Experiência inicial com o implante percutâneo da valva Venus-P valve® no trato de saída nativo pulmonar / Initial experience with percutaneous Venus-P Valve® valve implantation in the native pulmonary outflow tract

INTRODUÇÃO: O restabelecimento da função valvar pulmonar com implante percutâneo de próteses atualmente é realizado somente nos pacientes portadores de biopróteses, condutos ou homoenxertos posicionados na via de saída do VD. Próteses dedicadas para tratos de saída nativo estão sendo estudadas e implantadas com sucesso em diversos centros mundiais. OBJETIVOS: Apresentar a experiência inicial do implante percutâneo da VENUS-P Valve® em trato de saída nativo pulmonar. Serão demonstrados critérios de escolha dos pacientes, aspectos técnicos relacionados aos procedimentos e resultados de curto prazo. MATERIAIS E MÉTODOS: A prótese VENUS-P Valve® (Venus Medtech, Shanghai) é um stent de nitinol autoexpansível com uma valva de pericárdio porcino montada no seu interior. Possui aspecto de ampulheta e na extremidade distal o stent é de células abertas permitindo o fluxo para ambas artérias pulmonares. O seu sistema de entrega é 22 ou 24F. Os pacientes candidatos são avaliados com ecocardiografia transtorácica, angiotomografia, ressonância magnética e cateterismo cardíaco. São critérios de inclusão a presença de regurgitação pulmonar importante, com volumes diastólicos do VD>130ml/m2, sem estenoses na VSVD ou ramos pulmonares e trato de saída com no máximo 35mm de diâmetro após insuflação de cateter-balão medidor. O ecocardiograma transtorácico é o método padrão de escolha do diâmetro e comprimento da prótese. Todos os procedimentos foram realizados em sala híbrida, com anestesia geral endovenosa, profilaxia para endocardite bacteriana e heparina (TCA>250 seg). Os pacientes realizaram ecocardiografia transtorácica e avaliação clínica 24 horas e 30 dias após os procedimentos. RESULTADOS: Quatro pacientes (3 homens) foram submetidos ao implante percutâneo das valvas pulmonares em 2 dias consecutivos. O peso e idade médio foi de 62,2kg e 25,8 anos. A via de saída teve 30mm em média e a prótese utilizada apresentou diâmetro médio de 32mm. Três próteses tinham 25 mm de comprimento e a última 30mm. Houve sucesso no implante de todos os dispositivos com restabelecimento imediato da função valvar pulmonar. Uma prótese foi considerada em posição mais baixa no trato de saída porém sem interferência em estruturas cardíacas. Ao ecocardiograma de controle todos os dispositivos encontravam-se sem refluxo significativo, com fluxo preservado para as artérias pulmonares bilaterais e sem complicações relacionadas. Não houve complicações ou óbitos relacionados aos procedimentos. CONCLUSÕES: O restabelecimento da função valvar pulmonar em pacientes com trato de saída nativo com o implante percutâneo da VENUS-P Valve® mostrou-se uma excelente alternativa nesta experiência inicial. Os procedimentos são factíveis e seguros quando realizados por grupos experientes e familiarizados com o implante de stents na via de saída pulmonar. Por tratar-se de experiência inicial, acreditamos que um maior número de implantes e análise dos resultados tardios devem ser realizadas para incorporação definitiva destes dispositivos neste grupo selecionado de pacientes. (AU)

Monitoreo hemodinámico funcional en cirugía cardiovascular pediátrica bajo circulación extracorpórea / Hemodynamic monitoring Functional in surgery low pediatric cardiovascular Extracorporeal circulation

El síndrome de respuesta inflamatoria sistémica es una complicación frecuente en el período posoperatorio cardiovascular. Su gravedad es variable y múltiples factores generan una disfunción endotelial que se expresa de diferentes formas en la hemodinamia del paciente, ya sea con cuadros clínicos similares al shock frío o al shock caliente, según el mecanismo fisiológico predominante. Utilizamos un método de monitoreo mínimamente invasivo, configurando perfiles hemodinámicos basales e inducidos por fármacos, de tal modo de realizar un manejo racional de agentes vasoactivos. El propósito de este trabajo es mostrar la aplicación del monitoreo hemodinámico funcional, graficar la variabilidad de la hemodinamia en el período posquirúrgico y evidenciar la respuesta clínica al azul de metileno. Para tal fin, se presenta a modo de ejemplo un paciente con shock vasopléjico.(AU)

Cardiac surgery with cardiopulmonary bypass in children is associated with a systemic inflammatory response syndrome of different degree. The endothelial dysfunction may be followed by acute circulatory dysfunction that results in different hemodynamic profiles like cold or warm shock. Hemodynamic variables were measured with a minimal invasive method of hemodynamic monitoring and they were grouped in order to define profiles and evaluate the response to therapy. The goal of this work is to describe the functional hemodynamic monitoring in the care of pediatric cardiac surgery patients through the presentation of a child with vasodilatory shock and his response to the administration of methylene blue.(AU)

Paediatric and congenital heart disease in South America: an overview.

Congenital heart diseases are one of the most common structural defects present at birth, with an approximate incidence of 8 per 1000 live births. As most countries in South America have a high birth rate, they are a significant public health concern. This paper provides a brief overview of the burden of congenital heart disease in South America, focusing on its local prevalence, facilities for treatment and outcomes after medical, surgical or catheter intervention for the most common diseases.

Paediatric and congenital heart disease in South America: an overview / Paediatric and congenital heart disease in South America: an overview

Congenital heart diseases are one of the most common structural defects present at birth, with an approximate

significant public health concern. This paper provides a brief overview of the burden of congenital heart disease in

South America, focusing on its local prevalence, facilities for treatment and outcomes after medical, surgical or

catheter intervention for the most common diseases.

incidence of 8 per 1000 live births. As most countries in South America have a high birth rate, they are a

Percutaneous ultrasound-guided stenting of the atrial septum in fetal sheep.

OBJECTIVES: Hypoplastic left heart syndrome (HLHS) with a restricitive foramen ovale is associated with high mortality related to fetal left atrial hypertension. Fetal atrial balloon septoplasty has largely failed to achieve adequate decompression due to the small size of the holes produced. We attempted to produce larger atrial communications by stenting the atrial septum in fetal sheep using a minimally invasive technique. METHODS: We used a percutaneous, ultrasound-guided transpulmonary or transhepatic approach to attempt deployment of coronary stents (2-5 mm in diameter and 13-23 mm in length) in the atrial septum primum of 10 normal fetal sheep. RESULTS: Coronary stents were deployed in eight of the 10 fetal sheep (119-139 days' gestation). The transhepatic route was unsuccessful (n = 2). Transpulmonary implantation was only possible in prone fetuses, so three initially supine fetuses underwent external version. Small coronary stents (2.0-2.5 mm in diameter) were deployed rapidly without complication via an 18G needle (n = 4). Larger coronary stents (5 mm in diameter) were delivered through a 4F sheath, but a right pleural effusion occurred in three of the four cases, related to inferior vena cava injury by the balloon. One stent dislodged from a floppy septum. Another was partially occluded within a week by endocardial cells. CONCLUSIONS: Percutaneous ultrasound-guided deployment of coronary stents into the septum primum is feasible without laparotomy or uterine exteriorization in fetal sheep. Partial luminal occlusion by rapid proliferation of endocardial cells is a concern.

Woolly hair and palmoplantar hyperkeratosis may present with hypertrophic cardiomyopathy.

Woolly hair and palmoplantar hyperkeratosis is an infrequent autosomal recessive ectodermal disorder that may be associated with arrhythmogenic right ventricular dysplasia (Naxos disease) or dilated cardiomyopathy. We report the sporadic occurrence of the aforementioned skin-hair anomalies in a patient with physical findings compatible with Noonan's syndrome and hypertrophic cardiomyopathy-an association heretofore not described.

Immunoendocrine aspects of anorexia nervosa.

The T-lymphocyte proliferative response to phytohemoagglutinin (PHA) stimulation was the same in 11 anorexic women, 6 restricted (AN-R) and 5 bulimic (AN-B), and in 11 sex- and age-matched controls, in basal conditions and after acute administration of corticotropin-releasing hormone (CRH). Basal plasma levels of ACTH and cortisol were higher in patients than in controls, while beta-endorphin (beta-EP), growth hormone (GH) and prolactin (PRL) concentrations did not differ in the two groups. ACTH and beta-EP responses to CRH stimulation were blunted in patients, while those of cortisol did not differ in the two groups. ACTH, beta-EP and cortisol responses to the dexamethasone suppression test were impaired in 55% of the patients. Baseline T-lymphocyte concentrations of cholecystokinin-8 (CCK-8) and beta-EP were measured in another group of 56 anorexics, 33 restricted and 23 bulimic, and in 24 controls. CCK-8 values were significantly lower and beta-EP values significantly higher in patients than in controls.

T-lymphocyte cholecystokinin-8 and beta-endorphin concentrations in eating disorders: I. Anorexia nervosa.

Baseline concentrations of cholecystokinin-8 (CCK-8) and beta-endorphin (beta-EP) were measured in T-lymphocytes from 33 restricting patients with anorexia nervosa (AN-R), 23 binging/purging patients with anorexia nervosa (AN-BP), and 24 healthy volunteers. CCK-8 basal values were significantly lower and beta-EP values significantly higher in AN-R and AN-BP patients than in normal volunteers. Levels of the peptides were measured three more times during a 4-month combined cognitive-behavioral/psychopharmacological treatment (nortriptyline or fluoxetine in AN-R, fluoxetine or amineptine in AN-BP). CCK-8 values fluctuated (nonsignificantly) during each treatment, while beta-EP values decreased (to a significant degree only in fluoxetine-treated AN-R patients).

T-lymphocyte concentrations of cholecystokinin-8 and beta-endorphin in eating disorders: II. Bulimia nervosa.

Concentrations of cholecystokinin-8 (CCK-8) and beta-endorphin (beta-EP) in T-lymphocytes of 26 women with bulimia nervosa (BN) and in 26 age- and sex-matched healthy comparison subjects were measured. Ten patients were then treated with 300 mg/day of fluvoxamine, p.o., and five patients were treated with 300 mg/day of amineptine, p.o., for 4 months. Concentrations of the two peptides were measured again after 1, 2, and 4 months of therapy. Basal CCK-8 values were significantly lower in patients than in healthy subjects. During fluvoxamine therapy, CCK-8 values increased, reaching normal levels by month 4 of treatment. No such increase occurred during amineptine therapy. Baseline beta-EP values were normal in the bulimic patients but had declined by month 4 of fluvoxamine therapy.

Combined cognitive-behavioral, psychopharmacological and nutritional therapy in eating disorders. 1. Anorexia nervosa--restricted type.

Twenty-two female patients with anorexia nervosa, restricted type, 14-35 years old, were treated with a 4-month course of combined cognitive-behavioral therapy, nutritional counselling and antidepressant drugs (nortriptyline for 7, fluoxetine for 15). Patients were monitored for body mass index (BMI), for eating disorder symptoms by the Eating Disorder Inventory (EDI) and the Bulimic Investigation Test (BITE) and for depression and anxiety by the Hamilton Rating Scales for Depression and for Anxiety (HRS-D and -A). The scores were determined before and after 1, 2 and 4 months of therapy. BMI, depression, anxiety and EDI scores improved significantly and equally in both groups during the 4 months of therapy, while BITE scores did not change.

Combined cognitive-behavioral, psychopharmacological and nutritional therapy in eating disorders. 2. Anorexia nervosa--binge-eating/purging type.

Thirteen women with anorexia nervosa, binge-eating/purging type (AN-BP), 17-43 years old, were treated with a 4-month course of combined cognitive-behavioral, nutritional and antidepressant therapy (7 with amineptine and 6 with fluoxetine). Patients were monitored before and after 1, 2 and 4 months of treatment for body mass index (BMI), for eating disorder symptoms by the Eating Disorder Inventory (EDI) and the Bulimic Investigation Test (BITE) and for depression and anxiety by the Hamilton Rating Scales for Depression and for Anxiety. BMI, EDI scores, depression and anxiety improved significantly and equally in the two groups during the 4 months of therapy, while BITE scores did not change.

Combined cognitive-behavioral, psychopharmacological and nutritional therapy in bulimia nervosa.

Fifteen women with bulimia nervosa were treated with a 4-month course of combined cognitive-behavioral, nutritional and antidepressant therapy (5 with amineptine and 10 with fluvoxamine). Patients were monitored before and after 1, 2 and 4 months of therapy for body mass index (BMI), for eating disorder symptoms by the Eating Disorder Inventory (EDI) and the Bulimic Investigation Test (BITE), and for depression and anxiety by the Hamilton Rating Scale for Depression and for Anxiety (HRS-D and -A). BITE symptoms and gravity improved significantly and equally in the two groups during the 4 months of therapy. Global EDI scores, depression and anxiety decreased but not significantly. BMI was normal before therapy and did not change during treatment.

[Initial experience in the treatment of bronchiolitis using heparin]. / Prime esperienze sul trattamento della bronchiolite con eparina.

The aim of this paper was to evaluate the efficacy of treatment with heparin in children with bronchiolitis. We studied 30 children (average age 11.3 months) with bronchiolitis; 15 subjects were submitted to a supportive therapy with an adjunct of sodium heparin (50 U/Kg/24 h i.v.) for a time lasting 24-36 hours. The control group was managed with supportive therapy only. We considered the days of the disease and the persistence of symptoms since the admission as peculiar parameters between the two groups. The results appeared to be highly significant in the heparin treated group, with a mean duration of the diseases of 3.06 days compared to 5.53 days of the controls (p less than 0.001). These data are supportive for a possible utilization of the heparin in bronchiolitis but much more experiments are needed to confirm these results.