Balloon-Expandable Pulmonary Valves for Patched or Native Right Ventricular Outflow Tracts.

The implantation of percutaneous balloon expandable valves in native or patched right ventricular outflow tracts (nRVOT) is a challenging technique due to the diversity of anatomies and shapes, the large sizes, and the distensibility of the nRVOT, for which specific techniques have been developed. We present a single center experience with balloon expandable percutaneous pulmonary valves in nRVOT, describing the techniques used, complications observed, and a short-mid term follow-up.. This is a single center descriptive study of patients who underwent a percutaneous pulmonary valve implantation in a nRVOT with a balloon expandable pulmonary valve in our center between September 2012 and June 2022.. We implanted successfully 45 valves in 46 patients (20 Sapien and 25 Melody). Tetralogy of Fallot or pulmonary atresia with VSD were the main congenital heart disease (n = 32). All were pre-stented, 18 in a one step procedure. We used a Dryseal sheath in 13/21 Sapien. In 6 patients we used the anchoring technique, 5 with a very large nRVOT and one pyramidal nRVOT. In the 3.5 year follow-up 7 patients developed endocarditis and 3 required a valve redilation, no fractures were observed. PPVI of native RVOT with balloon expandable valves is feasible in a number of selected anatomies, including large or pyramidal nRVOT, using specific techniques, (presenting, LPA anchoring).

Unidad médico-quirúrgica de Cardiología Pediátrica y Cardiopatías Congénitas del Hospital Universitario Ramón y Cajal de Madrid / Medical-surgical unit of Pediatric Cardiology and Congenital Heart Diseases of the Hospital Ramón y Cajal of Madrid

Desde su creación, en el año 1977 por el Dr. Manolo Quero, coincidiendo con la apertura del Hospital, el Servicio de Cardiología Pediátrica y Cardiopatías Congénitas del Hospital Ramón y Cajal, ha sido y es centro de referencia donde se siguen enfermos de toda la geografia española. Se creó para dar atención especializada a la gran demanda existente en aquellos años. Y, después de casi 40 años, tenemos la gran satisfacción de poder seguir viendo a esos niños, ya convertidos en adultos, y seguimos ofreciéndoles la asistencia necesaria para tratar las cardiopatías complejas de la mayoria de ellos. Este Servicio se ha caracterizado, tanto por su actividad asistencial como por su actividad docente e investigadora, que ha propiciado que sea distinguido como Centro de Referencia Nacional (CSUR). Hoy en día, contarnos con un Servicio mixto de 7 facultativos, provenientes de la Pediatria y de la Cardiología y especializados en la Cardiología Pediátrica y las Cardiopatías Congénitas. El Servicio cuenta con varias Unidades especializadas como la de Hemodinámica Intervencionista Infantil y en Cardiopatías Congénitas, Hipertensión Pulmonar Pediátrica y Unidad de Arritmias Pediátricas y en Cardiopatias Congenitas, entre otros. Nuestro Servicio se integra en un equipo multidisciplinar, compuesto por cirujanos cardiacos, intensivistas pediátricos, anestesistas, obstetras, radiólogos, rehabilitadores y enfermería especializada, entre otros, que permiten la atención integral al enfermo. La gran mayoría de las consultas externas se organizan con la filosofía de la consulta de alta resolución. Realizándose la mayoría de las exploraciones y técnicas complementarias (electrocardiograma, ecocardicigrafía, Holter, ergometría) en el mismo día de la consulta (AU)

Since its creation in 1977 by Dr. Manolo Quero, coinciding with the opening of the Hospital, the Ramon y Cajal Hospital Pediatric Cardiology and Congenital Heart Disease Unit has been and is a referral center where patients coming from any Spanish region can get specialized and personalized integral care for children with congenital heart disease. After almost 40 years, the Service has integrated also the care of our grown up patients with congenital heart defects, into a transversal care unit. This service is characterized by its healthcare activity and its teaching and research that have led it to be distinguished as a National Reference Center (CSUR) activity. Today we are 7 physicians who perform our functions in different sections and allowed to specialize and create units as Hemodynamics, pulmonary hypertension and arrhythmias among others. Our cardiology department is integrated into a also has a rnultidisciplinary team including cardiac surgeons, pediatric intensivists, anesthesiologists, radiologist, physiotherapist, among others that allow for comprehensive patient care nursing. Our outpatient visits are Organized with the philosophy of "high resolution" visits and all the complementary examinations and functional tests) EKG, echocardiography, Holter cardiopulmonary exercise testing, and sometimes in the MRI) are done in the same day of the external visit (AU)

Timing of Pulmonary Valve Replacement: How Much Can the Right Ventricle Dilate Before it Looses Its Remodeling Potential?

Congenital heart disease patients that develop secondary pulmonary regurgitation require a pulmonary valve replacement (PVR) in their follow-up. The indications for PVR in asymptomatic patients are debated. Most guidelines consider a RV end-diastolic volume (RVEDV) over 150 ml/m(2) as an indication for PVR. We analyzed clinical, echocardiographic and MRI variables of patients that underwent a surgical PVR between September 2006 and February 2013. The included patients were asymptomatic, without pulmonary stenosis and with both pre- and post-surgery MRI. Thirty-five patients (74.3 % males) were included. Mean age at PVR was 25.8 years (SD = 7.18), and weight was 64.5 Kg (SD = 12.03). The main diagnosis was tetralogy of Fallot (n = 28), pulmonary atresia (n = 2), primary pulmonary regurgitation (n = 2) and pulmonary regurgitation after percutaneous treatment (n = 2). The maximal RVEDV pre-PVR was 267 ml/m(2), and right ventricular end-systolic volume (RVESV) was 183 ml/m(2). RV size and function were established by MRI: Pre-PVR Post-PVR p RVEDV (ml/m(2)) 162 (SD = 39.1) 94 (SD = 23.6) <0.001 RVESV (ml/m(2)) 87 (SD = 28.9) 44 (SD = 15.7) <0.001 RVEF 44.8 % (SD = 8.17) 52 % (SD = 9.9) <0.001 Patients with a RVEDV under 170 ml/m(2) combined with a RVESV under 90 ml/m(2) had a favorable RV remodeling, defined as RVEDV under 110 ml/m(2) (sensitivity 87.5 %), RVESV under 55 ml/m(2) (sensitivity 100 %) and RVEF over 50 % (sensitivity 100 %). When deciding the optimal PVR timing in asymptomatic patients, both RVEDV and RVESV should be considered. Our results suggest that higher volumes than used in the clinical practice can achieve a good remodeling. Therefore, PVR could be performed later in the follow-up reducing the number of cardiac interventions.

Distrofia simpático-refleja y mioclonías: una asociación infrecuente / Complex regional pain syndrome and myoclonus: an uncommon combination

La distrofia simpático-refleja es una patología infrecuente en la edad pediátrica, que no se relaciona con un trastorno orgánico subyacente y que requiere un tratamiento multidisciplinar debido al importante componente psicosomático que lo acompaña y al difícil control de los síntomas en este cuadro. Por otro lado, las mioclonías tienen un amplio espectro de diagnósticos diferenciales, siendo muy importante descartar enfermedades neurológicas y degenerativas subyacentes. Presentamos el caso de un varón adolescente con dolor neuropático en una localización atípica y con una presentación compleja al asociar mioclonías y distonías a lo largo de su evolución, que obliga a la revisión de ambos cuadros y a realizar un amplio diagnóstico diferencial (AU)

Complex regional pain syndrome (CRPS) is quite uncommon in paediatric patients.There is no identified organic aetiology. CRPS has a very significant psychosomatic component; therefore it is necessary to take a multidisciplinary approach to its treatment, which should include psychiatric assessment. CRPS is very difficult to diagnose, and can take months to control its symptoms. The association with myoclonus is rare, and since myoclonus is the clinical manifestation of a vast spectrum of different neurological disorders, some of which are degenerative, it is important to make a diagnosis as quickly as possible.We present the case of an adolescent male with an atypical presentation of CRPS. CRPS was located in the thorax, which is very unusual and was associated with myoclonus and dystonias.This made the diagnosis harder and widened the aetiological spectrum (AU)

[Complex regional pain syndrome and myoclonus: an uncommon combination]. / Distrofia simpático-refleja y mioclonías: una asociación infrecuente.

Complex regional pain syndrome (CRPS) is quite uncommon in paediatric patients. There is no identified organic aetiology. CRPS has a very significant psychosomatic component; therefore it is necessary to take a multidisciplinary approach to its treatment, which should include psychiatric assessment. CRPS is very difficult to diagnose, and can take months to control its symptoms. The association with myoclonus is rare, and since myoclonus is the clinical manifestation of a vast spectrum of different neurological disorders, some of which are degenerative, it is important to make a diagnosis as quickly as possible. We present the case of an adolescent male with an atypical presentation of CRPS. CRPS was located in the thorax, which is very unusual and was associated with myoclonus and dystonias. This made the diagnosis harder and widened the aetiological spectrum.

Meningitis pediátrica. ¿Cuándo podemos estar tranquilos? / Pediatric meningitis. When can we be calm?

Introducción: Desde que en 1980 se publicó la Escala de Boyer, se han propuesto muchas herramientas clínicas para distinguir entre meningitis viral (MV) y meningitis bacteriana(MB). En los últimos años se ha validado el Bacterial Meningitis Score (BMS), instrumento que identifica a los niños con muy bajo riesgo de padecer MB. Nuestro objetivo es determinarla eficacia de la Escala de Boyer modificada con la proteína C reactiva (PCR), y validar en nuestra población pediátrica el BMS. Métodos: Se realizó un análisis retrospectivo de los niños de3 meses a 15 años de edad hospitalizados con el diagnóstico de meningitis entre 1994 y 2007. Se aplicó la Escala de Boyer, añadiéndose una puntuación correspondiente a la PCR. La sensibilidad y la especificidad se calcularon en los puntos de corte determinantes para la toma de decisiones (puntuaciones 3 y 5). En el caso del BMS, se valoró su sensibilidad y especificidad a partir del cumplimiento de los criterios de inclusión propuestos por sus autores. Resultados: En total se incluyeron 145 casos, de los cuales46 fueron MB y 99 MV. Se determinó la PCR en 73 de ellos. La modificación de la Escala de Boyer con la PCR aumentó la sensibilidad en el punto de corte 5 de un 83 a un 100%, permaneciendo la sensibilidad en el 100% en ambos casos. En el caso del BMS, el cumplimiento de al menos uno de los cinco criterios de riesgo identificó los casos de MB con una sensibilidad del 100% y una especificidad del 61,22%. Conclusión: Una puntuación de más de 5 en la Escala de Boyer modificada con la PCR identifica de forma precisa los casos de meningitis de etiología bacteriana en nuestro medio. El BMS es muy eficaz para determinar los casos de muy bajo riesgo de MB. La combinación de ambas herramientas resulta muy útil en el manejo de los pacientes con sospecha de meningitis (AU)

Introduction: Since Boyer's Score was published in 1980; many clinical tools have been proposed to distinguish between viral meningitis (VM) and bacterial meningitis (BM). Recently, it has been validated the Bacterial Meningitis Score (BMS), an instrument that identifies children at very low risk of BM. Our objective is to determine the accuracy of Boyer's Score modified with C-reactive protein (CRP) and to validate the BMS in our pediatric population. Methods: A retrospective study analysis of children (aged from 3 months to 15 years) hospitalized with a meningitis diagnosis in our pediatric center between 1994 and 2007. We calculated Boyer's Score and added a punctuation corresponding to CRP. The sensitivity and specificity were estimated in the scores cut off point that determine the taking of decisions (punctuations 3 and 5). We applied the BMS to all children using the inclusion criteria proposed by the authors, and calculated the sensitivity and specificity. Results: Among the 145 cases included, 46 had BM and 99 had VM. CRP was determined in 73 of them. The modification of Boyer's Score with CRP increased the sensibility of punctuation 5 from 83% to 100%; the specificity of this punctuation remained in 100% even if CRP was included or not. In the case of BMS, the fact of presenting at least one of the 5 criteria identified the cases of BM with a sensibility of 100% and a specificity of 61.22%. Conclusion: A punctuation of more than 5 in Boyer's Score modified with CRP identifies with high efficacy the cases of bacterial etiology. The BMS is very accurate for the identification of very low risk cases of BM. The combination of both scales is very useful in the management of patients with suspicion of meningitis ( AU)