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J Thorac Cardiovasc Surg ; 139(2): 354-8, 2010 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-19775704


OBJECTIVE: The objective of this study was to review the long-term results of symptomatic patients with Ebstein anomaly in the neonatal period. METHODS: The medical records of 40 neonates with a diagnosis of Ebstein anomaly who were admitted to our institution between January 1988 and June 2008 were retrospectively reviewed. Primary outcomes studied included patient survival and need for reintervention. RESULTS: No early intervention was required in 16 of the 40 patients with a hospital survival of 94% (15/16) and no late mortality. The remaining 24 patients underwent surgical intervention in the neonatal period. A shunt alone was performed in 9 patients with an actuarial survival of 88.9% at 1 year and 76.2% at 5 and 10 years. For the patients undergoing intervention on the tricuspid valve, survival estimates for the 11 patients with a right ventricular exclusion procedure were 63.6% at 1, 5, and 10 years and 47.7% at 15 years compared with 25.0% at 1, 5, and 10 years for the 4 patients with tricuspid valve repair. All long-term survivors were in New York Heart Association class I or II, and only 1 patient required antiarrhythmic medication. CONCLUSION: Symptomatic neonates with Ebstein anomaly requiring no intervention or shunting alone have good long-term survival. For patients needing intervention on the tricuspid valve, overall survival is lower. For these patients, right ventricular exclusion may be superior to tricuspid valve repair.

Anomalia de Ebstein/cirurgia , Protocolos Clínicos , Anomalia de Ebstein/mortalidade , Mortalidade Hospitalar , Humanos , Recém-Nascido , Estudos Retrospectivos , Resultado do Tratamento
Fetal Diagn Ther ; 25(1): 163-6, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19293587


Although most prenatally diagnosed pulmonary sequestrations (PS) are asymptomatic, large lesions are associated with pleural effusions and pulmonary hypoplasia. We present the first reported case of a prenatally diagnosed giant extralobar pulmonary sequestration that required the ex utero intrapartum treatment (EXIT) procedure with resection and extracorporeal membrane oxygenation (ECMO). We discuss the compelling rationale for performing EXIT-resection-ECMO in the setting of a large thoracic mass and anticipated severe respiratory failure at birth.

Sequestro Broncopulmonar/cirurgia , Oxigenação por Membrana Extracorpórea , Adulto , Sequestro Broncopulmonar/diagnóstico por imagem , Sequestro Broncopulmonar/terapia , Feminino , Feto/irrigação sanguínea , Feto/cirurgia , Humanos , Recém-Nascido , Masculino , Gravidez , Ultrassonografia
Pediatr Cardiol ; 30(4): 431-5, 2009 May.
Artigo em Inglês | MEDLINE | ID: mdl-19184173


Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors' institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period (P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies.

Comunicação Interatrial/cirurgia , Comunicação Interventricular/cirurgia , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
Echocardiography ; 25(7): 727-31, 2008 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-18445061


There has traditionally been less concern regarding coronary anomalies with left-sided congenital heart lesions such as hypoplastic left heart syndrome (HLHS)or Shone complex than with other lesions. However, coronary anomalies in this setting can profoundly affect surgical intervention, particularly when surgical repair involves the ascending aorta. We describe four patients with congenital left-sided heart lesions in which left coronary artery (LCA) anomalies substantially affected intervention and outcome. In the first two cases, the coronary anomalies were not identified prospectively and resulted in surgical injury directly to the coronary or to its surrounding region. In the latter two cases, successful identification of the coronary anomaly preoperatively allowed for modification of surgical technique and/or intervention. We conclude that detailed coronary artery assessment should be part of the routine echocardiographic evaluation of congenital left-sided heart lesions that require surgery.

Anormalidades Múltiplas/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Anomalias dos Vasos Coronários/cirurgia , Ecocardiografia Doppler em Cores , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Interpretação de Imagem Assistida por Computador , Artéria Pulmonar/anormalidades , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/mortalidade , Procedimentos Cirúrgicos Cardíacos/mortalidade , Anomalias dos Vasos Coronários/diagnóstico por imagem , Feminino , Seguimentos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Recém-Nascido , Masculino , Gravidez , Artéria Pulmonar/diagnóstico por imagem , Medição de Risco , Amostragem , Taxa de Sobrevida , Resultado do Tratamento , Ultrassonografia Pré-Natal/métodos
Congenit Heart Dis ; 2(1): 32-7, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-18377514


OBJECTIVE: Double-outlet right ventricle (DORV) is a complex congenital heart defect with heterogeneous anatomy. Patients require a variety of surgical interventions, and the long-term prognosis is variable. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling. DESIGN: Medical records were reviewed in patients with a diagnosis of DORV who had fetal echocardiography at our institution from 1998 to 2004. Pre- and postnatal diagnoses were compared, anticipated surgical procedure was compared with surgery performed, and neonatal outcome was assessed. RESULTS: The study group consisted of 49 fetal patients with 6 in utero deaths (including 4 terminations), 2 patients lost to follow-up, and 41 live births. Postnatal echocardiograms or autopsy results were available on 42 patients. The overall accuracy of fetal echocardiography in making a correct diagnosis of DORV was 76%. Accurate prenatal prediction of the type of cardiac surgery that would be performed was made in 91% of patients who had surgery. Among live births, survival to hospital discharge was 76%. Survival was 50% in patients with extracardiac or chromosomal anomalies, compared with 92% in infants without additional anomalies. CONCLUSION: In most cases, careful evaluation by fetal echocardiography can determine essential anatomic details in fetuses with DORV with enough accuracy to allow for accurate counseling regarding the type of surgery needed. Survival is better than previously reported but is poor in patients with extracardiac or chromosomal abnormalities.

Dupla Via de Saída do Ventrículo Direito/diagnóstico por imagem , Coração Fetal/diagnóstico por imagem , Aorta/diagnóstico por imagem , Procedimentos Cirúrgicos Cardíacos , Dupla Via de Saída do Ventrículo Direito/cirurgia , Ecocardiografia , Comunicação Interventricular/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Humanos , Recém-Nascido , Valor Preditivo dos Testes , Artéria Pulmonar/diagnóstico por imagem , Ultrassonografia Pré-Natal