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1.
Pediatr Cardiol ; 42(3): 643-653, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33533966

RESUMO

Neurodevelopmental sequelae are prevalent among patients with congenital heart defects (CHD). In a study of infants and children with repaired tetralogy of Fallot (TOF), we sought to identify those at risk for abnormal neurodevelopment and to test associations between socioeconomic and medical factors with neurodevelopment deficits. Single-center retrospective observational study of patients with repaired TOF that were evaluated at the institution's Cardiac Kids Developmental Follow-up Program (CKDP) between 2012 and 2018. Main outcomes included neurodevelopmental test scores from the Bayley Infant Neurodevelopmental Screener (BINS), Peabody Developmental Motor Scale (PDMS), and Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Mixed effects linear regression and marginal logistic regression models tested relationships between patient characteristics and outcomes. Sub-analyses were conducted to test correlations between initial and later neurodevelopment tests. In total, 49 patients were included, predominantly male (n = 33) and white (n = 28), first evaluated at a median age of 4.5 months. Forty-three percent of patients (n = 16) had deficits in the BINS, the earliest screening test. Several socioeconomic parameters and measures of disease complexity were associated with neurodevelopment, independently of genetic syndrome. Early BINS and PDMS performed in infancy were associated with Bayley-III scores performed after 1 year of age. Early screening identifies TOF patients at risk for abnormal neurodevelopment. Socioeconomic factors and disease complexity are associated with abnormal neurodevelopment and should be taken into account in the risk stratification and follow-up of these patients. Early evaluation with BINS and PDMS is suggested for detection of early deficits.


Assuntos
Transtornos do Neurodesenvolvimento/diagnóstico , Fatores Socioeconômicos , Tetralogia de Fallot/complicações , Desenvolvimento Infantil , Feminino , Humanos , Lactente , Recém-Nascido , Modelos Lineares , Masculino , Estudos Retrospectivos
2.
Cardiovasc Pathol ; 50: 107295, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33002584

RESUMO

Congenital ventricular diverticulum is a rare anomaly with an unclear pathology. Here, we report a male fetus at 24 weeks of gestation, diagnosed with right ventricular diverticulum associated with tetralogy of Fallot and absent pulmonary valve. The diverticulum was located at the anterosuperior wall of the right ventricle and faced into the massive pulmonary regurgitation flow jet. Intrauterine fetal death from heart failure resulted at 26 weeks of gestation. An autopsy revealed significant subendocardial fibrosis in the diverticular wall without inflammatory cell infiltration. Clinical and pathologic features suggested that the regurgitative blood flow contributed to the formation of the right ventricular diverticulum.


Assuntos
Divertículo/congênito , Coração Fetal/anormalidades , Ventrículos do Coração/anormalidades , Hemodinâmica , Circulação Pulmonar , Valva Pulmonar/anormalidades , Tetralogia de Fallot/complicações , Autopsia , Divertículo/diagnóstico por imagem , Divertículo/fisiopatologia , Ecocardiografia Doppler em Cores , Evolução Fatal , Morte Fetal/etiologia , Coração Fetal/diagnóstico por imagem , Coração Fetal/fisiopatologia , Idade Gestacional , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Ultrassonografia Pré-Natal
4.
Am J Cardiol ; 132: 126-132, 2020 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-32778337

RESUMO

Risk stratification for malignant arrhythmias and risk of sudden cardiac death in tetralogy of Fallot (TOF) remains challenging. We aimed to ascertain factors associated with life-threatening arrhythmic events. A multicenter retrospective case-control study including 72 TOF patients with documented cardiac arrest and/or sustained ventricular tachycardia, compared with 216 controls matched for era of surgery. The mean age at event in the cases was 27.3 ± 12.5 years. The majority (57%) presented with sustained ventricular tachycardia. Fatal events occurred in 9. Random forest analysis and a decision tree demonstrated surgical era specific risk factors (< vs ≥ 1980). For both eras, arrhythmic symptoms and left ventricular dysfunction were strongly associated with malignant arrhythmias. In addition, right ventricular dysfunction and age at repair ≥ 6.5 years preceded by a shunt were associated with a higher risk group in the early era, whereas a trans-annular patch type repair was associated with a lower risk group in the recent era. For the moderate and high-risk groups, the decision tree showed a sensitivity of 88.4% and specificity of 68.1%. An "importance factor" was calculated for each predictor, creating a risk score and 4 risk categories. In conclusions, this risk stratification scheme, based on clinical history and noninvasive testing, allows categorization of TOF patients at high risk of malignant arrhythmia. A multicenter prospective evaluation of the accuracy of this scoring system is now being planned.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Morte Súbita Cardíaca/etiologia , Medição de Risco/métodos , Taquicardia Ventricular/etiologia , Tetralogia de Fallot/complicações , Adulto , Canadá/epidemiologia , Estudos de Casos e Controles , Morte Súbita Cardíaca/epidemiologia , Feminino , Humanos , Incidência , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Taquicardia Ventricular/epidemiologia , Tetralogia de Fallot/cirurgia , Estados Unidos/epidemiologia
5.
Pediatr Cardiol ; 41(7): 1532-1537, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32813029

RESUMO

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our aim is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) cardiac resynchronization in children with symptomatic ventricular dysfunction and dyssynchrony which seems to result in higher transplant-free survival, (2) outcomes of aortic leaflet reconstruction including Ozaki procedure to repair aortic valve disease in adolescents, (3) meta-analysis for risk factors of ventricular tachycardia and death after repaired tetralogy of Fallot which reiterates the known risk factors and showed that the severity of pulmonary regurgitation is not in itself associated with outcomes although the ventricular response to regurgitation (dilation and dysfunction) is, (4) preschool promotion of healthy life style did not associate with sustained effect when evaluated later in childhood although repeated intervention seems to have a dose-related effect to promote healthy life style, (5) the lack of beneficial effects of angiotensin-converting enzyme inhibitors in the interstage period, and (6) a new phenomenon of acute heart failure and multisystem inflammatory syndrome in children temporarily related to the COVID-19 pandemic.


Assuntos
Cardiopatias/complicações , Cardiopatias/terapia , Adolescente , Betacoronavirus , Terapia de Ressincronização Cardíaca/métodos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Pré-Escolar , Infecções por Coronavirus/complicações , Cardiopatias Congênitas/terapia , Cardiopatias/cirurgia , Insuficiência Cardíaca/complicações , Ventrículos do Coração/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/tratamento farmacológico , Pandemias , Pneumonia Viral/complicações , Fatores de Risco , Síndrome de Resposta Inflamatória Sistêmica/complicações , Taquicardia Ventricular/complicações , Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia
7.
Rev. chil. cardiol ; 39(2): 165-167, ago. 2020. graf
Artigo em Espanhol | LILACS | ID: biblio-1138530

RESUMO

Abstract: Right ventricular restrictive physiology (RVRP) occurs in diverse clinical scenarios, most frequently after repair of Tetralogy of Fallot (TOF). Cardiac magnetic resonance (CMR) can comprehensively evaluate RVRP using 4D flow along with anatomical and fibrosis characterization. Also, RVRP is associated with less pulmonary regurgitation and fewer right ventricle enlargement; its long term protective role is debated. RVRP is a challenging and relevant diagnosis, which hallmark is the presence of antegrade pulmonary arterial Flow in late diastole throughout the respiratory cycle. Also, other hemodynamic findings could aid such us flow in; caval veins, suprahepatic, coronary sinus and tricuspid valve. Obtaining all these flow curves is virtually impossible by echocardiography. CMR with 4DF is a unique and powerful technique enabling this comprehensive hemodynamic evaluation as depicted in this case.


Assuntos
Humanos , Imagem por Ressonância Magnética , Disfunção Ventricular Direita/diagnóstico por imagem , Imageamento Tridimensional/métodos , Artéria Pulmonar/patologia , Fluxo Sanguíneo Regional , Tetralogia de Fallot/complicações , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia , Hemodinâmica
8.
J Cardiovasc Magn Reson ; 22(1): 52, 2020 07 16.
Artigo em Inglês | MEDLINE | ID: mdl-32669114

RESUMO

BACKGROUND: Maternal right ventricular (RV) dysfunction (measured by echocardiography) is associated with impaired uteroplacental circulation, however echocardiography has important limitations in the assessment of RV function. We therefore aimed to investigate the association of pre-pregnancy RV and left ventricular (LV) function measured by cardiovascular magnetic resonance with uteroplacental Doppler flow parameters in pregnant women with repaired Tetralogy of Fallot (ToF). METHODS: Women with repaired ToF were examined, who had been enrolled in a prospective multicenter study of pregnant women with congenital heart disease. Clinical data and CMR evaluation before pregnancy were compared with uteroplacental Doppler parameters at 20 and 32 weeks gestation. In particular, pulsatility index (PI) of uterine and umbilical artery were studied. RESULTS: We studied 31 women; mean age 30 years, operated at early age. Univariable analyses showed that reduced RV ejection fraction (RVEF; P = 0.037 and P = 0.001), higher RV end-systolic volume (P = 0.004) and higher LV end-diastolic and end-systolic volume (P = 0.001 and P = 0.003, respectively) were associated with higher uterine or umbilical artery PI. With multivariable analyses (corrected for maternal age and body mass index), reduced RVEF before pregnancy remained associated with higher umbilical artery PI at 32 weeks (P = 0.002). RVEF was lower in women with high PI compared to women with normal PI during pregnancy (44% vs. 53%, p = 0.022). LV ejection fraction was not associated with uterine or umbilical artery PI. CONCLUSIONS: Reduced RV function before pregnancy is associated with abnormal uteroplacental Doppler flow parameters. It could be postulated that reduced RV function on pre-pregnancy CMR (≤2 years) is a predisposing factor for impaired placental function in women with repaired ToF.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Imagem Cinética por Ressonância Magnética , Circulação Placentária , Tetralogia de Fallot/cirurgia , Artérias Umbilicais/fisiopatologia , Artéria Uterina/fisiopatologia , Disfunção Ventricular Direita/diagnóstico por imagem , Função Ventricular Direita , Adulto , Velocidade do Fluxo Sanguíneo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Feminino , Humanos , Valor Preditivo dos Testes , Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos , Fatores de Risco , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Ultrassonografia Doppler , Artérias Umbilicais/diagnóstico por imagem , Artéria Uterina/diagnóstico por imagem , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia
9.
J Card Surg ; 35(8): 2025-2026, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32557816

RESUMO

We hereby describe a rare case of partial anomalous pulmonary venous connection (PAPVC) in a patient with tetralogy of Fallot (TOF). It is imperative to identify PAPVC preoperatively in patients with TOF as it can have significant hemodynamic outcomes. This case highlights the importance of computed tomography angiography in demonstrating the same.


Assuntos
Síndrome de Cimitarra/diagnóstico por imagem , Anormalidades Múltiplas , Procedimentos Cirúrgicos Cardiovasculares , Criança , Angiografia por Tomografia Computadorizada , Feminino , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico por imagem , Humanos , Período Pré-Operatório , Síndrome de Cimitarra/complicações , Síndrome de Cimitarra/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia
12.
Interact Cardiovasc Thorac Surg ; 30(5): 780-782, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-32298427

RESUMO

Ventricular tachyarrhythmia (VT) is a major cause of late morbidity and mortality in patients who underwent surgical repair of tetralogy of Fallot. The majority of VTs are monomorphic macro-reentrant VT (MVT) and depend on slow conducting areas of diseased myocardium bordered by unexcitable tissue (anatomical isthmuses). Myocardial fibrosis due to surgical incisions, patch material and valve annuli are typical boundaries of anatomical isthmuses (AI). The conducting myocardium between the pulmonary valve and ventricular septum defect patch is called isthmus 3, and the majority of MVTs originate from this area. During pulmonary valve replacement, there is excellent exposure of isthmus 3. Importantly, after pulmonary valve replacement, the homograft may cover important parts of isthmus 3, which makes percutaneous catheter ablation at a later stage impossible. In all patients who need pulmonary valve replacement late after tetralogy of Fallot repair, preoperative electrophysiology study and electroanatomical mapping can identify patients with inducible MVT or slow conduction carrying high risk of MVT. In these patients, intraoperative cryoablation of isthmus 3 should be performed and bidirectional conduction block across the cryoablation line should be demonstrated by intraoperative differential pacing.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criocirurgia , Valva Pulmonar/cirurgia , Taquicardia Ventricular/prevenção & controle , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Ablação por Cateter/efeitos adversos , Criança , Pré-Escolar , Feminino , Comunicação Interventricular/complicações , Comunicação Interventricular/cirurgia , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Taquicardia Ventricular/etiologia , Tetralogia de Fallot/complicações , Adulto Jovem
13.
Ann Vasc Surg ; 68: 568.e7-568.e10, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32278868

RESUMO

Pulmonary artery dissection is a rare and extremely dangerous disease with high mortality rates. It is one of the most serious complications of chronic pulmonary hypertension. It may be related to chronic pulmonary hypertension and pulmonary artery dilatation. Early diagnosis of pulmonary dissection is particularly important because of its high mortality. Once the symptoms worsen or severe deterioration of the disease occurs, imaging examination should be performed promptly for early diagnosis and timely treatment.


Assuntos
Aneurisma Dissecante/complicações , Artéria Pulmonar , Tetralogia de Fallot/complicações , Aneurisma Dissecante/diagnóstico por imagem , Aneurisma Dissecante/fisiopatologia , Evolução Fatal , Parada Cardíaca/etiologia , Hemodinâmica , Humanos , Masculino , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Adulto Jovem
14.
Pediatr Cardiol ; 41(5): 918-924, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32112115

RESUMO

22q11.2 deletion syndrome leads to both cardiac and non-cardiac developmental defects. We aimed to study the impact of 22q11.2 deletion syndrome on in-hospital outcomes in children undergoing surgical repair for tetralogy of Fallot (TOF) and truncus arteriosus (TA). Using the nationally representative Kids Inpatient Database (KID), we analyzed data from in-hospital pediatric patients for the years 2003, 2006, 2009, and 2012. We compared the in-hospital outcomes between those with and those without 22q11.2 deletion syndrome. There were 6126 cases of TOF and 968 cases of TA. 22q11.2 deletion syndrome were documented in 7.2% (n = 441) of the TOF and 27.4% (n  =  265) of the TA group. 22q11.2 deletion did not significantly increase the risk of mortality in either group: [OR = 1.98 (95% CI 0.99-3.94), adjusted p  =  0.053] for TOF and OR = 1.07 (95% CI 0.57-1.99), adjusted p = 0.82 for TA. However, the length of hospitalization was longer in the 22q11.2 deletion group by 8.6 days (95% CI 5.2-12), adjusted p < 0.001 for TOF and by 8.15 days (95% CI 1.05-15.25), adjusted p = 0.025 for the TA group. Acute respiratory failure [10.6% vs 5.5%, p < 0.001] and acute renal failure [6.3% vs 2.6%, p < 0.001] were higher in 22q11.2 deletion cohort within the TOF group but not in the TA group. Though survival is not affected, children with 22q11.2 deletion syndrome who undergo surgical repair for TOF and TA use significantly more hospital resources-specifically longer hospital stay and higher hospitalization cost-than those without 22q11.2 deletion syndrome. Prenatal or preoperative testing for 22q11deletion is indicated to make appropriate adjustments in parental, caregiver, and administrative expectations.


Assuntos
Síndrome de DiGeorge/complicações , Tetralogia de Fallot/cirurgia , Persistência do Tronco Arterial/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Deleção Cromossômica , Síndrome de DiGeorge/economia , Síndrome de DiGeorge/genética , Síndrome de DiGeorge/mortalidade , Feminino , Custos Hospitalares , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Complicações Pós-Operatórias/economia , Complicações Pós-Operatórias/epidemiologia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/mortalidade , Resultado do Tratamento , Tronco Arterial/cirurgia , Persistência do Tronco Arterial/complicações , Persistência do Tronco Arterial/mortalidade
15.
Pediatr Cardiol ; 41(5): 862-868, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32095853

RESUMO

Sudden cardiac death (SCD) is the most common cause of late mortality in tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) was previously found to be the most common hemodynamic abnormality associated with ventricular arrhythmias (VA), but cardiovascular magnetic resonance (CMR)-based studies did not show this association. The aim of this study is to investigate the risk factors for VA in TOF using CMR. Electronic records of TOF patients and their CMR studies between July 2006 and October 2018 in one center were retrospectively reviewed. Demographic, clinical and CMR data of patients were collected. Outcome was defined as sustained ventricular tachycardia (VT), aborted SCD and SCD. From a total of 434 TOF patients with complete CMR studies, 19 (4.4%) patients developed a positive outcome (12 sustained VT, 4 aborted SCD, 3 SCD) at a median age of 24 years. The number of surgical interventions was significantly greater in patients who developed VA. Right ventricular volumes were significantly larger in patients who suffered a positive outcome. Odds ratio for developing VA was 6.905 for RVEDVI ≥ 160 ml/m2 and 6.141 for RVESVI ≥ 80 ml/m2 (P = 0.0014 and 0.0012, respectively). Event-free survival was longer in patients with smaller right ventricular volumes. In conclusion, right ventricular dimensions are the most significant factors associated with the development of VA in TOF. The number of surgical interventions is also related to an increased risk.


Assuntos
Arritmias Cardíacas/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Arritmias Cardíacas/complicações , Arritmias Cardíacas/mortalidade , Procedimentos Cirúrgicos Cardíacos/métodos , Morte Súbita Cardíaca/etiologia , Feminino , Ventrículos do Coração/patologia , Hemodinâmica , Humanos , Masculino , Insuficiência da Valva Pulmonar/etiologia , Estudos Retrospectivos , Fatores de Risco , Taquicardia Ventricular/complicações , Taquicardia Ventricular/diagnóstico , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/mortalidade , Adulto Jovem
17.
J Thorac Cardiovasc Surg ; 159(4): 1466-1476.e2, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31926718

RESUMO

OBJECTIVES: Optimal management of tetralogy of Fallot in symptomatic neonates and young infants remains controversial. METHODS: A retrospective review of 53 patients (26 male) with symptomatic tetralogy of Fallot who underwent primary repair (group 1, n = 22) or initial palliation (group 2, n = 31) within 2 months (60 days) after birth between 2005 and 2018 was performed. Subsequent repairs were performed in 29 patients at postpalliation 7.1 months in group 2 except for 2 interstage mortalities. Optimal early outcome was defined as no significant pulmonary stenosis or significant pulmonary regurgitation, and no reintervention within 12 months after repair. RESULTS: In group 2, median Z-score of the pulmonary valve annulus and McGoon ratio increased after palliation from -3.52 to -2.95 (P = .074) and from 1.31 to 1.93 (P < .001), respectively. Pulmonary annulus preservation at repair and optimal early outcome were achieved in 38 patients (17/22, 77%, group 1; 21/29, 72%, group 2) and 26 patients (12/22, 55%, group 1; 14/29, 48%, group 2), respectively. On logistic regression analysis, initial Z-score of the pulmonary valve annulus was the only predictor of annulus preservation at repair (odds ratio, 1.715, P = .0204) and optimal early outcome (odds ratio, 1.583, P = .0259). The annulus preservation probability curve according to the initial postnatal Z-score of the pulmonary valve annulus of all patients with repair (n = 51) showed an annulus preservation probability less than 70% in 3 patients (3/22) in group 1 and greater than 85% in 8 patients (8/29) in group 2, signifying that the alternative strategy might have been beneficial for each subset. CONCLUSIONS: The surgical strategy for symptomatic tetralogy of Fallot should be individualized according to the initial size of the pulmonary valve annulus.


Assuntos
Tetralogia de Fallot/complicações , Tetralogia de Fallot/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Modelos Logísticos , Masculino , Cuidados Paliativos , Reoperação , Estudos Retrospectivos , Taxa de Sobrevida , Tetralogia de Fallot/mortalidade , Resultado do Tratamento
18.
Int J Cardiovasc Imaging ; 36(4): 691-700, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31907684

RESUMO

Aortopathy is a recognized comorbidity of Tetralogy of Fallot (TOF). Aortic flow in children with repaired TOF is abnormal despite normal aortic valve anatomy and early surgical repair that results in aortic size normalization. The purpose of this study was to investigate the flow hemodynamics inside the left ventricle (LV) of children with repaired TOF using 4D-Flow MRI derived vorticity. Vorticity is the spatial derivative of flow velocity and is sensitive to anatomic and geometric variations. Vorticity was calculated inside the LV of children with repaired TOF having normal aortic size (n = 14) and normal controls (n = 10) during systolic ejection phase. All subjects underwent comprehensive biventricular analysis including the MRI based feature-tracking based LV strain analysis and mechanical dyssynchrony. Right ventricular (RV) volumetric indices along with LV mechanical indices were correlated with LV vorticity. All TOF patients had supraphysiologic helical flow in the ascending aorta. The generated peak systolic vorticity integrated over the LV volume was elevated in TOF group compared to control (median: 1344 vs. 858 s-1, P < 0.001). TOF patients had increased LV mechanical dyssynchrony (47 ± 11 vs. 32 ± 7 ms, P < 0.001) and reduced LV global circumferential strain (19 ± 2 vs. 21 ± 2%, P = 0.020). In the TOF group, LV systolic vorticity was independent of RV size and LV mechanical indices. Pathologic aortic flow in children with repaired TOF is associated with abnormal ejection flow patterns inside the LV. Increased systolic vorticity was not associated with LV mechanical dyssynchrony and RV dilation, suggesting that systolic flow inside the LV is independent of impaired LV contractile mechanics and inter-ventricular interactions.


Assuntos
Aorta/fisiopatologia , Doenças da Aorta/fisiopatologia , Procedimentos Cirúrgicos Cardíacos , Hemodinâmica , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Adolescente , Aorta/diagnóstico por imagem , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/etiologia , Velocidade do Fluxo Sanguíneo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Criança , Feminino , Humanos , Hipertrofia Ventricular Direita/diagnóstico por imagem , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/fisiopatologia , Imagem Cinética por Ressonância Magnética , Masculino , Imagem de Perfusão do Miocárdio/métodos , Estudos Retrospectivos , Fatores de Risco , Sístole , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Direita , Remodelação Ventricular
19.
World J Pediatr Congenit Heart Surg ; 11(1): 130-132, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31835984

RESUMO

Coexistence of asymptomatic balanced double aortic arch with tetralogy of Fallot (TOF) is extremely rare and represents a surgical dilemma in decision-making due to the lack of consensus on the management of this subset of patients. We report a case of asymptomatic balanced double aortic arch coexistent with TOF in a two-year-old girl.


Assuntos
Aorta Torácica/anormalidades , Tetralogia de Fallot/diagnóstico , Anel Vascular/diagnóstico , Aorta Torácica/cirurgia , Pré-Escolar , Diagnóstico Diferencial , Ecocardiografia Doppler , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos , Tetralogia de Fallot/complicações , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Tomografia Computadorizada por Raios X , Anel Vascular/complicações , Anel Vascular/diagnóstico por imagem , Anel Vascular/cirurgia
20.
Pediatr Cardiol ; 41(2): 389-397, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31853582

RESUMO

We propose a novel method to quantify pulsatile liver deformation using the feature tracking method of cardiac cine magnetic resonance imaging (MRI) and investigate its association with liver dysfunction in long-term postoperative patients after Fontan and intracardiac repair for the tetralogy of Fallot (TOF). Standard cine MRI which was previously performed for cardiac evaluation of 85 patients who underwent Fontan operation (mean age, 22.9 years), 43 patients with TOF (mean age, 34.6 years), and 32 healthy controls (mean age, 42.3 years) were retrospectively analyzed. Pulsatile liver deformation in the craniocaudal direction was calculated using the feature tracking method of cardiac cine imaging derived from cine-balanced turbo field-echo sequences performed on a 1.5 Tesla MR scanner, and was defined as liver strain. The liver strain was compared across the three patient groups using one-way analysis of variance. Liver dysfunction by a liver strain were compared using the Mann-Whitney U test. Liver strain for patients who underwent Fontan operation and TOF patients was significantly lower than controls (Fontan, 13.3 ± 6.5%; TOF, 15.0 ± 11.2%; controls, 23.1 ± 10.2%, p < 0.0001). In Fontan and TOF patients, MELD score was significantly greater for patients with a liver strain < 15% than those with values > 15% (5.9 ± 5.8 vs. 2.9 ± 2.9, p < 0.001). Lower liver strain values were found in adolescent and adult patients after Fontan operation and TOF, and correlates with the severity of liver injury, expressed as MELD score. Our method can evaluate hepatic function in adult congenital heart disease, together with the assessment of cardiac function.


Assuntos
Técnica de Fontan/efeitos adversos , Hepatopatias/diagnóstico por imagem , Tetralogia de Fallot/complicações , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Feminino , Humanos , Hepatopatias/etiologia , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
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