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1.
Methodist Debakey Cardiovasc J ; 15(2): 138-144, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384377

RESUMO

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/uso terapêutico , Anomalia de Ebstein/terapia , Insuficiência da Valva Tricúspide/terapia , Valva Tricúspide/efeitos dos fármacos , Valva Tricúspide/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fármacos Cardiovasculares/efeitos adversos , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Hemodinâmica/efeitos dos fármacos , Humanos , Recuperação de Função Fisiológica , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/tratamento farmacológico , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita/efeitos dos fármacos
2.
J Cardiovasc Magn Reson ; 21(1): 34, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31242903

RESUMO

BACKGROUND: Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. METHODS: A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. RESULTS: Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14-57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3-4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3-100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. CONCLUSIONS: Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Imagem Cinética por Ressonância Magnética , Procedimentos Cirúrgicos Reconstrutivos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Tomada de Decisão Clínica , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Valor Preditivo dos Testes , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Procedimentos Cirúrgicos Reconstrutivos/mortalidade , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Adulto Jovem
3.
Heart Vessels ; 34(8): 1332-1339, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30848337

RESUMO

The variable anatomy of Ebstein's anomaly leads to its various surgical procedures. The long-term outcomes of different operations were not well established. Thirty-five patients with Ebstein's anomaly who underwent operations from 2006 to 2018 in our department were retrospectively reviewed. Individualized surgical plans were performed according to the preoperative echocardiography and surgeons' preference. Tricuspid repair, either Danielson's or Carpentier's technique, was the primary choice in patients who had sufficient tricuspid leaflets and adequate right ventricle, while tricuspid replacement was used when a reliable repair is not achievable. Additional bidirectional cavopulmonary shunt was performed in those who had unstable hemodynamics despite of high central venous pressure after separation from cardiopulmonary bypass. The perioperative and follow-up data were collected. The age was 26.9 (0.6-54) years [16 children (age < 14, and 19 adults (age ≥ 14)]. Preoperative tricuspid regurgitation was severe in 30, moderate in 4, and mild in the remaining 1 patient. Preoperative cardiac-associated malformations include 20 atrial septal defects, 2 ventricular septal defects, 2 pulmonary stenosis, and 1 sub aortic ridge, and these were operated simultaneously. Among all the surgical patients, 2 needed additional reoperation during the same admission, and ultimately, 29 patients had biventricular repair, including 21 tricuspid repair and 8 replacements. The other 6 patients had cavopulmonary connection and achieved 1.5 ventricular repair (3 tricuspid repair and 3 replacements). In all the 24 tricuspid repair patients, Danielson's procedure was used in 17, while Carpentier's technique was used in the other 7 patients. The average cardiopulmonary bypass time was 90 ± 28 min and cross-clamp time was 48 ± 24min. There were 2 perioperative deaths (5.7%) and no third-degree atrioventricular block. The postoperative in hospital stay was 13.7 ± 9.6 days. In the 33 survivors who were followed up at a median of 29.2 months, 6 patients had severe tricuspid regurgitation, and 2 of them underwent tricuspid replacement. The 5-year freedom from severe tricuspid dysfunction or reoperation was 78.5%, and no difference was found between children and adults, neither between different surgical choices. The surgeries of Ebstein's anomaly were variable, and individualized operation achieved reasonable short- and mid-term results. However, severe tricuspid regurgitation during the follow-up was not neglectable, and reoperation in such cases also achieved good outcomes. New repair strategy such as cone repair may be considered.


Assuntos
Anuloplastia da Valva Cardíaca , Anomalia de Ebstein/cirurgia , Técnica de Fontan , Adolescente , Adulto , Ponte Cardiopulmonar , Criança , Pré-Escolar , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Feminino , Humanos , Masculino , Reoperação , Estudos Retrospectivos , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita , Adulto Jovem
4.
Semin Thorac Cardiovasc Surg ; 31(3): 566-568, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30735711

RESUMO

We describe a change in our technique for plication of the atrialized right ventricle during a Cone repair. The plication was performed from the "Outside"-on a beating heart. Advantages expected from this change are-all major coronaries are better seen when full of blood and can be avoided, if a major vessel is compromised-electrocardiography changes should point it out immediately, there is a small but significant saving in terms of arrest time and if the plication needs to be taken down because of an adverse coronary event-this can be done without the need of another cardioplegia.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Átrios do Coração/cirurgia , Ventrículos do Coração/cirurgia , Valva Tricúspide/cirurgia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia
5.
Pediatr Cardiol ; 40(4): 792-798, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30726509

RESUMO

BACKGROUND: Exercise capacity is a well-defined marker of outcome in congenital heart disease. We analyzed seventeen cardiovascular magnetic resonance (CMR) derived parameters and their correlation to exercise capacity in patients with Ebstein's anomaly (EA). METHODS: Fifty-four surgery free patients, age 5 to 69 years (median 30 years) prospectively underwent CMR examination and cardiopulmonary exercise testing (CPET). The following volume/flow parameters were compared with peak oxygen uptake as the percentage of normal (peakVO2%) using univariate and multivariate analysis: right and left ventricular ejection fraction (RVEF and LVEF), the indexed end-diastolic and end-systolic volumes (RVEDVi, RVESVi, LVEDVi, and LVESVi), the indexed stroke volumes (RVSVi and LVSVi), the total normalized right and left heart volumes; the total right to left heart volume ratio (R/L-ratio). The indexed antegrade flow (ante), indexed net flow (net) as well as cardiac index (CI) in the aorta (Ao) and pulmonary artery (PA) were used. RESULTS: RVEF (R2 0.2788), indexed flow PA net (R2 0.2330), and PA ante (R2 0.1912) showed the best correlation with peakVO2% (all p < 0.001) in the univariate model. Further significant correlation could also be demonstrated with CI-PA, LVEF, LVSVi, Aorta net, RVESVi, and Aorta ante. Multivariate analysis for RVEF and indexed net flow PA revealed a R2 of 0.4350. CONCLUSION: Functional CMR parameters as RVEF and LVEF and flow data of cardiac forward flow correlate to peakVO2%. Evaluation of the indexed net flow in the pulmonary artery and the overall function of the right ventricle best predicts the maximal exercise capacity in patients with EA.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Tolerância ao Exercício/fisiologia , Coração/diagnóstico por imagem , Hemodinâmica/fisiologia , Imagem Cinética por Ressonância Magnética/métodos , Adolescente , Adulto , Idoso , Biomarcadores , Criança , Pré-Escolar , Anomalia de Ebstein/fisiopatologia , Teste de Esforço/métodos , Feminino , Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Consumo de Oxigênio/fisiologia , Estudos Prospectivos , Adulto Jovem
6.
Int J Cardiol ; 278: 76-83, 2019 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-30686335

RESUMO

BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ±â€¯41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. RESULTS: Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5-83) vs 28.4 mm/m2 (IQR 17.5-47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ±â€¯7.7% vs 36.7 ±â€¯9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7-0.98) vs 0.55 (IQR 0.4-0.7); p = 0.000], a lower functional RV-longitudinal strain (-10.2 ±â€¯6.2% vs -16.2 ±â€¯7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ±â€¯13.5% vs 36.3 ±â€¯12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. CONCLUSION: Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/normas , Adolescente , Criança , Pré-Escolar , Anomalia de Ebstein/epidemiologia , Ecocardiografia/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos
8.
Catheter Cardiovasc Interv ; 93(1): E46-E48, 2019 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-30280471

RESUMO

We report the use of an Amplatzer Vascular plug for percutaneous closure of a paravalvular leak after Cone repair in a 5-year-old boy with Ebstein's anomaly. A paravalvular leak of the tricuspid valve developed gradually after Cone repair-surgery. The combination of fluoroscopy, transesophageal and transthoracic imaging during general anesthesia was necessary for correct and safe positioning of the device by percutaneous approach. The results were promising.


Assuntos
Cateterismo Cardíaco , Anuloplastia da Valva Cardíaca/efeitos adversos , Anomalia de Ebstein/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Cateterismo Cardíaco/instrumentação , Pré-Escolar , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Humanos , Masculino , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/fisiopatologia
9.
Asian Cardiovasc Thorac Ann ; 27(3): 208-209, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30080103

RESUMO

Ebstein's anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein's anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function.


Assuntos
Anormalidades Múltiplas , Anomalia de Ebstein , Ventrículos do Coração/anormalidades , Miocárdio Ventricular não Compactado Isolado , Valva Tricúspide/anormalidades , Idoso , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Sístole , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Função Ventricular Esquerda
10.
BMJ Case Rep ; 12(12)2019 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-31888914

RESUMO

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein's anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)-functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein's anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.


Assuntos
Alprostadil/uso terapêutico , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/tratamento farmacológico , Cardiopatias Congênitas/diagnóstico por imagem , Vasodilatadores/uso terapêutico , Assistência ao Convalescente , Alprostadil/administração & dosagem , Cardiomegalia/diagnóstico por imagem , Cardiomegalia/etiologia , Anormalidades Congênitas/diagnóstico por imagem , Canal Arterial/fisiopatologia , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/métodos , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Oxigênio/sangue , Gravidez , Diagnóstico Pré-Natal , Atresia Pulmonar/fisiopatologia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Resultado do Tratamento , Vasodilatadores/administração & dosagem
11.
Prenat Diagn ; 38(13): 1042-1048, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30328635

RESUMO

OBJECTIVE: Anomalous neurological development associated with congenital heart disease (CHD) has been reported as early as third trimester of fetal development. While several studies have characterized variations in CHD neurodevelopmental outcomes in early childhood, these reports are often confounded by postnatal factors such as surgical outcome. Recent studies have focused on the comparing neurological variations between fetuses with CHD and normal controls. In this work, we present a comparison of in utero variations in brain development between fetuses with different types of CHD, by analyzing them under categories of single ventricle versus biventricular cardiac anatomy. METHODS: Using recent advances in fetal magnetic resonance imaging (MRI), we quantify the volumetric trajectories of various brain tissues (such as cortical plate, developing white matter, cerebrospinal fluid [CSF], and cerebellum). RESULTS: Our study is the first to differentiate between intraventricular and extra-axial CSF thereby allowing us to better identify variations in brain composition of the fetuses. CONCLUSIONS: Overall, our findings show that while total brain volume is similar between fetuses with single and biventricular anatomy, they exhibit statistically significant disparity in brain composition.


Assuntos
Encéfalo/diagnóstico por imagem , Feto/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem , Encéfalo/anormalidades , Encéfalo/embriologia , Estudos de Casos e Controles , Cerebelo/anormalidades , Cerebelo/diagnóstico por imagem , Cerebelo/embriologia , Córtex Cerebral/anormalidades , Córtex Cerebral/diagnóstico por imagem , Córtex Cerebral/embriologia , Circulação Cerebrovascular , Dupla Via de Saída do Ventrículo Direito/complicações , Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Dupla Via de Saída do Ventrículo Direito/fisiopatologia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Feminino , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Imagem por Ressonância Magnética , Circulação Placentária , Gravidez , Segundo Trimestre da Gravidez , Terceiro Trimestre da Gravidez , Ultrassonografia Doppler , Ultrassonografia Pré-Natal , Substância Branca/anormalidades , Substância Branca/diagnóstico por imagem , Substância Branca/embriologia
12.
JACC Clin Electrophysiol ; 4(10): 1271-1288, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30336873

RESUMO

Ebstein's anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. In addition to the hemodynamic burden of the actual valve defect, Ebstein's patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory atrioventricular pathways (APs) located along the posterior and septal border of the tricuspid valve where the valve leaflets are most abnormal. It is the only congenital heart defect with such a dramatic predisposition toward APs. Although it is logical to postulate a link between the anatomic deformity and the conduction abnormality, the exact nature of this link is still not fully understood and remains a fertile area for investigation that might shed light on abnormal conduction pathways in many other forms of heart disease. Furthermore, for reasons that are only now being fully appreciated, successful catheter ablation of APs in this setting is frequently more challenging than would be expected in a structurally normal heart. This review will explore the gross and microscopic anatomy of Ebstein's anomaly with attention to features that could be important to both arrhythmogenesis and ablation therapy in this unique population.


Assuntos
Anomalia de Ebstein , Taquicardia , Valva Tricúspide , Adolescente , Adulto , Criança , Pré-Escolar , Anomalia de Ebstein/complicações , Anomalia de Ebstein/patologia , Anomalia de Ebstein/fisiopatologia , Humanos , Taquicardia/etiologia , Taquicardia/fisiopatologia , Valva Tricúspide/patologia , Valva Tricúspide/fisiopatologia , Adulto Jovem
13.
J Pak Med Assoc ; 68(9): 1391-1393, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30317272

RESUMO

Ebstein anomaly is a rare malformation of the tricuspid valve of the heart. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. Pregnant patients with Ebstein anomaly become a challenge for the physician in terms of management. We report a case in which patient delivered successfully with Ebstein anomaly. The only complication was breathlessness. Authors concluded that women with Ebstein anomaly can have successful outcomes of pregnancy with close monitoring.


Assuntos
Anomalia de Ebstein , Administração dos Cuidados ao Paciente/métodos , Complicações Cardiovasculares na Gravidez , Valva Tricúspide , Adulto , Dispneia/diagnóstico , Dispneia/etiologia , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/métodos , Feminino , Monitorização Fetal/métodos , Humanos , Monitorização Fisiológica/métodos , Paquistão , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/fisiopatologia , Resultado da Gravidez , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Ultrassonografia
14.
J Cardiovasc Magn Reson ; 20(1): 69, 2018 09 27.
Artigo em Inglês | MEDLINE | ID: mdl-30257686

RESUMO

BACKGROUND: Myocardial fibrosis is a common pathophysiological process that is related to ventricular remodeling in congenital heart disease. However, the presence, characteristics, and clinical significance of myocardial fibrosis in Ebstein's anomaly have not been fully investigated. This study aimed to evaluate myocardial fibrosis using cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and T1 mapping techniques, and to explore the significance of myocardial fibrosis in adolescent and adult patients with Ebstein's anomaly. METHODS: Forty-four consecutive patients with unrepaired Ebstein's anomaly (34.0 ± 16.2 years; 18 males), and an equal number of age- and gender-matched controls, were included. A comprehensive CMR protocol consisted of cine, LGE, and T1 mapping by modified Look-Locker inversion recovery (MOLLI) sequences were performed. Ventricular functional parameters, native T1, extracellular volume (ECV), and LGE were analyzed. Associations between myocardial fibrosis and disease severity, ventricular function, and NYHA classification were analyzed. RESULTS: LGE was found in 10 (22.7%) patients. Typical LGE in Ebstein's anomaly was located in the endocardium of the septum within the right ventricle (RV). The LV ECV of Ebstein's anomaly were significantly higher than those of the controls (30.0 ± 3.8% vs. 25.3 ± 2.3%, P < 0.001). An increased ECV was found to be independent of the existence of LGE. Positive LGE or higher ECV (≥30%) was associated with larger fRV volume, aRV volume, increased disease severity, and worse NYHA functional class. In addition, ECV was significantly correlated with the LV ejection fraction (P <  0.001). CONCLUSIONS: Both focal and diffuse myocardial fibrosis were observed in adolescent and adult patients with Ebstein's anomaly. Increased diffuse fibrosis is associated with worse LV function, increased Ebstein's severity, and worse clinical status.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Miocárdio/patologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Remodelação Ventricular , Adolescente , Adulto , Fatores Etários , Estudos de Casos e Controles , Criança , China/epidemiologia , Meios de Contraste/administração & dosagem , Estudos Transversais , Progressão da Doença , Anomalia de Ebstein/epidemiologia , Anomalia de Ebstein/patologia , Anomalia de Ebstein/fisiopatologia , Feminino , Fibrose , Gadolínio DTPA/administração & dosagem , Humanos , Masculino , Valor Preditivo dos Testes , Prevalência , Prognóstico , Sistema de Registros , Fatores de Risco , Índice de Gravidade de Doença , Volume Sistólico , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/patologia , Disfunção Ventricular Esquerda/fisiopatologia , Adulto Jovem
16.
J Electrocardiol ; 51(4): 663-666, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29997008

RESUMO

Ebstein's anomaly is often accompanied by either Wolff-Parkinson-White syndrome or atriofascicular Mahaim. These bypass tracts give rise to antidromic atrioventricular (AV) re-entrant tachycardias, in which the bypass tract serves as the anterograde limb of the circuit and the AV node as the retrograde limb of the reentrant circuit. Since the antidromic AV reentrant tachycardia over a Mahaim fibre has a typically left bundle braunch block (LBBB) morphology, it is easy to make a misdiagnosis of supraventricular tachycardia with functional LBBB or even of ventricular tachycardia particularly in the presence of negative concordance. Some electrocardiographic clues might prevent misdiagnosis of ventricular tachycardia and inadvertent ICD implantation.


Assuntos
Anomalia de Ebstein/fisiopatologia , Eletrocardiografia , Pré-Excitação Tipo Mahaim/diagnóstico , Taquicardia por Reentrada no Nó Atrioventricular/diagnóstico , Desfibriladores Implantáveis , Diagnóstico Diferencial , Erros de Diagnóstico , Anomalia de Ebstein/complicações , Anomalia de Ebstein/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Pré-Excitação Tipo Mahaim/etiologia , Taquicardia por Reentrada no Nó Atrioventricular/terapia
18.
J Cardiovasc Magn Reson ; 20(1): 32, 2018 05 21.
Artigo em Inglês | MEDLINE | ID: mdl-29783986

RESUMO

BACKGROUND: In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. METHODS: All Ebstein anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs. RESULTS: Twenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p < 0.001), RV end-diastolic volume (EDV) (242 ± 110 ml/m2 vs. 137 ± 82 ml/m2, p < 0.001), and RV stroke volume (SV) (101 ± 35 vs. 51 ± 7 ml/m2, p < 0.001). RV EF was unchanged. Conversely, there was an increase in both LV EDV (68 ± 13 vs. 85 ± 13 ml/m2, p < 0.001) and LV stroke volume (37 ± 8 vs. 48 ± 6 ml/m2, p < 0.001). There was no change in LV EF, or global circumferential and longitudinal strain but basal septal circumferential strain improved (16 ± 7% vs. 22 ± 5%, p = 0.04). LV contraction become more synchronous (dyssynchrony index: 32 ± 17 vs. 21 ± 9 msec, p = 0.02), and the extent correlated with the reduction in RV EDV and TR. CONCLUSIONS: In patients with the Ebstein anomaly, the cone operation led to reduced TR and RV stroke volume, increased LV stroke volume, improved LV basal septal strain, and improved LV synchrony. Our data demonstrates that the detrimental effect of the RV on LV function can be mitigated by the cone operation.


Assuntos
Anomalia de Ebstein/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Contração Miocárdica , Valva Tricúspide/cirurgia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos , Criança , Pré-Escolar , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Direita , Adulto Jovem
20.
Int J Cardiol ; 257: 54-61, 2018 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-29402525

RESUMO

PURPOSE: We aimed to quantify atrial and ventricular myocardial deformation in Ebstein's Anomaly (EA) in a case-control study with cardiovascular magnetic resonance (CMR) feature tracking and to correlate changes in cardiac performance with the severity of disease and clinical heart failure parameters. MATERIALS AND METHODS: Atrial and ventricular deformation was measured using CMR feature tracking in 30 EA and 20 healthy control subjects. Atrial performance was characterized using longitudinal strain and strain rate parameters for reservoir function, conduit function and booster pump function. Ventricular performance was characterized using RV and LV global longitudinal strain (εl) and LV circumferential and radial strain (εc and εr). Volumetric measurements for the ventricles including the Total Right/Left-Volume-Index (R/L-Volume-Index) and heart failure markers (BNP, NYHA class) were also quantified. RESULTS: EA patients showed significantly impaired right atrial performance, which correlated with heart failure markers (NYHA, BNP, R/L-Volume-Index). LA function in EA patients was also impaired with atrial contractile function correlating with NYHA class. EA patients exhibited impaired RV myocardial deformation, also with a significant correlation with heart failure markers. CONCLUSION: CMR feature tracking can be used to quantify ventricular and atrial function in a complex cardiac malformation such as EA. EA is characterized by impaired quantitative right heart atrio-ventricular deformation, which is associated with heart failure severity. While LV function remains preserved, there is also significant impairment of LA function. These quantitative performance parameters may represent early markers of cardiac deterioration of potential value in the clinical management of EA.


Assuntos
Função do Átrio Direito/fisiologia , Anomalia de Ebstein/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética/métodos , Função Ventricular Direita/fisiologia , Adulto , Estudos de Casos e Controles , Estudos Transversais , Anomalia de Ebstein/epidemiologia , Anomalia de Ebstein/fisiopatologia , Feminino , Insuficiência Cardíaca/epidemiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Masculino , Estudos Prospectivos , Adulto Jovem
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