Reverse Potts for the Treatment of Severe Idiopathic Pulmonary Hypertension in Children.

Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.

Reverse Potts for the Treatment of Severe Idiopathic Pulmonary Hypertension in Children

ABSTRACT Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.

Estudo observacional transversal de pacientes submetidos à oclusão percutânea de forame oval patente: uma avaliação retrospectiva de 20 anos / Cross-sectional observational study of patients submitted to percutaneous occlusion of patent foramen ovale: a 20-year retrospective analysis

Introdução: A possibilidade de shunt da direita para esquerda pelo forame oval é causa potencial de embolia paradoxal. A presença de forame oval entre os pacientes com acidente vascular cerebral isquêmico criptogênico em mais de 40% dos casos despertou interesse em avaliar o impacto de seu fechamento na redução de recidiva da doença. O estudo objetiva relatar a experiência de 20 anos com fechamento percutâneo de forame oval realizado por um único operador. Métodos: Foram submetidos a fechamento percutâneo de forame oval associado a shunt da direita para esquerda 527 pacientes com acidente vascular cerebral isquêmico. Resultados: O procedimento foi realizado com sucesso em todos os pacientes. A média da idade foi de 48 anos (9 a 72 anos), sendo 57% do sexo masculino. Foram implantadas prótese Amplatzer® em 295 pacientes (56%) e Occlutech® em 232 (44%). Não houve mortalidade, e ocorreram as seguintes complicações: hematoma no local da punção em três pacientes (0,6%); fístula arteriovenosa femoral em dois (0,4%) com resolução espontânea; tamponamento cardíaco em dois (0,4%) com resolução após punção e drenagem; arritmia supraventricular transitória em três (0,6%); cefaleia em 27 (5,1%); fibrilação atrial em dois (0,4%); shunt residual grande com necessidade de segunda prótese em dois pacientes (0,4%); recorrência de acidente vascular cerebral isquêmico em seguimento em 5 anos em quatro (0,8%) e hipersensibilidade ao níquel em uma paciente (0,2%). Conclusão: A oclusão de forame oval como prevenção de recorrência de acidente vascular cerebral isquêmico nessa série se mostrou segura, eficaz e com baixo índice de recidiva em 5 anos de seguimento.

Background: Left-to-right shunting via the foramen ovale is a potential cause of paradoxical embolism. The presence of patent foramen ovale in more than 40% of patients with idiopathic ischemic stroke has led to deeper investigation of the impact of its closure on decreasing stroke recurrence rates. This study describes 20 years of experience with percutaneous patent foramen ovale occlusion performed by a single operator. Methods: This sample comprised 527 patients with ischemic stroke submitted to percutaneous occlusion of patent foramen ovale associated to left-to- right shunting. Results: The procedure was successful in all cases. The mean age was 48 years (range of 9 to 72 years), and 57% were male. Amplatzer® and Occlutech® prostheses were implanted in 295 and 232 patients (56% and 44%, respectively). There were no deaths, and complications were as follows: hematoma at the puncture site (three patients, 0.6%), femoral arteriovenous fistula with spontaneous resolution (two patients, 0.4%), cardiac tamponade resolved after puncture and drainage (two patients, 0.4%), transient supraventricular arrhythmia (22 patients, 4.2%), headache (27 patients, 5.1%), atrial fibrillation (two patients, 0.4%), large residual shunt requiring a second prosthesis (two patients, 0.4%), recurrence of ischemic stroke within 5 years (four patients, 0.8%) and hypersensitivity to nickel (one patient, 0.2%). Conclusion: In this series, patent foramen ovale occlusion was a safe and effective alternative for prevention of recurrent ischemic stroke. Recurrence rates over the course of 5 years were low.

Percutaneous closure of ostium secundum atrial septal defect using left internal jugular vein access in a child with situs inversus and absence of inferior caval vein.

Femoral vein access is the first choice for percutaneous atrial septal defect closure, and when it cannot be used due to anatomic reasons, the alternative sites should be considered, frequently increasing the complexity of the procedure. Here we report the case of a 3-year-old boy, with situs inversus and dextrocardia, electively referred for percutaneous closure of an ostium secundum atrial septal defect. During the procedure, agenesis of the infra-hepatic segment of the inferior caval vein was diagnosed, and no double inferior caval vein or right superior caval vein were identified by ultrasound or angiography. Therefore, we opted to perform the procedure through the left internal jugular vein, with fluoroscopy and transesophageal echocardiographic guidance. Catheters were navigated through a hydrophilic guidewire, and a Stiff guidewire was positioned in the left ventricle for better support. An Amplatzer septa occluder 19 was successfully deployed without major difficulties and the patient was discharged after 24 hours in good clinical condition. Percutaneous atrial septal defect closure through alternative access sites, especially in the presence of situs inversus, may pose significant challenges to the interventional team. In this case, the left internal jugular vein has shown to be a feasible option, allowing the navigation and manipulation of devices without complications. Provided the expertise of the interventional team, and awareness of the risks involved, alternative access sites can be successfully used for paediatric structural interventions.

Closure of Secundum Atrial Septal Defects by Using the Occlutech Occluder Devices in More Than 1300 Patients: The IRFACODE Project: A Retrospective Case Series.

BACKGROUND: The Occlutech Figulla ASD device series (OFSO) shows an improved device design for interventional ASD closure, larger follow-up series are missing. METHODS: We retrospectively reviewed the feasibility, safety, implantation properties, results, and follow-up of ASD closure using Occlutech devices over a 5 year period by establishing a multi-institutional collaborative result registry with 16 contributing centers from 11 countries (IRFACODE). RESULTS: In 1315 patients of all age groups (female 66.9%), successful (98%) ASD closure was performed (mean age 28.9 years, weight 52 kg, height 148.6 cm). Of the defects, 47.9% showed no or only a deficient aortic rim; in 11.9%, there was more than one defect; a septum aneurysm was present in 21.5%; and the mean implanted device size was 20.5 mm. Immediate closure was achieved in 78.6%, at discharge in 83.1%, and 96.4% and 97.3% at 6 and 12 months follow-up, respectively. During a mean follow-up of 2.7 years (in total 3597 patient years), significant complications were minimal (total = 8, <1%) with secondary device embolizations in five and AV-blocks in three patients. No erosion or death was reported. CONCLUSION: ASD closure using OFSO is feasible in a large variety of patients, safe with only a minimal risk of severe side effects and especially without any aortic erosions despite a large percentage of large and complicated defects. © 2016 Wiley Periodicals, Inc.

Oclusão percutânea de comunicação interatrial ou de forame oval guiada por ecocardiograma intracardíaco / Percutaneous closure of atrial septal defect or foramen ovale guided by intracardiac echocardiography

O ecocardiograma transesofágico (ECO-TE) é o método mais utilizado para guiar otratamento percutâneo da comunicação interatrial (CIA) e do forame oval (FOP), mas a necessidade de um outro profissional para realizá-lo e de anestesia geral constituem inconvenientes para seu emprego. Oecocardiograma intracardíaco (ECO-IC) apresenta-se como alternativa ao ECO-TE, pois pode ser realizado pelo próprio operador e demanda apenas anestesia local, com leve ou nenhuma sedação. Nosso objetivo foi relatar a experiência do serviço com a oclusão de CIA/FOP guiada por ECO-IC. Métodos: O ECO-IC utiliza cateter de ultrassom, que é introduzido por via venosa em câmaras cardíacas direitas e, por meio de posicionamento variável do transdutor, obtém as imagens adequadas para a intervenção. Foram avaliadas as taxas de sucesso do procedimento e as complicações. Resultados: De 2011 a 2015, foram realizados 201 procedimentos guiados pelo ECO-IC, sendo 139 empacientes com CIA e 62 com FOP. A maioria dos pacientes era do sexo feminino (64,2%), as idades variaramde 7 a 78 anos (36,6 ± 19,3 anos) e o peso variou de 28 a 92 kg (62,5 ± 13,0 kg). Foram utilizadas próteses Occlutech Figulla®, e todas as intervenções tiveram sucesso, com tempos de fluoroscopia de 5,7 ± 2,4 minutos e tempo de procedimento de 21,5 ± 6,4 minutos. Dois pacientes (2,0%) apresentaram taquicardia supraventricular transitória e outros dois pacientes evoluíram com fístula arteriovenosa na via de acesso, com resolução espontânea no primeiro mês. Conclusões: O ECO-IC forneceu informações anatômicas precisas para guiar o fechamento da CIA/FOP com sucesso e eliminou as principais desvantagens do ECO-TE...

Transesophageal echocardiography (TEE) is the most widely used method to guide the percutaneous treatment of atrial septal defect (ASD) and patent foramen ovale (PFO), but the necessity of another professional to perform it and the need for general anesthesia are potential disadvantages. Intracardiac echocardiography (ICE) is seen as an alternative to TEE, as it can be performed by the interventionist and requires only local anesthesia with mild or no sedation. The aim of this study was to report our experience with ASD/PFO occlusion guided by ICE. Methods: The ICE uses an ultrasound catheter, which is intravenously inserted in the right heart chambers and acquires images for the intervention through variable positioning of the transducer. Success and complication rates of the procedure were evaluated. Results: From 2011 to 2015, 201 procedures guided by ICE were performed, comprising 139 in patients with ASD and 62 in those with PFO. Most patients were female (64.2%), ages ranged from 7 to 78 years (36.6 ±19.3 years), and weight ranged from 28 to 92 kg (62.5 ± 13.0 kg). Occlutech Figulla® prostheses were used and all interventions were successful, with fluoroscopy time of 5.7 ± 2.4 minutes and procedure time of 21.5 ± 6.4 minutes. Two patients (2.0%) had transient supraventricular tachycardia and two others had arteriovenous fistula at the access site, with spontaneous resolution in the first month of follow-up. Conclusions: ICE provided accurate anatomical information to guide the closure of the ASD/PFO and successfully eliminated the main drawbacks of TEE...

O desafio diagnóstico da doença de Kawasaki incompleta / The diagnosis challenge of the incomplete Kawasaki disease

Objetivo: a doença de Kawasaki é uma vasculite sistêmica aguda autolimitada que acomete vasos de médio e pequeno calibres, preferencialmente as artérias coronárias. Os pacientes que não apresentam todos os critérios necessários para o diagnóstico são classificados como portadores da forma incompleta. Este trabalho descreve criança com a forma incompleta da doença de Kawasaki, ressaltando os aspectos clínicos e laboratoriais que possam ser de auxílio no diagnóstico da doença. Descrição do caso: paciente masculino, seis anos de idade, com febre, mialgia e sinais meníngeos admitido no hospital com diagnóstico de meningite. Apresentava hiperemia conjuntival bilateral ehemorragia conjuntival. A punção lombar evidenciou pleocitose e elevação da proteinorraquia. Iniciado tratamento para meningoencefalite, com desaparecimento da febre e dos sinais meníngeos. No terceiro dia de internação manifestou insuficiência cardíaca e quatro dias após a febre ressurgiu, persistindo por sete dias. A pesquisa de foco infecciosoera negativa. Após a defervescência da febre, o ecocardiograma revelou coronárias dilatadas. Diagnosticada a síndrome de Kawasaki incompleta. No mesmo dia, foi observada descamação laminar nas pontas dos dedos das mãos. Discussão: a síndrome de Kawasaki incompleta deve ser considerada em toda criança com febre prolongada por mais de cinco dias sem foco aparente, associado a algumas das principais manifestações típicas. O diagnóstico tardio representa alto risco de coronariopatias.

Objectives: Kawasaki disease is an acute self-limiting systemic vasculitis that affects medium and small caliber vessels, preferably the coronary arteries. Patients who do not present all the necessary criteria for diagnosis are classified as bearers of the incomplete form. This study describes a child with the incomplete form of Kawasaki disease emphasizing the clinical and laboratory aspects that may be of aid in the disease diagnosis. Case description: male patient, six years old, with fever, myalgia, and meningeal signs admitted to the hospital with a diagnosis of meningitis. He presented bilateral conjunctival hyperemia and conjunctival hemorrhage. The spinal tap showed pleocytosis and elevated proteinorachy. Started treatment for meningoencephalitis, with the disappearance of fever and meningeal signs. On the third day of hospitalization expressed heart failure, and the fever reemerged four days after persisting for seven days. The research of infection was negative. After fever defervescence, the echocardiogram revealed dilated coronary. The incomplete Kawasaki syndrome was diagnosed. On the same day, laminar desquamation was observed at fingertips. Discussion: the incomplete Kawasaki syndrome should be considered in every child with prolonged fever for more than five days without apparent focus, associated with some of the main typical manifestations. The late diagnosis represents higher risk for coronary artery disease.

Differential effects of variation in athletes training on myocardial morphophysiological adaptation in men: focus on ¹²³I-MIBG assessed myocardial sympathetic activity.

PURPOSE: High intensity systematic physical training leads to myocardial morphophysiological adaptations. The goal of this study was to investigate if differences in training were correlated with differences in cardiac sympathetic activity. METHODS: 58 males (19-47 years), were divided into three groups: strength group (SG), (20 bodybuilders), endurance group (EG), (20 endurance athletes), and a control group (CG) comprising 18 healthy non-athletes. Cardiac sympathetic innervation was assessed by planar myocardial (123)I-metaiodobenzylguanidine scintigraphy using the early and late heart to mediastinal (H/M) ratio, and washout rate (WR). RESULTS: Left ventricular mass index was significantly higher both in SG (P < .001) and EG (P = .001) compared to CG without a statistical significant difference between SG and EG (P = .417). The relative wall thickness was significantly higher in SG compared to CG (P < .001). Both left ventricular ejection fraction and the peak filling rate showed no significant difference between the groups. Resting heart rate was significantly lower in EG compared to CG (P = .006) and SG (P = .002). The late H/M ratio in CG was significantly higher compared to the late H/M for SG (P = .003) and EG (P = .004). However, WR showed no difference between the groups. There was no significant correlation between the parameters of myocardial sympathetic innervation and parameters of left ventricular function. CONCLUSIONS: Strength training resulted in a significant increase in cardiac dimensions. Both strength and endurance training seem to cause a reduction in myocardial sympathetic drive. However, myocardial morphological and functional adaptations to training were not correlated with myocardial sympathetic activity.

Portal cholangiopathy: case report / Colangiopatia portal: relato de caso

The present report describes the case of a child that after blunt abdominal trauma presented with portal thrombosis followed by progressive splenomegaly and jaundice. Ultrasonography and percutaneous cholangiography revealed biliary dilatation secondary to choledochal stenosis caused by dilated peribiliary veins, characterizing a case of portal biliopathy. The present case report is aimed at presenting an uncommon cause of this condition.

Relata-se o caso de uma criança que após trauma abdominal fechado apresentou trombose portal, seguida por esplenomegalia progressiva e icterícia. Os achados da ultrassonografia e da colangiografia percutânea mostraram dilatação de vias biliares secundária à constrição do colédoco por veias pericoledocianas dilatadas, configurando caso de colangiopatia portal. O objetivo deste relato é a apresentação de causa incomum desta condição.

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.

Forame oval permeável e sua relação com acidente vascular cerebral isquêmico (AVC) e enxaqueca / Permeable foramen ovale and its relation to ischemic stroke (stroke)and migraine

O forame oval permite, durante o período fetal, a passagem de sangue oxigenado oriundo da placenta para o átrio direito, possibilitando oxigenação adequada do cérebro e do coração. Após o nascimento, com o início da respiração e com a desconexão da placenta, há um aumento da pressão do lado esquerdo e uma diminuição do lado direito do coração, empurrando a lâmina do forame oval para a direita, o que ocasiona o seu fechamento funcional. Entretanto, em aproximadamente 25% da população ele pode permanecer patente. Sua relação com o acidente vascular isquêmico e com enxaqueca com aura tem sido sugerida em várias publicações. Na presença de acidente vascular isquêmico idiopático, tem sido sugerido por vários autores o fechamento percutâneo do forame oval patente (FOP), quando comprovada a existência de shunt da direita para a esquerda, espontaneamente ou com manobra de Valsalva. Contudo, hã necessidade de maior tempo de observação para avaliar a sua superioridade em relação ao tratamento clínico.

Foramen ovale allows the passage, during the fetal period, of oxygenated blood coming from the placenta to the right atrium, supplying adequate oxygenation for brain and heart. After birth, with onset of breathing and disconnection of the placenta, there are an increased pressure on the left side ofthe heart and a decreasing in its right side pushing the blade of the foramen ovale to the right, causing its functional closure. However, the patent foramen ovale (PFO) occurs in approximately 25% of the population. The relationship between cryptogenic stroke, migraine with aura and PFO has been suggestedin several publications. In the presence of cryptogenic stroke, percutaneous closure of PFO, when shown the existente of a shunt from right to left spontaneously or with the Valsalva maneuver, has been suggested by several authors. However, there is need of more observation time to assess its superiority over medica1 treatment.

Adenosine for vasoreactivity testing in pulmonary hypertension: a head-to-head comparison with inhaled nitric oxide.

BACKGROUND: APVT is an invasive method recommended for symptomatic patients with PAH that permits the identification of the minority of patients (<20%) that may benefit from long-term calcium channel blockers. Adenosine has been indicated in guidelines as a vasodilator agent of choice for APVT, although it has not been directly compared with iNO, the gold standard for this test. The objective of the study was to compare adenosine with inhaled nitric oxide (iNO) for acute pulmonary vasoreactivity testing (APVT) in pulmonary arterial hypertension (PAH), in order to determine the efficacy and safety of the first in the clinical setting. METHODS: The measurements of cardiac output, pulmonary and systemic resistance were done in the basal state and with a stepwise increase of the dose of each drug until either maximum dosage (adenosine: 500 microg/kg/min or iNO: 80 ppm) or side effects observed or a positive response were reached, according to current guidelines. The order of drugs used in each test was consecutively alternated during the study. RESULTS: Six of the 39 studied patients (15%) presented a positive response to iNO; none to adenosine (p = 0.047, McNemar's test). Twenty-three patients (59%) did not reach the maximum dose of adenosine due to side effects, including bronchospasm, thoracic pain and bradycardia. CONCLUSIONS: APVT testing with adenosine was not able to detect PAH patients responsive to iNo and provoked frequent adverse effects. Adenosine should not be used as a vasodilator drug in APVT.

Testing pulmonary vasoreactivity.

Pulmonary arterial hypertension is classified as idiopathic or secondary (associated with collagenoses, heart disease, portal hypertension, pulmonary thromboembolism, and pulmonary vascular diseases). Pulmonary vasoreactivity should be tested in order to define the best treatment option. Of the many drugs that have been used to test pulmonary vasoreactivity, inhaled nitric oxide is the best choice, due its specific pulmonary effect and very short half-life (5-10 s). The results of this test identify candidates for heart surgery among patients with congenital heart disease and candidates for the use of calcium antagonists among patients with other forms of pulmonary hypertension. Performing and interpreting the results of such tests are a great responsibility, since mistakes can lead to incorrect treatment decisions, resulting in the death of patients.

Teste de vasorreatividade pulmonar / Testing pulmonary vasoreactivity

A hipertensão arterial pulmonar é classificada como idiopática ou secundária (associada a colagenoses, cardiopatias, hipertensão portal, tromboembolismo pulmonar e doenças da vasculatura pulmonar). O teste de vasorreatividade pulmonar é indicado para definir a melhor opção terapêutica. Muitas drogas têm sido utilizadas para a realização desse teste, sendo o óxido nítrico inalado a melhor opção, por apresentar ação específica pulmonar e meia vida muita curta (5-10 s). O resultado desse teste identifica candidatos à cirurgia cardíaca nas cardiopatias congênitas e candidatos ao uso de antagonista de cálcio nas outras formas de hipertensão pulmonar. A realização e interpretação do teste de vasorreatividade pulmonar exigem grande responsabilidade, e erros podem levar a decisões erradas e à ocorrência de óbitos.

Pulmonary arterial hypertension is classified as idiopathic or secondary (associated with collagenoses, heart disease, portal hypertension, pulmonary thromboembolism, and pulmonary vascular diseases). Pulmonary vasoreactivity should be tested in order to define the best treatment option. Of the many drugs that have been used to test pulmonary vasoreactivity, inhaled nitric oxide is the best choice, due its specific pulmonary effect and very short half-life (5-10 s). The results of this test identify candidates for heart surgery among patients with congenital heart disease and candidates for the use of calcium antagonists among patients with other forms of pulmonary hypertension. Performing and interpreting the results of such tests are a great responsibility, since mistakes can lead to incorrect treatment decisions, resulting in the death of patients.

[Percutaneous closure of atrial septal defect using transhepatic puncture]. / Fechamento percutâneo de comunicação interatrial por punção trans-hepática.

The percutaneous closure of ostium secundum (OS) atrial septal defect (ASD) is a well-established procedure and is today considered the treatment of choice due to its good results and low morbidity and mortality. The procedure is routinely performed through the inferior vena cava (IVC). However, this route of access is not always available due to the obstruction or congenital absence of the IVC. We will present a case of ASD closure by means of transhepatic puncture due to the impossibility of using the IVC.

Fechamento percutâneo de comunicação interatrial por punção trans-hepática / Percutaneous closure of atrial septal defect using transhepatic puncture

O fechamento percutâneo da comunicação interatrial (CIA) ostium secundum (OS) é procedimento bem estabelecido, sendo atualmente considerado o tratamento de escolha por seus bons resultados e sua baixa morbidade e mortalidade. O procedimento é realizado rotineiramente pela veia cava inferior (VCI). Ocasionalmente, entretanto, essa via de acesso não é possível por causa da obstrução ou da ausência congênita da VCI. Apresentaremos um caso de fechamento de CIA por punção trans-hepática decorrente de impossibilidade de uso da VCI.

[Sildenafil in the management of idiopathic pulmonary arterial hypertension in children and adolescents]. / Sildenafil no tratamento da hipertensão arterial pulmonar idiopática em crianças e adolescentes.

OBJECTIVE: This study aims to provide data on the use of oral sildenafil in patients in New York Heart Association functional class III or IV with severe idiopathic pulmonary arterial hypertension unresponsive to conventional therapy. METHOD: In this series, six patients with idiopathic pulmonary arterial hypertension were prospectively treated with 2 to 8 mg of oral sildenafil in four to six doses a day. All patients were submitted to physical examination, electrocardiogram and echocardiogram, chest computed tomography, ventilation and pulmonary perfusion scintigraphy, coagulation studies, and tests for collagen vascular disease, acquired immune deficiency syndrome and schistosomiasis in order to rule out secondary causes of pulmonary arterial hypertension. All patients underwent cardiac catheterization for vasoreactivity tests using nitric oxide, O2 at 100% and oral nifedipine, and a 6 minute walking test was performed in those patients who were considered able to exercise. RESULTS: All patients achieved a good therapeutic response, with improvement by at least one functional class, and presented an increase in systemic arterial oxygen saturation. Five patients showed a decrease in the pulmonary systolic pressure to systemic systolic pressure ratio and improvement in the six-minute walking test. No major side effects were observed at 4 to 36 months of follow-up. One patient had sudden death after sildenafil had been withdrawn by mistake. CONCLUSIONS: These data suggest that sildenafil may be useful in the management of idiopathic pulmonary arterial hypertension. Patients should be advised against the withdrawal of sildenafil without medical supervision.