Implante percutáneo de válvula pulmonar autoexpandible Venus P en niños. Primer caso en Colombia / Percutaneous implantation of the Venus P self-expandable pulmonary valve in children. First case in Colombia

Resumen En pacientes con cardiopatía congénita, el implante percutáneo de válvula pulmonar es el tratamiento de elección en tractos de salida del ventrículo derecho (TSVD) disfuncionales. La válvula autoexpandible Venus-P, fue aprobada recientemente para su uso en tractos nativos de gran tamaño. El objetivo de este estudio es reportar la experiencia del primer caso de implantación exitosa de válvula pulmonar Venus-P en Colombia. Paciente de 16 años con Tetralogía de Fallot, con corrección completa a los 11 meses de vida. Presenta insuficiencia pulmonar severa y deterioro de la clase funcional. Se realiza cateterismo cardíaco para la colocación de válvula Venus-P 34 x 30 mm, procedimiento que se lleva a cabo sin complicaciones, evidenciando mejoría significativa de la presión diastólica de la arteria pulmonar, sin gradiente final entre el ventrículo derecho y arteria pulmonar. Existen varios reportes de experiencias en el implante de válvula Venus P a nivel mundial con resultados favorables. La colocación percutánea de la válvula representa disminución en tiempos del procedimiento y estancia hospitalaria. Consideramos el reemplazo valvular percutáneo en posición pulmonar en TSVD nativos es una opción posible y segura para mejorar la insuficiencia pulmonar y la disfunción ventricular derecha en este tipo de pacientes.

Abstract In patients with congenital heart disease, transcatheter pulmonary valve implantation is the treatment of choice in dysfunctional right ventricular outflow tracts (RVOT). The self-expandable Venus-P valve was approved recently for use in large native tracs. It is reported the experience of the first case of successful implantation of Venus-P pulmonary valve in Colombia. Ours was a 16-year-old patient with Tetralogy of Fallot, with complete correction at 11 months of life. The patient has severe pulmonary insufficiency and functional class deterioration. Cardiac catheterization was performed to place a 34 x 30 mm Venus-P valve, a procedure carried out without complications, evidencing significant improvement in the diastolic pressure of the pulmonary artery, without final gradient between the right ventricle and pulmonary artery. Reports exist of experiences in Venus-P valve implant globally with favorable results; percutaneous valve placement represents decreased procedure times and hospital stays. We believe percutaneous valve replacement in pulmonary position in native RVOTs is a possible and safe option to improve pulmonary insufficiency and right ventricular dysfunction in these types of patients.

Podemos Acreditar no Ecocardiograma de Rotina para Avaliar o Ventrículo Direito e a Insuficiência Pulmonar? Um Estudo Comparativo com Ressonância Magnética Cardíaca / Can we Trust in Routine Echocardiography to Assess the Right Ventricle and Pulmonary Insufficiency? A Comparative Study with Cardiac Magnetic Resonance

Resumo Fundamento A ressonância magnética cardíaca (RMC) é o método de escolha para avaliar as dimensões e a função do ventrículo direito (VD), e a insuficiência pulmonar (IP). Objetivos Avaliar a acurácia da ecocardiografia bidimensional (ECO 2D) em estimar a função e as dimensões do VD e o grau de IP, e comparar os resultados obtidos pela ECO 2D com os da RMC. Métodos Comparamos os relatórios de ECO e RMC de pacientes cuja indicação para RMC havia sido para avaliar VD e IP. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Incluímos 51 pacientes com cardiopatia congênita com idade mediana de 9,3 anos (7-13,3 anos). Observou-se uma baixa concordância entre ECO 2D e RMC quanto à classificação da dimensão (Kappa 0,19; IC 95% 0,05 a 0,33, p 0,004) e da função do VD (Kappa 0,16; IC 95% -0,01 a +0,34; p 0,034). O tamanho do VD foi subestimado pela ECO 2D em 43% dos casos, e a função do VD foi superestimada pela ECO 2D em 29% dos casos. O grau de concordância entre os métodos quanto à classificação da IP não foi significativo (Kappa 0,014; IC 95% -0,03 a +0,06; p 0,27). Houve uma tendência de a ECO 2D superestimar o grau da IP. Conclusões A ECO 2D mostrou baixa concordância com a RMC quanto às dimensões e função do VD, e grau de IP. Em geral, a ECO subestimou as dimensões do VD e superestimou a função do VD e o grau de IP quando comparada à RMC.

Abstract Background Cardiac magnetic resonance (CMR) is the method of choice for assessing right ventricular (RV) dimensions and function, and pulmonary insufficiency (PI). Objectives To assess the accuracy of two-dimensional echocardiography (2D ECHO) in estimating RV function and dimensions, and the degree of PI, and compare the 2D ECHO and CMR findings. Methods We compared ECHO and CMR reports of patients whose indication for CMR had been to assess RV and PI. A p-value < 0.05 was considered statistically significant. Results We included 51 congenital heart disease patients, with a median age of 9.3 years (7-13.3 years). There was poor agreement between 2D ECHO and CMR for classification of the RV dimension (Kappa 0.19; 95% CI 0.05 to 0.33, p 0.004) and function (Kappa 0.16; 95% CI -0.01 to +0.34; p 0.034). The RV was undersized by 2D ECHO in 43% of the cases, and RV function was overestimated by ECHO in 29% of the cases. The degree of agreement between the methods in the classification of PI was not significant (Kappa 0.014; 95% CI -0.03 to +0.06, p 0.27). 2D ECHO tended to overestimate the degree of PI. Conclusions The 2D ECHO showed a low agreement with CMR regarding the RV dimensions and function, and degree of PI. In general, ECHO underestimated the dimensions of the RV and overestimated the function of the RV and the degree of PI as compared with CMR.

Resonancia magnética cardíaca en el seguimiento alejado de pacientes con tetralogía de Fallot / Cardiac Magnetic Resonance in long term follow-up of Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot (TF) es la cardiopatía congénita cianótica más frecuente. La insuficiencia pulmonar (IP) y dilatación del ventrículo derecho (VD) son las complicaciones más frecuentes a largo plazo. La resonancia magnética cardiaca (RMC) es el "gold standard" para la evaluación del VD. OBJETIVO: Analizar la información obtenida de las RMC en el seguimiento de pacientes con TF. PACIENTES Y MÉTODO: Se incluyeron RMC realizadas entre 2007 y 2012 a pacientes con TF, reparados con parche transanular (PTA) o ampliación infundibular (AInf) y sin recambio valvular pulmonar (RVP). La fracción de regurgitación pulmonar (FRP), el volumen y función ventricular fueron evaluados. RESULTADOS: Se realizaron 122 RMC a 114 pacientes. Edad promedio al examen 15,4 ± 7,4 años. 53,3% presentó IP severa (> 40%). La media del volumen de fin de diástole del VD (VFDVD) fue 157,3 ± 38,6 ml/m2, fin de sístole (VFSVD) de 85,3 ± 27 ml/m2 y fracción de eyección (FEVD) 46,4 ± 7,1%. 48,4% presentaba un VFDVD mayor de 150 ml/m2 y el 32,8% mayor a 170 ml/ m2. El PTA se relacionó con mayores volúmenes de VD que la AInf. VFDVD mayor a 170 ml/m2 mostraron peor FEVD (FEVD 47,9 ± 7% vs 43,2 ± 6,4%, p < 0,01). DISCUSIÓN: Casi la mitad mostró una significativa dilatación del VD demostrando que la indicación de RMC es tardía en el seguimiento. El PTA se asoció con mayores VFDVD y VFSVD pero no a peor FEVD.

INTRODUCCIÓN: Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart disease. Pulmonary regurgitation (PR) and right ventricle (RV) enlargement and dysfunction are the most common long-term complications. Cardiac magnetic resonance (CMR) is the gold standard for RV evaluation. OBJECTIVE: To analyze CMR results in the follow-up of TOF patients. PATIENTS AND METHOD: All CMR performed between 2007 and 2012 in TOF patients with transannular patch (TAP) repair or infundibular widening, and without pulmonary valve replacement (PVR) were included. Pulmonary regurgitant fraction (PRF), ventricular end-diastolic (EDV) and end-systolic volume (ESV), and ejection fraction (EF) were examined. RESULTS: 122 CMR were performed in 114 patients. Average age at CMR was 15.4±7.4 years. 53.3% of them presented severe PR (> 40%). RVEDV was 157.3 ± 38.6 ml/m2, RVESV was 85.3 ± 27 ml/m2 and RVEF was 46.4 ± 7.1%. RVEDV was > 150 ml/ m2 in 48.4% and > 170 ml/m2 in 32.8% of patients. Patients with TAP showed larger RV volumes compared with those with infundibular widening. RVEDV > 170 ml/m2 showed worse RVEF that those with lower RVEDV (47.9 ± 7% vs 43.2 ± 6.4%, p < 0.01). CONCLUSION: Almost half of the pa tients showed significant RV enlargement, demonstrating that the indication of CMR is late in their follow-up. TAP was associated with higher RVEDV and RVESV, but no worse RVEF.

Evaluación de dispositivos extracorpóreos de soporte pulmonar en adultos con insuficiencia pulmonar aguda severa / Evaluation of extracorporeal lung support devices in adults with severe acute lung failure

Introducción: La injuria pulmonar aguda (IPA) se asocia con altas tasas de mortalidad. El tratamiento se basa principalmente en la ventilación mecánica (VM), con distintas variantes. Los dispositivos de soporte pulmonar extracorpóreo (ECLS) pueden proporcionar suficiente intercambio gaseoso en pacientes con IPA, cuando a pesar de la optimización del tratamiento habitual persiste hipoxemia o hipercapnia con acidosis respiratoria grave. Los dispositivos pueden ser con bomba (ECMO) o sin bomba (pECLA). El uso de ECMO y pECLA están autorizados en Argentina, Reino Unido (RU) y EEUU, y en la mayoría de los países europeos. Objetivo: Evaluar si disminuye la mortalidad con el uso de dispositivos de ECLS asociados a VM contra el uso de VM solamente, en pacientes adultos con IPA aguda grave. Secundariamente, se evaluará en dichos grupos los días libres de VM al día 28, los días de estancia hospitalaria, los días de estancia en UCI, las diferencias a nivel hemodinámico y respiratorio del ECMOvv y los pECLA. Finalmente se evaluarán reportes de costo-efectividad. Método: Se efectuó una revisión sistemática en TripDataBase, Pubmed, buscadores genéricos y sitios específicos. Se buscaron políticas de cobertura. Resultados: Se hallaron 4 ECCA, 8 SC, 4 estudios secundarios, 2 evaluaciones económicas y 1 ETS. Además, se localizaron políticas de cobertura en el RU y EEUU. Conclusiones: Se evidencia reducción de mortalidad en el corto tiempo (30 días o intrahospitalaria) en comparación con VM. No se encontraron diferencias a largo plazo, en días libres de VM-28d, en estancia hospitalaria, ni en UCI. En pECLA la evidencia es de menor calidad y no hay evidencia de reducción de mortalidad. Los parámetros hemodinámicos no son modificados por el ECMOvv, ni por pECLA. ECMOav es útil en casos de inestabilidad hemodinámica. La transferencia de gas pulmonar mejora con el uso de ECLS (reducción de PaCO2 y aumento de PaO2). El mayor beneficio se encuentra en el tratamiento de la hipercapnia, para la cual pECLA parecen ser los más indicados, en relación a su menor complejidad y costo. Muchas publicaciones coinciden en tasas menores de complicaciones con pECLA vs ECMOvv, en particular con respecto a la coagulación y hemólisis. Las indicaciones deben ser estrictamente controladas y monitorizadas. Los sistemas de salud hacen especial hincapié en la derivación a centros de alto volumen y complejidad para su uso. Encontramos costo efectividad para ECMO en los sistemas de salud de EEUU y RU, con valores de USD 7.000-35.000 por año de vida o QALY ganado y de £19.252 (IC95% 7.622-59.200) para pECLA.

Introduction: Acute lung injury (ALI) is associated with high mortality rates. Treatment is based on mechanical ventilation (MV). Extracorporeal lung support (ECLS) devices can provide sufficient gas exchange in patients with ALI when hypoxemia or hypercapnia persists with severe respiratory acidosis despite usual treatment. A pump can be used (ExtraCorporeal Membrane Oxigenator - ECMO) or not (pumpless ExtraCorporeal Lung Support - pECLA) towards to move blood stream in these devices. ECMO and pECLA are authorized in Argentina, UK, USA and most European countries. Objective: To evaluate decreasement in mortality with ECLS+MV vs. MV alone in adult patients with severe ALI. To evaluate MV-free-28-days, hospital length of stay (LOS), ICU LOS, differences in hemodynamic and respiratory parameters in vvECMO vs. pECLA. Cost-effectiveness will be evaluated. Methods: A systematic review in Tripdatabase, Pubmed, general search engines and specific sites was made. Coverage policies were sought. Results: 4 randomized controlled trials, 8 case series, 4 secondary studies, 2 economic evaluations and 1 sanitary technology evaluation were found. UK and USA coverage policies were located. Conclusions: ECLS+VM demonstrated reduction in 30-days-mortality compared to VM alone. No other differences (MV-free-28-days, LOS, ICU LOS) were found. Evidence about use of pECLA is low quality. There is no evidence in mortality reduction with pECLA. Hemodynamic parameters are not modified by vvECMO nor pECLA. avECMO is useful in hemodynamic instability. ECLS enhances lung gas transfer (CO2 arterial pressure reduction and O2 arterial pressure increase). More important benefits are achieved in hypercapnia treatment where pECLA seem to be more suitable because of its lower complexity and cost. There is lower complication rates in pECLA vs. vvECMO, particularly respect to coagulation and hemolysis complications. Indications should be strictly controlled and monitored. There is high recommendation on referral to high-volume centers for use of ECLS. ECMO is cost effective in US and UK health systems with USD 7,000-35,000 per life year or QALY gained and £ 19,252 (95% CI 7622-59200) for pECLA.

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.