Fetal aortic valvuloplasty as the first step in a complex therapeutic strategy.

BACKGROUND: Fetal aortic valvuloplasty (FAV) is proposed to prevent hypoplastic left heart syndrome due to fetal critical aortic stenosis. OBJECTIVE: to report our experience on FAV as the first step in a complex therapeutic strategy. METHOD: Series of patients with FAV over an 18-year period. RESULTS: 27 FAVs were performed in 26 fetuses, with technical success in 82% (22/27) and periprocedural fetal demise in 22% (6/27), decreasing to 15% in the second half-cohort. Loss to follow-up was due to birth or postnatal therapy in other centers (5) and termination of pregnancy (1), A normal-sized LV at birth was observed in 46% (6/13), 4 neonates underwent aortic valvuloplasty and 2 cardiac surgeries, with 5/6 achieving biventricular circulation at 28 days, and 3 transplant-free survival at mid-term follow-up. The 7/13 born with a borderline LV underwent LV rehabilitation strategy, with survival at 28 days in 4/7 and at mid-term in 3: one with biventricular circulation, one with a ventricle-and-a-half repair, and one lost to follow-up. CONCLUSION: FAV was feasible in most cases, with no maternal complications, and biventricular circulation at 28 days in ∼40% of survivors. After FAV, a diverse range of postnatal cardiac interventions are performed, reflecting the challenging innovation in current cardiovascular therapy.

[Problem-Based Learning virtual with a peer tutor in pandemic]. / Aprendizaje basado en problemas en modalidad virtual con tutor par en pandemia: Opinión estudiantes primer año de medicina.

PURPOSE OF THE STUDY: To know the experience of first-year medical students at Universidad Austral de Chile regarding virtual Problem-Based learning (PBL) tutorials carried out by peer tutors during the second semester of 2021. METHODS: Quantitative and qualitative research, transversal, descriptive. The non-probabilistic sample consisted of 45 medical students who participated in virtual PBL tutorials with peer tutors. Data collection through an anonymous and voluntary opinion survey comprised two areas: methodology and role of the tutor, considering open questions related to facilitating and hindering aspects. Quantitative data analysis was performed using descriptive statistics with frequency measurements. The qualitative ones with progressive reduction. Study approved by the Scientific Ethics Committee, Valdivia Health Service. RESULTS: There is a high degree of satisfaction in all three areas. Both in the qualitative and quantitative results, the participants positively value the performance of the peer tutor, highlighting empathy, commitment, trust, facilitation, feedback, and a good learning environment, allowing collaborative learning. Unlike the quantitative, the qualitative is more frequently identified as hindering factors, poor internet connection, excessive curricular load, and lack of knowledge about the methodology by the tutor. CONCLUSIONS: Although the preparation of peer tutors for PBL tutorials is essential, it is even more critical in virtual mode since they face challenges such as managing virtual platforms and achieving effective communication that allows learning collaboratively.

Congenitally corrected transposition of the great arteries outcomes of different surgical techniques in a paediatric population: A single-centre report.

BACKGROUND: Congenitally corrected transposition of the great arteries is a complex pathology characterised by atrioventricular and ventriculo-arterial discordance. Optimal surgical approaches are still a matter of debate. OBJECTIVE: To evaluate the outcomes of different surgical treatments in a single centre. METHODS: Between 1998 and 2020, 89 patients were studied. The cohort was divided into three groups: physiologic, anatomic, and univentricular repair. RESULT: Physiologic correction (56.18%) was associated with significant tricuspid valve regurgitation progress (42%) and complete AV block (30%) compared to anatomic repair. Right ventricular systolic dysfunction was developed in 14%. Instead, anatomic correction (30.34%) (double switch 59% and Rastelli type 40.7%) presented moderate to severe aortic regurgitation (4%) and left ventricular systolic dysfunction (11%). Complete AV block was developed in 14.8%. Rate of reintervention was 34% for physiologic and 26% for anatomic. Univentricular palliation (13.8%) presented no complications or late mortality during the follow-up. The overall survival at 5 and 10 years, respectively, was 80% (95% CI 69, 87) and 75% (95% CI 62, 84). There was no statistically significant difference in mortality between the groups (p log-rank = 0.5752). CONCLUSION: Management of congenitally corrected transposition of the great arteries remains a challenge. In this cohort, outcomes after physiologic repair were satisfactory in spite of the progression of tricuspid regurgitation and the high incidence of AV block. Anatomic repair improved tricuspid regurgitation but increased the risk of aortic regurgitation and left ventricular systolic dysfunction. The Fontan group showed the lowest incidence of complications.

Takayasu arteritis in paediatrics.

Takayasu arteritis is an idiopathic chronic granulomatous panarteritis predominantly affecting the aorta and its main branches. Although idiopathic, genetic contribution to disease susceptibility is being increasingly recognised. Rare in children, Takayasu arteritis is a worldwide disease with significant morbidity and mortality. Its diagnosis is a challenge and requires awareness of the condition as clinical features at presentation are non-specific and assessing disease activity is difficult. In the inflammatory stage, treatment is essential to prevent the insidious course and vascular damage: stenotic, occlusive lesions, aneurysms, and aortic regurgitation. New imaging modalities, such as CT scan, MRI, and 18F-fluorodeoxyglucose positron emission tomography, have expanded the possibilities for non-invasive diagnosis and monitoring; however, digital subtraction arteriography remains the gold standard for the diagnosis of Takayasu arteritis. Steroids are the first-line medical treatment. The combined use of methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, and biological agents is common. Revascularisation therapy should be considered in uncontrolled hypertension secondary to renal artery stenosis, symptomatic coronary ischaemia, cerebrovascular disease, severe aortic regurgitation, limb ischaemia, and aneurysms at risk of rupture, using surgical or endovascular procedures and taking into consideration that complications, especially restenosis, are frequent. Disease activity increases the likelihood of complications after revascularisation. Surgical intervention has shown better long-term outcomes, although the endovascular approach is evolving. The aim of this review was to describe key points of the diagnosis, treatment, and follow-up of Takayasu arteritis in childhood.

Heterogeneous atypical cell populations are present in blood of metastatic breast cancer patients.

INTRODUCTION: Circulating tumor cells (CTCs) are commonly isolated from the blood by targeting the epithelial cell adhesion molecule (EpCAM) through positive selection. However, EpCAM can be downregulated during metastatic progression, or it can be initially not present. We designed the present prospective trial to characterize CTCs as well as other circulating cell populations in blood samples from women with metastatic breast cancer without EpCAM-dependent enrichment and/or isolation technology. METHODS: A total of 32 patients with metastatic breast cancer were enrolled, and blood samples were processed using a previously described negative depletion immunomagnetic methodology. Samples from healthy volunteers were run as controls (n = 5). Multistep sequential labeling was performed to label and fix cell-surface markers followed by permeabilization for cytokeratins (CK) 8, 18 and 19. Multiparametric flow cytometry (FCM) analysis was conducted using a BD LSR II flow cytometer or a BD FACSAria II or FACSAria III cell sorter. Immunocytochemical staining on postenrichment specimens for DAPI, EpCAM, CD45, CK, epidermal growth factor receptor and vimentin was performed. Expression of these markers was visualized using confocal microscopy (CM). RESULTS: CD45-negative/CK-positive (CD45- CK+) populations with EpCAM + and EpCAM - expression were identified with both FCM and CM from the negatively enriched patient samples. In addition, EpCAM + and EpCAM - populations that were CK + and coexpressing the pan-hematopoietic marker CD45 were also noted. There were more CK + EpCAM - events/ml than CK + EpCAM + events/ml in both the CD45- and CD45+ fractions (both statistically significant at P ≤ 0.0005). The number of CK + CD45- and CK + CD45+ events per milliliter in blood samples (regardless of EpCAM status) was higher in patient samples than in normal control samples (P ≤ 0.0005 and P ≤ 0.026, respectively). Further, a significant fraction of the CK + CD45+ events also expressed CD68, a marker associated with tumor-associated macrophages. Higher levels of CD45-CK + EpCAM - were associated with worse overall survival (P = 0.0292). CONCLUSIONS: Metastatic breast cancer patients have atypical cells that are CK + EpCAM - circulating in their blood. Because a substantial number of these patients do not have EpCAM + CTCs, additional studies are needed to evaluate the role of EpCAM - circulating cells as a prognostic and predictive marker.

[Pediatric telecardiology experience in the COVID-19 pandemic context]. / Experiencia en telecardiología infantil en el contexto de la pandemia de COVID-19.

Introduction: The COVID-19 pandemic has caused a global impact on public health services. Using new strategies through telehealth for the management of patients with congenital heart disease was the challenge. Objective: To describe the experience in telecardiology, and the strategies implemented during the pandemic. Method: Retrospective, qualitative study that includes the period from April 2020 to April 2021. Inquiries were received through the service's official e-mail or telephone. They were classified according to the type of concern and complexity of heart disease using color coding. The responses were asynchronous (by e-mail) or synchronous (videoconferences). The videoconferences were made using a secure platform (Cisco-Webex). Results: From April 2020 to April 2021, a total of 3372 queries were answered. The responses were distributed via e-mail (64.9%), phone calls (1.2%) and videoconferences (14.5%). The most frequent reasons for consultation were the request for missed appointments (68%), and remote clinical monitoring (20%). A total of 2296 families was contacted. Only 14.1% of the inquiries were cited in person. With color coding, a stratification was performed according to urgency. Conclusions: Telehealth proved to be a useful tool for the clinical management of patients with congenital heart disease in their place of origin. It prevented a considerable number of transfers, identified patients at risk rapidly, comforted families, and strengthened ties with local hospitals that make up the health network.

Introducción: La pandemia de COVID-19 ha causado un impacto global en los servicios de salud pública. Utilizar nuevas estrategias a través de la telesalud para el manejo de los pacientes con cardiopatías congénitas fue el desafío. Objetivo: Describir la experiencia en telecardiología y las estrategias implementadas durante la pandemia. Método: Estudio retrospectivo, cualitativo, que comprende el periodo de abril de 2020 a abril de 2021. Se recibieron consultas a través del correo electrónico oficial del servicio o por teléfono. Se clasificaron según tipo de inquietud y complejidad de la cardiopatía utilizando una codificación por colores. Las respuestas a las mismas fueron asincrónicas (correo electrónico) o sincrónicas (videoconferencias). Las videoconferencias se realizaron utilizando una plataforma segura (Cisco-Webex). Resultados: Se contestaron 3372 consultas. Las respuestas fueron distribuidas en correos electrónicos (64.9%), llamados telefónicos (1.2%), videoconferencias (14.5%) y otros métodos (19.4%) Los motivos de consulta más frecuentes fueron la solicitud de turnos suspendidos (68%) y control clínico a distancia (20%). Se pudo establecer contacto con 2296 familias. Solo el 14.1% de las consultas se citó en forma presencial. Con la codificación por colores se logró realizar una estratificación según la urgencia. Conclusiones: La telesalud mostró ser una herramienta útil para el manejo clínico de pacientes con cardiopatías congénitas en su lugar de origen. Evitó un gran número de traslados, identificó pacientes en riesgo, confortó a las familias y fortaleció vínculos con hospitales locales que integran la red de salud.

Clinical experience with Berlin Heart Excor in pediatric patients in Argentina: 1373 days of cardiac support.

The objective of this study was to describe our experience (1373 days of support) with the Berlin Heart Excor (BH) ventricular-assist device (VAD) as bridging to cardiac transplantation in pediatric patients with end-stage cardiomyopathy. This study involved a retrospective observational cohort. Records of patients supported with the BH VAD were reviewed. Data regarding age, sex, weight, diagnosis, preoperative condition, single versus biventricular support, morbidity, and mortality were collected. Criteria for single versus biventricular support and intensive care unit management were registered. The procedure was approved by our Institutional Ethics Committee, and informed consent was obtained. Between March 2006 and March 2010, 12 patients with diagnosis of dilated (n = 10) and restrictive (n = 2) cardiomyopathy were supported. Median age was 56.6 months (range 20.1-165.9); mean weight was 18.3 kg (range 8.5-45); and nine patients were female. Every patient presented with severe heart failure refractory to pharmacological therapy. Biventricular support was necessary in four patients. Nine patients underwent heart transplantation. No child was weaned off the BH VAD because of myocardial recovery. Mean length of support was 73 days (range 3-331), and the total number of days of support was 1373. Three patients had fatal complications: 2 had thrombo-hemorrhagic stroke leading to brain death, and one had refractory vasoplegic shock. The BH VAD is a useful and reasonable safe device for cardiac transplantation bridging in children with end-stage heart failure. Team experience resulted in less morbidity and mortality, and time for implantation, surgical procedure, anticoagulation monitoring, and patient care improved.

[Alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient]. / Rabdomiosarcoma alveolar con metástasis cardíaca en un paciente pediátrico.

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.

El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.

Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report. / Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

Aprendizaje basado en problemas en modalidad virtual con tutor par en pandemia: Opinión estudiantes primer año de medicina / Problem-Based Learning virtual with a peer tutor in pandemic

PROPÓSITO DEL ESTUDIO: Conocer la experiencia de estudiantes de primer año de la carrera de Medicina, Universidad Austral de Chile con respecto a tutorías virtuales de ABP realizadas por tutores pares, durante el segundo semestre del 2021. MATERIAL Y MÉTODO: Investigación cuantitativa y cualitativa, transversal, descriptiva. Muestra no probabilística, conformada por 45 estudiantes de medicina que participaron en ABP virtual con tutores/as pares. Recolección de datos mediante encuesta de opinión anónima y voluntaria (Google Forms), compuesta por 2 ámbitos: metodología y rol del tutor/a, considerando preguntas abiertas relacionadas con aspectos facilitadores y obstaculizadores. Análisis de datos cuantitativo con programa Excel, utilizando estadística descriptiva con medidas de frecuencia. Los cualitativos con reducción progresiva. Estudio aprobado por Comité Ético Científico, Servicio de Salud Valdivia. Resultados: Existe un alto grado de satisfacción en los dos ámbitos. Tanto en los resultados cualitativos y cuantitativos los participantes valoran positivamente el desempeño del rol del tutor par, destacando: empatía, compromiso, confianza, facilitador, proporciona retroalimentación y buen ambiente de aprendizaje, permitiendo el aprender en forma colaborativa. A diferencia de lo cuantitativo en lo cualitativo se identifica con mayor frecuencia como factores obstaculizadores, la mala conexión a internet, excesiva carga curricular y desconocimiento de la metodología por parte del tutor. CONCLUSIONES: Si bien la preparación de tutores pares para el desarrollo de las tutorías de ABP es fundamental, más aún lo es para la implementación en modalidad virtual, ya que se enfrentan a otros desafíos como el manejo de plataformas y lograr una comunicación efectiva que permita aprender colaborativamente.

PURPOSE OF THE STUDY: To know the experience of first-year medical students at Universidad Austral de Chile regarding virtual Problem-Based learning (PBL) tutorials carried out by peer tutors during the second semester of 2021. METHODS: Quantitative and qualitative research, transversal, descriptive. The non-probabilistic sample consisted of 45 medical students who participated in virtual PBL tutorials with peer tutors. Data collection through an anonymous and voluntary opinion survey comprised two areas: methodology and role of the tutor, considering open questions related to facilitating and hindering aspects. Quantitative data analysis was performed using descriptive statistics with frequency measurements. The qualitative ones with progressive reduction. Study approved by the Scientific Ethics Committee, Valdivia Health Service. RESULTS: There is a high degree of satisfaction in all three areas. Both in the qualitative and quantitative results, the participants positively value the performance of the peer tutor, highlighting empathy, commitment, trust, facilitation, feedback, and a good learning environment, allowing collaborative learning. Unlike the quantitative, the qualitative is more frequently identified as hindering factors, poor internet connection, excessive curricular load, and lack of knowledge about the methodology by the tutor. Conclusions: Although the preparation of peer tutors for PBL tutorials is essential, it is even more critical in virtual mode since they face challenges such as managing virtual platforms and achieving effective communication that allows learning collaboratively

[Endovascular intervention in Takayasu Arteritis. Case report]. / Tratamiento endovascular en arteritis de Takayasu. Presentación de un caso clínico.

A four year old patient with no medical history was admitted to our hospital's Emergency Department, suffering from edema and hypertension. During physical examination a low pulse was detected in the left arm and a lack of pulses in lower limbs, with a blood pressure difference greater than 10 mm Hg between both arms. An echocardiogram demonstrated severe aortic abdominal coarctation and ventricular dysfunction. Takayasu arteritis was diagnosed and cardiac angiography was performed. Disconnection of left subclavian artery and severe tightness at the abdominal aorta were confirmed. The definitive diagnosis was Takayasu arteritis. Angioplasty with stent was performed in abdominal aorta, with good subsequent results. The pulses improved in the lower limbs, and normal blood pressure values without gradient between all members were registered. The echocardiogram improved ventricular function and the gradient in the coarctation area was significantly reduced.

Una paciente de cuatro años concurrió a la Guardia por edemas e hipertensión arterial. Al momento del examen físico, se detectó disminución del pulso en el brazo izquierdo y ausente en los miembros inferiores, con diferencia de tensión arterial mayor de 10 mmHg entre los miembros superiores. Se realizó un ecocardiograma, en el que se observó coartación grave de la aorta a nivel abdominal y disfunción ventricular. Con la sospecha de arteritis de Takayasu, se completaron estudios con angiotomografía cardíaca, que confirmó una desconexión de la arteria subclavia izquierda y estrechez grave en la aorta abdominal. El diagnóstico definitivo fue de arteritis de Takayasu. Se realizó una angioplastía con estent por Hemodinamia en la aorta abdominal, con buenos resultados posteriores. Mejoraron los pulsos de los miembros inferiores, y se obtuvieron valores normales de la tensión arterial. En el ecocardiograma, mejoró la función ventricular, y el gradiente en la zona de coartación se redujo significativamente.

Rabdomiosarcoma alveolar con metástasis cardíaca en un paciente pediátrico / Alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient

El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.

Formación de estudiantes de Medicina como tutores pares en aprendizaje basado en problemas / Training medical students as tutors via peer instructionin problem-based learning

Introducción: El aprendizaje basado en problemas es una estrategia educativa que se fortalece con tutores pares. La dificultad para contar con docentes interesados en ser tutores y con tiempo disponible, motivó a crear un programa de tutoría de pares en aprendizaje, basado en problemas en la Escuela de Medicina de la Universidad Austral de Chile. Objetivo: Difundir el programa de formación de estudiantes tutores en aprendizaje basado en problemas para implementarlo en otras universidades en las carreras que lo requieran. Métodos: Se describió el plan de formación de tutores pares en aprendizaje basado en problemas. Los estudiantes de cursos superiores fueron seleccionados según sus méritos académicos y asistieron a talleres de capacitación centrados en el manejo de la tutoría y el fortalecimiento de las competencias genéricas. Durante el aprendizaje basado en problemas se hicieron un seguimiento permanente y reuniones formales con los tutores. Al finalizar, se aplicaron encuestas de opinión para evaluar el desempeño de los alumnos, y cada tutor recibió un pequeño reconocimiento económico y créditos en horas de ayudantía. Resultados: Este programa generó un alto interés entre los estudiantes, fue bien evaluado y permitió formar a 120 estudiantes como facilitadores en aprendizaje basado en problemas. Las encuestas realizadas a 45 estudiantes y 45 tutores revelaron un alto grado de satisfacción en todos los ámbitos consultados. Conclusiones: Los tutores pares son la mejor manera de trabajar el aprendizaje basado en problemas. Estos requieren de una capacitación previa para contar con las herramientas necesarias de su rol de facilitadores, así como seguimiento, evaluación y apoyo permanente(AU)

Introduction: Problem-based learning is an educational strategy which may be strengthened by peer tutoring. The difficulty to engage teachers interested in being tutors and the scarcity of available time led to the development of a problem-based peer tutoring learning program at the Medical School of the Austral University of Chile. Objective: Disseminate information about the problem-based learning student tutor training program so as to implement it at other universities in the majors requiring it. Methods: A description was made of the problem-based learning peer tutor training program. Students selected from higher courses according to their academic merits attended training workshops dealing with tutoring management and the strengthening of generic competences. Problem-based learning sessions included permanent follow-up and formal meetings with tutors. At the end, opinion surveys were applied to evaluate the performance of students, and each tutor received a small economic incentive and credits in teaching assistance hours. Results: Students were highly motivated by the program, which was evaluated as satisfactory and made it possible to train 120 students as problem-based learning facilitators. The surveys applied to 45 students and 45 tutors revealed a high level of satisfaction in all the areas consulted. Conclusions: Peer tutoring is the best way to conduct problem-based learning. Tutors require previous training so they have the tools required by their role as facilitators, as well as permanent follow-up, evaluation and support(AU)

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Assessment of γ-H2AX levels in circulating tumor cells from patients receiving chemotherapy.

Circulating tumor cells (CTCs) are prognostic markers in a variety of solid tumor malignancies. The potential of CTCs to be used as a "liquid biopsy" to monitor a patient's condition and predict drug response and resistance is currently under investigation. Using a negative depletion, enrichment methodology, CTCs isolated from the peripheral blood of breast cancer patients with stage IV breast cancer undergoing DNA damaging therapy with platinum-based therapy were enriched. The enriched cell suspensions were stained with an optimized labeling protocol targeting: nuclei, cytokeratins 8, 18, and 19, the surface marker CD45, and the presence of the protein γ-H2AX. As a direct or indirect result of platinum therapy, double-strand break of DNA initiates phosphorylation of the histone H2AX, at serine 139; this phosphorylated form is referred to as γ-H2AX. In addition to γ-H2AX staining in specific locations with the cell nuclei, consistent with previous reports and referred to as foci, more general staining in the cell cytoplasm was also observed in some cells suggesting the potential of cell apoptosis. Our study underscores the utility and the complexity of investigating CTCs as predictive markers of response to various therapies. Additional studies are ongoing to evaluate the diverse γ-H2AX staining patterns we report here which needs to be further correlated with patient outcomes.

Tratamiento endovascular en arteritis de Takayasu: Presentación de un caso clínico / Endovascular intervention in Takayasu Arteritis: Case report

Una paciente de cuatro años concurrió a la Guardia por edemas e hipertensión arterial. Al momento del examen físico, se detectó disminución del pulso en el brazo izquierdo y ausente en los miembros inferiores, con diferencia de tensión arterial mayor de 10 mmHg entre los miembros superiores. Se realizó un ecocardiograma, en el que se observó coartación grave de la aorta a nivel abdominal y disfunción ventricular. Con la sospecha de arteritis de Takayasu, se completaron estudios con angiotomografía cardíaca, que confirmó una desconexión de la arteria subclavia izquierda y estrechez grave en la aorta abdominal. El diagnóstico definitivo fue de arteritis de Takayasu. Se realizó una angioplastía con estent por Hemodinamia en la aorta abdominal, con buenos resultados posteriores. Mejoraron los pulsos de los miembros inferiores, y se obtuvieron valores normales de la tensión arterial. En el ecocardiograma, mejoró la función ventricular, y el gradiente en la zona de coartación se redujo significativamente.

A four year old patient with no medical history was admitted to our hospital's Emergency Department, suffering from edema and hypertension. During physical examination a low pulse was detected in the left arm and a lack of pulses in lower limbs, with a blood pressure difference greater than 10 mm Hg between both arms. An echocardiogram demonstrated severe aortic abdominal coarctation and ventricular dysfunction. Takayasu arteritis was diagnosed and cardiac angiography was performed. Disconnection of left subclavian artery and severe tightness at the abdominal aorta were confirmed. The definitive diagnosis was Takayasu arteritis. Angioplasty with stent was performed in abdominal aorta, with good subsequent results. The pulses improved in the lower limbs, and normal blood pressure values without gradient between all members were registered. The echocardiogram improved ventricular function and the gradient in the coarctation area was significantly reduced.

Programa nacional de cardiopatías congénitas: diseños, implementación y resultados a mediano plazo / National program of congenital heart diseases: design, implementation and midterm results

El Programa Nacional de Cardiopatías Congénitas se diseñó e implementó sobre la base de redes, teniendo en cuenta la categorización de las instituciones participantes según estándares internacionales, la oferta derecursos humanos y el pago por desempeño. La cultura evaluativa aplicada ha sido un desafío para mejorar estrategias con el fin de perfeccionar el Programa. En los resultados a mediano plazo se describen los primeros indicadores epidemiológicos que marcan los pasos a seguir para una evolución continua.

Presentación clínica y evolución del isomerismo derecho / Clinical Presentation and Outcome of Right Isomerism

Introducción: El dextroisomerismo es una de las formas más complejas de cardiopatías congénitas. Los avances en el tratamiento médico y en los procedimientos quirúrgicos de los últimos años han permitido encarar el manejo de estos pacientes, no obstante lo cual el pronóstico sigue siendo incierto o poco satisfactorio. Objetivo: Comunicar las características clínicas, conductas y resultados del dextroisomerismo en nuestra población hospitalaria. Material y métodos: Estudio de diseño de cohorte retrospectivo realizado en el Hospital Nacional de Pediatría “Prof. Dr. Juan P. Garrahan”. Entre 1997 y 2011 se identificaron 72 pacientes con una mediana de seguimiento de 5,1 años (rango 1-26 años). Resultados: En el 91,7% la presentación fue neonatal, 66 pacientes con cianosis y 6 con insuficiencia cardíaca. Las características anatómicas más frecuentes fueron válvula AV común (n = 56), obstrucción pulmonar (n = 67), conexión ventriculoarterial discordante (n = 44) y tipo doble salida (n = 27), aurícula única (n = 25), vena cava superior bilateral sin innominada (n = 30), anomalía total del retorno venoso pulmonar (ARVP) (n = 43), asplenia (n = 53). Se detectaron anomalías extracardíacas en 11 pacientes. Al 76,38% se les indicó tratamiento quirúrgico, el estadio máximo alcanzado fue la cirugía paliativa en 14 pacientes, Glenn en 17, bypass de ventrículo subpulmonar (BPVP) en 23 y cirugía tipo ventrículo uno y medio en 1 paciente. La mortalidad global fue del 39,45% (n = 28); para los diferentes procedimientos paliativos fue del 29%, para el estadio de Glenn del 29% y para el BPVP, del 21,76%. En el análisis univariado, la mortalidad asociada con ARVP infradiafragmática fue significativa (p = 0,02). Para el estadio de Glenn la mortalidad se relacionó con el Glenn bilateral (p = 0,04), mientras que para el BPVP no se identificó una causa determinada. En los estadios de la cirugía univentricular desarrollaron estenosis de venas pulmonares 3 pacientes, colaterales aortopulmonares 4 pacientes y progresión a insuficiencia AV grave 2 pacientes. Conclusiones: En la mayoría, la edad de presentación fue neonatal y con clínica de cianosis. En el dextroisomerismo, la fisiología univentricular es predominante. Se detectó un 15% de anomalías extracardíacas. La mortalidad de los pacientes no quirúrgicos y con cirugía paliativa estuvo asociada con ARVP infradiafragmática. En el estadio de Glenn, la mortalidad se relacionó con el tipo bilateral. Solamente un tercio de los pacientes pudieron alcanzar el estadio de BPVP. Los eventos en el seguimiento a mediano plazo en los pacientes en los estadios de Glenn y BPVP son frecuentes.

Background: Right isomerism is one of the most complex forms of congenital heart disease. Recent advances in medical treatment and surgical procedures have allowed addressing the management of these patients. Nevertheless, the prognosis remains uncertain or unsatisfactory. Objective: The aim of this study was to report the clinical characteristics, management and outcomes of right isomerism in our hospital population. Methods: This was a retrospective cohort design study conducted at Hospital Nacional de Pediatría “Prof. Dr. Juan P. Garrahan”. Between 1997 and 2011, 72 patients with median followup of 5.1 years (1 and 26 years) were identified. Results: In 91.7% of cases, patients were in the neonatal period, 66 patients with cyanosis and 6 patients with heart failure. The most frequent anatomic lesions were: common AV valve (n=56), pulmonary obstruction (n=67), ventriculoarterial discordance (n=44) and double outlet right ventricle (n=27), commom atrium (n=25), bilateral superior vena cava without innominate vein (n=30), total anomalous pulmonary venous return (APVR) (n=43) and asplenia (n=53). Extracardiac lesions were detected in 11 patients. Cardiovascular surgery was indicated in 76.38% of cases: The maximum stage achieved was palliative surgery in 14 patients, Glenn procedure in 17 patients, subpulmonary ventricular bypass (PVBP) in 23 patients and one and a half ventricular correction in 1 patient. Overall mortality was 39.45% (n=28). Mortality for the different palliative procedures was 29%, for the Glenn stage, 29% and for PVBP, 21.76%. In the univariate analysis, a significant association was found between mortality and infradiaphragmatic APVR (p=0.02). Glenn stage mortality was related to bilateral Glenn procedure (p=0.04), whereas no related cause was identified for PVBP. In the univentricular stages of surgery, 3 patients developed pulmonary vein stenosis, 4 patients developed aortopulmonary collaterals, and 2 patients progressed to AV regurgitation. Conclusions: The majority of cases presented in the neonatal period and with cyanosis characteristics. In right isomerism, univentricular physiology is predominant. Extracardiac anomalies were detected in 15% of cases. Mortality of non-surgical and palliative procedure cases was associated with infradiaphragmatic APVR. Glenn stage mortality was related to bilateral procedures. Only one third of patients could reach PVBP. Events in Glenn and PVBP midterm follow-up are frequent.

[Clinical study and analysis of risk factors for mortality in 86 cases of infectious endocarditis in children and adolescents in Argentina: 1988-2000]. / Análisis clínico y de los factores de riesgo de mortalidad de 86 casos de endocarditis infecciosa en niños y adolescentes en Argentina (1988-2000).

INTRODUCTION: IE is a rare infection in children. Scarce reports with large number of patients are published. METHODS: Between January 1988 to December 2000 we analyzed all cases of IE cases admitted to our hospital. RESULTS: 86 cases of IE (4.9/10,000 admissions) in 86 children were diagnosed. The median age was 7.6 years. In 77% of patient previous cardiac disease was detected, interventricular defects and Tetralogy of Fallot were the more frequent. Three percent of children had rheumatic heart disease. Thirty-six percent of children had previous heart surgery. Fifty-seven percent have been received previous antibiotics. Eighty-seven percent had positive blood cultures, being the S. aureus and S. viridans the predominantly. Forty-eight percent of children had complications. The metabolic disorders and the nosocomial infections were the most frequent. Twenty-four percent required surgery, 24% of them in the first week of the diagnosis. The mortality in operated children was 19%. In the multivariate analysis we could observe that children with more than 7 years and S. aureus isolation in blood cultures had more incidence of complications and posterior surgery (p < 0.05). Children with S. aureus IE had longer duration of fever, more incidence of complications than patients with S. viridans IE (p < 0.05). Ten percent of children were treated as outpatients. The global mortality was 12,8%. Previous surgery (OR = 6.89; IC 95% 1.54-30.7) and previous antibiotic treatment (OR = 9.98; IC 95% 1.12-88.8) were the factors related with higher mortality in the multivariate analysis. S. aureus was the predominat pathogen and caused more morbidity and mortality than S. viridans IE. CONCLUSION: Children with IE with previous surgery and previous antibiotic treatment died with more frequency.

Miocardiopatía restrictiva en pediatría: hallazgos clínicos, hemodinámicos y evolución / Restrictive cardiomyopathy in pediatric patients

La miocardiopatía restrictiva idiopática es una entidad poco frecuente y de mal pronóstico en pacientes pediátricos.El objetivo de este estudio fue describir los hallazgos clínicos,hemodinámicos y evolutivos de la miocardiopatía restrictiva idiopática en un grupo de pacientes pediátricos.Población,material y metodos:Entre enero de 1990 y enero de 2000 se evaluaron 14 pacientes con una media de edad de 5 años(rango 4a-12a)a los que se les efectuó exámen clínico,electrocardiograma,radiografía de tórax y ecocardiograma.Además se realizó resonancia nuclear magnética en 7 pacientes,estudio hemodinámico con biopsia en 5 pacientes,ergometría y cámara gamma en 2 pacientes.Resultados.En el momento del diagnostico,11/14 pacientes(78,6 por ciento)eran sintomáticos.En todos el electrocardiograma mostró agrandamiento auricular y alteraciones del segmento ST,en la radiografía de tórax se observó cardiomegalia moderada con agrandamiento auricular y congestión pasiva.El estudio hemodinámico demostró aumento de la presión auricular izquierda,capilar pulmonar y pulmonar,En el ecocardiograma se observó agrandamiento auricular,ventrículo izquierdo con tamaño y funcionamiento conservada,patrón doppler alterado en el 42,5 por ciento de los pacientes e insuficiencia mitral y tricúspida en el 85,7 por ciento y 57,1 por ciento respectivamente.La biopsia de miocardio demostró fibrosis intersticial sin eosinófilos y la RNM confirmó el diagnóstico.Durante el seguimiento promedio de 4 años(rango 3 meses-10 años)fallecieron 6 pacientes(42,8 por ciento)Las causas de muerte fueron:arritmia en el 33 por ciento e insuficiencia cardíaca en el 66 por ciento.Se encuentran en clase funcional I 4/8 pacientes(50 por ciento)2 con alteraciones eléctricas(bloqueo auriculoventricular de primer grado,taquicardia supraventricular)y en clase funcional II-III sin alteraciones en la conducción,3/8(37,5 por ciento)Se envió un paciente a trasplante.Conclusiones.Los elementos clínicos demostraron ser más confiables como factores de pronóstico y mortalidad que los signos ecocardiográficos.Los pacientes con compromiso de la función ventricular sistólica,signos de bajo débito e hipertensión pulmonar deben ser enviados a trasplante y esta posibilidad terapéutica se debe consideran en aquellos con arritmias aún sin signos de compromiso hemodinámico por el alto riesgo de muerte súbita