Short-term outcome of polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of right ventricular outflow tract in tetralogy of fallot: a randomized controlled trial

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Implante percutáneo de válvula pulmonar: a propósito de dos casos colombianos / Percutaneous pulmonary valve implant: Two Colombian case reports

Resumen Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.

Abstract Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

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

Insuficiencia valvular pulmonar en el posoperatorio alejado de tetralogía de Fallot. Aporte del ecocardiograma transtorácico convencional para la toma de decisiones / Pulmonary valve insufficiency in the postoperative tetralogy of Fallot away. Contribution of conventional transthoracic echocardiography for decision making

La insuficiencia valvular pulmonar constituye uno de los principales problemas en el posoperatorio alejado de tetralogía de Fallot, siendo su incidencia muy frecuente y dependiente de múltiples aspectos. En este trabajo se efectúa una revisión relacionada con la utilidad de la ecocardiografía transtorácica convencional como instrumento para la evaluación delos pacientes con insuficiencia valvular pulmonar en el posoperatorio alejado de la tetralogía de Fallot. Se exponen los principales conceptos fisiopatológicos de esta entidad y, en función de ellos, se presentan los parámetros ecocardiográficos más relevantes a tener en cuenta en la valoración de estos pacientes. Finalmente se consideran las pautas y la oportunidad para el reemplazo valvular pulmonar.

Pulmonary valve regurgitation is one of the main problems in the late post-op of Tetrallogy of Fallot, having a great incidence and depending on many aspects . In these paper a review is done considering the usefulness of conventional transtoracic ecocardiography as a tool to evaluate patients with pulmonary regurgitation.in the late post-op of Tetralogy of Fallot. Main physiopathologic concepts about these entity are presented, and as a function of that, the most important ecocardiographic parameters to be considered evaluating these patients are also described. Finally guidelines on the opportunity for pulmonary valve replacement are considered.

Artículo de actualización para formación continuada. Tetralogía de Fallot / Update article for continuous education. Tetralogy of Fallot

La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente en la población general, con una incidencia general de 0,1/1.000 nacidos vivos. El eje morfológico diagnóstico es el desplazamiento anterior izquierdo del septo infundibular -hacia la vía de salida ventricular derecha- durante el periodo de embriogénesis, lo que causa complejo de cabalgamiento aórtico, comunicación interventricular, estenosis subpulmonar e hipertrofia ventricular derecha. Sin intervención quirúrgica, su sobrevida al año alcanza 66 porciento y sólo 10 porciento a 15 porciento en más de veinte años. La presentación clínica es variable y depende del grado de estenosis pulmonar; cuando ya es significativa en la etapa neonatal o en lactantes menores de tres a seis meses, pueden aparecer crisis de hipoxemia que requieren intervención médica o quirúrgica de urgencia. La corrección completa de la malformación ofrece buenos resultados de supervivencia durante décadas, si bien con las primeras técnicas -parche trans-anular y cierre del defecto interventricular- surgen problemas a largo plazo que pueden generar un riesgo adicional de morbimortalidad. La insuficiencia pulmonar severa, la presencia de dilatación ventricular derecha y el desarrollo de arritmias potencialmente fatales son problemas que se tornan importantes y que deben reconocerse a fin de valorar una reintervención temprana y reparar los defectos residuales inductores de arritmia. Las nuevas técnicas quirúrgicas tratan de conservar al máximo la integridad de la unión ventrículo-pulmonar y de la propia válvula, siempre y cuando la anatomía lo permita. Ello redunda, sin duda, en beneficio para el paciente ya que los problemas residuales descritos tienen menor significado clínico.

Emprego e avaliação em médio prazo da cúspide de homoenxerto decelularizado na correção da tetralogia de Fallot / The use and midium-term evaluation of decellularized allograft cusp in the surgical treatment of the tetralogy of Fallot

OBJETIVO: Descrever a técnica de preparo e a evolução ecocardiográfica das cúspides de homoenxerto decelularizado utilizadas em pacientes com tetralogia de Fallot. MÉTODOS: No período de março de 2005 a agosto de 2007, 15 pacientes foram submetidos ao implante deste tipo de enxerto e foram acompanhados clinicamente e com ecocardiograma para avaliar o resultado morfofuncional dos enxertos. RESULTADOS: O acompanhamento médio foi de 12,7 meses (1-25 meses). A análise ecocardiográfica em médio prazo revelou: insuficiência pulmonar leve em nove (60 por cento) pacientes, moderada em três (20 por cento) e importante em três (20 por cento); a função sistólica do ventrículo direito esteve preservada em 13 (86,7 por cento) pacientes e com disfunção leve em dois (13,3 por cento); 11 (73,4 por cento) pacientes não apresentaram gradientes na via de saída do ventrículo direito (VD), e em quatro (26,6 por cento) pacientes evidenciou-se a presença de estenose leve; a mobilidade da cúspide foi normal em todos os pacientes; não houve espessamento maior de 1,5mm nas cúspides analisadas; não se detectou nenhuma calcificação nas cúspides. Catorze (93,3 por cento) pacientes apresentaram Z score entre -1 e 0,7 e um (6,7 por cento) paciente apresentou anel pulmonar com Z score de + 2,5. CONCLUSÃO: O retalho de homoenxerto decelularizado parece ser uma boa opção para a ampliação da via de saída do VD nos pacientes submetidos à correção total da tetralogia de Fallot em médio prazo.

OBJECTIVE: To describe the technique of implantation and to show the echocardiographic follow-up of the decellularized cusps allografts used in patients with tetralogy of Fallot. METHODS: Fifteen patients underwent this implantation between March 2005 and August 2007 and they were clinically followed-up. An echocardiogram was performed to evaluate the morphofunctional result of the allografts. RESULTS: The mean follow-up was 12.7 months (1-25 months). The echocardiography results showed that pulmonary insufficiency was mild in nine (60 percent) patients, moderate in three (20 percent) patients, and severe in three (20 percent) patients. The results also showed that the systolic right ventricle function was normal in 13 (86.7 percent) and that there was mild dysfunction in two (13.3 percent). Eleven (73.4 percent) patients did not present any gradient in the right ventricular outflow tract and four (26.6 percent) presented mild stenosis. The mobility of the cusps were normal in all cases and there was no thickness larger than 1,5mm. There was no calcification; 14 patients (93,3 percent) presented Z score between -1 and 0,7 and one patient presented dilated pulmonary annulus with a Z score of + 2.5. CONCLUSION: In midium-term follow-up, the decellularized allograft seemed to be a good option for right ventricle outflow tract enlargement in patients underwent tetralogy of Fallot.

Anuloplastia de homoenxerto pulmonar criopreservado com anel de Delrin na atresia pulmonar com comunicação interventricular / Annuloplasty of cryopreserved pulmonary homograft with Delrin stent in pulmonary atresia with ventricular septal defect

Criança de seis anos portadora de atresia pulmonar com comunicação interventricular, submetida a correção total com um ano, empregando monoválvula de pericárdio bovino na reconstrução da via de saída do ventrículo direito. Evoluiu com importante regurgitação valvar pulmonar (RVP) e disfunção do ventrículo direito. Na reoperação foi implantado homoenxerto pulmonar criopreservado (HPC) com anuloplastia, utilizando anel de Delrin com o intuito de evitar distorção geométrica do conduto. Após dois anos, o ecocardiograma, semelhante ao pós-operatório imediato, demonstra RVP discreta e função ventricular direita normal, sugerindo que essa manobra pode ser utilizada como coadjuvante para otimizar o resultado do implante do HPC.

A six-year-old child was suffering from pulmonary atresia with ventricular septal defect after a total correction at 1 year of age using a bovine pericardial monocusp valve in the reconstruction of the right ventricle outflow tract. The infant evolved with significant pulmonary valve regurgitation (PVR) and right ventricle dysfunction. On reoperation, a cryopreserved pulmonary homograft (CPH) was implanted with annuloplasty utilizing a Delrin ring with the aim of avoiding geometric distortion of the vessel. After two years, an echocardiogram proved a similar state to the immediate postoperative period with slight pulmonary valve regurgitation and normal right ventricular function suggesting that this maneuver may be used as coadjuvant treatment to optimize the result of CPH implantation.

Disfunçäo diastólica do ventrículo direito no pós-operatório de tetralogia de Fallot / Right ventricular diastolic dysfunction in the postoperative period of tetralogy of Fallot

OBJECTIVE: To assess right ventricular diastolic function in the intermediate postoperative period of repair of tetralogy of Fallot. METHODS: We carried out a case-control study with 60 patients divided into 2 groups as follows: 1) group I - 30 patients who had undergone repair of tetralogy of Fallot and 2) group II - 30 healthy children. The 2 groups were paired for age, sex, and body surface. The flows in the pulmonary and tricuspid valves were analyzed with Doppler echocardiography. The presence of anterograde flow at the end of diastole in the pulmonary artery defined restrictive right ventricular physiology. Surgical, radiological, electrocardiographic, and echocardiographic variables were analized in the group I. RESULTS: The velocity of the A wave and the E/A ratio for the tricuspid valve showed significant differences between the groups. Cases with E/A < 1.30 predominated in inspiration (group I - 19/30, and group II - 5/30). The duration of the QRS complex on the electrocardiogram was significantly increased in patients with E/A <1.30. Nineteen (63.3 percent) patients had restrictive right ventricular physiology, which had a longer postoperative period, longer duration of the QRS complex, and a lower E/A ratio in inspiration. The surgical and radiological variables showed no statistical difference. CONCLUSION: Restrictive right ventricular physiology was detected on the intermediate follow-up of most patients undergoing repair of tetralogy of Fallot. The postoperative period and QRS duration were increased in patients with impairment in diastolic function

Efecto de la fórmula con almidón de maíz pregelatinizado en el tratamiento de las regurgitaciones en lactantes / Effect of a formula with a pre-gelatinized cornstarch in the treatment of regurgitations in infants

Evaluar la eficacia en el tratamiento de las regurgitaciones leves y moderadas, así como la tolerancia por parte de los lactantes, de una fórmula que utiliza un almidón de maíz pregelatinizado como agente que aporte densidad a la misma. Estudio longitudinal, prospectivo, aleatorio y comparativo tipo caso-control, de 60 lactantes menores con regurgitaciones no complicadas. Maternidad "Concepción Palacios", Consulta Externa, Departamento de Pediatría. Ambos grupos fueron comparables desde el punto de vista antropométrica y en cuanto a sus manifestaciones clínicas. La mejoría de la regurgitación se observó desde la primera semana de tratamiento, y progresó de forma directamente proporcional al tiempo de tratamiento. El hipo y el cólico abdominal mejoran con el tratamiento. El crecimiento fue similar en ambos grupos. La tolerancia a la fórmula fue excelente. Sólo un caso de los tratados desarrolló estreñimiento como único efecto colateral observado. Comprobamos que el uso regular de las fórmulas espesadas con almidón de maíz pregelatinizado, es eficaz en el tratamiento de las regurgitaciones leves y moderadas que se presentan en aquellos lactantes alimentados con fórmulas artificiales

Agenesia de valva pulmonar. Avaliação clínico-cirúrgica de 32 pacientes / Absent Pulmonary Valve. Clinical and Surgical Evaluation of 32 Patients

PURPOSE--To study the surgical and clinical evolution of 32 cases with absent pulmonary valve to propose the ideal period of time for surgical correction. METHODS--Clinical and laboratorial analysis were performed in 32 infants, under 12 months of age, between 1980 an 1993, in an evolutive character. From the clinical viewpoint, hypoxic and/or congestive features were considered in previous and late periods related to surgical repair. Laboratorial studies as ECG (cavities overload), chest X-ray (cardiac size and pulmonary vascular markings) and echocardiogram (associated defects, pressure gradients and anatomical aspects of pulmonary arteries) were also analyzed. Cardiac catheterization was performed in 15 patients. RESULTS--Early cyanosis in 84 of cases and "to and for "murmur in 90 of them facilitate clinical diagnosis in whom tetralogy of Fallot was associated in 30 patients. Refractory respiratory and cardiac insufficiency were responsible for operative indication in 12 patients, half of them, operated on under 12 months of age, died. Survival patients were repaired between two to 11 years old. Four deaths occurred early in life, before any surgical consideration and the 16 remaining patients will electively be considered for an opportune repair. CONCLUSION--Conservative clinical treatment is indicated, waiting for a more rigid bronchial wall can support the pressure of the dilated pulmonary arteries. This way, surgical repair is postponed for at least two years of age.

Reconstituiçäo da valva pulmonar e via de saída do ventrículo direito, com prótese bivalvular e prótese tubular valvada de tronco pulmonar de porco estudo experimental e aplicaçäo clínica / Pulmonary valve and outlet right ventricle reconstruction with bivalvular prosthesis and valved tubular prosthesis of the pig pulmonary trunk: experimental study and clinical application

A obstruçao da via de saída do ventrículo direito (VSVD) tem gerado muita polêmica em torno da técnica da sua correçao cirúrgica, sendo a reconstituiçao ainda motivo de controvérsias. Com essa finalidade, foram desenvolvidas duas próteses a partir do tronco pulmonar (TP) de porco: 1) a prótese bivalvular: poderia ser usada na correçao da tétrade de Fallot associada a hipoplasia do anel pulmonar: 2) a prótese tubular valvada, possuindo a própria valva pulmonar: poderia ser empregada na correçao de malformaçoes com descontínuidade entre o VD e TP. Estes dois tipos de próteses foram testados em modelo experimental. Seis ovelhas foram submetidas a implante de prótese bivalvular, com o auxílio da circulaçao extracorpórea (CEC), após ampla ressecçao do infundíbulo pulmonar, incluindo duas válvulas da valva pulmonar, procurando-se, com isto, imitar a reconstituiçao empregada no Fallot. O implante da protese tubular valvada foi realizado em 12 ovelhas, sem o auxílio da CEC, mediante pinçamento tangencial do infundíbulo e TP, permitindo o desvio do fluxo sangüíneo através do conduto, após ligadura do TP. A prótese bivalvular implantada foi avaliada mediante parâmetros hemodinâmicos e ecocardiográficos na fase intra-operatória, conferindo desempenho satisfatório (insuficiência pulmonar discreta ou ausente e gradientes VD-TP menores de 10 mmHg). A seguir, os animais foram sacrificados. O desempenho da prótese tubular valvada foi avaliada na fase intra-operatória com medidas hemodinâmicas, mostrando gradientes acima de 1O mmHg em apenas 3 casos. Sete ovelhas tiveram controle ecocardiográfico com 99 a 135 dias de evoluçao, registrando gradientes de 9,85 mmHg a 49 mmHg (média 19,7). Quatro casos foram submetidos a estudo hemodinâmico no 6( mês de evoluçao, registrando discreto aumento do gradiente (média 22,3); a seguir os animais foram sacrificados e encaminhados para estudo anatomopatológico. A aplicaçao clínica da prótese bivalvular foi realizada em 3 pacientes portadores de t.de Fallot associada a hipoplasia do anel pulmonar (2 casos) e agenesia da valva pulmonar (1 caso), com idades de 16, 2 e 7 anos. Após evoluçao de 3 a 10 meses, os gradientes variaram entre 10 mmHg e 20 mmHg e discreta insuficiência pulmonar valvar ao estudo ecodopplercardiográfico...

Regurgitaçäo congênita da válvula pulmonar / Congenital pulmonary valve regurgitation

Apresentaçäo de um caso de regurgitaçäo congênita da válvula pulmonar onde será discutido os aspectos do seu diagnóstico e conduta terapêutica.