Diagnostic Reference Levels in Interventional Pediatric Cardiology: Two-Year Experience in a Tertiary Referral Hospital in Latin America.

The goal of the present study was to propose the first local diagnostic reference levels (DRLs) for interventional pediatric cardiology procedures in a large hospital in Colombia. The data collection period was from April 2020 to July 2022. The local DRLs were calculated as the 3rd quartile of patient-dose distributions for the kerma-area product (Pka) values. The sample of collected clinical procedures (255) was divided into diagnostic and therapeutic procedures and grouped into five weight and five age bands. The Pka differences found between diagnostic and therapeutic procedures were statistically significant in all weight and age bands, except for the 1-5-year age group. The local DRLs for weight bands were 3.82 Gy·cm2 (<5 kg), 7.39 Gy·cm2 (5-<15 kg), 19.72 Gy·cm2 (15-<30 kg), 28.99 Gy·cm2 (30-<50 kg), and 81.71 Gy·cm2 (50-<80 kg), respectively. For age bands, the DRLs were 3.97 Gy·cm2 (<1 y), 9.94 Gy·cm2 (1-<5 y), 20.82 Gy·cm2 (5-<10 y), 58.00 Gy·cm2 (10-<16 y), and 31.56 Gy·cm2 (<16 y), respectively. In conclusion, when comparing our results with other existing DRL values, we found that they are similar to other centers and thus there is scope to continue optimizing the radiation dose values. This will contribute to establishing national DRLs for Colombia in the near future.

Congenitally corrected transposition of great arteries associated with interrupted aortic arch type A: a case report of an unusual association.

In this article, we report the third case, to our knowledge, of a congenitally corrected transposition of the great arteries associated with an interrupted aortic arch. This is a more complex and rare presentation than dextro-transposition of great arteries with interrupted aortic arch. The child ultimately succumbed to sepsis before the surgical repair was possible. We focus on an image modality report and propose what could be a brief embryologic explanation for this rare case.

Infarto de miocardio con arterias coronarias no obstruidas en pediatría: una afección poco conocida / Myocardial infarction with unobstructed coronary arteries in pediatrics: a little-known entity

Resumen El infarto de miocardio con arterias coronarias no obstruidas (MINOCA, por sus siglas en inglés) ha ganado importancia en los últimos 20 años gracias a la dilucidación de etiologías fisiopatológicas diferentes de las causas obstructivas del flujo coronario. Diversos estudios han evidenciado una prevalencia variable, la cual es más alta en las mujeres. Se han descrito dos grupos de alteraciones en la reactividad coronaria que afectan el flujo: las causas epicárdicas y las causas microvasculares. El diagnóstico de MINOCA es de exclusión; por lo tanto, inicialmente se deben descartar otras posibles causas de isquemia, como miocarditis, miocardiopatía séptica, choque hipovolémico por trauma o quemaduras, y enfermedades renales o pulmonares. Los reportes y estudios de esta enfermedad suelen incluir pacientes adultos o mayores de 18 años. Se presenta el caso de una paciente de 16 años con antecedente de tetralogía de Fallot corregida en etapa de lactante menor y reemplazo valvular pulmonar con bioprótesis y ampliación del tronco pulmonar a los 11 años, quien ingresó con dolor torácico de características coronarias. Cursó con un diagnóstico de MINOCA por exclusión en una institución de cuarto nivel en Cali, Colombia. El diagnóstico de MINOCA en edad pediátrica es raro; sin embargo, es importante saber acerca de su existencia para brindar a los pacientes el mejor manejo disponible, de manera que se aseguren los mejores desenlaces a largo plazo.

Abstract Myocardial infarction with non-obstructed coronary arteries (MINOCA) has gained importance in the last 20 years, due to the elucidation of physiopathological etiologies different from the obstructive causes of coronary flow. Different studies have shown variable prevalence, being higher in women. Different causes have been evidenced in the studies found in two groups of alterations in coronary reactivity: epicardial causes and microvascular causes. The diagnosis of MINOCA must be a diagnosis of exclusion. Therefore, other possible causes of ischemia, such as myocarditis, septic cardiomyopathy, hypovolemic shock due to trauma or burns, renal or pulmonary diseases, should be ruled out initially. The reports and studies done around this pathology usually include adult patients and people older than 18 years. We present the case of a 16-year-old patient with a history of Tetralogy of Fallot corrected as an infant and pulmonary valve replacement with bioprothesis and enlargement of the pulmonary trunk at 11 years of age who was admitted with chest pain of coronary characteristics. The diagnosis after multiples studies and exclusion of other causes was MINOCA in a fourth level institution in Cali, Colombia.

P060 Screening Criteria of Inflammatory Bowel Disease: Application in Colombian Patients With Spondyloarthritis.

BACKGROUND: The Spondyloarthritis (SpA) is a group of chronic inflammatory rheumatic diseases, in which 5-10% of extra-articular manifestations are gastrointestinal such as the inflammatory bowel disease. Objective: To apply the clinical criteria for the screening of inflammatory bowel disease (IBD) in patients with SpA with gastrointestinal symptoms and its association with disease activity and function. METHODS: A Cross-sectional study included 82 patients with SpA, according to ASAS classification criteria without diagnosis of IBD. We applied the Screening criteria for IBD developed by Sanz et al, in the SpA patients. Clinical evaluation by rheumatologist and in patients with ≥ 2 gastrointestinal symptoms clinical evaluation by gastroenterologist and IBD screening criteria were performed. Digital chromoendoscopy, magnification colonoscopy, and histological analysis were performed. Lab tests included, C-reactive protein, sedimentation rate, serum levels of transferrin, ferritin and vitamin B12. The association between clinical variables and colonoscopy and histological variables were evaluated using the Chi-square or Fisher's exact test (Ethical / Cod. 2017-023). RESULTS: Of the 82 individuals evaluated, 58 of them were referred to gastroenterology with a direction to perform colonoscopy with chromeondospia, and 41 of them were able to intervene to whom the IBD screening criteria were applied. 53.7% are men, 7.3% actively smoke. 100% of the population presented some gastrointestinal symptoms, the most frequent being diarrhea of more than 4 weeks in 61%. 68.3% had at least one of the three major criteria. Rectorrhagia was associated with BASFI>4, p=0.050, axial compromise p = 0.043, diagnosis of PsA p = 0.090 and alterations in the architecture of the ileum p=0.034. Diarrhea was associated with ESR> 20, p = 0.050, BASFI>4 p = 0.012. In addition, 70.75 of the patients had at least one of the minor screening criteria associated with higher BASFI levels, p = 0.01. Aphthous stomatitis was reported as extra-intestinal manifestations in 7.3% and abdominal pain in 87.8% of the patients, which was associated with BASDAI>4 p = 0.023, ASDASCRP> 2.1, p = 0.043 and inflammation in the ileum, p = 0.046. No patients with positive iron deficiency anemia were found. However, ferritin alteration was observed in 22% associated with chronic inflammation of the colon, p = 0.042. There were no cases of fever or family history of IBD. Noting that in 17.1% of the cases a decrease in vitamin B12 levels was detected, associated with the presence of ulcers (p = 0.035) and acute inflammation in the ileum, p = 0.032. Weight loss was found in 31.7% of the cases and was associated with smoking history p = 0.039. CONCLUSION: We found a high frequency of major and minor symptoms of IBD, both of which were associated with a high activity of spondyloarthritis and an important functional compromise as well as inflammation markers in this group of patients. The application of the screening criteria for IBD in SpA without IBD reflects a high frequency of intestinal symptoms of sufficient intensity that affect quality of life and disease activity. Early detection of gastrointestinal compromise allows patients to benefit from comprehensive treatment of the disease in its initial stages.

P062 Chromoendoscopy With Magnification Colonoscopy: Analysis of Mucosa of Colon And Ileum In Patients With Gastrointestinal Symptoms Without IBD.

BACKGROUND: Digital chromoendoscopy (Narrow Band Imaging By Olympus) or BLI (Blue Light Imaging By Fujifilm), with the magnification endoscope, allows a detailed evaluation of the mucosal surface and its vascular network, which facilitates the diagnosis and monitoring of early lesions. This technique has demonstrated a better detection, which allows optical diagnosis during a colonoscopy examination. Patients with SpA with nonspecific gastrointestinal symptoms, subclinical intestinal inflammation are defined as endoscopic and histologically. The aim was to detect early structural inflammatory changes by chromoendoscopy and magnification colonoscopy in colonic/ileum digestive mucosa, and establish its association with clinical variables in SpA and gastrointestinal symptoms. Study approved by Institutional Ethics Committee, code HMC 2017-023. METHODS: Clinical evaluation by rheumatologist in SpA patients (ASAS/criteria), fecal calprotectin levels, and HLA-B*27 were evaluated. In patients with ≥2 gastrointestinal symptoms, clinical evaluation by gastroenterologist, digital chromoendoscopy (NBI) or (BLI), magnification colonoscopy, and histological analysis were performed. The association between clinical and colonoscopy variables were established using the Chi-square or Fisher's exact test. RESULTS: In total, 62 SpA patients were included, with mean age of 45.1 ± 11.3 years, axial SpA (77.4%) peripheral SpA (12.9%), biological treatment (69.4%), ASDAS-CRP>2,1 (67.7%), presence of HLA-B*27 (41.9%). Patients with ≥2 gastrointestinal symptoms were found in 67.7%. The most important symptoms were abdominal pain (66.1%), abdominal distension (64.5%), and food intolerance (59.7%). 22.6% of patients showed high level of calprotectin. In those patients with gastrointestinal symptoms, chromoendoscopy and magnification colonoscopy were performed. The mean age of those patients was 45.4 ± 10.5, 57.6% were male, BMI>25 in 69.7%, presence of HLA-B*27 in 39.4%, 33.3% were former smokers, axial SpA in 84.8% and ASDAS-CRP>21 in 78.8%. In total, 27.27% of the patients presented high levels of calprotectin, of which 66.0% had more than two gastrointestinal symptoms (p = 0.015). 77.8% presented alterations in ileal mucosa (p=0.060). The most frequent alteration was the loss of vascular pattern (p = 0.002). By histological analysis, 5 patients had acute inflammation in the ileum, of which 4 had increased levels of fecal calprotectin (p = 0.013). 30.8% of patients positive for HLAB*27:05:02 had ulcers in ileum (p = 0.017) and 61.5% had chronic inflammatory patterns (p=0.020). CONCLUSION: Chromoendoscopy provided an enhanced, detailed contrast of the gastrointestinal mucosa surface, mainly in the loss of vascular pattern in ileum. The active search for symptoms, signs, and biomarkers of gastrointestinal involvement in addition to an objective endoscopic and histological evaluation may offer new perspectives at the evaluation of SpA patients and may provide guidance for specific clinical and therapeutic management.

Bevacizumab as a treatment for hereditary hemorrhagic telangiectasia in children: a case report.

CASE DESCRIPTION: Five-year-old female patient with hereditary hemorrhagic telangiectasia. CLINICAL FINDINGS: Deterioration of cardiopulmonary function with higher oxygen requirements secondary to pulmonary arteriovenous shunts, epistaxis. TREATMENT AND OUTCOME: The patient was treated with the monoclonal antibody bevacizumab, which inhibits the vascular endothelial growth factor, with good clinical outcome. CLINICAL RELEVANCE: Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by arteriovenous malformations in different organs, making its clinical presentations varied. Systemic therapeutic options for a generalized disease are limited. The monoclonal antibody bevacizumab, seems to be a good option in this disorder. Although reported as successful in adult population, its use in pediatric population has not yet been reported. Here we report the use of bevacizumab in a 5-year-old female patient with hereditary hemorrhagic telangiectasia, showing clinical benefits and good outcome.

DESCRIPCIÓN DEL CASO: Paciente de cinco años de sexo femenino con telangiectasia hemorrágica hereditaria. HALLAZGOS CLÍNICOS: Deterioro de la función cardiopulmonar con mayores requerimientos de oxígeno secundario a shunt pulmonar arteriovenoso, epistaxis. TRATAMIENTO Y RESULTADO: La paciente fue tratada con el anticuerpo monoclonal bevacizumab, que inhibe el factor de crecimiento endotelial vascular, con buen resultado clínico. RELEVANCIA CLÍNICA: La telangiectasia hemorrágica hereditaria es un trastorno autosómico dominante caracterizado por malformaciones arteriovenosas en diferentes órganos, lo que hace que sus presentaciones clínicas varíen. Las opciones terapéuticas sistémicas para la enfermedad generalizada son limitadas. El anticuerpo monoclonal bevacizumab, parece ser una buena opción en este trastorno. Aunque se ha reportado como exitoso en la población adulta, su uso en población pediátrica aún no ha sido reportado. Aquí se informa el uso de bevacizumab en una paciente de 5 años de edad con telangiectasia hemorrágica hereditaria, mostrando beneficios clínicos y buen resultado.

La hipertensión pulmonar en el recién nacido con cierre del ductus arterioso en el útero y secuencia de la perfusión arterial reversa en el embarazo gemelar / Pulmonary hypertension of the newborn with in utero closure of the ductus arteriosus and twin reversed arterial perfusion sequence

Resumen Introducción: La hipertensión pulmonar del recién nacido secundario con cierre en el útero del ductus arterioso es una complicación frecuente en las pacientes gestantes que usan analgésicos no esteroideos durante el embarazo. Se presenta un caso de secuencia de la perfusión arterial reversa en el embarazo gemelar con evidencia de supervivencia del gemelo acárdico con el flujo sanguíneo del gemelo bomba. El tratamiento es la cirugía con fetocoagulación láser al gemelo acárdico, previniendo complicaciones mayores para el gemelo sano. Caso clínico: Gemelo con secuencia twin reversed arterial perfusion. A las 22 semanas de gestación se le practicó fotocoagulación con láser al feto acárdico. La madre recibió por dos días acetaminofén y analgésicos no esteroideos para manejo del dolor. Se le realizó ecocardiograma fetal a las 31 semanas de gestación, con evidencia de disfunción diastólica del ventrículo derecho y cierre del ductus arterioso. Se le realizó cesárea de urgencia obteniendo recién nacido que presentó hipertensión pulmonar en el período neonatal, quien con manejo médico tiene resolución de la misma. Conclusión: La hipertensión pulmonar del recién nacido secundario a cierre del ductus arterioso en el útero por ingesta de medicamentos, es una entidad que pone en riesgo la vida del feto, por lo cual su diagnóstico y tratamiento oportuno es indispensable para mejorar la sobrevida de nuestros pacientes.

Abstract Introduction: Pulmonary hypertension of the newborn secondary to in utero closure of the ductus arteriosus is a frequent complication in pregnant patients taking non-steroidal antiinflammatory drugs during pregnancy. We present a case of reversed arterial perfusion sequence in a twin pregnancy with evidence of survival of the acardiac twin with the blood flow of the pump twin. The treatment of choice is surgery with intrafetal laser coagulation on the acardiac twin, to prevent further complications on the healthy twin. Clinical case: Twin with reversed arterial perfusion sequence. At 22 weeks gestation intrafetal laser coagulation on the acardiac twin was performed. The mother received acetaminophen for two days and non-steroidal anti-inflammatory drugs for the management of pain. Fetal echocardiogram was conducted at 31 weeks gestation, evidencing diastolic dysfunction of the right ventricle and ductus arteriosus closure. She underwent an emergency c-section; the newborn had pulmonary hypertension during the neonatal period that was solved with medical management. Conclusion: Pulmonary hypertension of the newborn secondary to in utero closure of the ductus arteriosus due to drug consumption is an entity that poses a risk to the fetus, so a timely diagnosis and treatment is essential to improve survival of our patients.

Bevacizumab as a treatment for hereditary hemorrhagic telangiectasia in children: a case report / Bevacizumab como tratamiento para telangiectasia hemorrágica hereditaria en niños: Reporte de caso

Abstract Case description: Five-year-old female patient with hereditary hemorrhagic telangiectasia. Clinical Findings: Deterioration of cardiopulmonary function with higher oxygen requirements secondary to pulmonary arteriovenous shunts, epistaxis. Treatment and Outcome: The patient was treated with the monoclonal antibody bevacizumab, which inhibits the vascular endothelial growth factor, with good clinical outcome. Clinical Relevance: Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by arteriovenous malformations in different organs, making its clinical presentations varied. Systemic therapeutic options for a generalized disease are limited. The monoclonal antibody bevacizumab, seems to be a good option in this disorder. Although reported as successful in adult population, its use in pediatric population has not yet been reported. Here we report the use of bevacizumab in a 5-year-old female patient with hereditary hemorrhagic telangiectasia, showing clinical benefits and good outcome.

Resumen Descripción del caso: Paciente de cinco años de sexo femenino con telangiectasia hemorrágica hereditaria. Hallazgos Clínicos: Deterioro de la función cardiopulmonar con mayores requerimientos de oxígeno secundario a shunt pulmonar arteriovenoso, epistaxis. Tratamiento y resultado: La paciente fue tratada con el anticuerpo monoclonal bevacizumab, que inhibe el factor de crecimiento endotelial vascular, con buen resultado clínico. Relevancia clínica: La telangiectasia hemorrágica hereditaria es un trastorno autosómico dominante caracterizado por malformaciones arteriovenosas en diferentes órganos, lo que hace que sus presentaciones clínicas varíen. Las opciones terapéuticas sistémicas para la enfermedad generalizada son limitadas. El anticuerpo monoclonal bevacizumab, parece ser una buena opción en este trastorno. Aunque se ha reportado como exitoso en la población adulta, su uso en población pediátrica aún no ha sido reportado. Aquí se informa el uso de bevacizumab en una paciente de 5 años de edad con telangiectasia hemorrágica hereditaria, mostrando beneficios clínicos y buen resultado.

Evaluación ecocardiográfica de prótesis valvulares en población pediátrica / Echocardiographic assessment of valve prostheses in the pediatric population

En niños con insuficiencia o estenosis valvular significativa, la intervención de la válvula, ya sea valvuloplastia o reemplazo valvular, suele ser inevitable. El seguimiento de estos pacientes puede ser complejo; los síntomas iniciales de disfunción valvular protésica son inespecíficos y en ocasiones es difícil diferenciar entre los efectos de la disfunción valvular, las patologías no cardíacas, la disfunción ventricular y la hipertensión pulmonar. Aunque el examen físico puede alertar al clínico acerca de alteraciones, se requieren otros métodos diagnósticos para evaluar la función de la prótesis. La ecocardiografía Doppler es el método de elección no invasivo para estudiar la función valvular y pese a que muchas de las mediciones y parámetros de normalidad y anormalidad son extrapolados de estudios en adultos, se han tratado de definir estándares en pacientes pediátricos. Este documento ofrece una revisión acerca de las técnicas usadas en el estudio de las válvulas protésicas con base en literatura científica, consensos internacionales y opiniones de expertos.

In children with significant valvular insufficiency or stenosis, valve surgery, either valvuloplasty or valve replacement is usually inevitable. The monitoring of these patients can be complex, the initial symptoms of prosthetic valve dysfunction are nonspecific and sometimes difficult to differentiate between the effects of valvular dysfunction, non-cardiac pathologies, ventricular dysfunction and pulmonary hypertension. Although physical examination may alert the clinician about alterations, other diagnostic methods to assess the function of the prosthesis is required. Doppler echocardiography is the noninvasive method of choice to study valvular function and although many of the measurements and parameters of normality and abnormality are extrapolated from adult studies have been attempted to define standards in pediatric patients. This document provides an overview about the techniques used in the study of prosthetic valves based on scientific literature, international consensus and expert opinion.

Tetralogía de Fallot asociada a síndrome de cimitarra / Tetralogy of Fallot associated with scimitar syndrome

Se expone el caso de un niño de catorce meses de edad, con tetralogía de Fallot asociada a síndrome de cimitarra, cuyo diagnóstico se realizó mediante ecocardiografía, cateterismo cardiaco y angio-TAC. Se hace referencia a las consideraciones clínicas y quirúrgicas de este caso que hasta la fecha es el quinto reportado en la literatura.

We describe the case of a fourteen-month-old boy with tetralogy of Fallot associated with scimitar syndrome, whose diagnosis was made by echocardiography, cardiac catheterization and angio-CT. We relate the clinical and surgical considerations in this case which is so far the fifth reported in the literature.

Acceso vascular inusual para cateterismo cardiaco en pacientes con cardiopatía congénita compleja: reporte de un caso(AU) / Unusual vascular access for cardiac catheterization in patients with complex congenital heart disease

La obtención del acceso vascular es fundamental en la realización de diferentes procedimientos de cateterismo cardíaco. Los cateterismos a repetición y las cirugías cardíacas múltiples pueden resultar en una marcada incidencia de pérdida de estos sitios de acceso, razón por la cual, en algunas oportunidades, es necesario el uso de técnicas no convencionales. Se reporta el caso de un paciente de un año de edad en quien fue necesario el abordaje retroperitoneal de la arteria ilíaca común.

Obtaining vascular access is essential in the realization of different cardiac catheterization procedures. The recurrent catheterizations and multiple cardiac surgeries may result in marked incidence of loss of these access sites, which is why sometimes it is necessary to use unconventional techniques. We report the case of a one year old patient that necessitated the retroperitoneal approach of the common iliac artery.

Origen anómalo de la coronaria izquierda en la arteria pulmonar: una serie de casos / Anomalous origin of the left coronary artery: a series of cases

La anomalía de la arteria coronaria izquierda que nace en la arteria pulmonar (ALCAPA por su sigla en Inglés: anomalous left coronary artery from the pulmonary artery), es una enfermedad cardiaca congénita, de baja incidencia y de espectro clínico amplio. La principal forma de presentación es la falla cardiaca por miocardiopatía dilatada. Mediante la revisión de historias clínicas se recolectaron cinco casos consecutivos de ALCAPA, de los que se describen sus cuadros clínicos, así como su diagnóstico y tratamiento. Todos fueron dados de alta en mejores condiciones y asisten a controles periódicos. Estos casos ilustran al origen anómalo de la coronaria izquierda, como diagnóstico diferencial de la miocardiopatía dilatada.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a congenital cardiac anomaly with low incidence and a broad clinical spectrum. Its main form of presentation is congestive heart failure due to dilated cardiomyopathy. We reviewed clinical histories and collected 5 consecutive ALCAPA cases; its clinical symptoms, diagnosis and treatment were described. All five patients were discharged in better clinical conditions and continue attending to periodic medical follow-up. These cases illustrate the ALCAPA as part of the differential diagnosis of dilated cardiomyopathy.

Defectos del complejo septal interventricular. Consideraciones quirurgicas / Defects of intraventricular septal complex

Los defectos del Septo Interventricular son defectos frecuentes y generalmente hacen parte de las cardiopatías congénitas complejas. Su reconocimiento es vital para definir la mejor estrategia para su corrección que debe garantizar un cierre hermético sin afectar el tejido de conducción ni el funcionamiento valvular. En este artículo se revisa de una manera práctica su enfoque y tratamiento