Retrospective analysis of percutaneous intervention of the renal artery in transplanted kidneys in children and adolescents at a tertiary public hospital.

BACKGROUND: This study evaluated the long-term effects of percutaneous intervention in children and adolescents with transplant renal artery stenosis (TRAS). METHODS: Twenty patients had significant stenosis (>50%) and underwent percutaneous transluminal angioplasty (PTA/stenting) (TRAS group-intervention); 14 TNS (non-significant group -control) patients did not have significant stenosis (≤50%) and were treated clinically. The combined primary endpoints were death from all causes and late graft failure. The secondary endpoints were serum creatinine (SCr), systolic blood pressure (SBP), and diastolic blood pressure (DBP). RESULTS: No statistically significant difference was found between TRAS-Intervention(N = 20) and TNS groups-Control (N = 14) for these clinical parameters: deaths, 1 (5.0%) vs. 0 (0.0%) (p = 1.000) and graft loss, 4 (20.0%) vs. 2 (14.3%) (p = 1.000). For the secondary endpoints, after 1 month and 1 year the values of SCr, SBP, and DBP were similar between the two groups but not statistically significant. DISCUSSION: In the TRAS group (intervention), the stent implantation was beneficial for treating refractory hypertension and reducing blood pressure (BP) in children and adolescents. Despite the outcomes being similar in the two groups, it can be inferred that the patients in the TRAS group (intervention) would have had a worse outcome without the percutaneous intervention. CONCLUSION: TRAS treatment with stenting can be considered for children and adolescents. Because the sample in the present study comprised of only a specific population, further studies are needed for generalization. TRIAL REGISTRATION: The trial was registered at clinictrials.gov with trial registration number NCT04225338.

Pharmacological Modulation of the Ca2+/cAMP/Adenosine Signaling in Cardiac Cells as a New Cardioprotective Strategy to Reduce Severe Arrhythmias in Myocardial Infarction.

Acute myocardial infarction (AMI) is the main cause of morbidity and mortality worldwide and is characterized by severe and fatal arrhythmias induced by cardiac ischemia/reperfusion (CIR). However, the molecular mechanisms involved in these arrhythmias are still little understood. To investigate the cardioprotective role of the cardiac Ca2+/cAMP/adenosine signaling pathway in AMI, L-type Ca2+ channels (LTCC) were blocked with either nifedipine (NIF) or verapamil (VER), with or without A1-adenosine (ADO), receptors (A1R), antagonist (DPCPX), or cAMP efflux blocker probenecid (PROB), and the incidence of ventricular arrhythmias (VA), atrioventricular block (AVB), and lethality (LET) induced by CIR in rats was evaluated. VA, AVB and LET incidences were evaluated by ECG analysis and compared between control (CIR group) and intravenously treated 5 min before CIR with NIF 1, 10, and 30 mg/kg and VER 1 mg/kg in the presence or absence of PROB 100 mg/kg or DPCPX 100 µg/kg. The serum levels of cardiac injury biomarkers total creatine kinase (CK) and CK-MB were quantified. Both NIF and VER treatment were able to attenuate cardiac arrhythmias caused by CIR; however, these antiarrhythmic effects were abolished by pretreatment with PROB and DPCPX. The total serum CK and CK-MB were similar in all groups. These results indicate that the pharmacological modulation of Ca2+/cAMP/ADO in cardiac cells by means of attenuation of Ca2+ influx via LTCC and the activation of A1R by endogenous ADO could be a promising therapeutic strategy to reduce the incidence of severe and fatal arrhythmias caused by AMI in humans.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics.

OBJECTIVE: Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. METHOD: In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. RESULTS: The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. CONCLUSION: TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

The complexity of having a child with a congenital heart defect in a developing country: A qualitative study of parental needs.

PURPOSE: To explore parental needs related to their experiences of living with a child with congenital heart defect (CHD) since the diagnosis. DESIGN AND METHODS: An interpretative qualitative study developed with nine parents of children between the ages of five months and 11 years diagnosed with CHD. Interviews were conducted at an ambulatory pediatric cardiology centre. Data were analyzed using inductive thematic analysis. The consolidated criteria for reporting qualitative research (COREQ) was followed for quality reporting. This research was approved by a research committee. RESULTS: One central theme emerged, namely 'A desire to feel safe in dealing with the demands of CHD,' along with two main themes. The first is 'Looking for effective relations with healthcare professionals and health care systems' which encompasses three types of need: (1) need for continuous, clear and accurate information; (2) need for resolution and the support of services such as the public health care system and social services; (3) need for trust in health care professionals. The second theme is 'Looking for balance in daily life' with two main needs: (1) maintain family functioning and (2) learn to deal with the child and CHD. CONCLUSION: The main parental needs are related to their interactions with healthcare professionals and healthcare systems, highlighted by a need for information and trust relationships to feel safe in their daily lives. PRACTICE IMPLICATIONS: Our results imply rethinking the nurse presence in ambulatory care, implementation of a family-centered care approach and addressing the diverse and multifaceted experiences and needs of parents and children with CHD in different health care contexts.

Ecocardiograma: da Solicitação do Exame pelo Pediatra à Realização pelo Cardiologista Pediátrico / Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Fundamento: O ecocardiograma é uma ferramenta diagnóstica utilizada para avaliar anomalias cardíacas e esclarecer dúvidas quanto ao caráter benigno de certos achados do exame clínico cardiovascular. Objetivos: Identificar os principais motivos da solicitação do ecocardiograma pelo pediatra e avaliar o grau de ansiedade dos familiares gerado por essa conduta. Métodos: Foram incluídos pacientes com idade inferior a 18 anos, sem diagnóstico prévio de cardiopatia e encaminhados para o primeiro exame de ecocardiograma. Os familiares responsáveis pelos pacientes responderam a um questionário para avaliar o grau de ansiedade deles desde o pedido até a realização do ecocardiograma, com pontuação entre 0 e 19. Os dados foram analisados através de porcentagens, desvio padrão e teste T de Student. Resultados: Estudados 30 pacientes com idade média de 4,45 anos. O motivo de encaminhamento mais prevalente foi sopro cardíaco (23 casos), dos quais 70% não tiveram a hipótese de cardiopatia congênita confirmada após o ecocardiograma. A pontuação média no questionário de ansiedade foi 11 ± 6, sendo relacionado significativamente à presença de cardiopatia, com média de 13,0 ± 5,3 vs. 9,3 ± 5,2 no grupo sem a hipótese da mesma (p = 0,007). Conclusão: O achado de sopro foi o principal motivo para solicitação de ecocardiograma pelo pediatra. A confirmação de cardiopatia foi maior nos menores de 1 ano e com achado de sopro. Apesar do grau de ansiedade ter sido maior no grupo dos pacientes que tiveram o diagnóstico de cardiopatia congênita, este não foi desprezível nos familiares no grupo dos pacientes sem cardiopatia

Background: Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination. Objectives: To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct. Methods: We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student's t test. Results: Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007). Conclusion: The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease

Indicações atuais para anticoagulacão em crianças com Cardiopatias Congênitas / Current indications for anticoagulation in children with Congenital Heart Disease

Os avanços nas técnicas cirúrgicas e manejo clínico aumentaram a sobrevida das crianças com cardiopatia congênita. Eventos tromboembólicos representam importante causa de morbimortalidade nestes pacientes, principalmente no pós-operatório da cirurgia de Fontan e implante de próteses, acrescido do risco adicional do seu tratamento. Particularidades próprias da idade, fatores hemodinâmicos e limitações na investigação de trombose contribuem para a dificuldadede definir diretrizes para anticoagulação em crianças. Os medicamentos mais usados na fase aguda são: heparina não fracionada e de baixo peso molecular, enquanto em longo prazo os antagonistas da vitamina K, principalmente a varfarina. O manejo seguro de anticoagulação nesta faixa etária é dificultado pela estreita faixa terapêutica, interação alimentar, uso simultâneo de vários medicamentos, além da dificuldade de punção venosa frequente e, com frequência os níveis desejáveis não são atingidos. Ainda faltam dados que deem suporte para recomendação de anticoagulação em crianças. Os novos anticoagulantes como os inibidores diretos da trombina e os inibidores seletivos do fator Xa, são empregados em adultos, principalmente com fibrilação atrial, o uso pediátrico ainda não está definido. Esta revisão sumariza as indicações atuais do uso de anticoagulantes e antiagregantes plaquetários em crianças com cardiopatia congênita.

Advances in surgical techniques and clinical management have led to increased survival ofchildren with congenital heart disease. Thromboembolic events represent a major cause of morbidity and mortality in these patients, particularly following Fontan surgery and the implantation of prosthesis, added to the risk of their treatment. Characteristics such as age, hemodynamic factors and limitations in the investigation of thrombosis have contributed to the difficulty of defining guidelines for anticoagulation in children. The most commonly used medications in the acute phase are and low molecular weight heparin, and in the long term, vitamin K antagonists, particularly warfarin. The safe management of anticoagulation in this age range is hindered by the narrow therapeutic range, food interactions, simultaneous use of other medications, and the difficulty of frequent venous puncture. As a result, the desired levels are often not reached. There is also a lack of data to support the recommendation of anticoagulation in children. The pediatric use of the new anticoagulants, such as direct thrombin inhibitors and selective factor Xa inhibitors used in adults, particularly with atrial fibrillation, is not yet defined. This review summarizes the current indications of the use of anticoagulants and platelet antiaggregants in children with congenital heart disease.

Newborns with congenital heart diseases: epidemiological data from a single reference center in Brazil.

OBJECTIVE: to describe the epidemiological data of the population born with the diagnosis of Congenital Heart Disease (CHD); to compare diagnoses made using fetal echocardiography with the findings from postnatal echocardiography or anatomopathological examination of the heart; and to evaluate mortality among newborns that underwent surgical treatment. METHODS: this was a cohort study with information gathered from the medical records of the pregnant women and their newborns diagnosed with CHD during the fetal or postnatal periods, between January 2008 and December 2012. Means, standard deviations and maximum and minimum values were calculated for the quantitative variables. Relative and absolute values were calculated for the qualitative variables. The heart malformations were categorized in four groups: complex lesions, significant lesions, minor lesions and others. RESULTS: we detected postnatal incidence of CHD of 1.9% at our service. The mean maternal age was 28.3 years and 10 (21.3%) of the pregnant women were ≥ 35 years old. The mean gestational age at the time of performing the fetal echocardiogram was 27.8 weeks. Mean gestational age at delivery was 38 weeks, and the mean weight of the newborns was 2,644.5 grams. Regarding the diagnosis of CHD, there were: 23 complex lesions (39%); 15 significant lesions (26%); 10 minor lesions (17%); 4 other lesions (7%) and 6 normal anatomies (10%). The diagnosis of CHD made on the fetus and postnatally coincided in 77.6% of the cases. A total of 27 patients (60%) underwent surgery, and the outcome was neonatal death in five cases. CONCLUSION: we detected postnatal incidence of CHD of 1.9%, and it was more common among older pregnant women and with late detection in the intrauterine period. Complex heart diseases predominated, thus making it difficult to have a good result regarding neonatal mortality rates.

Hipertensão pulmonar nas cardiopatias congênitas: implicações terapêuticas e prognósticas / Pulmonary hypertension in congenital cardiopathy therapeutic and prognostic implications

A Hipertensão arterial pulmonar (HAP) é uma importante complicação na evolução das cardiopatias congênitas de hiperfluxo pulmonar. A síndrome de Eisenmenger (SE) é o espectro máximo desta condição e se caracteriza por inversão do shunt com cianose e irreversibilidade até o presente momento. Nos últimos anos, novas opções terapêuticas se expandiram e a terapia avançada (TA), com o uso de vasodilatadores pulmonares, tornou-se uma opção atraente para estes pacientes. Estudos recentes têm demonstrado melhora da qualidade de vida e capacidade física desta população em uso de TA. Entretanto, a melhor forma de tratamento ainda é a prevenção pela correção cirúrgica da cardiopatia na época adequada. A presente revisão tem como objetivo abordar o prognóstico e tratamento na HAP associada a cardiopatias congênitas na atualidade e discutir sobre futuras abordagens terapêuticas, como a possibilidade de posterior correção cirúrgica de pacientes em TA, antes considerados inoperáveis.

Uso da angiografia rotacional 3D (3D-RA) em portadores de cardiopatias congênitas: experiência de 53 casos / Use of 3D rotational angiography (3D-RA) in congenital heart disease: experience with 53 patients

Introdução: Os avanços no diagnóstico e tratamento das cardiopatias congênitas estão relacionados com os avanços na obtenção de imagens. As representações anatômicas obtidas com tomografia computadorizada, ressonância magnética e ecocardiografia têm ajudado, mas esses métodos não conseguem obter de forma precisa os dados hemodinâmicos. A angiografia rotacional 3D (3D-RA), um novo método de reconstrução tridimensional realizado no laboratório de cateterismo, tem sido amplamente utilizada em procedimentos neurológicos e urológicos. Nosso objetivo foi avaliar o desempenho da 3D-RA na avaliação das cardiopatias congênitas. Método: Revisão dos exames dos pacientes com cardiopatia congênita encaminhados para estudo hemodinâmico e que foram submetidos a aquisição de imagens pela 3D-RA. Foi utilizado equipamento Philips Allure FD10 e contraste de baixa osmolaridade para as angiografias. Resultados: No total, foram avaliados 53 pacientes, sendo utilizados, em média, 2,2 ± 1,1 ml/kg de contraste por paciente. Foi possível observar detalhes anatômicos não revelados em angiografias planares em 23% dos pacientes. Adicionalmente, 49% dos pacientes tiveram benefícios das imagens 3D para decisões terapêuticas. A exposição à radiação não foi estatisticamente diferente das angiografias planares. Nenhum paciente apresentou complicações relacionadas ao método. Conclusão: A 3D-RA forneceu informações que algumas das angiografias convencionais não fornecem e que ajudaram no tratamento de pacientes selecionados portadores de cardiopatia congênita. O emprego desse método poderá reduzir o número de aquisições de imagem por procedimento e, como consequência, limitar a exposição do paciente à radiação e ao contraste.

Background: Advances in the diagnosis and treatment of congenital heart disease are associated to advances in imaging techniques. Anatomic images obtained by computed tomography scan, magnetic resonance imaging (MRI) and echocardiography have been useful but cannot provide accurate hemodynamic data. 3D rotational angiography (3D-RA) is a new 3D reconstruction method carried out inthe cath lab that has been widely used in neurological and urological procedures. This study was aimed at evaluating the use of 3D-RA in the diagnosis and treatment of congenitalheart disease. Method: Review of catheterization results of patients with congenital heart disease referred for diagnostic assessment in which the 3D reconstruction method was employed. Philips Allure FD 10 equipment and low osmolarity contrast medium were used for angiographies. Results: Overall,53 patients were reviewed and 2.2 ± 1.1 mL/kg of contrast medium were used per patient. Anatomic details notpreviously shown by 2D angiographies were observed in 23% of the patients. Furthermore, 3D-RA imaging was usedto make treatment decisions in 49% of the patients. Exposure to radiation was not statistically different from 2D angiography. None of the patients had complications related to the method. Conclusion: 3D-RA provided information not usually seen by conventional angiography which was useful in thetreatment of selected patients with congenital heart disease. The use of 3D-RA may reduce the number of imaging tests per procedure and as a consequence, limit patient exposureto radiation and contrast media.

What is the true frequency of carditis in acute rheumatic fever? A prospective clinical and Doppler blind study of 56 children with up to 60 months of follow-up evaluation.

BACKGROUND: This study aimed to evaluate prospectively clinical and echocardiographic findings of patients who had rheumatic fever with and without clinical features of cardiac involvement. METHODS: For this study, 56 consecutive patients (mean age, 11.4 years) with acute rheumatic fever diagnosed according to the 1992 modified Jones criteria were evaluated at diagnosis, after 3 and 6 months, then at 2 and 5 years. All assessments were performed blindly and included physical and cardiac examination, electrocardiogram, chest X-ray, and two-dimensional color-flow Doppler echocardiography. RESULTS: Initial clinical carditis was observed for 27 patients (48.2%), all of whom had positive echocardiographic abnormalities. Echocardiographic abnormalities were observed in 11 patients who had arthritis or chorea presentation without initial clinical carditis. Persistence of the abnormalities was observed at a late follow-up evaluation in 72.7% of the cases. Sydenham's chorea was present in 8 patients with clinical carditis and in 10 without this disorder, 8 of whom had echocardiographic abnormalities. CONCLUSION: Patients who had acute rheumatic fever without clinical signs of carditis showed acute and late follow-up echocardiographic abnormalities suggestive of cardiac involvement. Clinicians should be attentive for the presence of cardiac involvement among patients with chorea.

Hipertensão arterial pulmonar associada a cardiopatias congênitas / Pulmonary hypertension in congenital heart disease

A hipertensão arterial pulmonar é frequentemente encontrada nas cardiopatias congênitas associadas a hiperfluxo pulmonar secundário a grandes shunts da esquerda para a direita. A hipertensão pulmonar é um fator complicador importante no tratamento desses pacientes, podendo impedir a correção cirúrgica da cardiopatia naqueles com doença vascular pulmonar já estabelecida. O diagnóstico precoce e o conhecimento da história natural do defeito cardíaco usualmente levam à prevenção da doença vascular pulmonar, por permitir seu fechamento em tempo adequado. Nos pacientes com doença vascular pulmonar irreversível, o tratamento clínico convencional continua indicado, embora novos medicamentos demonstrem seus efeitos potenciais sobre variáveis hemodinâmicas e sobrevida. Após a falência ao tratamento clínico otimizado, existe a opção de transplante pulmonar.

Estudo angiográfico comparativo da artéria pulmonar no pré e pós-operatório de pacientes submetidos a operaçäo de derivaçäo cavopulmonar bidirecional / Comparative angiographic study of the pulmonary artery in pre and postoperative period in patients submitted to bidirectional cavopulmonary shunt surgery

INTRODUÇÄO: Este trabalho analisou as alteraçöes nos diâmetros da artéria pulmonar (AP) após a derivaçäo cavopulmonar bilateral (DCPB). CASUíSTICA E MÉTODOS: Foram incluídos 18 pacientes submetidos a DCPB, no período de março de 1990 a janeiro de 1997, que possuíam exames cineangiográficos disponíveis no período pré e pós-operatório. As medidas da AP direita e esquerda foram realizadas em três locais: na origem, imediatamente antes da bifurcaçäo e no início da artéria do lobo inferior. Em seguida, os diâmetros de cada local foram indexados à superfície corpórea ou ao diâmetro da aorta, medido na altura do diafragma. Os dados angiográficos e oximétricos foram submetidos a análise estatística. RESULTADOS: Quando analisados os diâmetros absolutos observou-se que a maioria sofreu aumento näo-significante, no período pós-operatório, ao passo que o diâmetro II da AP esquerda apresentou diminuiçäo. Os índices totais I e III apresentaram diminuiçäo significante, no período pós-operatório, e no índice total II a reduçäo näo foi significante. A análise da variável seguimento pós-operatório demonstrou reduçäo significativa dos índices totais nos pacientes com seguimento pós-operatório inferior a 23,6 meses. A presença de fluxo sangüíneo adicional determinou o aumento dos índices AP direita II e III, e pequena reduçäo nos outros índices. As medidas da AP indexadas pelo diâmetro da aorta revelaram comportamento semelhante à indexaçäo pela superfície corpórea. Na ausência de circulaçäo venosa colateral (CVC) observou-se aumento significante da saturaçäo de O2, por outro lado, a sua presença determinou um aumento näo-significante, durante o período de observaçäo

Atresia pulmonar com septo interventricular intacto: experiência inicial com perfusäo valvar por radiofreqüência em neonatos e lactentes / Pulmonary atresia with intact ventricular septum: experience with radiofrequency valve perfuration in neonates and infants

A atresia pulmonar com septo interventricular intacto é uma cardiopatia cianótica rara, onde existe obstrução completa ao fluxo sanguíneo do ventrículo direito para a artéria pulmonar no nível da valva pulmonar. O desapontamento e a frustração no tratamento dos neonatos com esta doença deve-se principalmente às suas grandes variações morfológicas. Depois que a valvotomia pulmonar pelo cateterismo intervencionista passou a ser eficaz, mesmo nos casos de estenose pulmonar crítica do recém-nascido, a sua indicação foi estendida para as formas mais favoráveis da atresia pulmonar com septo ventricular intacto. Inicialmente, para a perfuração valvar foi utilizado o guia de laser; em seguida a extremidade rígida do fio guia de angioplastia, e mais tarde por ser mais segura e menos onerosa a técnica de perfuração por radiofreqüência tornou-se factível e passou a ser empregada em vários centros especializadas. Objetivo: O objetivo deste estudo foi avaliar os resultados de nossa experiência inicial com a perfuração da valva pulmonar atrésica com radiofreqüência, seguida de dilatação pulmonar por cateter balão, em uma série de neonatos e lactentes com atresia pulmonar e septo ventricular intacto. Métodos: Entre maio de 1996 e julho de 1998, 11 pacientes portadores de atresia pulmonar com septo ventricular intacto foram vistos consecutivamente no Hospital São Paulo/ UNIFESP-EPM. Após avaliações clínica, eletrocardiográfica, radiológica e ecocardiográfica, do total de 11 pacientes, oito (73 por cento) foram selecionados e três foram excluídos. Dois por apresentarem ventrículo direito bipartite e extremamente hipoplásico, além de apresentarem sinusóides importantes, e o terceiro, apesar de ter o ventrículo direito tripartite, apresentava um grande trombo calcificado na ponta do ventlículo direito. Dos oito pacientes, dois (25 por cento) foram a óbito antes de se realizar o procedimento. Participaram deste estudo sete pacientes; o procedimento do paciente de n§ 7 foi por nós realizado no Royal Brompton Hospital de Londres. Todos apresentavam ventrículo direito tripartite, infundíbulo pérvio, ausência de sinusóides importantes e de circulaçäo coronariana ventrículo direito dependente. Os achados ecocardiográficos foram confirmados pela cineangiogradia antes de se iniciar o procedimento. Todos os pacientes, exceto o n§ 7, eram do sexo feminino. A idade dos pacientes variou de três dias a quatro anos e dois mês...(au)

Experiência clínica com o uso de adenosina em pacientes pediátricos / Clinical experience with use of adenosine in pediatric patients

Descrevemos nossa experiência com o uso de adenosina no diagnóstico e tratamento de quatro pacientes pediátricos com taquicardia supraventricular. Brevemente, revisamos o reconhecimento e manejo das taquidisritmias supraventriculares em crianças, bem como a farmacocinética, indicaçoes e efeitos colaterais associados ao uso de adenosina. Concluímos que a adenosina é uma nova droga antiarrítmica segura e eficaz no tratamento e diagnóstico das taquidisritmias com complexo QRS estreito em pediatria, inclusive no período neonatal. Com adequada monitorizaçao eletrocardiográfica, sua utilizaçao torna-se importante subsídio para o diagnóstico de diversos outros ritmos supraventriculares.

Acidente vascular cerebral em criança portadora de origem anômala da artéria coronária esquerda / Cerebrovascular Accident in Child with Anomalous Origin of the Left Coronary

A two-year-old male child presenting anomalous origin of the left coronary artery diagnosed by Doppler echocardiography and confirmed by angiography developed an ischemic stroke, which is a rare event in this situation. He [correction of She] had good recovery of the neurological deficit and his anomalous artery was successfully reimplanted