RESUMO
BACKGROUND: In hypoplastic left heart syndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplastic left heart syndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction. METHODS: TV of 100 patients with hypoplastic left heart syndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart. Associations of TV structure to TR grade and right ventricle function and volume were investigated. Shape parameterization and analysis was used to calculate the mean shape of the TV leaflets, their principal modes of variation, and to characterize associations of TV leaflet shape to TR. RESULTS: In univariate modeling, patients with moderate or greater TR had larger TV annular diameters and area, greater annular distance between the anteroseptal commissure and anteroposterior commissure, greater leaflet billow volume, and more laterally directed anterior papillary muscle angles compared to valves with mild or less TR (all P<0.001). In multivariate modeling greater total billow volume, lower anterior papillary muscle angle, and greater distance between the anteroposterior commissure and anteroseptal commissure were associated with moderate or greater TR (P<0.001, C statistic=0.85). Larger right ventricle volumes were associated with moderate or greater TR (P<0.001). TV shape analysis revealed structural features associated with TR, but also highly heterogeneous TV leaflet structure. CONCLUSIONS: Moderate or greater TR in patients with hypoplastic left heart syndrome with a Fontan circulation is associated with greater leaflet billow volume, a more laterally directed anterior papillary muscle angle, and greater annular distance between the anteroseptal commissure and anteroposterior commissure. However, there is significant heterogeneity of structure in the TV leaflets in regurgitant valves. Given this variability, an image-informed patient-specific approach to surgical planning may be needed to achieve optimal outcomes in this vulnerable and challenging population.
Assuntos
Técnica de Fontan , Síndrome do Coração Esquerdo Hipoplásico , Insuficiência da Valva Tricúspide , Humanos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Técnica de Fontan/efeitos adversos , Ventrículos do Coração , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/complicações , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/cirurgia , Estudos RetrospectivosRESUMO
Fetal diagnosis of scimitar syndrome requires a high index of suspicion. We present two fetal cases of complex congenital heart disease associated with scimitar syndrome, one of them is diagnosed with scimitar syndrome in utero. We emphasize prenatal echocardiographic findings that may assist with arriving at the correct prenatal diagnosis. We also discuss potential challenges in suspecting the presence of scimitar syndrome in utero. The postnatal echocardiographic findings and course are described for both patients. We reviewed the available literature on prenatal diagnosis of scimitar syndrome in the presence of complex congenital heart disease. We describe a new association of VACTERL, imperforate anus, scimitar syndrome, and double-outlet right ventricle all on the same patient, as well as the first prenatal diagnosis of scimitar syndrome associated with hypoplastic left heart syndrome with restrictive atrial septum. Advanced imaging modalities such as a fetal lung Magnetic Resonance Imaging is suggested as a confirmatory test when scimitar syndrome is suspected in utero in the presence of complex congenital heart disease.
Assuntos
Dupla Via de Saída do Ventrículo Direito , Síndrome do Coração Esquerdo Hipoplásico , Síndrome de Cimitarra , Feminino , Humanos , Gravidez , Síndrome de Cimitarra/complicações , Diagnóstico Pré-Natal , Síndrome do Coração Esquerdo Hipoplásico/complicações , Imageamento por Ressonância Magnética , Dupla Via de Saída do Ventrículo Direito/complicações , Coração FetalRESUMO
OBJECTIVE: To characterize neurodevelopmental outcomes after fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome and determine the risk factors for adverse neurodevelopment. STUDY DESIGN: Questionnaires were mailed to families of children who underwent fetal aortic valvuloplasty from 2000 to 2012, and medical records were reviewed retrospectively. The primary outcome was the General Adaptive Composite score of the Adaptive Behavior Assessment System Questionnaire-Second Edition. Other questionnaires included the Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, and Pediatric Quality of Life Inventory. RESULTS: Among 69 eligible subjects, 52 (75%) completed questionnaires at median age of 5.5 (range 1.3-12) years; 30 (58%) had biventricular status circulation. The General Adaptive Composite mean score (92 ± 17) was lower than population norms (P < .001) and similar to published reports in patients with hypoplastic left heart syndrome without fetal intervention; scores in the single ventricular versus biventricular group were 97 ± 19 vs 89 ± 14, respectively (P = .10). On multivariable analysis, independent predictors of a lower General Adaptive Composite score were total hospital duration of stay in the first year of life (P = .001) and, when forced into the model, biventricular status (P = .02). For all other neurodevelopmental questionnaires (Behavior Assessment System for Children, Behavior Rating Inventory of Executive Function, Ages and Stages, Pediatric Quality of Life Inventory), most subscale scores for patients with biventricular and single ventricular status were similar. CONCLUSION: Children who underwent fetal aortic valvuloplasty have neurodevelopmental delay, similar to patients with hypoplastic left heart syndrome without fetal intervention. Achievement of biventricular circulation was not associated with better outcomes. We infer that innate patient factors and morbidity during infancy have the greatest effect on neurodevelopmental outcomes.
Assuntos
Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/cirurgia , Feto/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/complicações , Transtornos do Neurodesenvolvimento/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Valvuloplastia com Balão , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Gravidez , Estudos Retrospectivos , Fatores de RiscoRESUMO
Las cardiopatías son las malformaciones congénitas más frecuentes del desarrollo prenatal, en las que se produce un defecto en la arquitectura normal del corazón fetal interfiriendo en su buen funcionamiento en la vida postnatal. La proporción entre las diferentes cardiopatías varía mucho, sin embargo el Síndrome de Corazón Izquierdo Hipoplásico representa el 2-3% del total de cardiopatías congénitas constituyendo la forma más severa de lesiones obstructivas del corazón izquierdo y la de peor pronóstico. Su diagnóstico en etapas precoces de la gestación es hoy un reto para el obstetra, ya que su carácter evolutivo dificulta este hecho, no siendo en ocasiones, hasta etapas avanzadas de la gestación cuando se produce la alteración anatómica y la subsiguiente expresión ecográfica. Es en este punto donde se debe hacer hincapié, intentando aumentar el número de casos detectados precozmente, lo cual permitiría un adecuado aseso-ramiento familiar y la toma de decisiones oportuna. En los últimos años la introducción de terapias en patología cardíaca fetal, ha despertado un gran interés por tratar de modificar la evolución de esta enfermedad, siendo la valvuloplastía con balón aórtico la técnica de elección en pacientes seleccionados.
Congenital heart defects are the most frecuently congenital malformations in the prenatal development and consist of defect in the normal architecture of the fetal heart which interfering with its proper functioning. The proportion between the different heart disease varies widely, however Hypoplastic Left Heart Syndrome (HLHS) represent 2-3% of all congenital heart diseases and constitute the most severe obstructive lesions of the left heart and worse prognosis. The quickly diagnosis in early stages of the disease is an obstetrician challenge, however, it s evolutional feature hinders this proposal even in advanced stages of pregnancy when the anatomical alteration and subsequent ultrasound expression can be occur. At this point that should be emphasized to try increasing the number of cases detected early, which would allow a proper family counseling and timely decision. Recent years the introduction of therapies on fetal cardiac pathology, has aroused great interest in trying to change the course of the disease and the fetal aortic valvuloplasty with aortic balloon would be the choice technique in selected patients.
Assuntos
Humanos , Feminino , Gravidez , Adulto , Ultrassonografia Pré-Natal , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Estenose da Valva Aórtica/etiologia , Estenose da Valva Aórtica/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/complicações , Diagnóstico Precoce , Cardiopatias CongênitasRESUMO
The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.
Assuntos
Atresia Biliar/cirurgia , Doença Hepática Terminal/cirurgia , Técnica de Fontan , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Transplante de Fígado , Atresia Biliar/complicações , Cianose/etiologia , Doença Hepática Terminal/etiologia , Humanos , Síndrome do Coração Esquerdo Hipoplásico/complicações , Lactente , Transtornos da Nutrição do Lactente/etiologia , Masculino , Cuidados Paliativos , Fatores de Tempo , Resultado do TratamentoRESUMO
A síndrome de hipoplasia do coração esquerdo (SHCE) é a cardiopatia congênita que sofreu as mais dramáticas mudanças na avaliação diagnóstica, manuseio e evolução na era recente. Embora o tratamento estagiado da SHCE esteja bem fundamentado na atualidade, ainda ocorre uma grande variabilidade no manuseio desta doença e os bons resultados globais são obtidos por um número reduzido de centros mundiais. O recém-nascido com síndrome de hipoplasia do coração esquerdo apresenta alto risco de choque antes e após o procedimento paliativo inicial. A síndrome de baixo débito cardíaco, caracterizada pela baixa liberação sistêmica de oxigênio, é achado típico após cirurgia cardíaca neonatal. O edema miocárdico e a disfunção pós-isquêmica resultam em baixo débito cardíaco. O recém-nascido com SHCE apresenta outros problemas: a massa ventricular total é reduzida, a anatomia em paralelo da circulação sistêmica e pulmonar resulta em saturação baixa de oxigênio no sangue arterial, além da necessidade do ventrículo único direito prover o débito cardíaco sistêmico e pulmonar. Ocorre, também, a necessidade de equilibrar as resistências periférica e pulmonar, com ajuste de drogas e ventilação, balanceando as duas circulações. O manuseio perioperatório destes pacientes com alto risco de mortalidade depende de avaliações constantes do paciente, com diagnóstico e condutas precisas. Este assunto é aqui abordado, baseado em nossa experiência com alto índice de sucesso e em trabalhos publicados por centros mundiais com excelentes resultados no tratamento desta doença desafiadora.
Assuntos
Humanos , Cuidados Pós-Operatórios/métodos , Cuidados Pós-Operatórios , Cuidados Pré-Operatórios/métodos , Cuidados Pré-Operatórios , Síndrome do Coração Esquerdo Hipoplásico/complicações , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/mortalidade , Mortalidade HospitalarRESUMO
OBJETIVO: Demonstrar a mortalidade hospitalar de crianças submetidas à operação de Fontan e determinar se a síndrome do coração esquerdo hipoplásico (SCEH) constitui fator de risco para mortalidade. MÉTODOS: De agosto 2001 a junho 2008, 76 pacientes foram submetidos à operação de Fontan, sendo divididos em dois grupos: grupo A com 54 pacientes, sendo 31 (40,7 por cento) portadores de atresia tricúspide e variantes, seis (7,8 por cento) de dupla via de entrada ventrículo esquerdo, quatro (5,3 por cento) de defeito do septo atrioventricular total e 13 (17,1 por cento) de outras cardiopatias congênitas complexas; e grupo B constituído por portadores de SCEH, num total de 22 (28,9 por cento) pacientes. RESULTADOS: Os pacientes do grupo A tiveram média de idade de 6,47 anos ± 4,83 e do grupo B de 2,08 anos ± 0,24 P<0,001; a média de peso foi de 22,42 ± 11,04 contra 12,99 ± 1,2 P=0,016; o tempo médio de CEC foi de 119,5 min contra 113,3 min P=0,0, com tempo médio de pinçamento aórtico de 74,8 min e 73,5 min P= 0,75. O tempo médio de permanência em UTI foi 4,1 dias para o grupo A contra 7,52 dias para o grupo B P= 0,0003. No total (grupo A + B), três pacientes foram a óbito, com mortalidade hospitalar de 3,9 por cento, sendo um paciente portador de SHCE (1,3 por cento) (P<0,001; IC95 por cento 0,001 - 0,228). CONCLUSÃO: Nosso estudo evidencia que, apesar de maior morbidade, a SCEH não constitui um fator de risco para mortalidade hospitalar.
OBJECTIVE: To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality. METHODS: From August 2001 to June 2008, 76 patients underwent Fontan operation and were divided into two groups: group A with 54 patients, 31 (40.7 percent) patients with tricuspid atresia and variants, six (7.8 percent) of double-inlet left ventricle, four (5.3 percent) and atrioventricular septal defect and total 13 (17.1 percent) of other complex congenital heart disease and group B all patients with HLHS a total of 22 (28.9 percent) patients. RESULTS: Group A patients had a mean age of 6.47 years ± 4.83 and group B of 2.08 years ± 0.24 P <0.001, the average weight was 22.42 ± 11.04 against 12.99 ± 1.2 P = 0.016, the mean CPB time was 119.5 min versus 113.3 min P = 0.0, with a mean clamping time of 74.8 min and 73.5 min p = 0.75. The mean ICU stay was 4.1 days for group A versus 7.52 days for group B p = 0.0003. In total (group A + B) three patients died, with hospital mortality of 3.9 percent, and one patient with HLHS (4.54 percent) (P <0.001, 95 percent CI 0.001 to 0.228). CONCLUSION: Our study shows that despite higher morbidity in HLHS is not a risk factor for hospital mortality.
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Masculino , Técnica de Fontan/mortalidade , Mortalidade Hospitalar , Síndrome do Coração Esquerdo Hipoplásico/complicações , Cardiopatias Congênitas/complicações , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Fatores de RiscoRESUMO
OBJECTIVE: To assess neurodevelopment in children with hypoplastic left heart syndrome (HLHS) or other types of functionally single ventricle (univentricular heart, UVH) and to estimate the effect of possible clinical predictors on outcome. STUDY DESIGN: A total of 22 patients with HLHS and 14 with UVH, surviving after palliative surgery performed in the same center, and 42 healthy control subjects were examined at a median age of 30.2 months neurologically and according to the Bayley Scales of Infant Development in a population based prospective neurodevelopmental follow-up study. RESULTS: The mean Mental Developmental Index was significantly lower (89.9) in patients with HLHS than in control subjects (105.5, P<.001), whereas there was no difference between patients with UVH (98.5) and control subjects. The mean Psychomotor Developmental Index in patients with HLHS (80.7, P<.001) as well as in those with UVH (94.5, P=.016) was significantly inferior to that in control subjects (105.3). CONCLUSIONS: In patients with HLHS, mean Mental Developmental Index lags behind control subjects. Psychomotor Developmental Index is impaired in patients with both HLHS and UVH. Routine neurodevelopmental follow-up is recommended for this seriously ill patient group.
Assuntos
Síndrome do Coração Esquerdo Hipoplásico/complicações , Doenças do Sistema Nervoso/etiologia , Pré-Escolar , Feminino , Seguimentos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Masculino , Sistema Nervoso/crescimento & desenvolvimento , Estudos ProspectivosRESUMO
OBJECTIVE: To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality. METHODS: From August 2001 to June 2008, 76 patients underwent Fontan operation and were divided into two groups: group A with 54 patients, 31 (40.7%) patients with tricuspid atresia and variants, six (7.8%) of double-inlet left ventricle, four (5.3%) and atrioventricular septal defect and total 13 (17.1%) of other complex congenital heart disease and group B all patients with HLHS a total of 22 (28.9%) patients. RESULTS: Group A patients had a mean age of 6.47 years ± 4.83 and group B of 2.08 years ± 0.24 P <0.001, the average weight was 22.42 ± 11.04 against 12.99 ± 1.2 P = 0.016, the mean CPB time was 119.5 min versus 113.3 min P = 0.0, with a mean clamping time of 74.8 min and 73.5 min p = 0.75. The mean ICU stay was 4.1 days for group A versus 7.52 days for group B p = 0.0003. In total (group A + B) three patients died, with hospital mortality of 3.9%, and one patient with HLHS (4.54%) (P <0.001, 95% CI 0.001 to 0.228). CONCLUSION: Our study shows that despite higher morbidity in HLHS is not a risk factor for hospital mortality.
Assuntos
Técnica de Fontan/mortalidade , Mortalidade Hospitalar , Síndrome do Coração Esquerdo Hipoplásico/complicações , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/complicações , Humanos , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Masculino , Fatores de RiscoRESUMO
Las cardiopatías congénitas tienen una incidencia de 1 por ciento de la población general, siendo la malformación congénita más frecuente. El síndrome del corazón izquierdo hipoplásico (SCIH), es un cuadro que se caracteriza por el desarrollo incompleto del lado izquierdo del corazón. Comprende varios grados de hipoplasia de la aorta, de la válvula aórtica, de la válvula mitral, de la aurícula izquierda y del ventrículo izquierdo. Su incidencia es 10-15 por ciento del total de las malformaciones cardíacas congénitas. Su etiología es desconocida y el riesgo de recidiva es del 0,5 por ciento. El diagnóstico prenatal se sospecha en el examen de rutina de las 20 a 24 semanas, el diagnóstico definitivo se realiza mediante la ecocardiografía fetal. El pronóstico es grave, falleciendo el 95 por ciento de los fetos aproximadamente en los primeros tres meses de vida. En este trabajo se comunica el diagnóstico, manejo y evolución de los casos de ventrículo izquierdo hipoplásico diagnosticados en nuestro servicio. Se realizó un estudio retrospectivo descriptivo que incluye seis casos con diagnóstico de hipoplasia del ventrículo izquierdo reunidos en nuestro servicio