Implante transcateter da valva pulmonar. Protocolo clínico de tratamento e requisitos para credenciamento de centros e operadores no Brasil / Transcatheter pulmonary valve implantation. Clinical treatment protocol and accreditation requirements for centers and interventionists in Brazil

O implante transcateter da valva pulmonar (ITVP) evoluiu significativamente desde sua introdução, no início dos anos 2000. Atualmente, esta técnica é uma opção segura e eficaz para o tratamento das disfunções valvares graves (estenose e/ou insuficiência) em biopróteses ou condutos cirúrgicos em posição pulmonar, em vários centros do mundo. No Brasil, os resultados iniciais com este procedimento em centros de referência foram similares àqueles observados na experiência global. O ITVP tem se demonstrado factível, seguro e eficaz em mãos treinadas na nossa realidade. Porém, antes da aplicação disseminada desta técnica em outros centros em nosso país, houve a necessidade de se estabelecerem alguns critérios para a seleção do paciente, a técnica de implante e o seguimento clínico, assim como para o treinamento e o credenciamento de novos operadores e centros. As orientações aqui descritas foram determinadas por um grupo de especialistas com experiência renomada em cardiopatias congênitas e ITVP, tendo sido encaminhadas ao Conselho Federal de Medicina (CFM). Representantes das diferentes sociedades médicas foram envolvidas na preparação deste documento, incluindo a Sociedade Brasileira de Cardiologia (SBC), a Sociedade Brasileira de Hemodinâmica e Cardiologia Invasiva (SBHCI) e a Sociedade Brasileira de Cirurgia Cardiovascular (SBCCV). Acreditamos que a rígida aderência às recomendações listadas neste documento oficial seja crucial para a segurança do paciente e para que ótimos resultados sejam alcançados imediatamente e a longo prazo. Uma vez consolidado em nosso meio, o ITVP abrirá caminho para a introdução de novas terapias valvares em cardiopatias congênitas

Transcatheter pulmonary valve implantation (TPVI) has evolved significantly since its introduction in the early 2000s. Currently, this technique is a safe and effective option for the treatment of severe valve dysfunction (stenosis and/or regurgitation) in bioprostheses or surgical conduits in pulmonary position, in several centers worldwide. In Brazil, the initial results with this procedure in reference centers were similar to those observed in the overall experience. TPVI was proven to be feasible, safe, and effective in trained hands in Brazil. However, prior to the widespread application of this technique to other centers in this country, it was necessary to establish some criteria for patient selection, implantation technique, and clinical follow-up, as well as for the training and accreditation of new interventionists and centers. The guidelines described here were determined by a group of experts with well-known experience in congenital heart disease and TPVI, and were referred to the Conselho Federal de Medicina (CFM, portuguese for Federal Council of Medicine). Representatives of the different medical societies were involved in the creation of this document, including the Sociedade Brasileira de Cardiologia (SBC, portuguese for Brazilian Society of Cardiology), the Sociedade Brasileira de Hemodinâmica e Cardiologia Invasiva (SBHCI, portuguese for Brazilian Society of Hemodynamics and Invasive Cardiology), and the Sociedade Brasileira de Cirurgia Cardiovascular (SBCCV, portuguese for Brazilian Society of Cardiovascular Surgery). The authors believe that strict adherence to the recommendations contained in this official document is crucial for patient safety and for optimal results to be achieved in both the short and long term. Once consolidated in Brazil, TPVI will open doors for the introduction of new valve therapies in congenital cardiopathies

Tratamento intervencionista das cardiopatias congênitas: estado da arte / Interventionist treatment of congenital heart defects: state of the art

Os avanços na tecnologia nas últimas décadas em relação ao tratamento intervencionista das cardiopatias congênitas têm permitindo excelentes resultados, em termos de morbimortalidade, a curto e longo prazo para este grupo especial de pacientes.Este artigo descreve os principais tratamentos disponíveis realizados com cateterismo intervencionista neste grupo de pacientes.

Technological advances in recent decades, in relation to interventionist treatment of congenital heart defects, have enabled excellent short- and long-term results for this group of patients, in terms of morbidity and mortality. This article describes the main treatmentsavailable involving interventionist catheterization in this group of patients.

Procedimentos percutâneos combinados em defeitos estruturais e congênitos / Combined percutaneous treatment of structural and congenital heart defects

Defeitos congênitos múltiplos são tradicionalmente corrigidos cirurgicamente, mas, atualmente, podem ser tratados percutaneamente. Existem poucos relatos na literatura atestandosua eficácia e segurança. Objetivamos descrever uma experiência com a realização de procedimentoscombinados para tratar diferentes defeitos, congênitos e estruturais, numa mesma sessão terapêutica. Métodos: Desde 2007, foram tratados, numa mesma sessão terapêutica, diferentes defeitos. Todos foram selecionados por ecocardiograma. Os procedimentos foram realizados segundo as técnicas tradicionais já descritas para cada defeito encontrado. Resultados: Foram tratados dez pacientes, cinco do sexo masculino, com idades de 1 a 67 anos, e pesos de 11 a 90 kg. O defeito mais prevalente de forma isolada foi a persistência do canal arterial (PCA, n = 5), seguido da comunicação interatrial ostium secundum (CIA OS, n = 4) e da comunicação interventricular (CIV, n = 4). As combinações mais frequentes foram CIV com PCA (n = 2) e CIV com CIA OS (n = 2). Foram dilatadas duasestenoses valvares pulmonares com CIA OS e com forame oval patente (FOP), e uma coarctação de aorta com PCA. Adicionalmente, foi ocluído um apêndice atrial esquerdo com FOP e foi embolizada uma fístula aortopulmonar com PCA. Todos os procedimentos foram bem-sucedidos. O tempo médio de seguimento foi de 31 ± 28,1 meses, havendo apenas duas complicações. Não houve nenhum óbito. Conclusões: A pequena série de casos relatada mostrou que os procedimentos combinados foram seguros e eficazes, podendo ser reproduzidos por operadores experientes em centros especializados, podendo vir a se constituir como primeira opção terapêutica para esses pacientes...

Multiple congenital defects are traditionally corrected surgically, but nowadays can be treatedpercutaneously. There are few reports in the literature attesting to its efficacy and safety. We aimed to describe an experience with combined procedures to treat different congenital and structural defects, in a single therapeutic session.Methods: Since 2007, different defects were treated in a single treatment session. All were selected byechocardiography. The procedures were performed using traditional techniques already described for each defect.Results: Ten patients were treated, five males, aged 1-67 years, weighting 11-90 kilograms. The most prevalent isolated defect was patent ductus arteriosus (PDA, n = 5), followed by ostium secundum atrial septal defects (ASD, n = 4) and ventricular septal defects (VSD, n = 4). The most common combinations were VSD with PDA (n = 2) and VSD with osASD (n = 2). Two pulmonary valve stenosis were dilated with ASD and patent foramen ovale (PFO), and one aorta coarctation with PDA. Additionally, a left atrial appendage with PFO was occluded and an aorto pulmonary fistula with PDA was embolized. All procedures were successful. The mean follow-up was 31 ± 28.1 months, with only two complications. There were no deaths.Conclusions: The small number of reported cases showed that the combined procedures were safe andeffective and can be reproduced by experienced operators in specialized centers and may be considered asthe first therapeutic option in these patients...

Resolución exitosa de la estenosis congénita de las dos ramas de la arteria pulmonar en un adulto mediante el uso de stents / Successful resolution of congenital stenosis of the two branches of the pulmonary artery in an adult using stents

La estenosis de las arterias pulmonares son rara vez diagnosticadas en edad adulta, convirtiéndose su tratamiento en una compleja situación con diferentes posibilidades terapéuticas. Las causas mas frecuentes corresponden a las adquiridas, siendo las mas común la hipertensión pulmonar trombo embólica crónica, quedando reducido numero de casos congénitos asociado principalmente a síndrome genéticos y malformaciones cardiacas asociadas. El tratamiento quirúrgico convencional puede convertirse en un desafío dadas las condiciones anatómicas y funcionales encontradas en estadios avanzados propias del diagnostico tardío y en especial en el caso presentado el cual esta iniciando la sétima década de la vida. La posibilidad de resolución percutánea de estas lesiones cobra importancia a la luz de la amplia utilización de esta en pacientes pediátricos, implementando el uso cada vez mayor de balones con dilatación percutánea y la utilización de stents cada vez más sostificados con buenos resultados. Se presenta nuestro primer caso de resolución percutánea de lesiones coronarias y de arteria pulmonar bilateral en un paciente previamente programado para cirugía de reparación con parche y by pass coronario, el cual evolucionó en formas satisfactoria recobrando su actividad normal a las pocas horas del procedimiento.

Stenosis of the pulmonary arteries is rarely diagnosed in adulthood, turning their treatment in a complex situationwith different therapeutic possibilities. The most frequent causes are acquired, being the most common chronicthromboembolic pulmonary hypertension, remaining a small number of congenital cases mainly associated to geneticsyndromes or complex cardiac malformations. The standard surgical treatment may be a challenge given the anatomicaland functional conditions found themselves in advanced stages of late diagnosis and especially in the case presented,who is beginning the seventh decade of his life. The possibility of percutaneous resolution of these lesions becomesimportant in light of the widespread use of this option in pediatric patients, implementing and increasing its use withpercutaneous dilatation balloons and stents with increasing sophistication and showing good results. We present ourfirst case of percutaneous resolution of coronary lesions and bilateral pulmonary artery stenosis in a patient previouslyscheduled for patch repair surgery and coronary bypass, which evolved in successfully, gaining normal activity within fewhours of the procedure.

Angiotensin converting enzyme inhibitor as an additive treatment after successful balloon dilation of a critical pulmonary valve stenosis

A 2 days old, 2.7 kg heavy baby boy with critical pulmonary stenosis, underwent successful balloon dilation. After the uneventful procedure, he remained oxygen dependent. The baby was given oral angiotensin converting enzyme inhibitor [ACE inhibitor] instead of an infusion of alpha blocker. Within few hours, in the afternoon of the same day after administration of ACE Inhibitor, the baby could be weaned off oxygen, maintaining on room air, oxygen saturation between 87% and 92%. At follow-up, two months later, his saturation was 99% on room air. We believe that some neonates with critical pulmonary valve stenosis who remain oxygen dependent despite successful balloon dilation, could benefit from such management

[ evaluation of late outcome of balloon pulmonary valvuloplasty in children]

Isolated pulmonary valve stenosis represents 8-10% of congenital cardiac anomalies. This study was performed to evaluate the late outcomes of Balloon Pulmonary Valvuloplasty [BPV] in children with residual infundibular stenosis. Seventy-eight patients underwent BPV in Rajaee Heart Center in Tehran Iran, from 2008-2010. The patients were divided into two groups: with and without infundibular stenosis. The group with infundibular stenosis was subdivided into two groups: with and without propranolol administration. Gradient measurement follow-ups by Doppler echocardiography were done on the first day and 1, 3, 6 and 12 months afterwards. Thirty five [44.9%] patients were male and 43 [55.1%] were female. The mean age of participants was 4.29 +/- 3.5 years and the mean weight 16.18 +/- 8.8 kg 33 patients [43%] did not show residual infundibular stenosis but 45 [57%] did so in the evaluations. There were no significant differences between the two groups regarding age, weight, sex, kind of balloon valvuplasty, balloon to body surface area ratio and pulmonary regurgitation [PR]. Propranolol was administered to 27 [60%] patients with residual stenosis for six months but 18 [40%] did not receive the medication. In both groups, the gradient significantly decreased immediately after BPV [P<0.0001]. In the propranolol group a constant decrease in residual gradient was seen. There was a significant relationship between balloon to body surface area ratio [387 +/- 94mm2/m[2]] with moderate to severe PR [P<0.015]. BPV is a safe and effective procedure to treat PS. Residual infundibular gradient after BPV decreases over time and propranolol helps decrease the gradient

Immediate- and medium-term effects of balloon pulmonary valvuloplasty in infants with critical pulmonary stenoses during the first year of life: a prospective single center study

Balloon pulmonary valvuloplasty [BPV] represents the standard of management for all patients with severe pulmonary stenosis [PS] irrespective of their age. Nevertheless neonates and infants with critical PS represent a high-risk group that needs to be studied. The study population included 72 infants with severe congenital valvular PS and four infants with imperforate pulmonary valve [PV] who were subjected to detailed history taking, full clinical examination, resting 12-lead ECG, Chest roentgenogram and transthoracic echocardiography. BPV was attempted in all infants with a peak-to-peak gradient across the PV of 50 mmHg or greater at catheterization-laboratory. Full echocardiographic evaluation was done 24 hours after the procedure as well as 3 and 6 months later. Seventy-six infants with severe PS or imperforate PV with a mean age of 5.63 +/- 2.99 months were subjected to BPV with or without wire perforation. Immediately after the procedure patients had a significant reduction of the right ventricular systolic pressure [RVSP] [104.69 +/- 24.98 mm Hg Vs 43.6 +/- 13 mm Hg, p < 0.001] and RV-PA systolic pressure gradient [PG] [82.5 +/- 23.76 mm Hg Vs 17.35 +/- 8.96 mm Hg, p < 0.001]. The immediate success rate defined as the drop in the RVSP to less than or equal to 50% of the baseline measurement was achieved in 85% of the cases. There was a progressive drop in the PG across the PV by Doppler echocardiogram throughout a follow-up period of six months from a mean of 93.3 +/- 28.2 mm Hg to a mean of 17.4 +/- 10.42 mm Hg [p < 0.001]. There was a significant increase of the mean PV annulus diameter after balloon dilatation [p < 0.001]. There was also a highly significant inverse correlation between the growth of the pulmonary annulus and the annular size at the baseline before dilatation [r = -0.74, p value <0.001]. The incidence of PR significantly increased immediately after BPV to 64% followed by a progressive decline over a 6 months period of follow-up to 20%. There was a significant decrease in the incidence of tricuspid regurgitation [TR] over the same period of follow-up [from 55.6% at baseline to less than 20% at follow-up]. BPV is safe and effective to relieve critical PS in infants during the first year of life. The balloon promotes advantageous changes in both, pulmonary annulus and PG across the RVOT. In addition, the Doppler gradient observations during the follow-up support the expectation that BPV is a "curative" therapy

Nacimiento anómalo de la arteria coronaria izquierda en un tronco común en el seno de Valsalva derecho / Birth of the anomalous left coronary artery in a common trunk within Valsalva right

Se presenta el caso de una paciente de 36 años con antecedente de valvulotomía a los 14 años por estenosis pulmonar congénita, consultó por dolor torácico punzante y disnea de pequeños esfuerzos de 6 meses de evolución. La coronariografía mostró la arteria coronaria izquierda originándose de un tronco común con la arteria coronaria derecha y una lesión obstructiva severa en el origen de la arteria descendente anterior. La paciente fue referida para revascularización quirúrgica.

Percutaneous transmitral balloon commissurotomy [PTMC]: to my Procedural success and immediate results at Ahmed Gasim Cardiac Center

Balloon valvuloplasty for stenosed mitral and pulmonary valves has been practiced with good results for the last two years in Ahmed Gasim Cardiac Centre, Khartoum Sudan. The aim of this study is to audit percutaneous trans-mitral balloon commissurotomy [PTMC] in our current set up. One hundred and eight patients underwent percutaneous transvenous mitral commissurotomy [PTMC] from April- 2004 to December-2005 in Ahmed Gasim cardiac center. 67% of the patients were females. Age range was from 13 years to 65 years and the mean age was 27 years. 27% were under 21 years of age and 12% had special problems. The procedural success was achieved in 94.5% patients, in two patients we failed to dilate a tough septum, in other two the balloon got stuck to the septum and failed to cross the MV, while two patients had successful emergency MVR [due to inadvertent puncture of the RA and cardiac tamponade]. There was no mortality related to the procedure, significant mitral regurgitation of> grade 2 didn't occur. No patient developed systemic embolization. Optimal results were achieved in 91.6% patients and in 92% of the patients with special problems. Hemodynamic data in the Cath. Lab showed left atrial mean [ +/- SD] pressure dropped from 32 [ +/- 3.2] mmHg to 12 [ +/- 2.4] mmHg. LA-LV gradient dropped from an average of 25 to 5 mmHg. Echocardiographic assessment showed mean [ +/- SD] mitral valve area increased from 0.86 [ +/- 0.19] cm[2] to 1.9 [ +/- 0.5] cm[2] [p<0.001] and PA pressure dropped from71 [ +/- 23] to 40 [ +/- 12] mmHg [p<0.01]. We conclude that PTMC is a safe procedure with good success rate and optimal results even in patients with special problems like redo and previous CVA

Valvuloplastía pulmonar percutánea / Percutánea pulmonary valvuloplatía

Introducción: la valvuloplastía pulmonar con balón (VPB) es el método terapéutico de elección para tratar la estenosis valvular pulmonar (EVP). El procedimiento consiste en introducir el catéter balón por vía venisa hasta posicionarlo en la parte estrecha de la válvula pulmonar, donde es insuflado produciendo dilatación valvular. Esta técnica reduce inmediatamente el gradiente entre el ventrículo derecho y la arteria pulmonar (VD/AP) la mayoria de las veces, otorgando beneficio hemodinámico, que mejora con el tiempo. Objetivos: evaluar los resultados de la VPB para el tratamiento de le EVP. Material y métodos. Estudio descriptivo-retrospectivo del Servicio de Hemodinamia-IPS e Instituto Codas Thompson. Se analizaron datos de 14 pacientes sometidos a VPB, entre octubre 1994 y agosto 2006. Se estudiaron variables hemodinámicas y ecocardiográficas. El resultado se consideró satisfactorio cuando el gradiente residual 40 mm Hg. El estudio se evaluó en 3 fases: pre-VPB, post-VPB inmediato y post-VPB tardío (>3 meses). Resultados: edad media 2 años 3 meses (r - 10 dias/8 años). Sexo: masculino 8 (57,4%); femenino 6(42.9%). Gradiente medio pre-VPB de 86mmHG bajó inmediatamente a 33 mmHg. Exito inmediato en 9(64%). Tardíamente 3(22%) del grupo no satisfactorio inmediato, redujeron al procedimiento satisfactorio en 12(86%). El resultado fue no satisfactorio en 2/14%), por hipertrofia infundibular residual severa que en un paciente debió ser corregida quirúrjicamente. Conclusión: La EVP puede ser tratada de manera segura y efectiva mediante la VPB, si el diagnóstico morfológico es correcto. Cuando el resultado es satisfactorio, la intervención puede considerarse curativa.

Estado da arte do procedimento percutâneo: paciente octogenário submetido com sucesso, em apenas uma sessão, a valvotomia pulmonar, implante de stent coronário e implante de marcapasso definitivo / State of the art percutaneous procedure: octogenarian patient successfully submitted, in a single session, to a pulmonary valvotomy, coronary stent implant and permanent pacemaker implant

É relatado caso de paciente de 82 anos, portador de insuficiência renal leve, estenose valvar pulmonar (EVP) severa, estenose severa de artéria descendente anterior e bloqueio atrioventricular total, submetido a angioplastia coronária com implante de stent coronário, valvotomia pulmonar e implante de marcapasso definitivo no mesmo procedimento, com sucesso.

Balloon dilatation of pulmonary valve in Tetralogy of Fallot's.

In some children of Tetralogy of Fallot's (TOF) presenting with progressive cyanosis, are palliative Blalock-Taussing (BT) shunt may be required. There are no reports of this modality of management in India, though this has been practiced in the other countries. The author reports an infant with Tetralogy of Fallot's who successfully underwent ballon dilatation of the pulmonary valve. Review of literature shows 332 patients with TOF undergoing pulmonary valve balloon dilatation as an alternative to BT shunt in 12 studies with significant increase in pulmonary artery 'Z' score and low incidence of conversion to shunt. This modality of management should be considered in selected patients to change a palliative surgery to an intervention.

Resultados imediatos e tardios da valvoplastia percutânea com balão na estenose valvar pulmonar / Short- and long- term results of percutaneous balloon valvuloplasty in pulmonary valve stenosis

OBJETIVO: Determinar os resultados em longo prazo da valvoplastia percutânea por balão em uma instituição isolada. MÉTODOS: Estudados 189 pacientes com estenose valvar pulmonar submetidos a valvoplastia percutânea por balão, entre 1984-1996, com idade média de 7,97±9,25 anos, classificando-se como bem sucedida, a redução do gradiente VD-AP em níveis < 36mmHg e reestenose gradientes > 36mmHg após procedimento eficaz. RESULTADOS: Após o término do procedimento, o gradiente pico a pico transvalvar reduziu-se de 70,12±30,06 para 25,11±20,23 mmHg (p<0,001). Obtiveram sucesso imediato 148 (78,72 por cento) pacientes. Houve redução posterior do gradiente para valores < 36mmHg em outros 24 pacientes categorizados sem sucesso no grupo. Assim a valvoplastia percutânea por balão foi considerada efetiva em 172 (91,01 por cento) pacientes. A efetividade aumentou para 93,53 por cento (159/170) nos casos de morfologia típica. O tempo de seguimento foi de 4,39±3anos até o período máximo de 13,01 anos. Observou-se reestenose em 24 (13,95 por cento). A presença de regurgitação pulmonar foi detectada em 95,1 por cento dos pacientes, sendo que em 29,5 por cento com grau maior do que leve. A probabilidade de se manter um resultado adequado, até qualquer ponto no tempo, sem a ocorrência de reestenose, foi de 92,29 por cento em 2 anos, de 87,38 por cento em 5 anos, de 82,46 por cento em 8 anos e de 64,48 por cento em 10 anos. CONCLUSAO: A valvoplastia percutânea por balão foi efetiva e segura no tratamento da estenose valvar pulmonar com excelentes resultados imediatos e a longo prazo.

Efectividad de la valvuloplastía pulmonar percutánea en el tratamiento de la estenosis pulmonar valvular moderada y severa en el Hospital Nacional de Niños, a los 10 años de practicar el procedimiento / Effectiveness of percutaneus pulmonary valvuloplasty in the treatment of the moderate to severe valvular pulmonary stenosis in the Hospital Nacional de Niños, to the 10 years to practice the procedure

La valvuloplastía pulmonar es, actualmente, el tratamiento de elección para la estenosis pulmonar valvular de moderada a severa. Introducida como técnica por Kan, hoy día constituye un procedimiento de fácil aplicación, barato y con resultados excelentes. Con el fin de conocer la situación en el Hospital Nacional de Niños de San José, Costa Rica, se planeó este estudio retrospectivo. Se encontraron un total de 98 casos a los que se les realizó valvuloplastía pulmonar con edades que variaron de 4 días a 24 años. No hubo predominancia de sexo alguno. Los menores de un año fueron 41 niños y los mayores de un año fueron 56. La edad más frecuente a la que se realizó el procedimiento fue a los tres años de edad. Los gradientes pre-dilatación variaron de 31 a 170 mmHg y post dilatación de 0 a 96 mmHg. No hubo ninguna defunción y las complicaciones más observadas fueron asistolia, flutter y taquicardia supraventricular cada uno con un caso. En conclusión la valvuloplastía pulmonar es un procedimiento de bajo riesgo y con resultados satisfactorios. Palabras claves: Estenosis pulmonar, valvuloplastía.