Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 373
Filtrar
1.
J Am Coll Cardiol ; 76(25): 2968-2976, 2020 12 22.
Artigo em Inglês | MEDLINE | ID: mdl-33334426

RESUMO

BACKGROUND: Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population. OBJECTIVES: The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors. METHODS: This was a retrospective study of adults with Ebstein anomaly who received care at Mayo Clinic from 2003 to 2018. RESULTS: Of 692 patients, the median MELD-XI score was 10.2 (interquartile range: 9.4 to 13.3); 53 (8%) died and 3 (0.4%) underwent heart transplant. MELD-XI was an independent predictor of death/transplant (hazard ratio: 1.32; 95% confidence interval: 1.11 to 2.06; p < 0.001). In the subset of patients with serial MELD-XI scores (n = 416), temporal change in MELD-XI score (ΔMELD-XI) was also a predictor of death/transplant. In the subset of patients who underwent tricuspid valve surgery (n = 344), a post-operative improvement in MELD-XI score (ΔMELD-XI) was associated with improved long-term survival. Impaired right atrial (RA) reservoir strain and elevated estimated RA pressure were associated with worse baseline MELD-XI and ΔMELD-XI scores. CONCLUSIONS: Hepatorenal dysfunction is a predictor of mortality in Ebstein anomaly, and RA dysfunction and hypertension are hemodynamic biomarkers that can identify patients at risk for deterioration in hepatorenal function and mortality. These data highlight the prognostic importance of noncardiac organ-system dysfunction, and provide complementary clinical risk stratification metrics for management of these patients.


Assuntos
Anomalia de Ebstein , Transplante de Coração , Insuficiência Hepática , Testes de Função Renal/métodos , Testes de Função Hepática/métodos , Insuficiência Renal , Insuficiência da Valva Tricúspide , Adulto , Anomalia de Ebstein/complicações , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Anomalia de Ebstein/cirurgia , Feminino , Transplante de Coração/métodos , Transplante de Coração/estatística & dados numéricos , Insuficiência Hepática/diagnóstico , Insuficiência Hepática/etiologia , Insuficiência Hepática/fisiopatologia , Humanos , Coeficiente Internacional Normatizado , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Insuficiência Renal/diagnóstico , Insuficiência Renal/etiologia , Insuficiência Renal/fisiopatologia , Fatores de Risco , Índice de Gravidade de Doença , Insuficiência da Valva Tricúspide/diagnóstico , Insuficiência da Valva Tricúspide/epidemiologia , Insuficiência da Valva Tricúspide/etiologia , Estados Unidos/epidemiologia
3.
Heart Lung Circ ; 29(1): e17-e18, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31547962

RESUMO

Patients with Fontan circulation post significant technical challenges at the time of permanent pacemaker implantation, as majority of them are treated with surgically implanted epicardial pacemakers. However, transvenous pacemaker implantation is technically feasible, and the treating physician should be aware of each individual's anatomy and available treatment options.


Assuntos
Estimulação Cardíaca Artificial , Anomalia de Ebstein , Técnica de Fontan , Marca-Passo Artificial , Adulto , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Anomalia de Ebstein/terapia , Feminino , Humanos
4.
Artigo em Inglês | MEDLINE | ID: mdl-33471450

RESUMO

We present a 52-year-old woman with Ebstein's anomaly not previously treated. In this subset of patients, there are no clear guidelines regarding the best surgical strategy for treating the tricuspid valve: replace it or repair it.  In this case, extensive repair of the tricuspid valve and the right ventricle is achieved using the cone repair technique popularized by Dr. José Pedro Da Silva. Because the patient also presented with symptomatic paroxysmal atrial fibrillation, a right atrial maze procedure combined with isolation of the pulmonary veins was performed using both radiofrequency and cryotherapy. At the last follow-up, 2 years after the repair, the patient is asymptomatic and maintains sinus rhythm. The last echocardiogram showed mild tricuspid regurgitation with normal right ventricular function.


Assuntos
Fibrilação Atrial/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Ablação por Cateter/métodos , Anomalia de Ebstein/cirurgia , Apêndice Atrial/cirurgia , Fibrilação Atrial/complicações , Fibrilação Atrial/fisiopatologia , Anomalia de Ebstein/complicações , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Feminino , Humanos , Pessoa de Meia-Idade , Veias Pulmonares/cirurgia , Função Ventricular Direita
5.
Can J Cardiol ; 35(12): 1834-1841, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31711824

RESUMO

BACKGROUND: The severity score of Ebstein anomaly (EA) that corresponds to clinical status is still under research, with the Celermajer index (Cel-ind) being one of those. The agreement between echocardiographic and cardiac magnetic resonance (CMR) assessment of Cel-ind is not known. We determined the agreement between echocardiography- and CMR-derived Cel-ind and its relationship with heart failure markers. METHODS: A total of 37 unoperated adults with EA (mean age, 43.0 ± 14.4 years) underwent echocardiography, CMR, and cardiopulmonary tests. For the Cel-ind, end-diastolic areas in echocardiography or end-diastolic volumes in CMR were used according to the following formula: Cel-ind = (right atrium + atrialized right ventricle)/(functional right ventricle + left atrium + left ventricle). On the basis of this assumption, patients were classified as follows: grade 1 = Cel-ind < 0.5, grade 2 = 0.5 to 0.99, grade 3 = 1.0 to 1.49, grade 4 > 1.5. The agreement between echocardiographic and CMR was determined with the intraclass correlation coefficient or Cohen's kappa (<0.2 poor agreement; 0.2-0.4 fair agreement; 0.4-0.6 moderate agreement; 0.6-0.8 good agreement; 0.8-1.0 very good agreement). RESULTS: The median echoCel-ind was 0.9 (range, 0.4-2.3), and the median cmrCel-ind was 0.7 (range, 0.3-5.3). Grade 1 or 2 was found in 19 patients (51.3%) by echocardiography and in 27 patients (72.9%) by CMR. The agreement between imaging methods was only fair (kappa = 0.39, P = 0.002) for the 4-grade classification and moderate (intra-class correlation coefficient = 0.43; 95% confidence interval, 0.13-0.66) for Cel-ind calculation. Significant correlations between Cel-ind in CMR and cardiopulmonary parameters were found (for peak oxygen uptake: R = -0.35, P = 0.034; for the ventilation/carbon dioxide slope: R = 0.46, P = 0.005). Neither of them correlated with echocardiographic severity score. CONCLUSIONS: The agreement between echocardiographic and CMR assessment of the Cel-ind is at most moderate; echocardiography usually overestimates, but rarely underestimates, EA severity. Cel-ind by CMR seems to be more valuable, because it is associated with heart failure markers.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/métodos , Imagem Cinética por Ressonância Magnética/métodos , Adulto , Fatores Etários , Estudos de Coortes , Bases de Dados Factuais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Polônia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Estatísticas não Paramétricas
6.
Can J Cardiol ; 35(12): 1824-1833, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31564390

RESUMO

BACKGROUND: Right ventricular (RV) diastolic function and right atrial (RA) function are poorly characterized in patients with Ebstein anomaly (EA) but may influence functional capacity. We aimed to evaluate RV diastolic function and RA function in EA and study their relationship with biventricular systolic function and exercise capacity. METHODS: Seventy-two patients with EA and 69 controls prospectively underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to investigate RV systolic and diastolic function, RA function, and exercise capacity. RESULTS: Altered RV diastolic function was indicated by the reduced tricuspid valve E/A ratio, percentage RV filling time, and early and late diastolic strain rate; and by the increased tricuspid valve E/E', isovolumic relaxation time, and RV myocardial performance index. The average of 6-RV-segment early diastolic strain rate correlated modestly with peak VO2 (r = 0.38, P < 0.01), RV ejection fraction (r = 0.41, P < 0.01), and left ventricular ejection fraction (r = 0.33, P < 0.05). Patients with EA had impaired RA reservoir, conduit, and pump function, which were associated with peak VO2 (r = 0.54, P < 0.001 for reservoir function). CONCLUSIONS: Altered RV diastolic function and RA function in patients with EA are associated with impaired biventricular systolic function and exercise capacity. The stronger correlation of RA vs RV function with exercise capacity suggests that it may be important to evaluate RA function in this population.


Assuntos
Função do Átrio Direito/fisiologia , Anomalia de Ebstein/fisiopatologia , Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Função Ventricular Direita/fisiologia , Adulto , Estudos de Casos e Controles , Anomalia de Ebstein/diagnóstico por imagem , Ecocardiografia/métodos , Feminino , Alemanha , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Valores de Referência , Índice de Gravidade de Doença , Volume Sistólico , Adulto Jovem
7.
J Am Soc Echocardiogr ; 32(12): 1598-1607, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31551185

RESUMO

BACKGROUND: Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD. OBJECTIVE: We sought to examine global and segmental LV function in fetal EA/TVD with comparison to normal controls and PAIVS. We hypothesized that LV dysfunction in EA/TVD is associated with abnormal LV remodeling and interventricular mechanics. METHODS: We retrospectively identified 63 cases of fetal EA/TVD (40 with retrograde ductal flow) and 22 cases of PAIVS encountered from 2004 to 2015 and compared findings to 77 controls of comparable gestational age. We measured the combined CO and global LV function using two-dimensional, Doppler-derived, deformational (six-segmental vector velocity imaging) and dyssynchrony indices (DIs; SD of time to peak), and a novel global DI. RESULTS: EA/TVD fetuses demonstrated abnormal LV global systolic function with reduced ejection fraction, fractional area change, and CO, while in PAIVS we observed a normal ejection fraction, fractional area change, and CO. PAIVS, but not EA/TVD, demonstrated increased LV sphericity, suggestive of remodeling, and associated enhanced radial function in the third trimester. In contrast, while EA/TVD fetuses had normal LV segmental longitudinal strain, there was abnormal radial segmental deformation and LV dyssynchrony with increased SD of time to peak and DI. CONCLUSIONS: Fetal EA/TVD is associated with a lack of spherical remodeling and presence of mechanical dyssynchrony, which likely contribute to reduced CO and ejection fraction. Clinical monitoring of LV function is warranted in fetal EA/TVD. Further studies incorporating quantification of LV function into prediction models for adverse outcomes are required.


Assuntos
Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Resultado da Gravidez , Ultrassonografia Pré-Natal/métodos , Disfunção Ventricular Esquerda/diagnóstico por imagem , Estudos de Casos e Controles , Estudos de Avaliação como Assunto , Feminino , Idade Gestacional , Humanos , Gravidez , Diagnóstico Pré-Natal/métodos , Valores de Referência , Estudos Retrospectivos , Disfunção Ventricular Esquerda/fisiopatologia
8.
PLoS One ; 14(8): e0220890, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31393951

RESUMO

In Ebstein's anomaly, maximal expansion in the atrialized right ventricle (RV) occurs during early diastole, whereas that of the functional RV occurs in late diastole, resulting in diastolic dyssynchronous expansion (DSE). We quantitatively assessed DSE and identified preoperative factors correlated with persistent DSE after surgery. Seventeen patients diagnosed with Ebstein's anomaly in whom transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) images available were retrospectively analyzed for quantitative DSE assessment and 10 patients who underwent surgery and postoperative TTE available were additionally analyzed for postoperative DSE. Severity of DSE was assessed by the time difference of maximal expansion between the atrialized and functional RV divided by the cardiac cycle length × 100 ("DSE index"). Relations between DSE and, clinical, electrophysiologic parameters and the severity of tricuspid valve (TV) tethering (the RV length / tethering height during diastole: "Tethering index") were assessed. In total patients, median DSE index and tethering index were 30.3 and 2.1 respectively, and the DSE index was correlated with tethering index (rs = 0.664, P = 0.004). In 10 patients who underwent surgery, this association remained after surgery and at 2-year follow up. Tethering index ≥2.5 separated patients with and without persistent DSE. In conclusion, DSE exists in Ebstein's anomaly. DSE index is related to the tethering index and DSE persists postoperatively if tethering index ≥ 2.5. As the persistent DSE might possibly impede the optimal recovery of RV function after surgery, severity of TV tethering should take into account in considering surgery.


Assuntos
Anomalia de Ebstein/fisiopatologia , Ventrículos do Coração/fisiopatologia , Período Pós-Operatório , Adolescente , Adulto , Diástole , Ecocardiografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Valva Tricúspide/fisiopatologia , Função Ventricular Direita , Adulto Jovem
9.
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
10.
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
11.
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
12.
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
13.
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
14.
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
15.
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
17.
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
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...