Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 101
Filtrar
1.
Braz J Cardiovasc Surg ; 34(5): 511-516, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31364344

RESUMO

OBJECTIVE: This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes. METHODS: A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction. RESULTS: Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure. CONCLUSION: In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.


Assuntos
Anomalia de Ebstein/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Idoso , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/métodos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Anomalia de Ebstein/complicações , Anomalia de Ebstein/mortalidade , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Insuficiência da Valva Tricúspide/etiologia , Disfunção Ventricular Direita/etiologia , Adulto Jovem
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.
Congenit Heart Dis ; 14(4): 619-627, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30791188

RESUMO

BACKGROUND: The increasing number of patients with Ebstein anomaly (EA) surviving into adulthood implies improvements in the treatments for the complex lesion. We revisited the clinical outcomes of patients with EA to demonstrate their "real world" survival. OBJECTIVES: To identify the survival and predictors of mortality in patients with EA who underwent medical or surgical management in the present era. METHODS: All patients who had EA with atrioventricular concordance between 1994 and 2016 were retrospectively reviewed. Baseline characteristics, initial echocardiographic findings, treatments, and outcomes were explored. The survival analysis was performed at the end of 2017. A multivariate analysis was used to assess mortality risks. RESULTS: A total of 153 patients (25.4 ± 20.4 years, 60% female) were analyzed. Of these, 89 patients had been diagnosed with EA in childhood. During the follow-up [median time of 5.2 years (3 days-23.5 years)], 32 patients (20.9%) died due to major cardiac adverse events. The overall survival at 1, 5, and 10 years were 89%, 82.2%, and 79%, respectively. Of the total 153 patients, 64 patients underwent at least one surgical intervention [median age of 17 years (1 day-64.4 years)]. The survival at 1, 5, and 10 years were 87.5%, 82.4%, and 77.7%, respectively, in patients with EA surgery. This survival is comparable to the survival of 89 nonoperated patients with EA: 89.9%, 87.5%, and 81.8%, at 1, 5, and 10 years, respectively. The significant predictors of mortality were: age at diagnosis ≤2 years, tricuspid valve (TV) z-score >3.80, TV displacement >19.5 mm/m2 , presence of severe tricuspid regurgitation, and absence of forward flow across the pulmonic valve at the initial diagnosis. CONCLUSION: Patients with EA had a moderately good survival in this era. In this paper, we report five simple predictors of death in this patient population.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Anomalia de Ebstein/cirurgia , Ecocardiografia/métodos , Eletrocardiografia/métodos , Medição de Risco/métodos , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Tailândia/epidemiologia , Adulto Jovem
5.
Braz J Cardiovasc Surg ; 33(4): 353-361, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30184032

RESUMO

OBJECTIVE: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). METHODS: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. RESULTS: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho -0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). CONCLUSION: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.


Assuntos
Anuloplastia da Valva Cardíaca/métodos , Anomalia de Ebstein/cirurgia , Técnica de Fontan/métodos , Ventrículos do Coração/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Anuloplastia da Valva Cardíaca/mortalidade , Criança , Pré-Escolar , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/mortalidade , Ecocardiografia , Feminino , Seguimentos , Técnica de Fontan/mortalidade , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Masculino , Ilustração Médica , Complicações Pós-Operatórias , Recuperação de Função Fisiológica , Índice de Gravidade de Doença , Resultado do Tratamento , Valva Tricúspide/diagnóstico por imagem , Adulto Jovem
6.
Ann Thorac Surg ; 106(3): 785-791, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29777671

RESUMO

BACKGROUND: Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multicentric practice patterns and outcomes. We analyzed multiinstitutional data concerning operations and outcomes in neonates and infants with EA. METHODS: Index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of EA and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31 to 365 days). RESULTS: The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (interquartile range, 4 to 13 days) and the majority required preoperative ventilation (61.6%, n = 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, n = 101), followed by systemic-to-pulmonary shunt (20.4%, n = 52) and tricuspid valve closure (9.4%, n = 24). Overall neonatal operative mortality was 27.4% (n = 70), with composite morbidity-mortality of 51.4% (n = 48). For infants, median age at operation was 179 days (interquartile range, 108-234 days); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, n = 91) followed by Ebstein repair (15.5%, n = 37). Overall operative mortality for infants was 9.2% (n = 22) with composite morbidity-mortality of 20.1% (48). CONCLUSIONS: Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.


Assuntos
Procedimentos Cirúrgicos Cardíacos/mortalidade , Procedimentos Cirúrgicos Cardíacos/métodos , Anomalia de Ebstein/cirurgia , Mortalidade Hospitalar , Estudos de Coortes , Bases de Dados Factuais , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/mortalidade , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , América do Norte , Cuidados Pré-Operatórios/métodos , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida , Resultado do Tratamento
7.
Arch Dis Child ; 103(9): 859-863, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29567665

RESUMO

BACKGROUND: Forecasting the prognosis of a child when diagnosed with Ebstein's anomaly is difficult. We, therefore, studied which factors at the time of diagnosis are associated with death during childhood. METHODS: All consecutive patients (0-18 years) diagnosed with Ebstein's anomaly in the Netherlands between 1980 and 2014 were included. Survival curves were obtained using the Kaplan-Meier method. By using the Cox proportional hazard model, we analysed the factors (at diagnosis) that were associated with death. RESULTS: We included 176 patients. Thirty-one patients (18%) died before the age of 18 years. The 1-year survival was 84% and remained stable at 82% from 35 months after diagnosis and onwards. Modified Ross Heart Failure Class 4 at the time of diagnosis was the most important risk factor for death during childhood (HR 12.5, 95% CI 4.4 to 35.9). Furthermore, diagnosis in the neonatal period (HR 4.2, 95% CI 1.5 to 12.0), severe tricuspid valve regurgitation (HR 2.4, 95% CI 1.2 to 5.0), severe right ventricular outflow tract obstruction (HR 3.7, 95% CI 1.8 to 7.7) and a patent ductus arteriosus (HR 2.8, 95% CI 1.3 to 6.0) at the time of diagnosis were univariately associated with death. Multivariable analysis showed that presentation with Heart Failure Class 4 and a ventricular septal defect is the strongest predictor of death in childhood and adolescence. CONCLUSION: Patients with Ebstein's anomaly presenting with Heart Failure Class 4 and a ventricular septal defect have a high risk of death during childhood.


Assuntos
Anomalia de Ebstein/mortalidade , Adolescente , Fatores Etários , Criança , Pré-Escolar , Comorbidade , Anomalia de Ebstein/diagnóstico por imagem , Ecocardiografia , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/mortalidade , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Países Baixos/epidemiologia , Prognóstico , Radiografia Torácica , Fatores de Risco
8.
Semin Thorac Cardiovasc Surg ; 29(2): 206-212, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28823330

RESUMO

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status. Pulmonary atresia or critical stenosis occurred in 18 neonates. Twenty-seven (34%) patients required intervention: 13 systemic-to-pulmonary shunts, 5 balloon pulmonary valvotomy, 3 relief of right ventricular outflow tract obstruction, 3 Starnes procedures, and 3 other procedures. Sixty-nine (86%) of the neonates survived to hospital discharge. Overall 15-year survival estimate was 67% (SE = 6.5), with a superior prognosis for those able to be managed medically (15-year survival of 79%, SE = 7.0) compared with those in whom surgical or catheter intervention was undertaken (15-year survival of 45%, SE = 11.2, P = 0.005). For early survivors of neonatal surgery, freedom from reoperation at 10 years was 16% (SE = 8.5). For long-term survivors, 96% were classified as New York Heart Association Class I or II. Neonates with Ebstein anomaly who can be managed without intervention have a good prognosis. Substantial mortality risk remains in those who require intervention, especially those complicated by pulmonary atresia.


Assuntos
Valvuloplastia com Balão , Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Procedimentos Cirúrgicos Vasculares , Fatores Etários , Valvuloplastia com Balão/efeitos adversos , Valvuloplastia com Balão/mortalidade , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Intervalo Livre de Doença , Anomalia de Ebstein/complicações , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Feminino , Humanos , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , New South Wales , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Modelos de Riscos Proporcionais , Atresia Pulmonar/complicações , Atresia Pulmonar/fisiopatologia , Retratamento , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade
9.
Circ J ; 81(9): 1354-1359, 2017 Aug 25.
Artigo em Inglês | MEDLINE | ID: mdl-28450668

RESUMO

BACKGROUND: In Ebstein's anomaly (EA) current surgical criteria may not translate into better long-term survival. The aim of this study was therefore to determine if surgical treatment for EA increases survival, and to analyze factors associated with mortality.Methods and Results:A retrospective study was carried out involving 103 patients with surgical indication using current criteria, comparing operated (n=49; 47.5%) and non-operated patients (n=54; 52.4%); the severity of disease was similar in all cases. Overall follow-up was 12 years (range, 1-49 years). There were no differences in mortality: in the surgical and non-surgical groups, survival at 10 years was 92.8% vs. 90.7%; 20 years, 85.7% vs. 81.0%; and 30 years, 78.5% vs. 72.2%, respectively. On multivariate analysis right ventricular fractional shortening (RVFS) was associated with mortality in both groups. Decreasing RVFS was associated with worse survival according to severity: when RVFS was <20%, survival at 20, 40 and 60 years was 58%, 39%, and 12.5%, respectively (P<0.0013). Left ventricular ejection fraction also correlated with survival (P<0.0013). CONCLUSIONS: Surgery did not translate into benefit in terms of survival, and this was clearly associated with RV function; therefore this should be a key factor in the surgical decision making.


Assuntos
Anomalia de Ebstein , Função Ventricular Direita , Adolescente , Adulto , Criança , Pré-Escolar , Intervalo Livre de Doença , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Anomalia de Ebstein/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
10.
Congenit Heart Dis ; 12(1): 32-39, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27558473

RESUMO

OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study. DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed. RESULTS: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths. CONCLUSIONS: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Sobreviventes , Adolescente , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Criança , Pré-Escolar , Intervalo Livre de Doença , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Feminino , Mortalidade Hospitalar , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Países Baixos , Cuidados Paliativos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Complicações Pós-Operatórias/cirurgia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
11.
Thorac Cardiovasc Surg ; 65(8): 649-655, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27509000

RESUMO

Objective Ebstein anomaly is a rare type of tricuspid malformation. The present surgical methods to resolve this anomaly include tricuspid valvuloplasty, palliative surgery, and tricuspid valve replacement. The purpose of this study was to evaluate the short- and midterm outcomes of different surgical treatments among children with Ebstein anomaly. Methods This was a retrospective study of 136 Ebstein anomaly patients undergoing surgery at our institution from January, 2006 to August, 2015. This cohort included 118 patients receiving tricuspid valvuloplasty, 14 patients receiving palliative surgery and 4 patients receiving tricuspid valve replacement. Results There were two in-hospital deaths and one delayed death 6 months after bidirectional cavopulmonary shunt during a second-stage operation; thus, the mortality rate was 2.2% (3/136). The follow-up variables included echocardiography, chest radiography, oxygen saturation, and cardiac function. After a mean follow-up duration of 35.8 ± 16.5 months (range: 6-98 months), all survivors had no indications for reoperation; the oxygen saturation following radical surgery and palliative surgery was 95 to 100% and 85 to 95%, respectively. Most of the patients exhibited an improved New York Heart Association (NYHA) functional class from III or IV preoperatively to I or II at follow-up; only two patients who underwent the Fontan procedure continued to exhibit NYHA functional class III. In most patients, tricuspid regurgitation (TR) degree decreased from moderate or severe preoperatively to mild or moderate at follow-up and only six patients continued to exhibit severe TR at follow-up. Conclusions Individualization of surgical methods based on different indications, including age, associated malformations, tricuspid anatomy, cardiac function, and intraoperative status, could effectively improve the outcomes of Ebstein anomaly patients.


Assuntos
Anomalia de Ebstein/cirurgia , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Valvuloplastia com Balão , Criança , Pré-Escolar , China , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Feminino , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/mortalidade , Mortalidade Hospitalar , Humanos , Lactente , Masculino , Cuidados Paliativos , Recuperação de Função Fisiológica , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X , 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/mortalidade , Insuficiência da Valva Tricúspide/fisiopatologia
12.
Interact Cardiovasc Thorac Surg ; 24(2): 245-250, 2017 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-27702829

RESUMO

Objectives: Favourable outcomes in the repair of Ebstein's anomaly are predicated on tricuspid valve competence, right ventricular function and presence of arrhythmia. We report our experience with a single-stage, three-fold repair of Ebstein's anomaly, namely, cone reconstruction of the tricuspid valve supplemented by bidirectional cavopulmonary anastomosis and right atrial electrocautery maze. Methods: From 2010 to 2014, 37 consecutive patients with Ebstein's anomaly, median age 17.3 (9.1-56.2) years, underwent this single-stage, three-fold surgical procedure. The principal elements of the procedure include (i) cone reconstruction of the tricuspid valve, limited plication at the level of the displaced valve, insertion of a homemade annuloplasty ring, defect repair and reduction atrioplasty supplemented by (ii) right atrial electrocautery maze and (iii) bidirectional cavopulmonary anastomosis. Postoperatively, all patients were followed up regularly for a mean period of 2.3 (1-4) years by clinical, electrocardiographic and echocardiographic examinations. Results: The in-hospital mortality rate was 2.7% (1 patient) with no late deaths. Mean cardiopulmonary bypass time was 110 ± 18.3 min and aortic cross-clamp time was 48.5 ± 7.4 min. Echocardiographic examination showed significant improvement of valve regurgitation (P < 0.0001). NYHA functional class was I in 77.8% of the survivors and II in 22.2%. The cardiothoracic ratio decreased significantly (P < 0.05). No deleterious effects of the Glenn shunts have been reported. Sinus rhythm has remained stable in 31 patients (86.1%) during the follow-up period. Conclusions: Single-stage, three-fold repair for the management of Ebstein's anomaly offers good outcome in terms of low mortality and morbidity rates. It can achieve a durable valve-sparing repair, good functional mid-term outcomes and good quality of life among survivors.


Assuntos
Anuloplastia da Valva Cardíaca , Anomalia de Ebstein/cirurgia , Técnica de Fontan , Adolescente , Adulto , Criança , Estudos de Coortes , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Eletrocardiografia , Feminino , Átrios do Coração/cirurgia , Mortalidade Hospitalar , Humanos , Masculino , Pessoa de Meia-Idade , Valva Tricúspide/cirurgia , Função Ventricular Direita , Adulto Jovem
13.
Circulation ; 134(17): 1257-1264, 2016 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-27777295

RESUMO

BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. RESULTS: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5-9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6-8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2-2.3) W/m2, and mean pulmonary pressure was 12 (9-18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. CONCLUSIONS: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.


Assuntos
Anomalia de Ebstein , Técnica de Fontan , Ventrículos do Coração , Função Ventricular Esquerda , Pré-Escolar , Intervalo Livre de Doença , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Anomalia de Ebstein/cirurgia , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/cirurgia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Terapia de Salvação , Taxa de Sobrevida
14.
J Korean Med Sci ; 31(11): 1749-1754, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27709852

RESUMO

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.


Assuntos
Anomalia de Ebstein/cirurgia , Adolescente , Adulto , Idoso , Arritmias Cardíacas/etiologia , Transtornos Cerebrovasculares/etiologia , Intervalo Livre de Doença , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/patologia , Ecocardiografia , Feminino , Coração/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Reoperação , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
15.
Can J Cardiol ; 32(12): 1500-1506, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27476984

RESUMO

BACKGROUND: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare anomalies and data on outcomes after a fetal or neonatal EA/TVD diagnosis are conflicting. METHODS: To examine the outcome and identify markers predictive of mortality, we reviewed our single-centre experience from 2000-2014. Variables were analyzed separately for cases diagnosed in utero without pregnancy termination and for all live-born patients. RESULTS: Of 47 fetal cases, 8 (17%) died in utero and 10 (21%) as neonates. Independent predictors associated with fetal demise included severe tricuspid regurgitation with a Doppler gradient < 40 mm Hg (odds ratio, 1.22 per mm Hg deduction; P = 0.003) and pulmonary regurgitation (odds ratio, 11.4; P = 0.03) at the baseline examination. A novel prognostic score (range, 0-10) combining the severity of 5 echocardiographic findings was independently associated with overall mortality (hazard ratio [HR], 1.39 per point increase; P = 0.01). Survival rates of 66 live births at 1 month, 1 year, and 5 years were 86%, 82%, and 80% respectively, and 75%, 60%, and 55% remained free from surgery at the same points in time. Factors associated with postnatal death in multivariate analysis included a younger gestational age at birth (HR per week, 1.59; P < 0.001), tricuspid annulus diameter (HR per z-score increase, 1.76; P = 0.004), and no pulmonary forward flow (HR, 4.63; P = 0.03). CONCLUSIONS: Our experience with fetal and neonatal EA/TVD shows better survival rates than previously reported. Mortality after a fetal diagnosis was significantly associated with hemodynamic changes indicative of a circular shunt, including pulmonary and tricuspid regurgitation severe enough to cause diastolic umbilical arterial flow reversal.


Assuntos
Anomalia de Ebstein , Ecocardiografia Doppler/métodos , Insuficiência da Valva Tricúspide , Valva Tricúspide , Canadá/epidemiologia , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Mortalidade Perinatal , Valor Preditivo dos Testes , Gravidez , Prognóstico , Estudos Retrospectivos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/congênito , Insuficiência da Valva Tricúspide/diagnóstico , Insuficiência da Valva Tricúspide/mortalidade , Insuficiência da Valva Tricúspide/fisiopatologia , Ultrassonografia Pré-Natal/métodos
16.
J Thorac Cardiovasc Surg ; 152(2): 516-21, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27160941

RESUMO

OBJECTIVE: In neonates with Ebstein's anomaly and absent right ventricular forward flow, pulmonary valve morphology is normal or abnormal. Although initial postnatal presentations of these 2 conditions are similar, clinical courses and therapeutic strategies for each category differ greatly. METHODS: Among 29 neonates with Ebstein's anomaly without right ventricular forward flow on initial postnatal echocardiography, 16 had a normal pulmonary valve and 13 had an abnormal pulmonary valve. RESULTS: During the postnatal follow-up of the normal pulmonary valve group, right ventricular forward flow commenced approximately 10 days after birth (1-15 days). The ductus arteriosus was surgically ligated in 3 neonates to facilitate right ventricular forward flow. Biventricular or 1 1/2 ventricular physiology was eventually achieved in 14 patients in the normal pulmonary valve group (14/16, 88%) and 2 patients in the abnormal pulmonary valve group (2/13, 15.3%). With respect to the preoperative echocardiographic findings, the normal pulmonary valve group had a significantly larger pulmonary valve annulus (8.2 ± 1.4 mm in the normal pulmonary valve group and 6.4 ± 1.8 mm in the abnormal pulmonary valve group, P = .002) and smaller cardiothoracic ratio (0.79 ± 0.05 in the normal pulmonary valve group and 0.85 ± 0.07 in the abnormal pulmonary valve group, P = .03). Mild to moderate pulmonary regurgitation was present in all patients (16/16, 100%) in the normal pulmonary valve group, but 3 patients (3/13, 23%) in the abnormal pulmonary valve group also had pulmonary regurgitation. On logistic regression analysis, only pulmonary valve annulus size remained as an indicator of a normal pulmonary valve (P = .03). CONCLUSIONS: In patients with Ebstein's anomaly and absent right ventricular forward flow, large pulmonary valve annulus size indicated a normal pulmonary valve. Patients with a normal pulmonary valve showed better survival and had a higher probability of achieving biventricular hemodynamics.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Hemodinâmica , Valva Pulmonar/fisiopatologia , Função Ventricular Direita , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Feminino , Humanos , Recém-Nascido , Estimativa de Kaplan-Meier , Ligadura , Modelos Lineares , Modelos Logísticos , Masculino , Valva Pulmonar/anormalidades , Valva Pulmonar/diagnóstico por imagem , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Função Ventricular Esquerda
17.
Arch Cardiovasc Dis ; 109(6-7): 390-8, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27079467

RESUMO

BACKGROUND: Ebstein anomaly is an uncommon congenital cardiac lesion that may be associated with cyanosis, arrhythmias and right heart dysfunction. Investigation into patient characteristics and outcomes in pregnant women with Ebstein anomaly has been limited. AIMS: To characterize patient characteristics and clinical events for pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; also, to determine the effect of Ebstein anomaly on maternal clinical outcomes and individual predictors of poor outcome at time of delivery. METHODS: We screened the Healthcare Cost and Utilization Project's National Inpatient Sample for hospital admissions of pregnant women for delivery (vaginal or caesarean section) in the USA from 2003-2012, and identified a cohort of 7,850,381. Clinical characteristics and maternal outcomes were identified in those with and without Ebstein anomaly. The primary outcome of interest was major adverse cardiac events (MACE), a composite of in-hospital death, acute myocardial infarction, cerebrovascular events, embolic events, cardiac complications of labour and delivery heart failure or arrhythmia. RESULTS: Our study population consisted of 82 hospitalizations of pregnant women with Ebstein anomaly and 7,850,299 without. The Ebstein cohort more frequently had ostium secundum-type atrial septal defect and/or patent foramen ovale and anomalous atrioventricular excitation (P<0.001 for both). The MACE rate was significantly higher among Ebstein patients (P<0.001). Preterm delivery, postpartum haemorrhage and caesarean delivery occurred more frequently among the Ebstein cohort (19.5% vs 7.2%, 8.5% vs 2.8% and 47.6% vs 31.1%, respectively; P≤0.001). In a multivariable analysis, anomalous atrioventricular excitation (odds ratio [OR] 21.75, 95% confidence interval [CI] 1.03-457.91) and preterm delivery (OR 11.71, 95% CI 1.39-98.89) were associated with MACE among those with Ebstein anomaly. CONCLUSIONS: Pregnant women with Ebstein anomaly are at higher risk of MACE during pregnancy and delivery. Preterm delivery occurred more frequently in women with Ebstein anomaly.


Assuntos
Anomalia de Ebstein/epidemiologia , Complicações Cardiovasculares na Gravidez/epidemiologia , Resultado da Gravidez/epidemiologia , Adulto , Transtornos Cerebrovasculares/epidemiologia , Cesárea , Distribuição de Qui-Quadrado , Bases de Dados Factuais , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Feminino , Cardiopatias/epidemiologia , Mortalidade Hospitalar , Humanos , Modelos Logísticos , Mortalidade Materna , Análise Multivariada , Razão de Chances , Hemorragia Pós-Parto/epidemiologia , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/mortalidade , Nascimento Prematuro/epidemiologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Estados Unidos/epidemiologia , Adulto Jovem
18.
J Thorac Cardiovasc Surg ; 150(5): 1212-9, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26349593

RESUMO

OBJECTIVE: We describe a repertoire of repair techniques according to type of Ebstein's anomaly to correct tricuspid valve (TV) incompetence, and report long-term ventricular function and functional outcomes. METHODS: Sixty-eight patients (mean age, 26.9 ± 7.3 years) with Ebstein's anomaly (type A, n = 21; type B, n = 23; type C, n = 15; type D, n = 9) underwent correction of TV incompetence under normothermic cardiopulmonary bypass. The atrialized ventricle, TV, and subvalvar apparatus were inspected to analyze the precise morphology and determine which leaflet was the most mobile. Various repair strategies (anterior and/or posterior annulorrhapy, Sebening stitich, double-orifice valve technique, with bidirectional Glenn anastomosis if necessary) were used according to the presenting morphology and applied according to the type of Ebstein's anomaly. In all, the atrialized right ventricle (RV) was incorporated into the contractile RV by partial closure of the natural annulus using the most mobile leaflet for valve competence. RESULTS: The mean duration of follow-up was 13.25 ± 1.3 years (median, 9.34 years; range, 1-24 years). The mean New York Heart Association class improved from 3.4 to 1.3 (P < .001). The mean severity of TV incompetence was reduced from 3.2 to 1.3 (P < .001). Exercise tolerance tests demonstrated improved maximal oxygen uptake from a mean of 15 ± 7.8 ng/kg/min preoperatively to a mean of 24.9 ± 2.0 ng/kg/min postoperatively (P < .02). Displacement tissue Doppler imaging was used to evaluate overall cardiac performance of the RV and left ventricle and interventricular septum. The mean basal, middle, and apical ventricular strain improved significantly from preoperative values of 18.08%, 15.6%, and 13.9%, respectively to postoperative values of 25.7% (P < .011), 23.7% (P < .001), and 19.36% (P < .05), respectively. Freedom from reoperation was 100% at 1 year, 98.3% at 5 years, and 92.9% at 20 years. Early mortality was 2.9%, and late mortality was 5.8%. The overall survival rate was 97.6% at 30 days, 92.7% at 5 years, and 91.26% at 20 years. CONCLUSIONS: The various repair techniques, all of which preserve the atrialized chamber and are used individually according to morphology, provide satisfactory long-term ventricular function and functional outcome even in severe types of Ebstein's anomaly.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Idoso , Anastomose Cirúrgica , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Anuloplastia da Valva Cardíaca , Criança , Pré-Escolar , Intervalo Livre de Doença , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Ecocardiografia Doppler , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Recuperação de Função Fisiológica , Reoperação , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Técnicas de Sutura , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico , Insuficiência da Valva Tricúspide/mortalidade , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Esquerda , Função Ventricular Direita , Adulto Jovem
19.
Circulation ; 132(6): 481-9, 2015 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-26059011

RESUMO

BACKGROUND: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. METHODS AND RESULTS: Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). CONCLUSION: In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.


Assuntos
Anomalia de Ebstein/mortalidade , Valva Tricúspide/anormalidades , Aborto Eugênico , Adulto , Peso ao Nascer , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Síndrome de Down/complicações , Síndrome de Down/mortalidade , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/embriologia , Anomalia de Ebstein/cirurgia , Feminino , Idade Gestacional , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/embriologia , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/cirurgia , Mortalidade Hospitalar , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/mortalidade , Masculino , Cuidados Paliativos , Derrame Pericárdico/etiologia , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Valva Tricúspide/fisiopatologia , Valva Tricúspide/cirurgia , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/cirurgia , Ultrassonografia Pré-Natal , Adulto Jovem
20.
Heart Lung Circ ; 24(10): 996-1001, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25911143

RESUMO

BACKGROUND: Ebstein's anomaly (EA) occurs in about one to five per 200 000 live births. Long-term follow-up data of adults with EA is scarce due to the relatively low frequency of the disease and the variation of its anatomic and haemodynamic severity. METHODS: Since 1995, in our adult congenital heart disease (ACHD) centre, we have practised a uniform approach to management of adults with EA, with surgery reserved for those with refractory arrhythmia (failed medical and/or catheter-based treatment) or worsening symptoms of breathlessness. A retrospective review of medical records of all such patients with EA and normal cardiac connections was performed. RESULTS: Fifty-one EA patients (17 males) were identified. Mean age at diagnosis was 21+/-21 years and mean follow-up time at our centre was 21±14 years. During this time, 18 patients (35%) had documented supraventricular arrhythmia. Sixteen patients (30%) underwent ablation therapy with long-term relief from arrhythmia in nine (56%). Nine patients (18%) underwent tricuspid valve (TV) surgery (four repair and five replacement), with seven patients having undergone a tricuspid valve surgery prior to referral to our unit. Three patients died, one of cardiogenic shock after redo surgery (58 years), one of progressive heart failure (45 years) and one with malignancy. Overall survival was 100% to age 40 years, 95% to age 50 years and 81% to age 60 years. CONCLUSIONS: Ebstein's Anomaly in adulthood often has severe morphological abnormalities but is compatible with good medium-term survival, with a generally symptom driven approach to the indications for interventions.


Assuntos
Arritmias Cardíacas/etiologia , Anomalia de Ebstein/complicações , Insuficiência da Valva Tricúspide/etiologia , Valva Tricúspide/cirurgia , Adolescente , Adulto , Idoso , Arritmias Cardíacas/tratamento farmacológico , Arritmias Cardíacas/cirurgia , Ablação por Cateter , Criança , Pré-Escolar , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/mortalidade , Ecocardiografia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Valva Tricúspide/anormalidades , Insuficiência da Valva Tricúspide/congênito , Insuficiência da Valva Tricúspide/cirurgia , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA