In this international cohort study, obesity was more prevalent for patients with MIS-C vs KD, and associated with more severe presentation, laboratory test features, and outcomes. These findings suggest that obesity as a comorbid factor should be considered at the clinical presentation in children with MIS-C.
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COVID-19 , Síndrome de Linfonodos Mucocutâneos , Criança , Estados Unidos/epidemiologia , Humanos , Masculino , Pré-Escolar , Feminino , COVID-19/epidemiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , SARS-CoV-2 , Estudos de Coortes , Sobrepeso , Obesidade/complicações , Obesidade/epidemiologiaRESUMO
AIMS: An improved understanding of the pathophysiology of trastuzumab-mediated cardiotoxicity is required to improve outcomes of patients with human epidermal growth factor receptor 2 (HER2)-positive breast cancer. We aimed to characterize the cardiac and cardiometabolic phenotype of trastuzumab-mediated toxicity and potential interactions with cardiac pharmacotherapy. METHODS AND RESULTS: This study was an analysis of serial magnetic resonance imaging (MRI) and circulating biomarker data acquired from patients with HER2-positive early-stage breast cancer participating in a randomized-controlled clinical trial for the pharmaco-prevention of trastuzumab-associated cardiotoxicity. Circulating biomarkers (B-type natriuretic peptide, troponin I, MMP-2 and -9, GDF-15, neuregulin-1, and IGF-1) and MRI of cardiac structure and function and abdominal fat distribution were acquired prior to trastuzumab, post-cycle 4 and post-cycle 17. Ninety-four participants (51 ± 8 years) completed the study with 30 on placebo, 33 on perindopril, and 31 on bisoprolol. Post-cycle 4, global longitudinal strain deteriorated from baseline in both placebo (+2.0 ± 2.7%, P = 0.002) and perindopril (+0.9 ± 2.5%, P = 0.04), but not with bisoprolol (-0.2 ± 2.1%, P = 0.55). In all groups combined, extracellular volume fraction and GDF-15 increased post-cycle 4 (+1.3 ± 4.4%, P = 0.004; +130 ± 150%, P ≤ 0.001, respectively). However, no significant change in troponin I was detected throughout trastuzumab. In all groups combined, visceral and intermuscular fat volume increased post-cycle 4 (+7 ± 17%, P = 0.02, +8 ± 23%, P = 0.02, respectively), while muscle volume and IGF-1 decreased from post-cycle 4 to 17 (-2 ± 10%, P = 0.008, -18 ± 28%, P < 0.001, respectively). CONCLUSION: Trastuzumab results in impaired cardiac function and early myocardial inflammation. Trastuzumab is also associated with deleterious changes to the cardiometabolic phenotype which may contribute to the increased cardiovascular risk in this population.
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Neoplasias da Mama , Cardiotoxicidade , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/genética , Neoplasias da Mama/patologia , Cardiotoxicidade/prevenção & controle , Feminino , Humanos , Peptídeo Natriurético Encefálico/uso terapêutico , Trastuzumab/efeitos adversos , Troponina IRESUMO
BACKGROUND: MANTICORE 101 - Breast (Multidisciplinary Approach to Novel Therapies in Cardiology Oncology Research) is a randomized trial to determine if conventional heart failure pharmacotherapy (angiotensin converting enzyme inhibitor or beta-blocker) can prevent trastuzumab-mediated left ventricular remodeling, measured with cardiac MRI, among patients with HER2+ early breast cancer. METHODS/DESIGN: One hundred and fifty-nine patients with histologically confirmed HER2+ breast cancer will be enrolled in a parallel 3-arm, randomized, placebo controlled, double-blind design. After baseline assessments, participants will be randomized in a 1:1:1 ratio to an angiotensin-converting enzyme inhibitor (perindopril), beta-blocker (bisoprolol), or placebo. Participants will receive drug or placebo for 1 year beginning 7 days before trastuzumab therapy. Dosages for all groups will be systematically up-titrated, as tolerated, at 1 week intervals for a total of 3 weeks. The primary objective of this randomized clinical trial is to determine if conventional heart failure pharmacotherapy can prevent trastuzumab-mediated left ventricular remodeling among patients with HER2+ early breast cancer, as measured by 12 month change in left ventricular end-diastolic volume using cardiac MRI. Secondary objectives include 1) determine the evolution of left ventricular remodeling on cardiac MRI in patients with HER2+ early breast cancer, 2) understand the mechanism of trastuzumab mediated cardiac toxicity by assessing for the presence of myocardial injury and apoptosis on serum biomarkers and cardiac MRI, and 3) correlate cardiac biomarkers of myocyte injury and extra-cellular matrix remodeling with left ventricular remodeling on cardiac MRI in patients with HER2+ early breast cancer. DISCUSSION: Cardiac toxicity as a result of cancer therapies is now recognized as a significant health problem of increasing prevalence. To our knowledge, MANTICORE will be the first randomized trial testing proven heart failure pharmacotherapy in the prevention of trastuzumab-mediated cardiotoxicity. We expect the findings of this trial to provide important evidence in the development of guidelines for preventive therapy. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01016886.
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Anticorpos Monoclonais Humanizados/efeitos adversos , Bisoprolol/uso terapêutico , Neoplasias da Mama/tratamento farmacológico , Insuficiência Cardíaca/prevenção & controle , Perindopril/uso terapêutico , Remodelação Ventricular/efeitos dos fármacos , Antagonistas de Receptores Adrenérgicos beta 1/uso terapêutico , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Apoptose , Feminino , Insuficiência Cardíaca/tratamento farmacológico , Humanos , Imageamento por Ressonância Magnética , Placebos , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Receptor ErbB-2/metabolismo , Projetos de Pesquisa , TrastuzumabRESUMO
AIMS: Anthracyclines are a cornerstone of paediatric cancer treatment. We aimed to quantify myocardial cardiac magnetic resonance (CMR) native T1 (NT1) and extracellular volume fraction (ECV) as markers of fibrosis in a cohort of childhood cancer survivors (CCS). METHODS AND RESULTS: A cohort of CCS in remission underwent CMR T1 mapping. Diastolic function was assessed by echocardiography. Results were compared to a cohort of normal controls of similar age and gender. Fifty-five CCS and 46 controls were included. Both groups had similar mean left ventricular (LV) NT1 values (999 ± 36 vs. 1007 ± 32 ms, P = 0.27); ECV was higher (25.6 ± 6.9 vs. 20.7 ± 2.4%, P = 0.003) and intracellular mass was lower (37.5 ± 8.4 vs. 43.3 ± 9.9g/m2, P = 0.02) in CCS. The CCS group had lower LV ejection fraction (EF) and LV mass index with otherwise normal diastolic function in all but one patient. The proportion of subjects with elevated ECV compared to controls did not differ between subgroups with normal or reduced LV EF (22% vs. 28%; P = 0.13) and no correlations were found between LVEF and ECV. While average values remained within normal range, mitral E/E' (6.6 ± 1.6 vs. 5.9 ± 0.9, P = 0.02) was higher in CCS. Neither NT1 nor ECV correlated with diastolic function indices or cumulative anthracycline dose. CONCLUSIONS: There is evidence for mild diffuse extracellular volume expansion in some asymptomatic CCS; myocyte loss could be part of the mechanism, accompanied by subtle changes in systolic and diastolic function. These findings suggest mild myocardial damage and remodelling after anthracycline treatment in some CCS which requires continued monitoring.