[Congenital cystic adenomatoid malformation of the lung, intrauterine diagnostic and treatment. A case report and literature review]. / Enfermedad adenomatoidea quística pulmonar, diagnóstico y manejo intrauterino. Reporte de un caso y revisión bibliográfica.

The use of prenatal ultrasonography has improve the detection of fetal abnormalities, which affects the perception about the natural history and evolution of them, changing the management of the fetus and neonate. Today, it is possible to perform an early prenatal diagnosis, and be treated even intrauterine with high rates of success. Such is the case of Congenital Cystic Adenomatoid Malformation (CCAM), characterized by abnormal proliferation and dilatation of the terminal structures of the airway, generating multiple cysts of diferent sizes and locations. The CCAM can be asymptomatic until adulthood, but sometimes it is presented as an adverse perinatal outcome, manifested with ascites, hydrothorax, hydrops and hypoplasia of the affected lung. We report a case of a pregnant women, 32-year-old, diagnosed on the 18 week pregnancy by ultrasound Type 3 Congenital Cystic Adenomatoid Malformation disease. Treated with an intrauterine intervention percutaneous fetal sclerotherapy successfully.

[Ductus arteriosus aneurysm. Case report and review of the literature]. / Aneurisma de conducto arterioso: reporte de un caso y revisión de la bibliografía.

The saccular or fusiform dilatation of the ductus arteriosus is called aneurysm (DAA). It is diagnosed in the second trimester during a structural ultrasound. Even though the reported incidence is 2.2%, it remains infrequent, because it is not searched routinely. The pathogenesis is uncertain, resulting from an increase on the circulating blood volume thus in the cardiac afterload, associated to a thinning of the vessel wall, for an inadequate intima development. Ductus arteriosus aneurysm can be classified according by their diameter as small (<7 mm.) and large (> or =8 mm). The smaller commonly closes spontaneously in 70% of the cases, unlike the larger one, they are associate in a greater number of complications, such as thrombosis, embolism, infection, compression effects of adjacent structures and spontaneous rupture. The absence of spontaneous closure, according to the symptoms and possible complications, a neonatal surgical closure should be considered. We report a case of a 35-year-old patient, diagnose on the 35 week pregnancy, a ductus arteriosus aneurysm by ultrasound with an image of the three vessels performed communicating, saccular dilatation of 7-8 mm. and a turbulent Doppler flow.

Guía mexicana para el diagnóstico y el tratamiento del hemangioma infantil.

Infantile hemangioma is a benign vascular tumor, the most common in childhood, whose natural evolution is the disappearance of the lesion in the pediatric age and which has effective and safe treatments that limit its growth and favor its disappearance at younger ages. Infantile hemangioma continues to be a reason for attention to complications, due to erroneous diagnoses, lack of knowledge of the condition, late referral or fear of the effects of the medications used for its treatment. Furthermore, its presence is normalized without taking into account that it can cause uncertainty, anxiety, feelings of guilt and, as a consequence, a significant impact on the quality of life, mainly in the parents or caregivers of the child. The need for a clinical practice guideline in our country arises from the high presentation of late-remitted complications in infantile hemangioma even with the availability of adequate treatments, the continuous evolution of medicine and the appearance of new evidence. Throughout the guide you will find recommendations regarding the diagnosis, treatment and follow-up of patients with infantile hemangioma, taking into account the paraclinical tests that can be performed, topical or systemic management options, as well as adjuvant therapies. For the first time, objective tools for patient follow-up are included in a guide for the management of infantile hemangioma, as well as to help the first contact doctor in timely referral.

El hemangioma infantil es un tumor vascular benigno, el más frecuente de la infancia, cuya evolución natural favorece la desaparición de la lesión en la misma edad pediátrica y que cuenta con tratamientos eficaces y seguros que limitan su crecimiento y favorecen su desaparición a edades más tempranas. Continúa siendo motivo de atención de complicaciones, debido a diagnósticos erróneos, desconocimiento del padecimiento, referencia tardía o temor de los efectos de los fármacos utilizados para su tratamiento. Además, se normaliza su presencia sin tomar en cuenta que puede llegar a causar incertidumbre, ansiedad, sentimientos de culpa y, como consecuencia, importante afectación de la calidad de vida, principalmente en los padres o cuidadores del niño. La necesidad de una guía de práctica clínica en nuestro país surge ante la alta presentación de complicaciones del hemangioma infantil referidas de manera tardía aun con la disponibilidad de tratamientos adecuados, la evolución continua de la medicina y la aparición de nueva evidencia. A lo largo de la guía se encontrarán recomendaciones en relación con el diagnóstico, el tratamiento y el seguimiento de los pacientes con hemangioma infantil, tomando en cuenta los paraclínicos que pueden realizarse, las opciones de manejo tópico o sistémico, y las terapias adyuvantes. Por primera vez se incluyen en una guía para el manejo del hemangioma infantil herramientas objetivas para el seguimiento de los pacientes, así como para ayudar al médico de primer contacto en su referencia oportuna.

[Pediatric cardiovascular surgical data base registry in México. First report]. / El Registro Mexicano de Cirurgía Cardiaca Pediátrica. Primer informe.

INTRODUCTION: Current world tendency is the detection of health problems in order to offer solution alternatives by means of the development of computarized data bases. OBJECTIVE: To present the results of a computerized data base developed for the registry of pediatric cardiac surgery with the support of Asociación Mexicana de Especialistas en Cardiopatías Congénitas (AMECC, A.C.). MATERIAL AND METHODS: A one-year analysis (from August 1, 2011 to July 31, 2012) of a computerized data base was performed with the support of AMECC and the participation of the most important Mexican institutions for pediatric surgical heart disease health care, particularly for the uninsured population. RESULTS: There were 7 health institutions voluntarily incorporated to the national data base registry, and in the first year of observation, 943 surgical procedures in 880 patients and 7% re-operations (n = 63), were reported. Patients up to one-year old accounted for 38%. The most frequent types of operated congenital heart diseases were: patent ductus arteriosus (n = 96), ventricular septal defect (n = 86), tetralogy of Fallot (n = 72), atrial septal defect (n = 68), and aortic coarctation (n = 54). Elective procedures were 90%, and 62% of them were performed with the use of cardiopulmonary bypass. Overall mortality was 7.5% with the following RACHS-1 score risk distribution: 1 (n = 4.2%), 2 (n = 19.6%), 3 (n = 22.8%), 4 (n = 12.19%), 5 (n = 1.25%), 6 (n = 6.44%) and not classifiable (n = 2.9%). CONCLUSIONS: Although this analysis gives a representative vision of the cardiovascular surgical health care for the uninsured national pediatric population, the incorporation of other health institutions to this data base may lead us to have a most realistic overview in relation to the surgical cardiovascular health care for the up to 18 year-old population.

Characteristics and Outcomes of Cases of Children and Adolescents With Pediatric Inflammatory Multisystem Syndrome in a Tertiary Care Center in Mexico City.

Background: Pediatric inflammatory multisystem syndrome (PIMS) is a complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children that resembles Kawasaki syndrome and places them at high risk of cardiorespiratory instability and/or cardiac damage. This study aims to describe the clinical presentation and outcomes of patients with PIMS in Mexico City. Methods: This was an observational study of children hospitalized for PIMS based on the Centers for Disease Control and Prevention case definition criteria, in a single tertiary care pediatric center in Mexico City between May 1, 2020, and September 30, 2021. Demographic characteristics, epidemiological data, medical history, laboratory tests, cardiologic evaluations, treatment, and clinical outcomes were analyzed. Results: Seventy-five cases fulfilled the case definition criteria for PIMS [median age: 10.9 years, Interquartile range (IQR): 5.6-15.6]. Fifteen (20%) patients had a severe underlying disease, 48 (64%) were admitted to the intensive care unit, 33 (44%) required invasive mechanical ventilation and 39 (52%) received vasopressor support. The patients were clustered through latent class analysis based on identified symptoms: Cluster 1 had rash or gastrointestinal symptoms (n = 60) and cluster 2 were those with predominantly respiratory manifestations (n = 15). Two patients (2.7%) died, and both had severe underlying conditions. Five patients (6.7%), all from cluster 1, developed coronary aneurysms. Conclusion: There were a high proportion of patients with severe respiratory involvement and positive RT-PCR SARS-CoV-2 and very few cases of coronary aneurysms in our study which suggests that a high proportion of the children had severe acute COVID-19. The clinical manifestations and outcomes are comparable to previously reported international studies.

Implementation of diagnostic screening for congenital heart disease in Hidalgo, Mexico.

Objective: Implementing screening through pulse oximetry (PO) and a knowledge management model (KMM) for early detection of life-threatening congenital heart disease (CHD) in the neonatal period. Material and methods: Pilot study of PO implementation supported by clinical criteria performed in newborns at two public hospitals of Hidalgo State. Those who tested positive were referred for echocardiography and those diagnosed with critical CHD (CCHD) were referred to specialized hospitals for treatment. Results: 1748 newborns were screened: 29 positive, 62% with CHD and 13.8% with CCHD, one death, three referrals to palliative treatment. Conclusion: PO as a method of screening helps in early diagnosis of CHD added to clinical and echocardiography studies. KMM fosters innovation and resource management.

Objetivo: Implementar el tamizaje mediante la oximetría de pulso (OP) y un modelo de gestión del conocimiento (MGC) para la detección oportuna de cardiopatías congénitas (CC) que amenazan la vida en el período neonatal. Material y métodos: Estudio piloto de implementación de OP apoyado en criterios clínicos, realizado en recién nacidos (RN) de dos hospitales públicos de Hidalgo. Los pacientes que resultaron positivos fueron objeto de ecocardiografía (EC) y los diagnosticados con cardiopatías congénitas críticas (CCC) se refirieron a tratamiento. Resultados: Se tamizó a 1,748 RN (29 positivos), CC en 62% y CCC en 13.8 %, 1 muerte y 3 programados para operación paliativa. Conclusiones: La OP ayuda en el diagnóstico de CC en combinación con criterios clínicos y EC. Un MGC favorece la innovación y la gestión de recursos.

Subepicardial aneurysm: echocardiographic evaluation and evolution.

Subepicardial aneurysms (SEA) are an infrequent and serious form of subacute cardiac rupture complicating myocardial infarction. An early diagnosis and surgical repair may be life saving. SEA comprise an abrupt interruption of the myocardium, with a narrow neck and thin wall containing only the epicardium. It may progress to fatal cardiorrhexis. We describe the echocardiographic evolution of this type of cardiac rupture and the contribution of contrast-enhanced echocardiography. A possible pathophysiological mechanism is proposed.

[Echocardiographic assessment of ventricular synchrony. Implications to patient selection and treatment outcome]. / Evaluación ecocardiográfica de la sincronía ventricular. Implicaciones en la selección de pacientes y en los resultados del tratamiento.

Heart failure is one of the most prevalent diseases in industrialized countries. Up to 30% of the patients with advanced heart failure present disturbances in intra-ventricular conduction, and this produce asynchrony of ventricular contractility, leading to further deterioration in heart function. Cardiac resynchronization (TRC) is an increasingly important therapeutic option for a subgroup of patients with heart failure. Several methods have been show to be useful in study the mechanical asynchrony. However, there are discrepancies between the results of the different methods. The echocardiography provides the best parameters in predicting a good response.

Three-dimensional transesophageal echocardiography of the atrial septal defects.

Transesophageal echocardiography has advantages over transthoracic technique in defining morphology of atrial structures. Even though real time three-dimensional echocardiographic imaging is a reality, the off-line reconstruction technique usually allows to obtain higher spatial resolution images. The purpose of this study was to explore the accuracy of off-line three-dimensional transesophageal echocardiography in a spectrum of atrial septal defects by comparing them with representative anatomic specimens.

[Tetralogy of fallot and total anomalous pulmonary venous drainage to coronary sinus. Case report]. / Tetralogía de Fallot con conexión anómala total de venas pulmonares a seno coronario. Reporte de un caso con esta rara asociación.

We report the case of an infant 3 months old with a rare association, tetralogy of Fallot with total anomalous pulmonary veins connection the diagnosis was made by echocardiography and a successful complete reparation was achieved.

[Prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children: a sample obtained from The Federico Gomez Children's Hospital of Mexico]. / Prevalencia y espectro de las enfermedades que predisponen a la muerte súbita cardiaca en niños mexicanos: una muestra obtenida del Hospital Infantil de México Federico Gómez.

OBJECTIVE: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. METHODS: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. RESULTS: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. CONCLUSION: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.

Maternal Obesity as a Risk Factor for the Development of Total Anomalous Pulmonary Venous Connection in Their Offspring.

The incidence of total anomalous pulmonary venous connection (TAPVC) in the Caucasian population is 2.5/100,000 live births (LB), and the incidence in the Hispanic population is 19.8/100,000 LB. Without knowing the exact etiology for the development of congenital heart disease, our objective was to determine the maternal factors associated with the development of TAPVC. METHODS: 55 mother-child binomials with isolated TAPVC (group I) and 152 healthy mother-child binomials (group II) were included. Both groups had no maternal history of addiction, pre-eclampsia, or type 1, 2 or gestational diabetes mellitus. Complete clinical histories were obtained for the women in both groups and perinatal and birth data were recorded. In addition, genealogies across three generations were constructed to determine affected first- or second-degree relatives with complex congenital heart disease. RESULTS: Among the maternal characteristics analyzed, women in group I had a higher number of pregnancies before gestation of the index case (p = <0.05), and the Body Mass Index (BMI) before pregnancy was higher compared to Group II (p < 0.05), with an adjusted risk of OR = 3.6 (p = 0.011). The family history showed a higher prevalence in the group of patients with TAPVC compared to healthy children (p < 0.05). CONCLUSION: Maternal obesity before pregnancy is a risk factor for the development of CATVP in children in the Mexican population.

[Echocardiography in valvular heart disease. New perspectives]. / Ecocardiografía: aportaciones en la evaluación del daño valvular: nuevas perspectivas.

Echocardiography occupies an excellent place in the field of valvular heart disease study. Its presence is being increased in the catheterism and surgery rooms as well as in the intensive cares units. The ultrasound machines development has improved its technology and echocardiographic diagnoses has a greater repercussion in clinical decisions every time. The greater challenge than appears to us is to be able to have prepared enough professionals that know the tool, the physiopathology, the therapeutics modalities, and who can apply and take advantage of the new technological developments for a better cardiological practice.

[Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring]. / Arco aórtico derecho con subclavia izquierda aberrante y divertículo de Kommerell. Una causa de anillo vascular.

The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.

[From 2D to 4D echocardiography in adults with congenital heart disease]. / Imágenes en cardiopatía congenita en el adulto. Ecocardiografía: De la imagen bidimensional a la imagen en cuatro dimensiones.

In the present publication an analysis of the utility is done of the Echocardiography, and its different modalities, in the diagnosis of patient adult with Congenital Heart disease. Special emphasis since the view 2-D to the view in 4-D.

[Echocardiography in diagnosis of primary cardiac tumors in pediatrics]. / Valor de la ecocardiografía en el diagnóstico contemporáneo de tumores cardíacos primarios en pediatría.

We report the experience in the diagnosis of primary cardiac tumor during the period from 1999 to 2004, 8500 studies were revised echocardiographic carried out. We found 21 patients, 11 of female sex (55%). In 15/21 (71%), the age of presentation was less than 1 year. In 9/21 the tumor was multiple (42.8%), lodged in the ventricle right in 2/21 (9.5%), in the ventricle left 3 (14.2%), 8 in the septum interventricular (38%) and 4 compromised the auriculas. They were classified like rabdomiomas 14 (66%), 5 associates with sclerosis tuberosa, 4 mixomas (19%), 2 fibromas (9.5%) and 1 rabdomiosarcoma (4.7%). In five patients the diagnosis was prenatal. The global mortality went of 9.5%. The echocardiograpy is a good diagnosis method in our series the rabdomioma occupied the first place in frequency.

[Unruptured sinus of Valsalva aneurysm. Bi and three-dimensional echocardiography approach]. / Valoración ecocardiográfica bi y tridimensional de aneurisma no roto del seno de Valsalva.

Aneurysm of the sinus of Valsalva is a rare anomaly that can be congenital or acquire. The most common among the acquire is aortic endocarditis. At the present time echocardiography has be come the election diagnostic method. We present two pediatric patients with unrupture aneurysm of the sinus of Valsalva and cardiac failure secondary to a acute aortic insufficiency manifestations. Transthoracic and transesophageal bidimensional images were compared, in one of them also three-dimensional image. Assessing the quality of images, localization and anatomical morphology of aneurysm, obstruction or compression and presence of associated defects. The usefulness of the three dimensional echocardiography assessment for these congenital malformations is demonstrated.

[Recurrent myxoma. Bi and three-dimensional echocardiography approach]. / Mixoma recidivante. Diagnóstico ecocardiográfico bi y tridimensional.

The case of a 16-years old female patient with left atrial myxoma and cerebral embolism is reported. She was subjected to surgical excision of the mass. At 40 months of follow-up she initiates with dyspnea and an aortic murmur is detected. The transthoracic and transesophageal echocardiographic analysis revealed the presence of three tumors: in the left atrium, left and right ventricle. The myxoma of the left ventricle obstructs the aortic valve. We discuss the usefulness of bi-and three-dimensional echocardiography as initial diagnostic method.

[Persistent fifth aortic arch with patent ductus arteriosus]. / Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding occasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

Implementation of diagnostic screening for congenital heart disease in Hidalgo, Mexico / Implementación del tamizaje diagnóstico de cardiopatías congénitas en Hidalgo, México

Abstract Objective: Implementing screening through pulse oximetry (PO) and a knowledge management model (KMM) for early detection of life-threatening congenital heart disease (CHD) in the neonatal period. Material and methods: Pilot study of PO implementation supported by clinical criteria performed in newborns at two public hospitals of Hidalgo State. Those who tested positive were referred for echocardiography and those diagnosed with critical CHD (CCHD) were referred to specialized hospitals for treatment. Results: 1748 newborns were screened: 29 positive, 62% with CHD and 13.8% with CCHD, one death, three referrals to palliative treatment. Conclusion: PO as a method of screening helps in early diagnosis of CHD added to clinical and echocardiography studies. KMM fosters innovation and resource management.

Resumen Objetivo: Implementar el tamizaje mediante la oximetría de pulso (OP) y un modelo de gestión del conocimiento (MGC) para la detección oportuna de cardiopatías congénitas (CC) que amenazan la vida en el período neonatal. Material y métodos: Estudio piloto de implementación de OP apoyado en criterios clínicos, realizado en recién nacidos (RN) de dos hospitales públicos de Hidalgo. Los pacientes que resultaron positivos fueron objeto de ecocardiografía (EC) y los diagnosticados con cardiopatías congénitas críticas (CCC) se refirieron a tratamiento. Resultados: Se tamizó a 1,748 RN (29 positivos), CC en 62% y CCC en 13.8 %, 1 muerte y 3 programados para operación paliativa. Conclusiones: La OP ayuda en el diagnóstico de CC en combinación con criterios clínicos y EC. Un MGC favorece la innovación y la gestión de recursos.