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Introducción: El trasplante hepático (TH), es una terapia establecida en el tratamiento de diversas enfermedades del hígado agudas y crónicas terminales y del carcinoma hepatocelular (CHC). Las principales indicaciones en nuestro medio son la cirrosis de diferentes etiologías, el CHC, la atresia de vías biliares en niños y la falla hepática fulminante (FHF). Menos del 10% corresponden a indicaciones inhabituales, que incluyen pacientes con una miscelánea de enfermedades entre las cuales están la enfermedad poliquística hepática (EPH), enfermedades metabólicas (Niemann – Pick, otras), el síndrome hepato/portopulmonar, metástasis de diferentes tumores, etc.Objetivo: Describir y evaluar los resultados obtenidos con el trasplante hepático en estas indicaciones.Material y Método: Estudio de cohorte no concurrente que incluyó los TH por indicaciones inhabituales realizados entre marzo de 1997 y diciembre de 2016. De 295 TH realizados, 34 (11,5%) fueron por estas indicaciones.Resultados: Las causas más frecuentes fueron el síndrome porto/hepatopulmonar en 11 (40,7%) pacientes y la EPH en 9 (26,5%). Las enfermedades metabólicas representaron la tercera indicación, con 5 (14,7%) casos. 7 (20,6%) pacientes eran menores de 18 años. Las complicaciones más frecuentes fueron biliares y la trombosis de arteria hepática en 6 (17,6%) y 4 (11,8%) casos respectivamente; estos últimos eran portadores de una EPH masiva. 4 (12,5%) pacientes requirieron retrasplante. La mortalidad a 90 días fue de 2 (5,9%) enfermos. Conclusión: El TH es una opción factible en este grupo de pacientes con resultados similares a los obtenidos en las indicaciones clásicas.

Resumen: Introducción: La evaluación ecocardiográfica de pacientes post-trasplante cardiaco pediátrico es una herramienta útil. Sin embargo, aún es necesario definir un protocolo óptimo de seguimiento. Obje tivo: Caracterizar los resultados de la aplicación de un protocolo de evaluación ecocardiográfica fun cional en pacientes en control post-trasplante cardiaco pediátrico ortotópico. Pacientes y Método: Posterior al trasplante cardiaco pediátrico se realizó una ecocardiografía con un protocolo funcional que incluyó strain longitudinal global. Se evaluaron además los datos de la biopsia endomiocárdica y del estudio hemodinámico contemporáneos. Resultados: De un total de 9 pacientes sólo 1 presentó disfunción sistólica de ventrículo izquierdo según parámetros clásicos, pero casi la totalidad presentó un strain longitudinal global alterado. No se observaron episodios de rechazo moderado o severo en estos pacientes. Todos los pacientes presentaban disfunción sistólica ventricular derecha. No se ob servó correlación entre estos parámetros y la presión de arteria pulmonar. Conclusiones: Se observó disfunción sistólica biventricular subclínica en la mayoría de los pacientes de esta serie. No hubo aso ciación con episodios de rechazo o con hipertensión pulmonar, lo que podría estar en relación con la ausencia de episodios de rechazo moderado o severo en la biopsia contemporánea y con el reducido tamaño muestral. Sólo el seguimiento a largo plazo de estos pacientes permitirá definir la relevancia clínica de los hallazgos descritos.

Introduction: Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. Objectives: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Material and Methods: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Results: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. Conclusions: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed urgently and 34.5% died on the waiting list.

The echocardiographic evaluation of patients after heart transplantation is a useful tool. However, it is still necessary to define an optimal follow-up protocol.To describe the results of the application of a functional echocardiographic protocol in patients being followed after pediatric heart transplantation.Alls patients being followed at our institution after pediatric heart transplantation underwent an echocardiographic examination with a functional protocol that included global longitudinal strain. Contemporaneous endomyocardial biopsy results and hemodynamic data were recorded.9 patients were evaluated with our echocardiographic functional protocol. Of these patients, only 1 showed systolic left ventricular dysfunction according to classic parameters. However, almost all patients had an abnormal global longitudinal strain. Right ventricular systolic dysfunction was observed in all patients. No epidodes of moderate to severe rejectiom were recorded. No correlation was observed between these parameters and pulmonary artery pressure.Subclinical biventricular systolic dysfunction was observed in the majority of the patients in this study. No association with rejection episodes or pulmonary hypertension was observed, which may be related to the absence of moderate or severe rejection episodes during the study period, and to the small sample size. Long term follow-up of these patients may better define the clinical relevance of our findings.

Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children.To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation.Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality.Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively.Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed as a medical emergency and 34.5% died on the waiting list.