Experiência inicial de implante percutâneo de válvula pulmonar myval 30, 5mm e 32mm sem pré-stent em vias de saída nativas ectásicas: registros imediatos / Initial experience of percutaneous implantation of 30.5mm and 32mm myval pulmonary valve without pre-stent in ectatic native outflow tracts: immediate records

INTRODUÇÃO: O implante percutâneo de valva pulmonar foi introduzido em 2000 como uma alternativa de baixo risco para pacientes previamente operados com disfunção da via de saída do ventrículo direito (VSVD). Até recentemente, pacientes com VSVD nativas e ectásicas não eram candidatos a essa terapêutica devido à ausência de um dispositivo adequado a esta população no Brasil. O advento da MyVal (Meril Life Sciences, Gujarat, India), uma válvula balão-expansível, com diâmetros de 30,5mm e 32mm proporcionou o tratamento destes pacientes que frequentemente já possuem cirurgias prévias com risco cirúrgico de nova intervenção muito alto. OBJETIVO: Descrever os resultados imediatos da experiência inicial brasileira do implante de MyVal em vias de saída nativas e ectásicas em diferentes centros do país. MÉTODOS: 14 pacientes foram submetidos ao implante percutâneo de MyVal para o tratamento da disfunção da via de saída nativa e ectásica. A idade média dos pacientes foi de 31,3 anos (14-67) e o peso médio de 66,4 Kg (40-90). Todos os pacientes, à exceção de um idoso que havia sido diagnosticado com estenose de valva pulmonar, foram submetidos a, pelo menos, um procedimento cirúrgico previamente. RESULTADOS: O implante foi bem-sucedido em todos os pacientes, tendo sido realizado sem pré-stent. A via de saída mediu em média 27,6mm (20-32) e após a insuflação de balão Cristal 30mm, mediu em média 29,8mm (28-32,5). Foram implantadas 3 valvas 30,5mm com diâmetro final médio de 30,1mm (30- 30,5) e 11 valvas 32mm com diâmetro final de 32,8mm (32-35). Não houve complicações ou mortalidade relacionadas ao procedimento Todos os pacientes apresentaram insuficiência pulmonar trivial no ecocardiograma antes da alta hospitalar. CONCLUSÕES: Atualmente, esta é a maior experiência mundial implante de valva pulmonar com MyVal de calibres 30,5 e 32mm. O implante de valva pulmonar é um procedimento seguro e efetivo que substitui a troca valvar cirúrgica e beneficia principalmente pacientes com toracotomias prévias. O implante da MyVal de calibres de 30,5 e 32mm propiciou o tratamento de pacientes com vias de saída ectásicas e que não possuíam alternativa percutânea atualmente no Brasil. Diâmetro final pode chegar a 35mm sem o surgimento de insuficiência pulmonar. Pré-stent não é necessário na maioria dos casos.

Experiência inicial de implante percutâneo de válvula pulmonar MyVal 30, 5mm e 32 mm sem pré-stent em vias de saída nativas ectásicas: registros imediatos / Initial experience of percutaneous MyVal 30.5mm and 32mm pulmonary valve implantation without pre-stent in ectatic native outflow tracts: immediate records

FUNDAMENTO: O implante percutâneo de valva pulmonar foi introduzido em 2000 como uma alternativa de baixo risco para pacientes previamente operados com disfunção da via de saída do ventrículo direito (VSVD). Até recentemente, pacientes com VSVD nativas e ectásicas não eram candidatos a essa terapêutica devido à ausência de um dispositivo adequado a esta população no Brasil. O advento da MyVal (Meril Life Sciences, Gujarat, India), uma válvula balãoexpansível, com diâmetros de 30,5mm e 32mm proporcionou o tratamento destes pacientes que frequentemente já possuem cirurgias prévias com risco cirúrgico de nova intervenção muito alto. OBJETIVO: Descrever os resultados imediatos da experiência inicial brasileira do implante de MyVal em vias de saída nativas e ectásicas em diferentes centros do país. Amostra e MÉTODOS: 13 pacientes foram submetidos ao implante percutâneo de MyVal para o tratamento da disfunção da via de saída nativa e ectásica. A idade média dos pacientes foi de 32,6 anos (14-67) e o peso médio de 69,3Kg (45-90). Todos os pacientes, à exceção de um idoso que havia sido diagnosticado com estenose de valva pulmonar, foram submetidos a, pelo menos, um procedimento cirúrgico previamente. RESULTADOS: O implante foi bem-sucedido em todos os pacientes, tendo sido realizado sem pré-stent. A via de saída mediu em média 27,7mm (20-32) e após a insuflação de balão Cristal 30mm, mediu em média 29,8mm (28-32). Foram implantadas 4 valvas 30,5mm com diâmetro final médio de 30,3mm (30-30,5) e 9 valvas 32mm com diâmetro final de 33,3mm (32- 35). Não houve complicações ou mortalidade relacionadas ao procedimento Todos os pacientes apresentaram insuficiência pulmonar trivial no ecocardiograma antes da alta hospitalar. CONCLUSÃO: Atualmente, esta é a maior experiência mundial implante de valva pulmonar com MyVal de calibres 30,5 e 32mm. O implante de valva pulmonar é um procedimento seguro e efetivo que substitui a troca valvar cirúrgica e beneficia principalmente pacientes com toracotomias prévias. O implante da MyVal de calibres de 30,5 e 32mm propiciou o tratamento de pacientes com vias de saída ectásicas e que não possuíam alternativa percutânea atualmente no Brasil. Diâmetro final pode chegar a 35mm sem o surgimento de insuficiência pulmonar. Préstent não é necessário na maioria dos caso. Palavras-chave: cateterismo; congênitos; implante de valva pulmonar.

Implante transcateter de bioprótese valvular pulmonar: revisão sistemática da literatura / Transcatheter pulmonary valve implantation: systematic literature review

A correção cirúrgica de algumas cardiopatias congênitas complexas envolve a reconstrução da via de saída do ventrículo direito com a interposição de homoenxertos, biopróteses, enxertos de jugular bovina ou outros condutos valvulados entre o ventrículo direito e o tronco da artéria pulmonar. Apesar de essas cirurgias poderem ser realizadas com baixa mortalidade, a vida útil das válvulas ou dos condutos implantados é normalmente pequena (< 10 anos), seja por degeneração e/ou calcificação. Graus variáveis de estenose pulmonar na maioria das vezes associada a insuficiência pulmonar são consequências da degeneração dos condutos. Em 2000, Bonhoeffer et al. foram os primeiros a relatar o implante transcateter de bioprótese valvular pulmonar (ITVP) com um dispositivo que posteriormente foi denominado de válvula Melody® (Medtronic, Minneapolis, Estados Unidos). A técnica foi inicialmente desenvolvida para limitar a necessidade de múltiplos procedimentos cirúrgicos, substituindo, em última análise, uma nova troca cirúrgica valvular. Estudos subsequentes na Europa e Estados Unidos atestaram para a segurança e eficácia dessa técnica em um número maior de pacientes. Como a Agência Nacional de Vigilância Sanitária (Anvisa) concedeu a aprovação para o uso clínico da válvula biológica pulmonar transcateter Melody® em fevereiro de 2013, consideramos necessária e oportuna a avaliação judiciosa da utilização dessa nova tecnologia antes que ela fosse aplicada em larga escala em nosso país. O objetivo deste estudo foi realizar uma revisão sistemática da literatura sobre o ITVP em pacientes com disfunções de homoenxertos, condutos valvulados e biopróteses implantados cirurgicamente na via de saída do ventrículo direito.

Surgical repair of some complex congenital heart diseases involves reconstruction of the right ventricular outflow tract using homografts, bioprostheses, bovine jugular grafts or other valved conduits between the right ventricle and the main pulmonary artery. Although these surgical procedures may be performed with low mortality rates, the life span of these implanted valves or conduits is usually short (< 10 years) due to either degeneration and/or calcification. Variable degrees of pulmonary stenosis, often associated with pulmonary insufficiency, are consequences of conduit degeneration. In 2000, Bonhoeffer et al. were the first to report the transcatheter pulmonary valve implantation (TPVI) of a bioprosthetic pulmonary valve later named Melody® valve (Medtronic, Minneapolis, USA). The technique was initially developed to limit the need for multiple surgical procedures, and, ultimately, to work as a surrogate of a new surgical valve replacement. Subsequent clinical studies in Europe and the United States confirmed the safety and efficacy of this technique in a larger number of patients. Since the National Sanitary Surveillance Agency (Agência Nacional de Vigilância Sanitária - Anvisa) granted approval for clinical use of the Melody® transcatheter pulmonary biological valve in February 2103, we deemed that a judicious assessment of this new technology was timely and necessary before the widespread use in our country. The objective of this study was to perform a systematic literature review on the use of TPVI in patients with dysfunctional homografts, valved conduits and bioprostheses implanted surgically in the right ventricular outflow tract.

[22q11.2 deletion syndrome and complex congenital heart defects]. / Síndrome de deleção 22q11 e cardiopatias congênitas complexas.

OBJECTIVE: Investigate the frequency of 22q11 deletion syndrome among patients with complex congenital heart disease. METHODS: A prospective and consecutive cohort of patients with complex heart defects was evaluated in their first hospitalization at a cardiac intensive care unit of a pediatric hospital. For each patient a protocol of demographic and clinical evaluation was filled. High resolution karyotype and 22q11 microdeletion by fluorescence in situ hybridization was investigated. The heart defects were classified by a cardiologist of the study. RESULTS: The cohort comprised 66 patients. Karyotypic anomalies were observed in 5 patients (7.6%), however none of those was the 22q11 deletion. Evaluation by means of FISH was successful in 65 patients and 22q11 microdeletion was identified in 2 (3.1%). Of the 66 patients with complex defects, 52 were carriers of conotruncal malformations and in 51 the 22q11 microdeletion analysis by FISH was successful. Both 22q11 microdeletion carriers belonged to this group, representing a frequency of 3.9%. They presented tetralogy of Fallot. CONCLUSION: 22q11DS is a frequent abnormality among patients with complex and conotruncal heart defects. Variations of the 22q11DS frequency among studies seem to be mainly associated to criteria for patient selection and specific characteristics of the population in analysis.

Síndrome de deleção 22q11 e cardiopatias congênitas complexas / 22q11.2 deletion syndrome and complex congenital heart defects

OBJETIVO: Verificar a frequência da síndrome de deleção 22q11 (SD22q11) entre pacientes portadores de cardiopatia congênita do tipo complexa. MÉTODOS: A amostra foi constituída por uma coorte prospectiva e consecutiva de pacientes com cardiopatia complexa em sua primeira hospitalização em uma unidade de tratamento intensivo cardiológica de um hospital pediátrico. Para cada paciente foi preenchida uma ficha de avaliação, com coleta de dados clínicos, e realizado o cariótipo de alta resolução e técnica de hibridização in situ fluorescente (FISH) com pesquisa de microdeleção 22q11. Os defeitos cardíacos foram classificados por um cardiologista participante do estudo. RESULTADOS: A amostra foi composta de 66 pacientes. Quanto à análise cariotípica, alterações foram observadas em cinco pacientes (7,6 por cento); contudo, nenhum deles apresentava deleção 22q11. A avaliação pela técnica de FISH pôde ser realizada com sucesso em 65 pacientes, sendo que a microdeleção 22q11 foi identificada em dois (3,1 por cento). Dos 66 pacientes com defeitos complexos, 52 eram portadores de malformações do tipo conotruncal, sendo que em 51 a pesquisa para microdeleção 22q11 foi realizada. Os dois pacientes portadores da microdeleção 22q11 fizeram parte deste grupo, representando uma frequência de 3,9 por cento. Eles apresentavam tetralogia de Fallot. CONCLUSÃO: A SD22q11 é uma anormalidade frequente entre pacientes com cardiopatias congênitas complexas e conotruncais. Variações da frequência da SD22q11 entre os estudos parecem estar associadas, principalmente, com a forma adotada para a seleção da amostra e às características da população em análise.

OBJECTIVE: Investigate the frequency of 22q11 deletion syndrome among patients with complex congenital heart disease. METHODS: A prospective and consecutive cohort of patients with complex heart defects was evaluated in their first hospitalization at a cardiac intensive care unit of a pediatric hospital. For each patient a protocol of demographic and clinical evaluation was filled. High resolution karyotype and 22q11 microdeletion by fluorescence in situ hybridization was investigated. The heart defects were classified by a cardiologist of the study. RESULTS: The cohort comprised 66 patients. Karyotypic anomalies were observed in 5 patients (7.6 percent), however none of those was the 22q11 deletion. Evaluation by means of FISH was successful in 65 patients and 22q11 microdeletion was identified in 2 (3.1 percent). Of the 66 patients with complex defects, 52 were carriers of conotruncal malformations and in 51 the 22q11 microdeletion analysis by FISH was successful. Both 22q11 microdeletion carriers belonged to this group, representing a frequency of 3.9 percent. They presented tetralogy of Fallot. CONCLUSION: 22q11DS is a frequent abnormality among patients with complex and conotruncal heart defects. Variations of the 22q11DS frequency among studies seem to be mainly associated to criteria for patient selection and specific characteristics of the population in analysis.

Espectro óculo-aurículo-vertebral em pacientes com defeitos cardíacos congênitos / Oculo-auriculo-vertebral spectrum in patients with congenital heart defects / Espectro óculo-aurículo-vertebral en pacientes con defectos cardíacos congénitos

FUNDAMENTO: Há poucos estudos avaliando a frequência do espectro óculo-aurículo-vertebral (EOAV) nos pacientes com defeitos cardíacos congênitos (DCC). OBJETIVO: Verificar a frequência do EOAV em uma amostra de pacientes com malformações cardíacas graves. MÉTODOS: Avaliamos uma coorte prospectiva de pacientes com DCC, admitidos em uma unidade de terapia intensiva (UTI) cardiopediátrica no Brasil. O diagnóstico de EOAV foi feito com base em dados clínicos, considerando critérios padrões. Os pacientes que preenchiam esses critérios foram submetidos ao cariótipo de alta resolução com bandamento GTG e hibridização fluorescente in situ para a microdeleção 22q11.2. O teste exato de Fisher (P < 0,05) foi usado para as análises estatísticas. RESULTADOS: Durante o período de avaliação, 330 pacientes foram hospitalizados pela primeira vez na UTI, mas trinta deles não participaram deste estudo. Dos 300 pacientes que constituíram a amostragem final, o EOAV foi verificado em três casos (1 por cento). Todos apresentaram estudos citogenéticos normais. CONCLUSÃO: O EOAV parece ser uma condição frequente em pacientes com DCC. Contudo, não podemos excluir a possibilidade de que a frequência de EOAV encontrada em nosso estudo possa ter sido subestimada devido à baixa taxa de detecção pré-natal de DCC e ao acesso limitado dos pacientes ao tratamento médico apropriado em nosso meio. Estudos prospectivos futuros com critérios clínicos bem definidos e pacientes com defeitos leves e graves serão importantes para avaliar o papel do EOAV na população em geral de indivíduos com malformações cardíacas.

BACKGROUND: There have been few studies evaluating the frequency of oculo-auriculo-vertebral spectrum (OAVS) in patients with congenital heart defects (CHDs). OBJECTIVE: To verify the frequency of OAVS in a sample of patients with major heart malformations. METHODS: We evaluated a prospective cohort of patients with CHD admitted in a pediatric cardiac intensive care unit (ICU) in Brazil. The diagnosis of OAVS was made based on the clinical data, considering standard criteria. The patients that met these criteria were submitted to high resolution GTG-Banding karyotype and fluorescence in situ hybridization for 22q11.2 microdeletion. Fisher's exact test (P < 0.05) was used for the statistical analysis. RESULTS: During the period of evaluation, 330 patients were hospitalized for the first time in the ICU, but thirty of them did not participate in the study. Of the 300 patients that constituted the final sample, OAVS was verified in 3 cases (1 percent). All presented normal cytogenetic studies. CONCLUSION: OAVS seems to be a frequent condition among patients with CHDs. However, we cannot exclude the possibility that the frequency of OAVS found in our study might have been underestimated due to the low rate of prenatal detection of CHDs and the limited access of patients to appropriate health care in our region. Future prospective studies with well defined clinical criteria and subjects with mild and major defects will be important to assess the role of OAVS in the general population of subjects with heart malformations.

FUNDAMENTO: Hay pocos estudios evaluando la frecuencia del espectro óculo-aurículo-vertebral (EOAV) en los pacientes con defectos cardíacos congénitos (DCC). OBJETIVO: Verificar la frecuencia del EOAV en una muestra de pacientes con malformaciones cardíacas graves. MÉTODOS: Evaluamos una cohorte prospectiva de pacientes con DCC, admitidos en una unidad de terapia intensiva (UTI) cardiopediátrica en el Brasil. El diagnóstico de EOAV fue hecho con base en datos clínicos, considerando criterios estándares. Los pacientes que llenaban esos criterios fueron sometidos al cariotipo con bandeamiento GTG en alta resolución e Hibridización fluorescente in situ para la microdelección 22q11.2. El Test Exacto de Fisher (P < 0,05) fue usado para los análisis estadísticos. RESULTADOS: Durante el período de evaluación, 330 pacientes fueron hospitalizados por primera vez en la UTI, pero treinta por ciento de ellos no participó de este estudio. De los 300 pacientes que constituyeron el muestreo final, el EOAV fue verificado en tres casos (1 por ciento). Todos presentaron estudios citogenéticos normales. CONCLUSIÓN: El EOAV parece ser una condición frecuente en pacientes con DCC. Con todo, no podemos excluir la posibilidad de que la frecuencia de EOAV encontrada en nuestro estudio pueda haber sido subestimada debido a la baja tasa de detección pre-natal de DCC y al acceso limitado de los pacientes al tratamiento médico apropiado en nuestro medio. Estudios prospectivos futuros con criterios clínicos bien definidos y pacientes con defectos leves y graves serán importantes para evaluar el papel del EOAV en la población en general de individuos con malformaciones cardíacas.

[Oculo-auriculo-vertebral spectrum in patients with congenital heart defects]. / Espectro óculo-aurículo-vertebral em pacientes com defeitos cardíacos congênitos.

BACKGROUND: there have been few studies evaluating the frequency of oculo-auriculo-vertebral spectrum (OAVS) in patients with congenital heart defects (CHDs). OBJECTIVE: to verify the frequency of OAVS in a sample of patients with major heart malformations. METHODS: we evaluated a prospective cohort of patients with CHD admitted in a pediatric cardiac intensive care unit (ICU) in Brazil. The diagnosis of OAVS was made based on the clinical data, considering standard criteria. The patients that met these criteria were submitted to high resolution GTG-Banding karyotype and fluorescence in situ hybridization for 22q11.2 microdeletion. Fisher's exact test (P < 0.05) was used for the statistical analysis. RESULTS: During the period of evaluation, 330 patients were hospitalized for the first time in the ICU, but thirty of them did not participate in the study. Of the 300 patients that constituted the final sample, OAVS was verified in 3 cases (1%). All presented normal cytogenetic studies. CONCLUSION: OAVS seems to be a frequent condition among patients with CHDs. However, we cannot exclude the possibility that the frequency of OAVS found in our study might have been underestimated due to the low rate of prenatal detection of CHDs and the limited access of patients to appropriate health care in our region. Future prospective studies with well defined clinical criteria and subjects with mild and major defects will be important to assess the role of OAVS in the general population of subjects with heart malformations.

22q11.2 deletion syndrome in patients admitted to a cardiac pediatric intensive care unit in Brazil.

The 22q11.2 deletion syndrome (22q11DS) is one of the most recognizable causes of congenital heart defects (CHDs), but the frequency varies in non-selected populations. The purpose of this study was to determine the incidence and clinical features of patients with CHD and 22q11DS admitted to a pediatric cardiology intensive care unit in Brazil. In a prospective study, we evaluated a consecutive series of 207 patients with a CHD following a clinical protocol and cytogenetic analysis by high resolution karyotype and fluorescent in situ hybridization (FISH). 22q11DS was identified in four patients (2%), a frequency similar to studies that evaluated subjects with major CHDs in other countries. Despite this similarity, we believe that the low rate of prenatal identification of CHDs and the limited access of these patients to appropriate diagnosis and care, which occur in our region, could have had an influence on this frequency. It is possible that 22q11DS patients with a severe CHD could have died before having a chance to access a tertiary hospital, leading to an underestimate of its frequency.

Hybrid management for hypoplastic left heart syndrome : an experience from Brazil.

Initial surgical reconstruction for hypoplastic left heart syndrome (HLHS) is associated with satisfactory outcomes only in a few referral centers. Moreover, there is a persistent high-risk period for sudden death while the patient waits for the next surgical procedure. The development of a less invasive approach, so-called "hybrid," postponing a major surgery outside the neonatal period, might reduce the immediate and late surgical burden on these patients. This is a retrospective study of a contemporary series of patients with HLHS seen in two separate institutions. Patients with HLHS or its variants who underwent a "hybrid" management were included in the study. Data are described as the mean and standard deviation or absolute numbers and percentage, as appropriate. From January 2004 to June 2006, 15 patients (10 male; 5 +/- 3.8 days old and 2.9 +/- 0.5 kg) were included in the study. Ten had both mitral and aortic atresia; the ascending aorta and atrial septal defect measured 2.5 +/- 1.4 and 4.9 +/- 1.2 mm, respectively. There were six hospital survivors after stage I (mortality rate 60%). During the interstage period, all but one patient needed additional procedures. One patient died of bacterial meningitis 4 months after stage I. Four patients were submitted to stage II operation at 6.6 +/- 0.5 months of age and one is waiting for the operation. All four required early reinterventions for pulmonary artery stenosis. Only one was discharged home and was not yet submitted to the third stage. The hybrid approach for HLHS was associated with poor results in this early experience from two independent institutions in a developing country. This might have been related to infrastructure and technical problems, as well as our own learning curve. Institutions working under the same conditions might face similar problems during their initial experience.

Endovascular stenting for aortic coarctation.

Surgery had been the traditional treatment for native coarctation of the aorta, one of the most common cardiovascular congenital malformations. As a less invasive mode of treatment, balloon angioplasty has emerged as an alternative to surgery but has not gained universal acceptance due to its rates of restenosis secondary to vessel recoil and concerns over aortic wall injury resulting in aneurysm formation. To overcome these problems, endovascular stents were introduced in the management of this condition. The early- and intermediate-term results are encouraging, with low rates of restenosis and complications. In this article, the authors review the current evidence on coarctation stenting and discuss future trends in this area.

Stenting vs. balloon angioplasty for discrete unoperated coarctation of the aorta in adolescents and adults

More information is needed to clarify whether stenting is superior to balloon angioplasty (BA) for unoperated coarctation of the aorta (CoA). From September 1997, 21 consecutive adolescents and adults (24 6 11 years) with discrete CoA underwent stenting (G1). The results were compared to those achieved by BA performed in historical group of 15 patients (18 6 10 years; P = 0.103; G2). After the procedure, systolic gradient reduction was higher (99% 6 2% vs. 87% 6 17%; P = 0.015), residual gradients

lower (0.4 6 1.4 vs. 5.9 6 7.9 mm Hg; P = 0.019), gain at the CoA site higher (333% 6 172% vs. 190% 6 104%; P = 0.007), and CoA diameter larger (16.9 6 2.9 vs. 12.9 6 3.2 mm; P < 0.001) in G1. Aortic wall abnormalities were found in eight patients in G2 (53%) and in one in G1 (7%; P < 0.001). There was no major complication. Repeat catheterization (n = 33) and/or MRI (n = 2) was performed at a median follow-up of 1.0 year for G1 and 1.5 for G2 (P = 0.005). Gradient reduction persisted in both groups, although higher late gradients were seen in G2 (median of 0 mm Hg for G1 vs. 3 for

G2; P = 0.014). CoA diameter showed no late loss in G1 and a late gain in G2 with a trend to being larger in G1 (16.7 6 2.9 vs. 14.6 6 3.9 mm; P = 0.075). Two patients required late stenting due to aneurysm formation or stent fracture in G1. Aortic wall abnormalities did not progress and one patient required redilation in G2. Blood

pressure was similar in both groups at follow-up (126 6 12/81 6 11 for G1 vs. 120 6 15/80 6 10 mm Hg for G2; P = 0.149 and 0.975, respectively). Although satisfactory and similar clinical outcomes were observed with both techniques, stenting was a better means to relieve the stenosis and minimize the risk of developing immediate aortic

wall abnormalities.

Stenting vs. balloon angioplasty for discrete unoperated coarctation of the aorta in adolescents and adults.

More information is needed to clarify whether stenting is superior to balloon angioplasty (BA) for unoperated coarctation of the aorta (CoA). From September 1997, 21 consecutive adolescents and adults (24 +/- 11 years) with discrete CoA underwent stenting (G1). The results were compared to those achieved by BA performed in historical group of 15 patients (18 +/- 10 years; P = 0.103; G2). After the procedure, systolic gradient reduction was higher (99% +/- 2% vs. 87% +/- 17%; P = 0.015), residual gradients lower (0.4 +/- 1.4 vs. 5.9 +/- 7.9 mm Hg; P = 0.019), gain at the CoA site higher (333% +/- 172% vs. 190% +/- 104%; P = 0.007), and CoA diameter larger (16.9 +/- 2.9 vs. 12.9 +/- 3.2 mm; P < 0.001) in G1. Aortic wall abnormalities were found in eight patients in G2 (53%) and in one in G1 (7%; P < 0.001). There was no major complication. Repeat catheterization (n = 33) and/or MRI (n = 2) was performed at a median follow-up of 1.0 year for G1 and 1.5 for G2 (P = 0.005). Gradient reduction persisted in both groups, although higher late gradients were seen in G2 (median of 0 mm Hg for G1 vs. 3 for G2; P = 0.014). CoA diameter showed no late loss in G1 and a late gain in G2 with a trend to being larger in G1 (16.7 +/- 2.9 vs. 14.6 +/- 3.9 mm; P = 0.075). Two patients required late stenting due to aneurysm formation or stent fracture in G1. Aortic wall abnormalities did not progress and one patient required redilation in G2. Blood pressure was similar in both groups at follow-up (126 +/- 12/81 +/- 11 for G1 vs. 120 +/- 15/80 +/- 10 mm Hg for G2; P = 0.149 and 0.975, respectively). Although satisfactory and similar clinical outcomes were observed with both techniques, stenting was a better means to relieve the stenosis and minimize the risk of developing immediate aortic wall abnormalities.

Obliteration of a competitive forward flow from the ventricle after a bidirectional cavopulmonary shunt with an Amplatzer duct occluder.

A 7-month-old boy who was status post-bidirectional Glenn shunt implantation with residual ventricular flow to the pulmonary arteries (PA) presented with massive edema of the head and systemic desaturation soon after surgery. After test occlusion, an Amplatzer duct occluder was implanted at the PA banding site with subsequent decrease in the PA pressure, resolution of the edema and extubation.

Oclusão percutânea de grande fístula artério-venosa pulmonar / Pulmonary arteriovenous big fistula percutaneous occlusion

É descrito um caso de grande fístula arterio-venosa pulmonar congênita em uma menina de 6 anos, com cianose presente desde o nascimento. A fístula foi ocluída com sucesso no laboratório de cateterismo utilizando-se uma umbrella de Rashkind e múltiplos coils de Gianturco desta forma, hove resolução do shunt intra-pulmonar, evitando-se o tratamento cirúrgico com lobectomia ou pneumectomia.

Management of a large pseudoaneurysm secondary to balloon dilation for native coarctation of the aorta with coil occlusion after stent implantation in a child.

We report a case of a 9-year-old girl status post-balloon dilation for native coarctation of the aorta who had a large aortic pseudoaneurysm 1 year after the initial procedure. The aneurysm was occluded with multiple coils after stent implantation to the aorta. The technique and possible advantages of this novel approach are discussed.

Management of a large pseudoaneurysm secondary to ballon dilatation for native coarctation of the aorta with coil occlusion after stent implantation in a child

We report a case of a 9-year-old girl status post-balloon dilatation for native coarctation of the aorta who had a large aortic pseudoaneurysm 1 year after the initial procedure. The aneurysm was occluded with multiple coils after stent implantation to the aorta. The anewrysm as ocludded with multiple coils after stent implantation to the aorta. The technique and possible advantages of this novel approach are discussed...

A new use for the amplatzer duct occluder device.

We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel.

A new use for the Amplatzer duct occluder device / Um novo uso para a prótese Amplatzer de canal arterial

We report a case in which the Amplatzer device for percutaneous occlusion of ductus arteriosus was successfully used for occluding a large systemic-pulmonary collateral vessel in a patient who had previously undergone surgery for correction of pulmonary atresia and ventricular septal defect (Rastelli technique), and was awaiting the change of a cardiac tube. In the first attempt, the device embolized to the distal pulmonary bed and, after being rescued with a Bitome, it was appropriately repositioned with no complications and with total occlusion of the vessel