RESUMEN
BACKGROUND: In infants with complete atrioventricular canal (CAVC) defects, post-operative left atrioventricular valve regurgitation (LAVVR) is a known major cause of morbidity and mortality and a common indication for re-operation. However, there is scarce data to identify risk factors for poor outcomes. Our study aims to find echocardiographic characteristics that predict post-operative LAVVR at discharge and 1-year follow-up. METHODS: Retrospective cohort study of patients with initial CAVC repair at our hospital who were followed for 1 year between 2013 and 2022. Patients with major co-morbid conditions were excluded. Serial echocardiograms were reviewed. Anatomic details, quantitative and qualitative measure of LAVVR including the number of regurgitant jets, regurgitant jet length and vena contracta width, and ventricular function were collected. The time points measured include pre-operative transthoracic echocardiogram (TTE), post-operative transesophageal echocardiogram (PO-TEE), routine protocol based post-operative day 1 (POD1) TTE, discharge TTE and 1-year post-operative (1yPO) TTE. Paired t-tests, chi-square analysis, and linear regression analysis were performed comparing measured variables to LAVVR outcomes. RESULTS: Fifty-two patients were included; 92% had Trisomy 21. The majority were classified as Rastelli A (71%), others Rastelli C (29%). Only two patients had moderate or greater LAVVR pre-operatively. The mean age at repair was 125 ± 44 days. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year after backward stepwise regression. Of those with < moderate LAVVR on PO-TEE, 20% had worsening to ≥ moderate at discharge, but only 9% remained that way at 1 year. Of those with ≥ moderate LAVVR on PO-TEE, 40% improved to < moderate by 1 year. Two patients who worsened at 1 year, both secondary to likely cleft suture dehiscence. Only one patient required reoperation in the immediate post-operative period secondary to severe LAVVR due to suture dehiscence. Routine protocol-based POD1 echo did not have any association with altered outcomes. CONCLUSION: Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year. A significant percentage (40%) of patient with ≥ moderate LAVVR on PO-TEE improved to < moderate by 1 year. Furthermore, routine protocol-based POD1 echo did not have any association with altered outcomes.
Asunto(s)
Ecocardiografía , Insuficiencia de la Válvula Mitral , Complicaciones Posoperatorias , Humanos , Masculino , Femenino , Estudios Retrospectivos , Lactante , Insuficiencia de la Válvula Mitral/cirugía , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/fisiopatología , Ecocardiografía/métodos , Factores de Riesgo , Estudios de Seguimiento , Ecocardiografía Transesofágica/métodos , Defectos de los Tabiques Cardíacos/cirugía , Defectos de los Tabiques Cardíacos/complicaciones , Defectos de los Tabiques Cardíacos/diagnóstico por imagen , Preescolar , Valor Predictivo de las PruebasRESUMEN
AIM: The aims of this nationwide retrospective cohort study were to determine the time and causes of detection of severe congenital heart defects (CHDs) in live-born infants in Norway between 2017 and 2020. METHODS: Information regarding live-born infants with severe CHDs was retrieved from national registries and medical records. RESULTS: A total of 219 776 infants were born in Norway from 01.01.2017 to 31.12.2020. Severe CHDs were diagnosed in 442 (0.2%) infants. Of these, 376 (85%) infants were diagnosed either prenatally (n = 203, 46%) or before discharge from hospital after birth (n = 173, 39%). A total of 56 (13%) infants were discharged with undetected CHDs. Time of detection was unknown in 10 cases (2%). The most frequent undetected CHDs at discharge were coarctation of the aorta/aortic arch hypoplasia (n = 24), atrioventricular septal defect (n = 13), anomalous pulmonary venous connection (n = 5) and coronary artery anomalies (n = 4). Seven (13%) children with undetected CHD experienced circulatory collapse out of hospital. CONCLUSION: Most infants with severe CHDs in Norway were identified prior to hospital discharge. However, some infants were discharged undiagnosed. Awareness of undetected CHDs and immediate cardiac assessment in infants with signs of circulatory failure early in life are still important.
Asunto(s)
Síndrome de Down , Cardiopatías Congénitas , Defectos de los Tabiques Cardíacos , Lactante , Niño , Humanos , Estudios Retrospectivos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/etiología , Defectos de los Tabiques Cardíacos/complicaciones , Síndrome de Down/complicaciones , Noruega/epidemiologíaRESUMEN
Abstract Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Defectos de la Almohadilla Endocárdica/cirugía , Defectos de los Tabiques Cardíacos/cirugía , Válvula Mitral/anomalías , Resultado del Tratamiento , Implantación de Prótesis de Válvulas Cardíacas/métodos , Defectos de la Almohadilla Endocárdica/complicaciones , Anuloplastia de la Válvula Mitral/métodos , Defectos de los Tabiques Cardíacos/complicaciones , Válvula Mitral/cirugíaRESUMEN
O ecocardiograma transesofágico (ECO-TE) é o método mais utilizado para guiar otratamento percutâneo da comunicação interatrial (CIA) e do forame oval (FOP), mas a necessidade de um outro profissional para realizá-lo e de anestesia geral constituem inconvenientes para seu emprego. Oecocardiograma intracardíaco (ECO-IC) apresenta-se como alternativa ao ECO-TE, pois pode ser realizado pelo próprio operador e demanda apenas anestesia local, com leve ou nenhuma sedação. Nosso objetivo foi relatar a experiência do serviço com a oclusão de CIA/FOP guiada por ECO-IC. Métodos: O ECO-IC utiliza cateter de ultrassom, que é introduzido por via venosa em câmaras cardíacas direitas e, por meio de posicionamento variável do transdutor, obtém as imagens adequadas para a intervenção. Foram avaliadas as taxas de sucesso do procedimento e as complicações. Resultados: De 2011 a 2015, foram realizados 201 procedimentos guiados pelo ECO-IC, sendo 139 empacientes com CIA e 62 com FOP. A maioria dos pacientes era do sexo feminino (64,2%), as idades variaramde 7 a 78 anos (36,6 ± 19,3 anos) e o peso variou de 28 a 92 kg (62,5 ± 13,0 kg). Foram utilizadas próteses Occlutech Figulla®, e todas as intervenções tiveram sucesso, com tempos de fluoroscopia de 5,7 ± 2,4 minutos e tempo de procedimento de 21,5 ± 6,4 minutos. Dois pacientes (2,0%) apresentaram taquicardia supraventricular transitória e outros dois pacientes evoluíram com fístula arteriovenosa na via de acesso, com resolução espontânea no primeiro mês. Conclusões: O ECO-IC forneceu informações anatômicas precisas para guiar o fechamento da CIA/FOP com sucesso e eliminou as principais desvantagens do ECO-TE...
Transesophageal echocardiography (TEE) is the most widely used method to guide the percutaneous treatment of atrial septal defect (ASD) and patent foramen ovale (PFO), but the necessity of another professional to perform it and the need for general anesthesia are potential disadvantages. Intracardiac echocardiography (ICE) is seen as an alternative to TEE, as it can be performed by the interventionist and requires only local anesthesia with mild or no sedation. The aim of this study was to report our experience with ASD/PFO occlusion guided by ICE. Methods: The ICE uses an ultrasound catheter, which is intravenously inserted in the right heart chambers and acquires images for the intervention through variable positioning of the transducer. Success and complication rates of the procedure were evaluated. Results: From 2011 to 2015, 201 procedures guided by ICE were performed, comprising 139 in patients with ASD and 62 in those with PFO. Most patients were female (64.2%), ages ranged from 7 to 78 years (36.6 ±19.3 years), and weight ranged from 28 to 92 kg (62.5 ± 13.0 kg). Occlutech Figulla® prostheses were used and all interventions were successful, with fluoroscopy time of 5.7 ± 2.4 minutes and procedure time of 21.5 ± 6.4 minutes. Two patients (2.0%) had transient supraventricular tachycardia and two others had arteriovenous fistula at the access site, with spontaneous resolution in the first month of follow-up. Conclusions: ICE provided accurate anatomical information to guide the closure of the ASD/PFO and successfully eliminated the main drawbacks of TEE...
Asunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/terapia , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/terapia , Ecocardiografía Transesofágica/métodos , Foramen Oval , Prótesis e Implantes , Arteria Femoral/cirugía , Cateterismo Cardíaco , Defectos de los Tabiques Cardíacos/complicaciones , Defectos de los Tabiques Cardíacos/terapia , Estudios Retrospectivos , Heparina/administración & dosificación , Atrios CardíacosRESUMEN
Abstract Introduction: Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect. Objective: To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect. Methods: We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%). Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%. Results: At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%). The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26), unlike patients without such abnormalities (P=0.016). During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02). However, after a multivariate analysis, none of the factors reached significance. Conclusion: None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit ...
Resumo Introdução: A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular. Objetivo: Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular. Métodos: Avaliamos os resultados em 51 pacientes consecutivos menores de 14 anos com defeito de septo atrioventricular incompleto, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 4,1 anos e a do peso de 13,4 Kg; 37,2% tinham síndrome de Down; antes da operação, 23 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (45,1%); anormalidades na valva atrioventricular foram encontradas em 17,6% dos casos; anuloplastia foi realizada em 21,6% dos pacientes. Resultados: Após a correção cirúrgica, 12 casos apresentaram insuficiência da valva atrioventricular esquerda pelo menos moderada (23,5%). A variância entre os graus de insuficiência da valva atrioventricular esquerda pré e pós-operatória nos pacientes com anormalidades na valva atrioventricular não teve significância estatística (P=0,26), ao contrário daqueles sem tais anormalidades (P=0,016). Pela análise univariada, apenas a ausência de síndrome de Down teve significância estatística (P=0,02). Porém, após análise multivariada, nenhum dos fatores teve significância. Conclusão: Nenhum dos fatores estudados foi determinante de insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após a correção de defeito de septo atrioventricular incompleto na população avaliada. Pacientes sem anormalidades na valva atrioventricular ...
Asunto(s)
Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Defectos de los Tabiques Cardíacos/cirugía , Insuficiencia de la Válvula Mitral/etiología , Complicaciones Posoperatorias/etiología , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Síndrome de Down/fisiopatología , Métodos Epidemiológicos , Defectos de los Tabiques Cardíacos/complicaciones , Insuficiencia de la Válvula Mitral/fisiopatología , Insuficiencia de la Válvula Mitral , Periodo Posoperatorio , Periodo Preoperatorio , Complicaciones Posoperatorias/fisiopatología , Reoperación , Factores de Riesgo , Factores de Tiempo , Resultado del TratamientoRESUMEN
El origen anómalo de una de las ramas pulmonares desde la aorta ascendente en vez de ocurrir desde el tronco pulmonar, también conocido como hemitruncus constituye una singularidad cardiovascular congénita muy rara, pues ocurre en aproximadamente 0,05% de todas las forma de cardiopatías congénitas (¹). En la forma más frecuente depresentación de este defecto la rama pulmonar derecha (RPD) emerge directamente de la aorta ascendente en un 70%-80% de los casos (²). La ecocardiografía con Doppler color es el método de mayor utilidad para realizar el diagnóstico, los cortes apicales, paresternales y subcostales en eje corto de grandes vasos así como las visiones supraesternales longitudinales son esenciales para conseguir una completa evaluación del defecto. Presentamos una secuencia de imágenes ecocardiográficas de una paciente que es referida tardíamente a nuestro centro para evaluación cardiológica con historia de cianosis desde el nacimiento, fatiga y episodios repetidos de hemoptisis que resultó ser portadora de un origen anómalo de rama derecha de arterial pulmonar emergiendo desde la aorta ascendente y que desarrolló hipertensión arterial pulmonar en rangos suprasistémicos con resistencia vascular pulmonar también muy elevada.
Anomalous origin of one pulmonary branch from the ascending aorta rather than occurring from the pulmonary trunk, also known as hemitruncus is a very rare congenital cardiovascular entity, which occurs in approximately 0.05% of all forms of congenital heart disease (¹). In the most common presentation of this defect, the right pulmonary branch (RPB) emerges directly from the ascending aorta in 70%-80% of cases (²). Color Doppler echocardiography is the most useful method to establish the diagnosis. Apical, subcostal, and parasternal short axis views of the great vessels and longitudinal suprasternal views are essential for a complete assessment of the defect. We present a sequence of echocardiographic images of a patient referred late to our center for a cardiac evaluation with a history of cyanosis from birth, fatigue and repeated episodes of hemoptysis that resulted from having an anomalous origin of the right pulmonary artery branch emerging from the ascending aorta with development of pulmonary arterial hypertension exceeding systemic levels along with very high pulmonary vascular resistance.
Asunto(s)
Humanos , Adolescente , Femenino , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/complicaciones , Cateterismo Cardíaco/métodos , Defectos de los Tabiques Cardíacos/cirugía , Defectos de los Tabiques Cardíacos/complicaciones , Defectos de los Tabiques Cardíacos/diagnóstico , Ecocardiografía/métodos , Hipertensión Pulmonar/patología , Ventrículos Cardíacos/lesionesRESUMEN
Introducción: La evolución a largo plazo de pacientes con síndrome de Down y cardiopatía congénita es poco conocida. Material y métodos: Estudio retrospectivo de pacientes con síndrome de Down y cardiopatía congénita y pacientes con defecto del tabique auriculoventricular y sin trisomía 21. Resultados: Entre 2004 y 2008 se hizo seguimiento a 317 pacientes con cardiopatía congénita; 19 pacientes (6%) con edad media de 26.8 ± 8.1 años tenían síndrome de Down. Las cardiopatías congénitas más frecuentes fueron el defecto del tabique auriculoventricular (63%) y la comunicación interventricular (26%); 10 (53%) habían sido intervenidos quirúrgicamente en la infancia, tres de ellos con defecto auriculoventricular completo requirieron reintervención en la edad adulta (dos por obstrucción al tracto de salida del ventrículo izquierdo y uno por insuficiencia severa de la válvula auriculoventricular izquierda); cuatro (21%) desarrollaron hipertensión arterial pulmonar, con mejoría del grado funcional al recibir tratamiento con bosentan; dos (10.5%) tuvieron endocarditis bacteriana; dos (10.5%) fallecieron. No existieron diferencias en el grado de insuficiencia de la válvula auriculoventricular izquierda (1.5 ± 0.9 versus 1.7 ± 0.8, p = 0.689) entre pacientes con defecto auriculoventricular con y sin síndrome de Down. Conclusiones: La insuficiencia de la válvula auriculoventricular izquierda y la obstrucción al tracto de salida del ventrículo izquierdo fueron las causas más frecuentes de reintervención en la edad adulta en pacientes con defectos del tabique auriculoventricular.
BACKGROUND: Long-term complications of Down syndrome patients with congenital heart disease are poorly known. METHODS: We carried out a retrospective study of Down syndrome patients with congenital heart disease and patients with atrioventricular septal defect with and without Down syndrome. RESULTS: Between 2004 and 2008, 317 patients with congenital heart disease were followed-up in the Adult Congenital Heart Disease Unit. Of these patients, 19 (6%) with a mean age of 26.8 +/- 8.1 years had Down syndrome. Atrioventricular septal defect was the most frequent congenital heart disease(63%) followed by ventricular septal defect (26%). Ten patients (53%) were operated on during childhood. Three of these patients required reoperation during adulthood (two patients due to left ventricle outflow tract obstruction and one patient due to left atrioventricular valve insufficiency). Four patients (21%) had Eisenmenger syndrome with improvement of functional class in those treated with bosentan, two patients (10.5%) had bacterial endocarditis and two patients (10.5%) died. No significant differences were seen in left atrioventricular valve insufficiency between atrioventricular septal defect in patients with and without Down syndrome (1.5 +/- 0.9 vs. 1.7 +/- 0.8, p = 0.689). CONCLUSIONS: Left atrioventricular valve insufficiency and left ventricle outflow tract obstruction were the most frequent long-term complications requiring surgical reintervention in patients with atrioventricular septal defect.
Asunto(s)
Humanos , Masculino , Femenino , Adulto Joven , Cardiopatías/complicaciones , Cardiopatías/congénito , Defectos de los Tabiques Cardíacos/complicaciones , Síndrome de Down/complicaciones , Estudios de Seguimiento , Estudios RetrospectivosRESUMEN
El cierre percutáneo del foramen oval permeable esuna de las opciones terapéuticas propuestas para el ictuscriptogénico. El objetivo del presente trabajo es describir la experiencia de nuestro centro. Hasta febrero de 2006, se ha tratado con cierre percutáneo de foramen oval permeable a 52 pacientes que habían presentado uno o más ictus o ataques isquémicos transitorios criptogénicos. El procedimiento se realizó con anestesia y guiado por ecocardiografía transesofágica (ETE). La tasa de éxito primario fue del 100%. Inmediatamente tras el implante deldispositivo, en 27 pacientes no se observó paso derechaizquierda por ETE. Tras un seguimiento clínico medio de 26 meses en 49 pacientes, ninguno ha tenido nuevos episodios isquémicos ni complicaciones atribuibles al dispositivo. El seguimiento ecocardiográfico mostró en todos un sellado completo del foramen oval
Percutaneous closure of patent foramen ovale (PFO)has been proposed as a therapeutic option forcryptogenic stroke. The aim of this article was to describe the experience with this treatment at our center. Up until February 2006, percutaneous closure of PFO was carried out in 52 patients who presented with one or more cryptogenic strokes or transient ischemic attacks. The procedure was carried out under anesthesia and was guided by transesophageal echocardiography. Theoverall success rate was 100%. Transesophagealechocardiography carried out immediately after deviceimplantation found no evidence of right-to-left shunting in 27 patients. During a mean follow-up of 26 months in 49 patients, none presented with a new ischemic episode or with complications attributable to the device.Echocardiographic follow-up showed that the foramenovale had been completely sealed in all cases (AU)
Asunto(s)
Humanos , Accidente Cerebrovascular/cirugía , Defectos del Tabique Interatrial/cirugía , Accidente Cerebrovascular/etiología , Defectos de los Tabiques Cardíacos/complicaciones , Ecocardiografía TransesofágicaRESUMEN
Os defeitos do septo atrioventricular total (DSAVT) representam 4 por cento das mal formações cardíacas e acima de 50 por cento dos defeitos observados na síndrome de Down (SD)¹. A apresentação clínica é de insuficiência cardíaca precoce na infância e hipertensão pulmonar por hiperfluxo. Raramente a cianose é observada e sugere hipertensão pulmonar ou associação à tetralogia de Fallot³, dupla via de saída de ventrículo direito², anomalia de Ebstein4, drenagem anômala de cava esquerda persistente em átrio esquerdo (Barbero Marcial, comunicação pessoal). Crianças com SD são particularmente difíceis de avaliação por apresentarem obstrução de vias aéreas superiores, que podem contribuir com o aumento da resistência pulmonar observada no cateterismo cardíaco. A presença de cianose pré-operatória constitui-se um desafio ao tratamento cirúrgico devido ao risco de hipertensão pulmonar irreversível com falência ventricular direita com, a correção dos defeitos intracardíacos.
Atrioventricular septal defects account for 4 percent of congenital cardiac malformations and over 50 percent of cardiac defects seen in Down syndrome¹. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle², Ebstein anomaly³, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction4, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.
Asunto(s)
Humanos , Femenino , Lactante , Cianosis/etiología , Síndrome de Down/complicaciones , Insuficiencia Cardíaca , Defectos de los Tabiques Cardíacos/cirugía , Procedimientos Quirúrgicos Cardíacos , Ecocardiografía , Electrocardiografía , Defectos de los Tabiques Cardíacos/complicaciones , Oximetría , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
We describe an adult patient with a large atrial septal defect, an atrial septal aneurysm and thrombus formation on a transvenous right atrial pacing lead. Because of right-to-left shunting through the atrial septal defect, she developed multiple systemic emboli to the spleen and left kidney (with infarcts), to the left leg, and probably to the brain as a cerebrovascular accident. The fundamental guiding principle of avoiding endocardial pacing leads in patients with congenital intracardiac communications, was violated in this patient, leading to serious dire consequences and complications.
Asunto(s)
Humanos , Femenino , Anciano , Aneurisma Cardíaco/complicaciones , Embolia/etiología , Defectos de los Tabiques Cardíacos/complicaciones , Marcapaso Artificial/efectos adversos , Infarto Cerebral/etiología , Trastornos Cerebrovasculares/etiología , Ecocardiografía Transesofágica , Embolia/diagnóstico , Estudios de Seguimiento , Defectos de los Tabiques Cardíacos , Defectos de los Tabiques Cardíacos/cirugía , Infarto/etiología , Infarto del Bazo/etiología , Riñón/irrigación sanguínea , Factores de Tiempo , Tomografía Computarizada por Rayos XRESUMEN
OBJETIVOS: Comprarar o grau de repercussäo, fatores responsáveis e época de aparecimento dos sintomas do defeito do septo atrioventricular (DSAV), em pacientes com e sem síndrome de Down...
Asunto(s)
Humanos , Masculino , Femenino , Recién Nacido , Lactante , Defectos de los Tabiques Cardíacos/complicaciones , Hipertensión Pulmonar/fisiopatología , Síndrome de Down/complicaciones , Defectos de los Tabiques Cardíacos/cirugía , Estudios Retrospectivos , Síndrome de Down/fisiopatologíaRESUMEN
A associaçäo de doença de Ebstein e defeito septal atrioventricular é extremamente rara, havendo 13 casos na literatura e nenhum em nosso meio. Descrevemos uma paciente de 15 anos com diagnóstico eco e angiográfico e confirmaçäo cirúrgica. A näo identificaçäo de uma das lesöes poderia comprometer o planejamento e resultado cirúrgico
Asunto(s)
Humanos , Femenino , Adolescente , Anomalía de Ebstein/complicaciones , Defectos de los Tabiques Cardíacos/complicaciones , Anomalía de Ebstein/cirugía , Anomalía de Ebstein/diagnóstico , Defectos de los Tabiques Cardíacos/cirugía , Defectos de los Tabiques Cardíacos/diagnósticoAsunto(s)
Humanos , Anticoagulantes/uso terapéutico , Cateterismo/instrumentación , Bombas Centrífugas , Circulación Extracorporea , Filtros , Defectos de los Tabiques Cardíacos/complicaciones , Homeostasis , Oxigenadores , Oxigenadores de Membrana , Estenosis de la Válvula Pulmonar/complicaciones , Bombas , Instrumentos QuirúrgicosRESUMEN
Säo apresentados 3 casos com estenose pulmonar congênita, defeitos septais intracardíacos e tuberculose. Em um caso, com estenose pulmonar valvar e defeito do septo atrial, "shunt" direito-esquerdo e severa cianose, a tuberculose manifestou-se muito discreta, afetando ambas as pleuras e o ápice do pulmäo direito, antes da valvotomia e fechamento da comunicaçäo interatrial. O exame radiológico praticado 1 ano e 5 meses após demonstrou lobite superior direita com escavaçöes e a baciloscopia foi positiva. Em outro caso, praticou-se anastomose Blalock-Taussig numa criança de 5 anos com estenose pulmonar infundibular associada à comunicaçäo interventricular e a infecçäo tuberculosa manifestou-se no exame radiológico praticado quando o paciente atingiu 18 anos de idade, observando-se na radiografia lesäo cavitária no ápice esquerdo. No terceiro caso, com estenose pulmonar valvar e defeito do septo ventricular, "shunt" bidirecional entre os ventrículos e ausência de cianose, a tuberculose instalou-se como processo primária agudo (quadro pneumônico) no final do primeiro mês de vida. Em seguida, tornou-se crônico e causou fibrotórax esquerdo, com forte retraçäo costofrênica mediastinal, conforme radiografias obtidas aos 7 anos de idade. Consideraçöes quanto a freqüência, hipótese sobre a predisposiçäo para a tuberculose nos caso com estenose pulmonar congênita, isolada ou associada com outras malformaçöes, sua importância na indicaçäo da cirurgia e na evoluçäo pós-operatória, säo feitas de acordo com a literatura ao alcance