Causa inusual de cianosis en el recién nacido: hemoglobina M / Unusual cause of cyanosis in the newborn: hemoglobin M

Resumen La hemoglobina M es un desorden hereditario infrecuente, causante de metahemoglobinemia y, por ende, cianosis. Las manifestaciones clínicas de esta enfermedad son amplias y variadas, por lo que se debe considerar como diagnóstico diferencial en un recién nacido con cianosis sin otra causa aparente. A continuación, se presenta el caso de un recién nacido de 5 horas de vida con cianosis generalizada desde el nacimiento y con saturaciones de oxígeno de entre 60-70%, en el que se descartaron patologías como hipoxia perinatal, patología pulmonar o cardíaca y sepsis y se documentó un nivel de metahemoglobina elevado, reportado en 21,6%, con lo cual se estableció el diagnóstico de metahemoglobinemia. El tratamiento administrado fueron dos dosis de azul de metileno, pero no hubo respuesta clínica. Por este motivo, se realizó electroforesis de hemoglobina, la cual fue compatible con hemoglobina M (Iwate o Kankakee), lo que se confirma su causa congénita de metahemoglobinemia.

Abstract Hemoglobin M is a rare hereditary disorder that causes ethemoglobinemia and therefore cyanosis. The clinical manifestations of this condition differs considerably, so it should be considered as a differential diagnosis in a newborn with cyanosis, with no other apparent cause. The case of a 5 hours old newborn is presented below, with generalized cyanosis from birth with oxygen saturations between 60-70%, in whom, upon ruling out pathologies such as perinatal hypoxia, pulmonary disease, heart disease and sepsis, a high level of methemoglobin is documented, reported in 21,6%; the diagnosis of methemoglobinemia was established. The treatment administered was two doses of methylene blue with no response. For this reason, hemoglobin electrophoresis was performed, which was compatible with Hemoglobin M (Iwate or Kankakee), confirming its congenital cause.

Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis

ABSTRACT Clinical data: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. Chest radiography: Cardiomegaly with prevalence of pulmonary vascular network. Electrocardiogram: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. Echocardiogram: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. Computed tomography: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. Diagnosis: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. Operation: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

Sulfohemoglobinemia secundaria a zopiclona. Casos clínicos / Sulfhemoglobinemia secondary to the use of zopiclone. Report of two cases

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.

Anomalía de Ebstein en recién nacido / Ebstein's anomaly in newborn

Introducción: La anomalía de Ebstein es una rara malformación con presentación sintomática respiratoria leve a grave a causa de defectos cardiacos congénitos manifestados durante la niñez. Objetivo: Exponer el caso en el cual el diagnóstico oportuno de esta enfermedad permitió el tratamiento integral del paciente. Presentación del caso: Recién nacido a término, quien a las siete horas de su nacimiento presentó saturación de oxígeno disminuida, examen físico sin cianosis, soplo pansistólico en la auscultación y cardiomegalia detectada por rayos X de tórax. Se realizó ecocardiograma que permitió diagnosticar al bebé como portador de la enfermedad de Ebstein con manifestaciones leves. Una vez que tiene mejoría clínica, se le proporcionó el alta médica con indicación de valoración por cardiología pediátrica. Conclusiones: La anomalía de Ebstein puede comenzar en el período neonatal con sintomatología de severidad variable. La sospecha clínica permite la confirmación diagnóstica y el seguimiento estrecho, sobre todo en casos severos(AU)

Introduction: Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood. Objective: To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient. Case presentation: Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology. Conclusions: Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases(AU)

Importancia clínica del diagnóstico de la metahemoglobinemia / Clinical importance of the diagnosis of methemoglobinemia

Introducción: La metahemoglobina es una forma de hemoglobina en la que el grupo hemo, usualmente en forma ferrosa, es oxidado a forma férrica, lo que afecta el transporte de oxígeno. El incremento por encima de los valores de referencia se denomina metahemoglobinemia. Objetivo: Actualizar conceptos como prevención, manifestaciones clínicas, diagnóstico de laboratorio y tratamiento de elección de esta enfermedad, con la información disponible de la última década. Métodos: Se realizó una revisión de la literatura en inglés y español, a través del sitio web PubMed, el motor de búsqueda Google académico y Scielo, de artículos publicados en los últimos 10 años. Los términos de búsqueda usados incluyeron metahemoglobinemia, déficit de citocromo b5 reductasa, cianosis y cooximetría. Análisis y síntesis de la información: La metahemoglobinemia se puede clasificar en congénita y adquirida, esta última es la más frecuente. Es importante el diagnóstico de esta enfermedad que aunque es un padecimiento poco común, puede cursar con complicaciones graves e incluso la muerte. Puede ser evitable con diagnóstico temprano y tratamiento oportuno para reducir las complicaciones asociadas a este cuadro. Conclusiones: El diagnóstico y el tratamiento, profiláctico y terapéutico de la metahemoglobinemia en su etapa aguda o de mantenimiento, requieren la adecuada actualización del profesional de la salud(AU)

Introduction: Methemoglobin is a form of hemoglobin in which the heme group, usually in the ferrous form, is oxidized to the ferric form, which affects oxygen transport. The increase above the reference values ​​is called methemoglobinemia. Objective: To update concepts such as prevention, clinical manifestations, laboratory diagnosis and treatment of choice for this disease, with the information available from the last decade. Methods: A review of the literature in English and Spanish was carried out, through the PubMed website, the academic Google search engine and Scielo database, of articles published in the last 10 years. Search terms used included methemoglobinemia, cytochrome b5 reductase deficiency, cyanosis, and co-oximetry. Analysis and synthesis of information: Methemoglobinemia can be classified into congenital and acquired, the latter being the most common. It is important to diagnose this disease, which, although it is a rare condition, can cause serious complications, and even death, which are avoidable with early diagnosis and timely treatment that reduce the complications associated with this condition. Conclusions: The diagnosis and treatment, prophylactic and therapeutic, of methemoglobinemia, in its acute or maintenance stage, require adequate updating of the health professional(AU)

Bilateral choanal atresia – An unusual case of severe respiratory distress in a newborn without nasal flaring

Respiratory distress in a newborn can be due to various causes and some need active intervention. Choanal atresia (CA) is a rare congenital anomaly with its incidence estimated to be 1 case/5,000–8,000 births. It is characterized by narrowing or blockage of the nasal passages. It is important to make an early clinical diagnosis with emphasis on timely management as it can be life-threatening. The pediatrician may be not able to pass a feeding tube through the neonate’s nostril even on repeated attempts. Detailed evaluation should be performed for the CHARGE association. High-resolution computed tomography can aid the diagnosis and transnasal endoscopic surgery is the preferred treatment modality. Here is a case report of a term neonate born with severe respiratory distress who was diagnosed to have bilateral CA on evaluation and managed with nasal endoscopic surgery.

Common Arterial Trunk with Interrupted Aortic Arch

Abstract Clinical data: Patient diagnosed with common arterial trunk, submitted to pulmonary artery banding in another center and lost to clinical follow-up. Referred to our center at four years old, extremely cyanotic. Chest radiography: Cardiomegaly; attenuated peripheral vascular markings. Electrocardiography: Right ventricular hypertrophy. Echocardiography: Common arterial trunk, but it was not possible to analyze all the structures. Computed tomography angiography: Van Praagh type A4 common arterial trunk. Extremely hypoplastic right and left pulmonary arteries. Diagnosis: Association of aortic arch interruption type A is uncommon and should be considered. Operation: Debanding of pulmonary arteries allowing for possible future complete repair.

El Síndrome de Leriche: Subdiagnóstico en la Enfermedad Arterial Periférica

Resumen La enfermedad oclusiva aorto-ilíaca, denominada también como Síndrome de Leriche, es la oclusión de la aorta abdominal en su segmento infrarrenal, siendo la aterosclerosis la causa principal. Los factores de riesgo más frecuentes, son: la hipertensión, la diabetes mellitus, la dislipidemia y el tabaquismo. Se describe el caso de un paciente con factores de riesgo para enfermedad ateroesclerótica quien ingresó por dolor en miembros inferiores, claudicación intermitente, cambios de coloración en pie y ausencia de pulsos en miembros inferiores. Clínicamente, esta entidad se caracteriza por la tríada clásica: Claudicación, disfunción eréctil y pulsos distales disminuidos. Debido a su cronicidad, muchos suelen ser asintomáticos debido a la circulación colateral que desarrollan, lo que conlleva a un subregistro. Los estudios vasculares como: la ecografía doppler, la angiotomografía computarizada y la angiografía aórtica pueden ayudar a confirmar el diagnóstico y la ubicación de la estenosis, siempre teniendo como pilar fundamental la sospecha clínica.

Abstract Aortoiliac occlusive disease, also known as Leriche´s Syndrome, is the occlusion of the abdominal aorta in its infrarenal segment, with atherosclerosis being the main cause. The most frequent risk factors are: hypertension, diabetes mellitus, dyslipidemia and smoking. The case of a patient with risk factors for atherosclerotic disease who was admitted due to pain in the lower limbs, intermittent claudication, discoloration of the foot and absence of pulses in the lower limbs is described. Clinically, is characterized by the classic triad: Claudication, erectile dysfunction and decreased distal pulses. Due to their chronicity, many are usually asymptomatic due to the collateral circulation they develop, which leads to underreporting. Vascular studies such as: Doppler ultrasound, computed tomography angiography and aortic angiography can help confirm the diagnosis and location of stenosis, always having clinical suspicion as a fundamental pillar.

A death case of oral lime sulfur mixture poisoning / 中华劳动卫生职业病杂志

The Second Affiliated Hospital of Shandong First Medical University treated a patient with oral sulfur mixture poisoning on January 14, 2020. The patient presented with cyanosis and disturbance of consciousness as the first manifestations, accompanied by metabolic acidosis, shock, hypercalcemia and severe liver function and myocardial damage. The patient was given active treatment, including gastric lavage, blood purification, methylene blue application, correction of shock, organ support and other therapies. However the treatment was poor. Finally, the patient's family chose to give up and requested to be discharged from the hospital, and the patient died on the same day after follow-up.

A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant

Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Fístula da Artéria Coronária: Associação entre Padrões de Trajetos, Características Clínicas e Cardiopatias Congênitas / Coronary Artery Fistula: Association between Pathway Patterns, Clinical Features and Congenital Heart Disease

Resumo Fundamento A fístula da artéria coronária (FAC) é uma conexão direta entre uma ou mais artérias coronárias e câmaras cardíacas ou um grande vaso; pode estar associada à cardiopatia congênita. Objetivo Estabelecer os padrões de trajetos de FAC a partir de dados ecocardiográficos e correlacioná-los com aspectos clínicos e cardiopatias congênitas. Métodos Um total de 7.183 prontuários médicos de crianças menores de 5 anos de idade com cardiopatia submetidas a ecodopplercardiograma colorido foram analisados utilizando o teste de correlação de Spearman para associar sinais, sintomas e cardiopatia à FAC, com nível de significância de 5%. Resultados Vinte e seis crianças (0,0036%) apresentaram FAC, nos seguintes trajetos: da artéria coronária direita para o ventrículo direito (26,92%), da artéria coronária esquerda para o ventrículo direito (23,08%), do ramo interventricular anterior para o ventrículo direito (23,08%), da artéria coronária direita para o átrio direito (11,54%), da artéria coronária esquerda para o tronco pulmonar (7,69%) e do ramo interventricular anterior para o tronco pulmonar (7,69%). Em 57,69% dos pacientes, houve uma correlação positiva entre sintomas e a FAC (p = 0,445), relacionada à dispneia ou cianose (53,84%). Em 96,15%, a cardiopatia congênita estava associada à FAC; principalmente, a comunicação interventricular e a comunicação interatrial, em 34,62% dos casos, correlacionaram-se positivamente com a FAC (p = 0,295). O trajeto da FAC foi representado em três dimensões pelo software de modelagem, texturização e animação Cinema 4D R19. Conclusão A FAC é uma entidade anatômica incomum que apresenta quadro clínico compatível com dispneia e cianose e está associada a cardiopatias congênitas, principalmente com a CIV ou a CIA. De acordo com as análises ecocardiográficas, as fístulas na ACD, na ACE ou no RIVA representam aproximadamente um terço dos pacientes, com trajeto prioritário para as câmaras cardíacas direitas.

Abstract Background Coronary artery fistula (CAF) is a direct connection of one or more coronary arteries to cardiac chambers or a large vessel; it may be associated with congenital heart disease. Objective To establish CAF pathway patterns from echocardiographic data and to correlate them with clinical aspects and congenital heart disease. Methods A total of 7.183 medical records of children under the age of five years with cardiac disease submitted to color Doppler echocardiography and Spearman's Correlation test were used to associate signs and symptoms and cardiopathy to CAF with a significance level of 5%. Results Twenty six children (0.0036%) presented CAF: from the right coronary artery (RCA) to the right ventricle (RV) 26.92%, from the left coronary artery (LCA) to the RV 23.08%, from the anterior interventricular branch (AIVB) to RV 23.08%, RCA to right atrium (RA) 11.54%, LCA for pulmonary trunk (PT) 7.69% or AIVB for PT 7.69%. In 57.69% of the patients, there was a positively correlated symptomatology to CAF with p=0.445 related to dyspnea or cyanosis (53.84%); in 96.15%, congenital heart disease associated with CAF, mainly interventricular communication (IVC) or interatrial communication (IAC) in 34.62% positively correlated to CAF with p=0.295. CAF pathway was represented in three dimensions by software modeling, texturing and animation Cinema 4D R19. Conclusion CAF is an uncommon anatomical entity that presents a clinical picture compatible with dyspnea and cyanosis, and this is associated with congenital heart disease, mainly with IVC or IAC. According to echocardiographic analyzes, fistulas in RCA, LCA, or AIVB represent about one-third of the patients, with a priority pathway for right heart chambers.

Development of nomogram predicting postoperative blood loss among pediatric patients following corrective operation of tetralogy of Fallot / 中国胸心血管外科临床杂志

@#Objective    To identify the risk factors of postoperative blood loss among pediatric patients following corrective operation of tetralogy of Fallot (TOF) and to develop nomogram predicting the risk of postoperative blood loss. Methods    A retrospective case-control study was conducted in pediatric TOF patients who underwent corrective operation in our hospital from November 2018 to June 2019. And the clinical data from each enrolled patient were gathered and analyzed. Clinically significant postoperative blood loss was defined as drainage volume from chest tube ≥ 16 mL/kg during the first 24 h after surgery, which corresponded to the 75th percentile of the blood loss in our population. The primary outcome was to determine the independent predictors of postoperative blood loss by the least absolute shrinkage and selection operator (LASSO) regression, univariate and multivariate logistic regression analysis. On the basis of the independent predictors of postoperative bleeding, nomogram was developed and its discrimination and calibration were estimated. Results    A total of 105 children were selected (67 males and 38 females aged 3-72 months). The drainage volume from chest tube in the bleeding group was significantly higher than that in the non-bleeding group during the first 24 h (P<0.000 1). Multivariate logistic regression analysis showed that low body weight (OR=0.538, 95%CI 0.369-0.787, P=0.001), high preoperative hemoglobin concentration (OR=1.036, 95%CI 1.008-1.066, P=0.013) and prolonged intraoperative aortic cross clamp time (OR=1.022, 95%CI 1.000-1.044, P=0.048) were independent risk factors for postoperative blood loss. In the internal validation, the model displayed good discrimination with a C-index of 0.835 (95%CI 0.745-0.926) and high quality of calibration plots in nomogram models was noticed. Conclusion    The nomogram demonstrated good discrimination and calibration in estimating the risk of postoperative blood loss among pediatric patients following corrective operation of TOF.

Factores de riesgo de Bronquiolitis en pacientes menores de 2 años

Resumen Introducción: Se estima que a nivel mundial, cada año alrededor del 10% de los lactantes tienen bronquiolitis. Se produce un pico entre los 2 y los 6 meses de edad. Objetivo: Determinar los factores de riesgo de bronquiolitis en menores de dos años que consultaron al servicio de Pediatría del Instituto de Medicina Tropical entre el 2018 y 2019. Método: Estudio de tipo descriptivo, transversal en el que se incluyeron pacientes menores de 2 años internados en el Servicio de Pediatría del Instituto de Medicina Tropical de enero de 2018 a febrero de 2019 con diagnóstico de bronquiolitis en el que se analizaron los factores de riesgo de la enfermedad. Resultados: Se estudió una población de 38 pacientes que presentaron bronquiolitis antes de los 2 años, 19 fueron niños (50%) La edad media al ingreso fue de 3,5 ± 2,8 meses (límites, 1-10 meses). No se han encontrado datos de tabaco en el embarazo. Se detectó VRS en el aspirado nasofaríngeo de 8 niños (21%), la detección viral fue negativa en 14 niños (37%) y no se realizó la determinación en 18 casos (47%). Los siguientes factores de riesgo se asociaron de forma independiente con la bronquiolitis: Asma de los padres, infección por VSR, sibilancia, cianosis y hacinamiento. Los factores de riesgo estudiado, exposición al tabaco, sexo, edad en el momento de la bronquiolitis o lactancia materna exclusiva, no se asociaron con el ingreso a UTI de los pacientes con bronquiolitis. Conclusión: Hemos encontrado factores de riesgo asociado relacionados al huésped como asma de los padres, y otros como sibilancia, cianosis, hacinamiento e infección por VRS.

Abstract Introduction: It is estimated that globally, each year around 10% of infants have bronchiolitis. A peak occurs between 2 and 6 months of age Objective: To determine the risk factors of bronchiolitis in children less than two years of age who admitted at the pediatric service of the Institute of Tropical Medicine between 2017 and 2019 Method: A descriptive, cross-sectional study in which patients under 2 years of age admitted to the Pediatric Service of the Institute of Tropical Medicine from January 2018 to February 2019 were included with a diagnosis of bronchiolitis in which the risk factors of the disease were analyzed. Results: We studied a population of 38 patients who presented bronchiolitis before 2 years, 19 were children (50%) The average age at admission was 3.5 ± 2.8 months (range, 1-10 months). No tobacco data were found in pregnancy. RSV was detected in the nasopharyngeal aspirate of 8 children (21%), viral detection was negative in 14 children (37%) and the determination was not made in 18 cases (47%). The following risk factors were independently associated with bronchiolitis: Asthma of the parents, RSV infection, wheezing, cyanosis and overcrowding. The risk factors studied exposure to tobacco, sex, age at the time of bronchiolitis or exclusive breastfeeding were not associated with admission to the ICU of patients with bronchiolitis. Conclusion: We have found associated risk factors related to the host such as asthma of the parents, and others such as wheezing, cyanosis, overcrowding and RSV infection.

Conhecimento sobre a Doença e a Prática de Atividade Física em Crianças e Adolescentes com Cardiopatia Congênita / Knowledge about the Disease and the Practice of Physical Activity in Children and Adolescents with Congenital Heart Disease

Resumo Fundamento O conhecimento sobre a própria doença pode ser importante para o autocuidado em pacientes com vários problemas e abrange a informação sobre o diagnóstico até as implicações clínicas mais importantes. Objetivo Identificar o nível de conhecimento de crianças e adolescentes com cardiopatia congênita (CC) sobre a sua doença, e analisar a relação entre o nível de conhecimento e a prática de atividade física. Métodos Estudo transversal com 335 pacientes com CC, de 8 a 13 anos, acompanhados em um serviço de cardiologia pediátrica de referência no Sul do Brasil. Os pacientes foram entrevistados em relação ao seu conhecimento sobre a CC e foi realizada revisão dos prontuários para obtenção de detalhes sobre a cardiopatia e os procedimentos. Foi utilizado o nível de significância p < 0,05. Resultados Mais de 50% das crianças e adolescentes não sabiam referir o nome de sua doença ou explicá-la. Após OR ajustado (ORaj), mostraram potencial para respostas incorretas ou não saber sua doença os pacientes cianóticos em relação aos acianóticos (ORaj: 2,29; IC95%: 1,76-6,71; p=0,019); crianças com menor nível de escolaridade (ORaj: 2,20; IC95%: 1,81-5,86; p=0,025); e não praticantes de atividade física (ORaj: 1,88; IC95%: 1,09-3,45; p=0,011). Conclusão As crianças e adolescentes cianóticos, com menor nível de escolaridade e que não praticavam de atividade física apresentaram pouco conhecimento sobre a sua doença. Há necessidade do desenvolvimento de estratégias de intervenções educativas para aumento do conhecimento e mudança comportamental na promoção da atividade física, de acordo com a complexidade da CC. (Arq Bras Cardiol. 2020; 114(5):786-792)

Abstract Background Knowledge about the disease itself can be important for self-care in patients with several problems and comprehends information about the diagnosis up to the most important clinical implications. Objective To identify the level of knowledge of children and adolescents with congenital heart disease (CHD) about their illness, and to analyze the association between the level of knowledge and the practice of physical activity. Methods Cross-sectional study with 335 patients with CHD, aged 8 to 13 years, followed at a referral pediatric cardiology service in Southern Brazil. Patients were interviewed regarding their knowledge about CHD and a review of medical records was performed to obtain details on heart disease and procedures. A significance level p<0.05 was used. Results More than 50% of the children and adolescents did not know how to say the name of their disease or explain it. After adjusted OR (AOR), cyanotic patients in comparison to acyanotic ones (AOR: 2.29; 95%CI: 1.76-6.71; p = 0.019); children with lower level of schooling (AOR: 2.20; 95%CI: 1.81-5.86; p = 0.025); and those who did not practice physical activity (AOR: 1.88; 95%CI: 1.09-3.45; p = 0.011) showed potential for incorrect answers or did not know their disease. Conclusion Cyanotic children and adolescents, with a lower level of schooling and who did not practice physical activity, had little knowledge about their disease. It is necessary to develop educational intervention strategies to increase knowledge and change behavior in physical activity promotion, according to the CHD complexity. (Arq Bras Cardiol. 2020; 114(5):786-792)

Clinical profile and outcome of congenital cyanotic heart disease in neonatal period: a retrospective study

Background: Congenital Cyanotic Heart Disease (CCHD) is under reported during neonatal period and mortality rate is high in India. Aims and objectives of the study determine clinical presentation, maternal and neonatal risk factors and outcome of CCHD during neonatal period.Methods: A retrospective study conducted over 15-month period during March 2017 to June 2018 in a tertiary out born NICU. Neonates with echocardiographically confirmed case of structurally abnormal heart disease were analyzed.Results: Among 106 CCHD neonates, 60% neonates were asymptomatic, 22% had persistent cyanosis and 19% had tachypneic at birth. Cyanosis (35%) and tachypnea (30%) were common initial presentation in postnatal period. 22% neonates were readmitted with initial clinical symptom after discharge from birthing centre. CCHD were more common in male and term neonate, 34% were small for age, median age at admission and discharge were 5days (2-12) and 5 days (2.7-9.2) days respectively. Nineteen percent neonates had fetal distress and 6% neonates required aggressive resuscitation at birth. First trimester abortion (16%), maternal diabetes (10%) and hypothyroidism (7%) were common maternal risk factor. Nearly 20% neonates were diagnosed at referring hospital and 4.7% were transported with PGE1 during transport. TGA (17%) was most common lesion noted followed by pulmonary atresia (10%). Fourteen percent neonates died during the neonatal period. Disease specific death rate was highest for Single ventricle (42%) followed by TGA with IVS (37.5%) and aortic arch anomaly (28.5%) in this study.Conclusions: Early diagnosis and management may improve the survival in CCHD neonates.

Congenital Heart Disease in the Pediatric Population in Eastern India: A Descriptive Study

This cross-sectional study assessed distribution and pattern ofechocardiography confirmed congenital heart disease, among593 pediatric patients in outpatient departments of a tertiary carehospital in eastern India. Commonest defects were ventricularseptal defect (43, 40.7%), atrial septal defect (241, 31.7%), andtetralogy of Fallot (125, 21%).

Hipoxemia recurrente y formen oval permeable. Unidad diagnóstica fácilmente inadvertida. Caso clínico / Recurrent hypoxemia secondary to a patent foramen ovale. Report of one case

Patent foramen ovale (PFO) is a prevalent congenital septal atrial defect usually without pathological significance. In certain pathogenic situations, PFO can trigger episodes of recurrent hypoxemia, a specific condition known as platipnea-orthodeoxia syndrome (POS). We report a 73 years old female presenting with dyspnea and low arterial oxygen saturation. On admission the patient had a hemoglobin saturation of 81% and an arterial oxygen partial pressure of 50 mmHg. After breathing 100% a 17% arterial-venous shunt was found (normal: less than 12%). A transesophageal echocardiogram and a cardiac catheterization showed the presence of a PFO. A percutaneous closure was performed.

Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis / Manejo anestésico de paciente com cianose grave após estenose de valva bioprotética

Abstract We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care.

Resumo Apresentamos o caso de uma paciente de 39 anos, com hipoxemia em risco de vida após a substituição da valva tricúspide devido à anomalia de Ebstein e cianose grave devido à estenose de valva bioprotética e comunicação interatrial. O manejo anestésico de um paciente com disfunção obstrutiva grave de prótese valvar pode ser um desafio. Os pacientes com anomalia de Ebstein também precisam de atenção especial para manter a pressão equalizada entre o átrio direito e o esquerdo. A ecocardiografia transesofágica e a saturação cerebral de oxigênio forneceram informações em tempo real nos cuidados perioperatórios.

Edema agudo de pulmón por presión negativa secundario a laringoespasmo / Acute lung edema due to negative pressure secondary to laryngospasm

Resumen El edema agudo de pulmón por presión negativa es una forma de edema pulmonar no cardiogénico, potencialmente grave, que suele ocurrir en sujetos sanos jóvenes capaces de generar presiones intratorácicas negativas elevadas al inspirar vigorosamente contra una vía aérea superior obstruida. En adultos la causa más frecuente es el laringoespasmo posextubación en el periodo posoperatorio inmediato. Puede afectar a 0.05-0.1% de los pacientes sanos sometidos a anestesia general. Se comunica el caso de un paciente de 24 años de edad, sin antecedentes patológicos, que acudió al servicio de consulta externa de Cirugía del Hospital Regional Santa Teresa, Comayagua, Honduras, para realizar colecistectomía abierta por colecistitis crónica calculosa agudizada. Se realizó procedimiento quirúrgico sin complicaciones; 15 minutos del periodo posoperatorio inmediato, después de la extubación, el paciente tuvo cuadro clínico de disnea súbita, cianosis y hemoptisis. A la exploración física se encontró hipoxemia, taquipnea (FR = 40 rpm) y crépitos bilaterales en la auscultación. Se realizaron exámenes complementarios y de imagen, concluyendo edema agudo de pulmón. Se inició tratamiento con oxigenoterapia, corticoesteroides y diuréticos durante 72 horas, con lo que evolucionó de forma favorable. Los hallazgos clínicos del edema agudo de pulmón por presión negativa pueden confundirse con una amplia gama de afecciones clínicas, por lo que es de suma importancia realizar el abordaje adecuado y diagnóstico diferencial. El inicio de las medidas terapéuticas oportunas ofrece un pronóstico favorable y generalmente disminución de la mortalidad.

Abstract Acute negative pressure pulmonary edema is a form of potentially serious noncardiogenic pulmonary edema that usually occurs in young healthy subjects capable of generating elevated negative intrathoracic pressures by vigorously inhaling a blocked upper airway. In adults, the most frequent cause is postextubation laryngospasm in the immediate postoperative period. It can present in 0.05-0.1% of healthy patients under general anesthesia. This paper reports the case of a 24-year-old male with no pathological history, who was presented to the general surgery service of the Hospital Regional Santa Teresa, Comayagua, Honduras, to perform open cholecystectomy for chronic cholecystitis. The surgical procedure was performed without complications; 15 minutes of the immediate postoperative period, post-extubation, patient presented clinical manifestations of sudden dyspnea, cyanosis and hemoptysis. On physical examination were found hypoxemia, tachypnea (BR = 40 bfm) and bilateral craniocereus on pulmonary auscultation. Complementary tests and image were performed and NPPE was diagnosed. Treatment with oxygen therapy, corticosteroids and diuretics was started, during 72 hours evolving favorably. The clinical findings of the acute negative pressure pulmonary edema can be confused with a wide range of clinical entities, so it is extremely important to carry out an adequate approach and differential diagnosis. The introduction of appropriate therapeutic measures offers a favorable prognosis and generally a wide decrease in mortality.