Pseudocoartación aórtica en niños con ductus arterioso persistente: dos casos de una patología extremadamente infrecuente en menos de 48 horas / Aortic pseudocoarctation in children with persistent ductus arteriosus: two cases of an extremely uncommon pathology in less than 48 hours

Introducción: mientras la persistencia del ductus arterioso (PDA) es la cardiopatía congénita más frecuente, encontrar una pseudocoartación aórtica es muy poco frecuente y lo es más hallarlo en niños y solo hay unos cuantos casos reportados de este hallazgo en niños asociado a PDA. Material y Métodos: se describen los casos de 2 pacientes (1 con diagnóstico preoperatorio y otro sin él) con esta asociación: tanto los datos preoperatorios, los hallazgos transoperatorios, y su manejo trans y postoperatorio que operamos en menos de 48 horas. (AU)

Introduction: while the persistence of ductus arteriosus (PDA) is the most frequent congenital heart disease, finding an aortic pseudocoarctation is very rare and more find it in children and there are only a few reported cases of this finding in children associated with PDA. Material and Methods: we describe the cases of 2 patients (1 with preoperative diagnosis and another without it) with this association: the preoperative data, the transoperative findings, and their trans and postoperative management that we operated on in less than 48 hours. (AU)

Alternative treatment for patent ductus arteriosus: a therapeutic challenge / Tratamento alternativo do canal arterial pervio: um desafio terapêutico

Abstract The ductus arteriosus is a fetal structure that spontaneously closes in 90% of newborns. Patency 3 months after birth is considered a congenital heart disease that, if untreated, can progress to serious cardiovascular complications. This report aims to review an alternative treatment with an aortic endoprosthesis in a 49-year-old man who presented with dyspnea on moderate exertion associated with a heart murmur. He was diagnosed with persistent ductus arteriosus (PDA) with cardiac complications. Clinical management was unsuccessful and surgical treatment was indicated. Endovascular treatment with a thoracic endoprosthesis was indicated and performed successfully. Percutaneous closure is the preferred method in adult patients. Endovascular intervention using an endoprosthesis is a safe and effective option, in addition to being applicable regardless of the anatomy of the PDA. This case demonstrates the natural history of the pathology and presents a safe and effective alternative for its management.

Resumo O canal arterial é uma estrutura fetal que se fecha espontaneamente em 90% dos recém-nascidos. A permeabilidade por mais de 3 meses é considerada uma cardiopatia congênita que, se não tratada, pode evoluir para complicações cardíacas graves. Este trabalho relata um tratamento alternativo com endoprótese aórtica. Trata-se de um homem, 49 anos, que apresentou dispneia aos moderados esforços, associada com um sopro cardíaco. Foi diagnosticado com canal arterial persistente (PCA) já com complicações cardíacas. Tentou-se manejo clínico sem sucesso, sendo indicado tratamento cirúrgico. Optou-se pelo tratamento endovascular com uma endoprótese torácica, o qual foi realizado com sucesso. O fechamento percutâneo é o método preferido em pacientes adultos. A intervenção endovascular com o uso da endoprótese é uma possibilidade segura e eficaz, além de ser aplicável independentemente da anatomia do PCA. Este caso, além de demonstrar a história natural desta patologia, apresenta uma alternativa segura e eficaz empregada no seu manejo.

Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso / Persistent fifth aortic arch with patent ductus arteriosus

La persistencia del quinto arco aórtico es una rara anomalía congénita vascular que consiste en la presencia de una comunicación entre la aorta ascendente y la aorta descendente a través de un conducto arterial; se diagnostica de manera incidental. Informamos de un caso asociado a persistencia de conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding ocasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

Adult patent Ductus Arteriosus complicated by endocarditis and hemolytic anemia / Conducto arterioso patente complicado por endocarditis y anemia hemolítica en un adulto

An adult with a large patent ductus arteriosus may present with fatigue, dyspnea or palpitations or in rare presentation with endocarditis. The case illustrated unique role of vegetation of endocarditis in hemolytic anemia in adult with patent ductus arteriosus (PDA). Despite treatment of endocarditis with complete course of appropriate antibiotic therapy and normality of C- reactive protein, erythrocyte sedimentation rate and leukocytosis and wellness of general condition, transthoracic echocardiography revealed large vegetation in PDA lumen, surgical closure of PDA completely relieved hemolysis, and fragmented red cell disappeared from peripheral blood smear. The 3-month follow-up revealed complete occlusion of PDA and abolishment of hemolytic anemia confirmed by clinical and laboratory examination.

Un adulto con un gran ductus arterioso permeable puede presentar fatiga, disnea y palpitaciones y menos frecuentemente presentar endocarditis. El caso muestra el papel de la vegetación de la endocarditis en la anemia hemolítica con el conducto arterioso patente (CAP) en adultos. A pesar del tratamiento de la endocarditis con la terapia antibiótica completa, la normalidad en la proteína C-reactiva, la tasa de sedimentación globular y leucocitaria, y un estado de bienestar general del paciente, la ecocardiografía trans torácica reveló gran vegetación en el lumen de CAP y el cierre completo quirúrgico de PDA, sin hemólisis y la desaparición de glóbulos rojos fragmentados en frotis de sangre periférica. Los 3 meses de seguimiento revelaron oclusión completa de CAP y la desaparición de la anemia hemolítica confirmada por examen clínico y laboratorio.

Haga su diagnóstico / Make your diagnosis

El diagnóstico es Ductus arterioso permeable (DAP)

Evolución posquirúrgica de la hipertensión arterial pulmonar asociada a conducto arterioso permeable a una altitud de 2680 metros sobre el nivel del mar / Postoperative evolution of pulmonary arterial hypertension associated with patent ductus arteriosus at an altitude of 2680 meters above sea level

Introducción: El conducto arterioso permeable puede provocar hipertensión arterial pulmonar; éste ocupa el primer lugar de las cardiopatías congénitas quirúrgicas en la ciudad de Toluca, México. Métodos: En el periodo comprendido entre enero del 2006 y diciembre del 2011 se operaron 51 pacientes con conducto arterioso permeable: 34 (66.7%) del sexo femenino. Se estudia la reversibilidad de la presión arterial pulmonar y de la dilatación de cavidades izquierdas posterior al cierre quirúrgico del conducto arterioso permeable y se describen las complicaciones posquirúrgicas en la población de la ciudad de Toluca, localizada a una altitud de 2680 metros sobre el nivel del mar (msnm). Resultados: La reversibilidad de la presión pulmonar ocurrió en el 88% de los casos. También se observó una disminución de los diámetros de las cavidades izquierdas, manifestado por una relación de aurícula izquierda/anillo aórtico prequirúrgica de 2.0±0.41 y posquirúrgica de 1.36±0.25 (p<0.001). El cierre exitoso del conducto arterioso permeable inmediato fue del 94.1% y mediato del 98.0%. Se presentaron complicaciones posquirúrgicas en el 13.8% de los pacientes, todas fueron menores, siendo las principales: infección y hematoma de la herida quirúrgica (5.9%), neumotórax (3.9%), neumonía (2.0%) y derrame pleural (2.0%). Conclusiones: La hipertensión arterial pulmonar secundaria a conducto arterioso permeable es reversible en la mayoría de los casos después del cierre quirúrgico, incluso en habitantes de localidades por arriba de los 2500 msnm.

Introduction: Patent ductus arteriosus in Toluca can cause pulmonary hypertension and ranks first surgery of congenital heart disease in Toluca, Mexico. Methods: In the period between January 2006 and December 51 patients with patent ductus arteriosus went to surgery: 34 (66.7%) were female. We study the reversibility of pulmonary arterial pressure and the left cavities dilatation after surgical closure of the ductus arteriosus and postoperative complications are described in a population of Toluca, located at an altitude of 2680 meters over the sea. Results: The reversibility of pulmonary pressure ocurred in 88% of cases. We also observed a decrease in the diameters of the left chambers manifested by a ratio of left atrium/aortic annulus before surgery of 2.0 ± 0.41 SD and after surgery of 1.36 ± 0.25 SD, (p<0.001). The successful closure of the ductus arteriosus was 94.1% immediately and mediate 98.0%. Postoperative complications ocurred in 13.8%, being the main infection and surgical wound hematoma (5.9%), pneumothorax (3.9%), pneumonia (2.0%) and pleural efusion (2.0%). Conclusions: Pulmonary arterial hypertension secundary to patent ductus arteriosus is reversible in the mayority of cases after surgical closure, including residents of village above 2500 meters over the sea.

Fechamento de canais arteriais com o dispositivo CeraTM PDA Occluder: mais uma boa opção na caixa de ferrramentas / Percutaneous closure of patent ductus arteriosus with the CeraTM PDA Occluder: another good option in the toolbox

Introdução: O fechamento percutâneo de persistência dos canais arteriais (PCA) tem sido considerado tratamento de escolha pela maioria dos autores, e diversos dispositivos com diferentescaracterísticas estruturais têm sido utilizados. Apresentamos a experiência inicial do grupo com a nova próteseCeraTM PDA Occluder. Métodos: Entre março de 2010 e dezembro de 2011 foram submetidos ao procedimento pacientes com mais de 5 kg de peso, com PCA diagnosticada por meio deecocardiograma transtorácico com mapeamento de fluxo em cores (ETT), sem defeitos associados. O seguimento foi feito com ETT no primeiro, no terceiro e no sexto meses subsequentes, e, a seguir, anualmente. Resultados: No total, 18 pacientes foram encaminhados para oclusão percutânea, dos quais 61,2% eram do sexo feminino. As médias das idades e dos pesos foram,respectivamente, de 13,7 ± 9,3 anos e 42,9 ± 20,1 kg. Quanto à morfologia, 11 canais foram do tipo A, 6 foram do tipo E,e 1 pertuito residual após cirurgia. A média dos diâmetros foi de 4,2 mm. O implante foi possível em todos os casos.Foram utilizadas 10 próteses 6-4 mm, 1 prótese 8-6 mm, 3 próteses 10-8 mm e 4 próteses 12-10 mm. Todos os canaisestavam completamente fechados por ocasião do primeiro ETT de controle. Não houve óbitos ou complicaçõesnesta casuística. Conclusões: A prótese CeraTM pode ser utilizada para ofechamento de canais de pequeno ou grande calibres com excelente resultado, em crianças e adultos. O procedimento é fácil, seguro, com alta eficácia e baixa morbidade, e pode serexcelente opção para o fechamento percutâneo de PCA. Suas características de flexibilidade sugerem que sejam utilizadas próteses superdimensionadas acima dos 2 mm habitualmenterecomendados.

Tratamento percutâneo vs. cirúrgico da persistência do canal arterial em crianças e adolescentes / Percutaneous vs. surgical treatment of patent ductus arteriosus in children and adolescents

INTRODUÇÃO: Estudos comparando os métodos percutâneo e cirúrgico no tratamento da persistência do canal arterial (PCA) são raros na literatura. Nosso objetivo foi realizar análise comparativa entre os dois métodos de tratamento da PCA, enfatizando os aspectos de eficácia e morbidade. MÉTODOS: Estudo observacional com 2 coortes de crianças e adolescentes > 5 kg e < 14 anos, portadores de PCA, tratados durante um projeto de avaliação de incorporação de novas tecnologias ao Sistema Único de Saúde (SUS), realizado em um hospital cardiológico de excelência, em parceria com o Ministério da Saúde do Brasil. Foi feita análise prospectiva no grupo percutâneo entre 2009 e 2011 e retrospectiva no grupo cirúrgico entre 2006 e 2011. RESULTADOS: Foram incluídos 80 pacientes no grupo percutâneo (60% do sexo feminino) e 39 no grupo cirúrgico (51% do sexo feminino; P = 0,37). A mediana de idade e de peso dos grupos percutâneo e cirúrgico foi de 39,4 meses vs. 25,5 meses (P = 0,04) e de 14 kg vs. 11,1 kg (P = 0,052), respectivamente. No grupo percutâneo, 78 pacientes (92%) tinham PCA do tipo A e o diâmetro mínimo do canal à angiografia foi de 2,5 ± 1,2 mm. As próteses mais utilizadas foram Amplatzer®, molas de Gianturco e CeraTM. A técnica cirúrgica mais utilizada foi a clipagem. A taxa de sucesso dos procedimentos foi de 100% nos dois grupos. O grupo cirúrgico apresentou maiores taxas de complicação, incluindo quilotórax, infecções, necessidade de hemoderivados, hipertensão arterial sistêmica e uso de opioides, como também maior necessidade de terapia intensiva. A mediana do tempo de internação foi de 1,3 dia no grupo percutâneo e de 7,9 dias no grupo cirúrgico (P < 0,01). À alta hospitalar, as taxas de oclusão foram semelhantes nos dois grupos (91% no grupo percutâneo e 87% no grupo cirúrgico; P = 0,71). CONCLUSÕES: Em decorrência da menor morbidade, do menor tempo de internação e da igual eficácia, o tratamento percutâneo da PCA deve ser considerado a modalidade terapêutica de escolha para pacientes selecionados.

BACKGROUND: Studies comparing percutaneous and surgical methods for the treatment of the patent ductus arteriosus (PDA) are rare in the literature. This study aimed to perform a comparative analysis between both PDA treatment methods with emphasis on efficacy and morbidity. METHODS: Observational study with 2 cohorts of children and adolescents > 5 kg and < 14 years of age with PDA, treated under a study protocol to assess the incorporation of novel technologies to the Brazilian Public Health System (Unified Health System - SUS) at an excellence hospital, in partnership with the Brazilian Ministry of Health. A prospective analysis was conducted for the percutaneous group from 2009 to 2011 and a retrospective analysis was performed for the surgical group between 2006 and 2011. RESULTS: Eighty patients were included in the percutaneous group (60% female) and 39 patients in the surgical group (51% female; P = 0.37). The median age and weight of the percutaneous and surgical groups was 39.4 months vs 25.5 months (P = 0.04) and 14 kg vs 11.1 kg (P = 0.052), respectively. In the percutaneous group, 78 patients (92%) had type A PDA and the minimal ductal diameter at angiography was 2.5 + 1.2 mm. Amplatzer®, Gianturco coils and CeraTM were the most commonly used devices. Clipping was the most commonly used surgical technique. The success rate of the procedure was 100% in both groups. The surgical group had higher complication rates, including chylothorax, infections, transfusions, systemic arterial hypertension, use of opioids and a greater need for intensive care. The median hospitalization time was 1.3 days in the percutaneous group and 7.9 days in the surgical group (P < 0.01). Upon discharge, occlusion rates were similar in both groups (91% in the percutaneous group and 87% in the surgical group; P = 0.71). CONCLUSIONS: Due to the lower morbidity, the shorter hospitalization time and similar efficacy, percutaneous treatment of the PDA should be considered the modality of choice for selected patients.

Influência de fatores maternos e neonatais no desenvolvimento da displasia broncopulmonar / Influence of maternal and neonatal factors on bronchopulmonary dysplasia development

OBJETIVO: Analisar as características epidemiológicas da displasia broncopulmonar (DBP) e suas relações com condições maternas e neonatais em uma unidade neonatal. MÉTODOS: Estudo transversal, descritivo e analítico, sendo os dados coletados através da análise de prontuários envolvendo recém-nascidos (RNs) pré-termo com peso ao nascimento inferior a 1.500 g e idade gestacional abaixo de 37 semanas internados em uma unidade neonatal. RESULTADOS: Foram estudados 323 recém-nascidos com média do peso ao nascimento de 1.161 g (± 231 g), idade gestacional entre 24 e 36,5 semanas com incidência da DBP de 17,6 por cento. Entre os RNs que desenvolveram DBP, a média de dias de uso de assistência ventilatória mecânica invasiva (AVMI), ventilação não invasiva (VNI) e oxigênio foi, respectivamente, 17,6 dias, 16,2 dias e 46,1 dias, sendo significativamente maior naqueles RNs que desenvolveram a DBP (p < 0,001). A ocorrência da DBP foi significativamente maior nos RNs com diagnóstico de persistência do canal arterial (PCA). CONCLUSÃO: A incidência da DBP neste estudo foi semelhante à encontrada na literatura mundial. Não houve associação entre a presença de infecção materna e o uso de corticoide antenatal com a DBP. Os RNs que fizeram uso de surfactante tiveram maior incidência da DBP porque tinham menor PN e menor IG. A ocorrência da PCA e DBP simultaneamente está associada ao maior tempo de uso de AVMI, VNI e oxigênio.

OBJECTIVE: To review epidemiological features of bronchopulmonary dysplasia (BPD) and its relationship with maternal and neonatal conditions in a neonatal unit. METHODS: Cross-sectional, descriptive and analytical study involving preterm newborns (NBs) with a birth weight lower than 1,500 g and gestational age under 37 weeks. Data was collected through a review of medical records of these newborns admitted to a neonatal unit. RESULTS: The study included 323 newborns with a mean birth weight of 1,161 g (± 231 g), gestational age between 24 and 36.5 weeks, with a BPD incidence of 17.6 percent. Among the NBs developing BPD, the mean of days using invasive mechanical ventilation (IMV), non-invasive ventilation (NIMV), and supplemental oxygen was 17.6, 16.2, and 46.1 days, respectively, with a time significantly longer for those NBs developing BPD (p < 0.001). BPD occurred significantly more often in NBs with a patent ductus arteriosus (PDA). CONCLUSION: BPD incidence in this study was similar to that found in the literature. No BPD association with maternal infection and antenatal corticosteroid use was found. NBs receiving exogenous surfactant had a higher BPD incidence because they had lower BW and GA. Concomitant occurrence of PDA and BPD is associated with staying longer on IMV, NIMV and supplemental oxygen.

Oclusão percutânea do canal arterial com molas de liberação controlada / Percutaneous occlusion of patent ductus arteriosus with controlled-release coils

INTRODUÇÃO: A oclusão percutânea do canal arterial tem sido realizada com eficácia e segurança, reservando-se o tratamento cirúrgico a situações específicas tecnicamente desfavoráveis à utilização da via percutânea, como a abordagem de prematuros. O dispositivo mola de liberação controlada configurou-se como técnica segura e de baixo custo. Apresentamos a experiência de nosso serviço com esse dispositivo, discutindo aspectos técnicos do implante e características do material utilizado. Método: No período de 2002 a 2009 foram encaminhados 90 pacientes para oclusão percutânea do canal arterial. Todos os pacientes foram diagnosticados em base clinicas, com confirmação por meio de ecocardiografia transtorácica. Foi realizado angiograma imediatamente após o procedimento para verificação da presença de fluxo residual e de possíveis complicações imediatas. Resultados: A idade variou de 4 meses a 36 anos (mediana de 3 anos e 4 meses). O diâmetro do canal arterial variou de 0,2 mm a 6 mm (média de 2,3 mm). Os tipos de canal arterial...

BACKGROUND: The percutaneous occlusion of patent ductus arteriosus is an effective and safe procedure and surgical intervention is restricted to specific unfavorable technical conditions, such as in premature infants. The controlledrelease coil has proven to be a low cost and safe method. We report our experience with this device, discussing technical aspects of the implant and coil characteristics. METHOD: From 2002 to 2009, 90 patients were referred for the percutaneous occlusion of patent ductus arteriosus. The size of the coil was based on the narrowest ductal luminal diameter. Diagnosis of all of the patients was based on clinical evaluation and transesophageal echocardiography. Aortic angiogram was performed immediately after the procedure to assess residual shunt and early complications. RESULTS: Age ranged from 4 months to 36 years (median: 3 years and 4 months). The patent ductus arteriosus diameter ranged from 0.2 mm to 6 mm (mean: 2.3 mm). According to Krichenko's morphological classification, the types of patent ductus arteriosus were divided as follows: type A, 80 (88.8%); type C, 4 (4.6%); and type E, 6 (6.6%). Success was achieved in 97.6% (81/83) of the cases. There was no evidence of hemolysis, endarteritis or vascular obstruction. We observed only one case of embolization where the device was retrieved back into the delivery catheter and repositioned successfully. CONCLUSION: This study showed that the controlled-release coil is safe and effective for the percutaneous occlusion of patent ductus arteriosus.

Pulmonary hemorrhage in preterm neonates: risk factors and management

Pulmonary hemorrhage in neonates is an acute catastrophic event requiring prompt recognition and emergency intervention. It represent one end of a spectrum of disorders characterized by pulmonary oedema. It is characterized by sudden onset, rapid progression into respiratory failure and high mortality. Twenty two babies with documented pulmonary hemorrhage were analysed for risk factors. High frequency oscillatory ventilation [HFOV] as a rescue strategy for respiratory support of those patients was assessed by comparing oxygenation before and after its use. Pulmonary hemorrhage was exclusively seen among preterm babies during the period of the study. Al 22 babies suffered a primary respiratory illness namely respiratory distress syndrome [RDS], all [100%], received surfactant. Fourteen babies [70%] had patent ductus arteriosus [PDA]. Out of 22 babies, 7 babies [2%] expired, 15 babies [68%] survived. Oxygenation improved after HFOV. In survivors, oxygen index [OI] before HFOV was 14.2 +/- 1.1, compared to 14.9 +/- 1.2 in non survivors. While, after HFOV, OI was 6.7 +/- 0.7 in survivors, compared to 12.8 +/- 1.2 in non survivors. P-value<0.05. A similar trend was seen for a/ApO[2]. Pulmonary hemorrhage is a life threatening condition, with high mortality rate. Mortality is more common in extremely low birth weight infants [ELBW], who had RDS, received surfactant therapy and had significant PDA.HFOV is a good option to ventilate those babies who suffered this serious complication

Actualización en presentación y patogénesis de la displasia broncopulmonar / Update on presentation and pathogenesis of brochopulmonary dysplasia

Bronchopulmonary dysplasia (BPD) remains as the most frequent chronic lung disease seen among babies with very low birth weight, contributing to their morbidity and mortality. An increase in the survival of very immature babies due to improvement in pre and post natal care, has resulted in an increase in the number of newborns with BDP, although there have been no changes in the actual incidence of the disease. Objective: Lo describe the evolution of DBP in recent decades, the current definition, and to describe and analyze the risk factors involved in the pathogenesis of this disease. Until a few years ago, the terms BPD and chronic lung disease were used as synonyms. After the workshop sponsored by the National Institute of Health in the United States in 2001, it was recommended that the term BPD be used to describe the pulmonary sequelae of immature babies. Classic severe BPD, as described by Northway et al over forty years ago, has evolved into milder forms of chronic pulmonary damage, the so-called "new BPD", characterized by impairment of alveolarizacion and vascularization of the immature lung in response to multiple injuries. BPD is a multifactorial disease where major risk factors are related to pulmonary immaturity, hyperoxia, baro/volutrauma, as well as inflamation and infection. Genetic susceptibility has recently been shown to be another important risk factor. Conclusion: Bronchopulmonary Dysplasia continues to be the most frequent sequelae affecting low birth weight infants. In the past four decades, the disease has been better defined, and new pathogenetic risk factors have been established.

La Displasia Broncopulmonar (DBP) continúa siendo la enfermedad pulmonar crónica más frecuente que afecta al recién nacido de muy bajo peso, contribuyendo a su morbilidad y mortalidad. El aumento en la sobrevida de los recién nacidos muy inmaduros, debido a la mejoría en el cuidado pre y post natal, ha aumentado el número de recién nacidos con displasia, sin cambios en su incidencia. El objetivo de esta revisión es representar los cambios en la presentación clínica de la DBP en las últimas décadas y describir la definición recientemente instituida, junto con analizar y actualizar los factores de riesgos involucrados en la patogénesis de esta enfermedad. Hasta hace algunos años el término DBP o Enfermedad Pulmonar Crónica se usaban como sinónimos; sin embargo luego del taller patrocinado por Instituto Nacional de Salud de Estados Unidos en el año 2001, se recomendó utilizar el término de DBP para describir las secuelas pulmonares del recién nacido muy inmaduro. La DBP clásica severa descrita por Northway y cols hace más de 40 años, ha evolucionado a formas más leves de daño pulmonar crónico, la denominada "Nueva DBP", caracterizada por un deterioro y/o detención de la alveolarización y vascularización del pulmón inmaduro en respuesta a múltiples injurias. La DBP es una enfermedad multifactorial siendo los principales factores de riesgo la inmadurez pulmonar, la hiperoxia, el baro-volutrauma, y la inflamación-infección. Recientemente se ha demostrado que la susceptibilidad genética puede ser otro factor de riesgo. La ventilación mecánica continúa siendo un importante factor de riesgo, por lo cual debe ser utilizada con precaución y sólo cuando esté claramente indicada. La persistencia del ductus arterioso se ha asociado también a DBP, por lo cual el cierre farmacológico precoz podría disminuir la incidencia de esta complicación. Conclusión: La DBP continúa siendo la secuela pulmonar crónica más frecuente que afecta al RN de muy bajo peso...

A new association: Dandy-Walker's variant and tatralogy of Fallot with atrial septal defect and patent ductus arteriosus and primary hypothyroidy.

An 11-month-old girl who has Dandy-Walker's variant (DWV) associated with tetralogy of Fallot (TOF), atrial septal defect (ASD), patent ductus arteriosus (PDA), and primary hypothyroidy is presented. There has been no report describing a case of DWV associated with TOF, ASD and PDA and primary hypothyroidy. The first case of Dandy-Walker malformation associated with TOF was reported by Kohyama et al in 1988, since then, a few cases were reported in the literature. Our patient is the first reported case.

Oclusão percutânea do pequeno canal arterial com molas de gianturco: impacto da otimização da seleção das molas e dos pacientes e da não-tolerância ao fluxo residual significativo imediato nos resultados / Percutaneous closure of the small patent ductus arteriosus with gianturco coils: impact of the optimal patient and coil selection and the no tolerance policy for significant immediate residual leaks on outcomes

Introdução: A oclusão percutânea do canal arterial persistente (PCA) com implante retrógrado não controlado de molas de Gianturco tem sido freqüentemente empregada. Método: Desde janeiro de 2001, temos aplicado essa técnica em pacientes com canais < 3 mm, dos tipos A, D e E, e não tolerado fluxos residuais imediatos significativos, implantando outra mola. Neste artigo, descrevemos os resultados desse tipo de abordagem. Resultados: Desde janeiro de 2001, 178 pacientes (110 do sexo feminino; mediana de idade e peso: cinco anos e 17 kg, respectivamente) foram submetidos ao procedimento. A média do diâmetro mínimo do PCA foi de 1,8 ± 0,6 mm (0,5 mm a 3,0 mm), sendo 157 do tipo A, nove do tipo D e 12 do tipo E. Em três pacientes, houve embolização inicial das molas com resgate, sendo reimplantadas em dois (taxa de sucesso: 99,4%). Mais de uma mola foi utilizada em 32 (18%) pacientes, com canais significativamente maiores que os restantes (2,3 ± 0,5 mm vs. 1,6 ± 0,5 mm; p < 0,001). Oclusão imediata foi observada em 160 pacientes e 17 (9,6%) possuíam fluxo residual discreto, difuso e de baixa velocidade. Redução de pulsos e transfusão foram complicações observadas em dois pacientes. Um paciente com fluxo residual discreto não retornou. Todos os 176 pacientes, à ecocardiografia, apresentavam oclusão e nenhum apresentava distúrbios de fluxo na aorta ou na artéria pulmonar no seguimento (mediana: seis meses). Conclusão: A tolerância zero para fluxos residuais imediatos e a seleção otimizada das molas e dos pacientes, provavelmente, explicam esses ótimos resultados. O procedimento é simples, custo efetivo, seguro e altamente eficaz.

Introduction: Percutaneous occlusion of the patent ductus arteriosus (PDA) by non-controlled retrogade release of Gianturco coils has been frequently employed. Methods: Since January of 2001, we have applied this technique for patients with type A, D and E PDAs with < 3 mm and have not tolerated significant immediate residual leaks, implanting additional coils. In this paper we report the outcome of this approach. Results: From January 2001, 178 patients (110 females; median age and weight: 5 years and 17 kg, respectively) underwent the procedure. The mean minimal diameter of the PDA was 1.8 ± 0.6 mm (0.5 to 3.0 mm) with 157, 9 and 12 being of types A, D and E, respectively. In 3 patients, there was initial embolization with percutaneous retrieval followed by coil re-implantation in 2 (success rate: 99.4%). More than one coil was required in 32 patients with PDAs significantly larger than the remainder (2.3 ± 0.5 versus 1.6 ± 0.5 mm; p < 0.001). Immediate occlusion was observed in 160 patients with 17 (9.6%) having discrete, diffuse, low velocity residual leaks. Pulse reduction and transfusion were complications observed in 2 patients. One patient with mild residual leak was lost to follow up. On echocardiography, all 176 patients had occlusion and none had flow disturbances in the aorta or in the pulmonary artery on follow up (average 6 months). Conclusion: Zero tolerance for significant immediate residual leaks and optimized selection of coils and patients probably explain these excellent outcomes. The procedure is easy to perform, cost effective, safe and highly efficient.

Avaliação tomográfica pulmonar tardia em prematuros com displasia broncopulmonar e persistência de canal arterial / Late pulmonary tomography assessment in premature infants with bronchopulmonary dysplasia submitted to patent ductus arteriosus managemnent

OBJETIVO: Avaliação tomográfica do parênquima pulmonar de crianças nascidas prematuras de muito baixo peso, com persistência do canal arterial submetidas a tratamento clínico ou cirúrgico e que apresentaram displasia broncopulmonar. MÉTODOS: Entre dezembro de 2006 e janeiro de 2007, 14 crianças foram submetidas à tomografia computadorizada de alta resolução (TCAR), que nasceram prematuras, peso inferior a 1500 gramas, com displasia broncopulmonar (DBP) e persistência do canal arterial (PCA), os quais necessitaram tratamento para oclusão do canal, sendo divididos em dois grupos: A - clínico (n = 6) e B - cirúrgico (n = 8). Nove pacientes eram do sexo masculino e cinco, do feminino, com idade média de 36,5±4,3 meses. As TCAR foram analisadas por dois observadores independentes e as lesões quantificadas em cada paciente. Para análises estatísticas, foi utilizado o teste de Mann-Whitney e considerados significantes valores de p<0,05. RESULTADOS: No grupo A, o achado mais freqüente foi vidro fosco multifocal. No grupo B, as lesões tipo vidro fosco multifocal, atelectasia e áreas de redução da atenuação e dos vasos foram preponderantes (62,5 por cento). Houve diferença estatisticamente significante entre os grupos, com maiores médias nos tempos de intubação, utilização de oxigênio e de internação no grupo B. Porém, quanto ao número de lesões encontradas na TCAR, não houve diferença estatisticamente significante (p=0,0787). CONCLUSÃO: A TCAR empregada tardiamente demonstrou não haver diferença significativa entre o tratamento clínico ou cirúrgico visando ocluir o canal arterial nas lesões do parênquima pulmonar dos recém-nascidos prematuros com PCA que desenvolveram displasia broncopulmonar.

OBJECTIVE: To assess through high-resolution computed tomography the pulmonary parenchyma of children prematurely born with both very low birth weight and patent ductus arteriosus submitted to medical or surgical treatment that developed bronchopulmonary dysplasia. METHODS: Between December 2006 and January 2007, 14 children prematurely born with a weight less than 1500g with bronchopulmonary dysplasia (BPD) and patent ductus arteriosus (PDA) were submitted to high-resolution computed tomography (HRCT). All of them underwent surgical closure of the canal divided into two groups: A - medical (n=6) and B - surgical (n=8). The pool of patients comprised 9 baby boys and 5 girls who were 36.5±4.3 month-old. The HRCT were analyzed by two independent observers and quantified in each patient. The statistical analyses were assessed using the Mann-Whitney test, and p<0.05 was considered statistically significant. RESULTS: Three patients presented normal tomographies, being two of A group and one of B. In A, the most frequent finding was multifocal ground-glass opacity. In B, multifocal ground-glass opacity, atelectasis, and low attenuation areas with relatively decreased number and caliber of vessels were prevalent (62.5 percent). There was a statistically significant difference between both groups, with B having higher averages in the intubation times, use of oxygen, and admission. However, as to the number of injuries found on HRCT there was no statistically significant difference (p=0.0787). CONCLUSION: The lately use of HRCT have shown no significant difference between both medical and surgical treatment aiming at to occlude the PDA in pulmonary parenchyma injuries of premature with PDA that developed bronchopulmonary dysplasia.

Case report vancomycin -resistant Enterococcus faecium VanA phenotype: first documented isolation in India.

In recent years, vancomycin-resistant enterococci, especially Enterococcus faecium has emerged as an important nososcomial pathogen and represents a serious threat to patients with impaired host defences. We report infection with vancomycin-resistant Enterococcus faecium in a 3-year-old child with patent ductus arteriosus. The organism, isolated from a central venous catheter tip, exhibited a high-level resistance to vancomycin (minimum inhibitory concentration > or = 256 microg/ ml) and was also resistant to teicoplanin. The child probably died due to sepsis from this highly resistant organism. To the best of our knowledge, this is the first reported isolation of VanA phenotype Enterococcus faecium in India.

Heart murmur in the first week of life: Siriraj Hospital.

Congenital heart disease (CHD) is the most common form of cardiovascular diseases in children. In Thailand, there has not been available information about congenital heart disease in neonates. Between January 1st and December 31st, 2000, all full-term babies born at Siriraj Hospital with detected heart murmur within the first week of life were consulted to pediatric cardiologists. Echocardiography was performed for diagnosis in every baby. Total livebirths during that period were 11,245 cases. Heart murmurs were detected in 83 cases. The incidence of heart murmur within the first week of life was 7.38:1,000 livebirths. Innocent murmurs were found in 34 cases and echocardiogram revealed no detectable cardiac anomalies (2 cases), mild tricuspid regurgitation (2 cases), physiologic branch pulmonary stenosis (4 cases), and small size PDA (< 2 mm., 26 cases). Forty-nine cases had CHDs. The incidence of CHD was 4.36:1,000 livebirths. At the time of initial diagnosis, 22 cases (44.8%) were asymptomatic. Among these patients, 1 case had serious cardiac anomaly, i.e., tetralogy of Fallot. There were 27 cases with symptoms, including 15 cases (30.6%) with tachypnea, 8 cases (16.4%) with cyanosis and 4 cases (8.2%) with congestive heart failure. The 3 most common cardiac diseases were ventricular septal defect (9 cases, 18.4%), patent ductus arteriosus greater than 2 mm. (8 cases, 16.3%), and atrial septal defect (8 cases, 16.3%). Those with CHDs were treated with anticongestive medications (22 cases, 44.8%), prostaglandin E1 (5 cases, 10.2%), laser pulmonary vulvulotomy (1 case, 2%), palliative surgery within the first week of life (4 cases, 8.2%) and corrective surgery (4 cases, 8.2%). During follow-up for the period of 1 year, 2 cases died from sepsis. Early diagnosis and proper management are important to reduce morbidity and mortality in the newborn with CHD.

Cierre percutáneo de conducto arterioso y de comunicación interventricular muscular con dispositivos de Amplatzer en paciente con hipertensión pulmonar acentuada / Percutaneous closure of ductus arteriosus and muscular ventricular defect with Amplatzer occluder in a patient with severe pulmonary hypertension

El tratamiento quirúrgico de la comunicación interventricular muscular múltiple con lesiones asociadas e hipertensión arterial pulmonar severa tiene una alta morbilidad y mortalidad. El cierre de estos defectos con dispositivo Amplatzer para comunicación interventricular muscular es una alternativa a la cirugía, evitando la utilización de circulación extracorpórea. Presentamos el caso de una mujer de 38 años de edad con signos de falla cardíaca en clase funcional IV (NYHA), con dos comunicaciones interventriculares de tipo muscular, persistencia de conducto arterioso e hipertensión arterial pulmonar severa, que fueron ocluidos con tres dispositivos Amplatzer específicos para cierre de comunicación interventricular muscular, lográndose disminución importante de la presión pulmonar y mejoría de su clase funcional.

Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.