RESUMEN
Resumen El síndrome de hipoplasia de corazón izquierdo (SHCI) es una cardiopatía congénita con letalidad superior al 95%. La etapificación quirúrgica es la principal vía de tratamiento, y se inicia con la operación de Norwood; la sobrevida a largo plazo de los pacientes tratados es desconocida en nuestro medio. Objetivos 1) Revisar nuestra experiencia en el manejo de todos los pacientes con SHCI evaluados entre enero 2000 y junio 2010. 2) Identificar factores de riesgo de mortalidad quirúrgica. Pacientes y método Estudio retrospectivo de una única institución con una cohorte de pacientes con SHCI. Se revisan antecedentes clínicos, quirúrgicos, y registros de seguimiento. Resultados Se evaluaron 76 pacientes con SHCI; 9/76 tenían comunicación interauricular (CIA) restrictiva, y 8/76, aorta ascendente de < 2 mm; 65/76 fueron tratados: 77% tuvieron operación de Norwood con conducto entre ventrículo derecho y ramas pulmonares como fuente de flujo pulmonar, 17% Norwood con shunt de Blalock-Taussig, y 6% otra cirugía. La mortalidad en la primera etapa quirúrgica fue del 23%, y en operación de Norwood, del 21,3%. En el período 2000-2005 la mortalidad en la primera etapa quirúrgica fue del 36%, y entre 2005-2010, del 15% (p = 0,05). La sobrevida global fue del 64% a un año y del 57% a 5 años. Por análisis multivariado fueron factores de riesgo para mortalidad la presencia de aorta ascendente diminuta y CIA restrictiva. Conclusiones Nuestros resultados inmediatos y a largo plazo en la etapificación quirúrgica de SHCI son similares a la experiencia de grandes centros. Hay una mejoría en mortalidad operatoria en la segunda mitad de la serie. Se identifican factores de riesgo de mortalidad.
Abstract Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and method Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. Results Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤ 2 mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P = .05). Actuarial survival was 64% at one year, and 57% at 5 years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. Conclusions Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.
Asunto(s)
Humanos , Masculino , Femenino , Recién Nacido , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Defectos del Tabique Interatrial/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Aorta/anomalías , Factores de Tiempo , Análisis Multivariante , Estudios Retrospectivos , Factores de Riesgo , Estudios de Seguimiento , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Defectos del Tabique Interatrial/fisiopatología , Defectos del Tabique Interatrial/mortalidadRESUMEN
UNLABELLED: Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. OBJECTIVES: 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. PATIENTS AND METHOD: Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. RESULTS: Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. CONCLUSIONS: Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos/métodos , Defectos del Tabique Interatrial/cirugía , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Aorta/anomalías , Femenino , Estudios de Seguimiento , Defectos del Tabique Interatrial/mortalidad , Defectos del Tabique Interatrial/fisiopatología , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Recién Nacido , Masculino , Análisis Multivariante , Estudios Retrospectivos , Factores de Riesgo , Factores de TiempoRESUMEN
OBJECTIVE: To examine the variation in survival in infants with atrioventricular septal defects (AVSD) with demographic factors and clinical characteristics, including the presence of Down syndrome. STUDY DESIGN: We selected infants with all types of AVSD with Down syndrome (n = 177) and without Down syndrome (n = 161), born between Jan 1, 1979, and Dec 31, 2003 and identified through the Metropolitan Atlanta Congenital Defects Program (MACDP). Infants were classified by the complexity of their cardiac defects and presence of major non-cardiac malformations. Deaths (n = 111) were identified through 2004 with linkage with state vital records and the National Death Index. Kaplan-Meier survival probabilities and adjusted hazard ratios (HRs) were calculated in relation to demographic and clinical characteristics. RESULTS: Children with AVSD and Down syndrome had a similar overall survival probability (70%) as those without Down syndrome (69%). Mortality was higher in children with a complex AVSD (adjusted HR = 7.0; 95% CI, 3.1-15.5) and in children with > or =2 major non-cardiac malformations (adjusted HR = 3.4; 95% CI, 1.8-6.5) and was lower in children in the 1992 to 2003 birth cohort (adjusted HR = 0.6; 95% CI, 0.4-0.998). CONCLUSIONS: Down syndrome was not a prognostic factor. Our findings might be helpful in assessing the long-term prognosis of infants with AVSD.
Asunto(s)
Síndrome de Down/epidemiología , Defectos del Tabique Interatrial/mortalidad , Defectos del Tabique Interventricular/mortalidad , Comorbilidad , Síndrome de Down/mortalidad , Femenino , Defectos del Tabique Interatrial/epidemiología , Defectos del Tabique Interventricular/epidemiología , Humanos , Lactante , Estimación de Kaplan-Meier , Masculino , Pronóstico , Modelos de Riesgos Proporcionales , Factores de RiesgoRESUMEN
Introducción: La cirugía de la comunicación interauricular (CIA) tiene excelentes resultados a largo plazo en niños y también en pacientes operados en la etapa adulta. Objetivo: Determinar y comunicar nuestra experiencia respecto de los resultados a largo plazo del tratamiento quirúrgico de la CIA en adultos. Pacientes y Métodos: Estudio descriptivo de 58 pacientes intervenidos entre los años 1991 y 2004. Cincuenta y seis tenían CIA tipo ostium secundum y 2 tipo seno venoso; 4 presentaron insuficiencia tricuspídea funcional. Veintiocho pacientes tenían hipertensión pulmonar moderada y 25 severa. El shunt promedio fue de 3.2 ± 1.016. Resultados: La edad promedio fue 31.8 ± 11.34 años; 42 pacientes eran de sexo femenino y 14 mayores de 40 años; 15 pacientes estaban en CF II y 43 en CF III (NYHA). En 56 pacientes el cierre del defecto se realizó con parche de dacrón, en 1 con pericardio autólogo y cierre directo en otro. En 3 pacientes se efectuó una anuloplastía tricuspidea y reemplazo en 1. Una paciente falleció por tromboembolismo pulmonar masivo postoperatorio, 3 pacientes presentaron fibrilación auricular transitoria y 1 paciente presentó un derrame pleural. La probabilidad de sobrevida actuarial fue de 98 por ciento a 176 meses y la probabilidad de estar libre de arritmias fue de 95 por ciento a 176 meses de seguimiento. Conclusiones: En nuestra experiencia, el cierre quirúrgico de la CIA en el adulto provee una excelente mejoría sintomática, encontrándose la mayoría de los pacientes en CF I, con una excelente probabilidad de sobrevida y tiempo libre de eventos cardiovasculares en el largo plazo.
Asunto(s)
Adulto , Masculino , Humanos , Femenino , Persona de Mediana Edad , Defectos del Tabique Interatrial/cirugía , Chile , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interatrial/mortalidad , Defectos del Tabique Interatrial , Ecocardiografía , Epidemiología Descriptiva , Estudios de Seguimiento , Complicaciones Posoperatorias , Tasa de SupervivenciaRESUMEN
Objetivo: Selecionar os pacientes com Defeitos do Septo Atrial (DSA) -Comunicação Interatrial (CIA) e Forâmen Oval Patente (FOP) -através de um Escore Ecocardiográfico Transesofágico (E-Eco) que se beneficiariam com o fechamento percutâneo utilizando a prótese de Amplazer. Métodos: A nossa experiência consiste em 62 pacientes em duas fases, a primeira entre abril/0O a dez/02 com 25 pacientes (pcts); a segunda entre dez/02 a fev/04após a aplicação do E-Eco com 37 pcts. Neste último período foram encaminhados 90 pcts com suspeita diagnóstica de DSA;destes, foram selecionados37 pcts (24 CIA e 13 FOP) com idade mediana de 39 anos. O escore foi estabelecido de acordo com a distância da borda muscular do septo atrial às seguintes estruturas: Veia Pulmonar Superior Direita (VPSD), Seio Coronariano (SO, Veia Cava Superior(VCS) e Valvas AV (Mitral ou Tricúspide). Para uma distância < 5mm a pontuação é O; 5mm = 1 ponto; entre 5-10mm = 2 pontos; >10mm = 3 pontos. Para qualquer pontuação O ou quando a C/A > 40 mm o pct é excluído. Resultados: Dos 37 pcts selecionados, 36 obtiveram sucesso (97,8 por cento), sendo observado pequeno shunt residual imediato em 2 pcts (ambos com escore de 6 pontos). Conclusões: O E-Eco mostrou-se bastante eficaz na seleção destes pacientes. Antes da sua introdução houve 20 por cento de insucesso (5 de 25 pcts) contra 3,2 por cento (1 de 36 pts) após a sua utilização.
Asunto(s)
Humanos , Adulto , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interatrial/mortalidad , Ecocardiografía Transesofágica , Fibrilación Atrial/mortalidadRESUMEN
We prospectively examined whether surgical treatment of secundum atrial septal defects (ASDs) in patients > or =40 years old improves their long-term clinical outcome. Surgical treatment of secundum ASDs in adults > 40 years old is a subject of controversy because of the perception of good long-term clinical outcomes in patients with unrepaired ASDs and the lack of data from randomized trials. We recruited 521 patients > 40 years old with secundum ASDs referred for treatment; 48 were excluded. Patients were randomly assigned to surgical closure (n = 232) or medical treatment (n = 241). The primary and secondary end points were a composite of major cardiovascular events (death, pulmonary embolism, major arrhythmic event, embolic cerebrovascular event, recurrent pulmonary infection, functional class deterioration or heart failure) and overall mortality, respectively. We assessed possible prognostic markers. The analysis was performed on an intention-to-treat basis. The median follow-up period was 7.3 years (range 2 to 13). The risk of having the primary end point was significantly higher in the medical group, which had a univariate hazards ratio of 1.99 (95% confidence interval [CI] 1.23 to 3.22) and a multivariate hazards ratio of 1.85 (95% CI 1.08 to 3.17). Although the survival analysis did not reveal differences in overall mortality between the surgical and medical treatments (hazards ratio 1.71, 95% CI 0.76 to 3.86), the multivariate analysis, adjusted by age at entry, mean pulmonary artery pressure and cardiac index, demonstrated significant differences between the study groups (hazards ratio 4.09, 95% CI 1.41 to 11.89). Surgical closure was superior to medical treatment in improving both the composite of major cardiovascular events and overall mortality in patients > 40 years old with secundum ASDs. This superiority was related to the mean pulmonary artery pressure, age at diagnosis and cardiac index. Because of the higher risk of morbidity and mortality, we believe that anatomic closure should always be attempted as the initial treatment for ASDs in adults > 40 years old with pulmonary artery systolic pressure < 70 mm Hg and a pulmonary/systemic output ratio > or =1.7. The operation must be performed as soon as possible, even if the symptoms or the hemodynamic impact seems to be minimal.
Asunto(s)
Defectos del Tabique Interatrial/cirugía , Complicaciones Posoperatorias/etiología , Adulto , Factores de Edad , Anciano , Causas de Muerte , Estudios Cruzados , Estudios de Seguimiento , Defectos del Tabique Interatrial/mortalidad , Humanos , Persona de Mediana Edad , Complicaciones Posoperatorias/mortalidad , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Pacientes com defeito septal atrioventricular de forma total (DSAVT) frequentemente apresentam insuficiência cardíaca intratável e hipertensäo arterial pulmonar nos primeiros meses de vida, e apenas uma minoria sobrevive sem tratamento cirúrgico precoce. Por essa razäo, indica-se a correçäo definitiva para alterar favoravelmente a história natural da doença. Entretanto, vários fatores säo responsáveis pela alta mortalidade cirúrgica. O presente trabalho estuda a experiência na correçäo cirúrgica do DSAVT com o objetivo de identificar alguns fatores de risco estatisticamente significativos para a ocorrência de morte operatória. Analisaram-se, retrospectivamente, 52 pacientes submetidos, entre janeiro de 1974 e dezembro de 1990, a cirurgia definitiva para correçäo de DSAVT no Royal Brompton and National Heart and Lung Institute, sendo estudadas as seguintes variáveis: idade, peso, sexo, ano de operaçäo, presença de síndrome de Down, grau de regurgitaçäo da valva AV, bandagem prévia do tronco pulmonar, presença de anomalias associadas, pressäo sistólica pulmonar, duplo orifício mitral, classificaçäo do defeito segundo Rastelli, emprego de parada circulatória e técnica de correçäo (1 x 2 retalhos). Todos os fatores foram avaliados isoladamente, mediante a análise univariada. Para determinar quais os fatores que, independentemente da açäo de outros, contribuíram significativamente para maior mortalidade cirúrgica, foi utilizada a análise multivariada com regressäo logística. A análise multivariada demonstrou que o baixo peso na época da operaçäo e a técnica de correçäo com um retalho aumentam significativamente a mortalidade cirúrgica.