Abordaje terapéutico de cardiopatías congénitas en el síndrome de Down / Congenital heart disease in Down's syndrome

Resumen Antecedentes: El síndrome de Down es la anomalía cromosómica más frecuente y se asocia con defectos cardiacos congénitos, elementos clínicos de una alta morbilidad y mortalidad infantil. Objetivo: Describir los desenlaces clínicos de los pacientes con síndrome de Down sometidos a cirugía e intervencionismo como tratamiento de las cardiopatías congénitas en esta institución. Material y métodos: Estudio retrospectivo que incluyó pacientes menores de 18 años con síndrome de Down y patología cardiaca asociada durante los últimos 10 años. Las variables estudiadas fueron: peso, talla, sexo, edad, tipo de cardiopatía, procedimiento correctivo, tiempo de estancia intrahospitalaria y en unidad de terapia intensiva, morbilidad y mortalidad. Resultados: 368 pacientes pediátricos fueron llevados a corrección quirúrgica o intervencionista, de los cuales 197 (54%) pertenecían al sexo femenino, la mediana de edad fue de 24 meses (rango intercuartílico [RIQ]: 14-48) en el grupo quirúrgico y de 36 meses (RIQ: 17-85) en el intervencionista. Las cardiopatías congénitas más frecuentes fueron: persistencia del conducto arterioso (31%), comunicación interventricular (28%), canal atrioventricular (CAV) (20%), comunicación interauricular (16%) y tetralogía de Fallot con el 4% respectivamente. La estancia hospitalaria fue de 9 días (RIQ: 7-15) en el grupo quirúrgico y de 3 días (RIQ: 2-5) en el hemodinámico. Las morbilidades fueron infección postoperatoria en 30 pacientes (14%) y en 19 pacientes (9%) bloqueo atrioventricular completo. La mortalidad global incluyendo tanto el quirúrgico como el intervencionista fue del 2%. Conclusiones: Los resultados terapéuticos, quirúrgicos e intervencionistas, en los niños con síndrome de Down y cardiopatías congénitas han mejorado en forma muy satisfactoria. Es de destacar la menor prevalencia del CAV en la población mexicana. Es indispensable realizar evaluación cardiológica a los niños con síndrome de Down y aquellos con cardiopatías congénitas llevarlos a corrección de manera oportuna para favorecer la sobrevida y calidad de vida.

Abstract Background: Down syndrome is the most common chromosomal abnormality, it is associated with a wide variety of congenital heart defects, being considered as clinical elements of high infant morbidity and mortality. Objective: To describe the clinical outcomes of patients with Down syndrome undergoing surgery and interventionism as treatment for congenital heart disease at this Institution. Material and methods: 368 patients with Down syndrome and associated congenital heart disease were diagnosed. The variables studied were weight, stature, sex, age, type of heart disease, corrective procedure, length of stay in the hospital and intensive care unit, morbidity and mortality. Results: 368 pediatric patients underwent surgical or interventional correction. Of which 197 (54%) were female, the median age was 24 months (interquartile range [IQR]: 14-48) in the surgical group and 36 months (IQR: 17-85) in the interventional group. The most frequent congenital heart diseases were: PCA (31%), IVC (28%), CAV (20%), ASD (16%) and tetralogy of Fallot with 4% respectively. Hospital stay was 9 days (IQR: 7-15) in the surgical group and 3 days (IQR: 2-5) in the hemodynamic group. Morbidities were postoperative infection in 30 patients (14%) and complete atrioventricular block in 19 patients (9%). Overall mortality including both surgical and interventional was 2%. Conclusions: The therapeutic, surgical and interventional results in children with Down syndrome and congenital heart disease have improved very satisfactorily. The lower prevalence of the atrioventricular canal in the Mexican population is noteworthy. It is essential to carry out a cardiological evaluation of children with Down syndrome and those with congenital heart disease to correct them in a timely manner to promote survival and quality of life.

Origen anómalo de una de las ramas de la arteria pulmonar (OAAP): tratamiento quirúrgico y resultados / Anomalous origin of one of the branches of the pulmonary artery (AOPA): Surgical treatment and outcome

Resumen Introducción: El origen anómalo de una de las ramas de la arteria pulmonar procedente de la aorta ascendente es poco frecuente. Objetivo: Identificar las características clínicas y quirúrgicas de los pacientes sometidos a reimplante de la rama afectada. Método: Se realizó un estudio observacional, transversal, descriptivo, retrospectivo y retrolectivo, en el que se incluyeron los pacientes diagnosticados de origen anómalo de alguna de las ramas de la arteria pulmonar y que fueron tratados mediante cirugía correctiva en el Instituto Nacional de Cardiología Ignacio Chávez, en el periodo del 1 de enero de 2003 al 31 de enero de 2019. De los expedientes se extrajeron las características demográficas, los antecedentes quirúrgicos, los diagnósticos, los reportes ecocardiográficos y tomográficos, la técnica quirúrgica y el estado posquirúrgico. Resultados: Se encontraron nueve pacientes sometidos a cirugía de corrección, con un promedio de edad de 2 ± 2 años, un peso de 11.4 ± 1.5 kg y una talla de 82 ± 15 cm. El 67% fueron de sexo masculino. La rama afectada con más frecuencia fue la derecha; el 68% se originaban directamente de la aorta y el 42% de un conducto arterioso persistente. La corrección con circulación extracorpórea tuvo un tiempo promedio de pinzamiento aórtico de 35 minutos y de soporte circulatorio de 45 minutos. Se utilizó el implante directo o con material sintético. Las complicaciones fueron falla ventricular, sangrado, arritmias y neumonía nosocomial. La mortalidad fue del 11%. Conclusiones: El tratamiento quirúrgico para el reimplante de la rama anómala de la arteria pulmonar es el procedimiento de elección, y con cuidados quirúrgicos y posteriores tiene un buen pronóstico a mediano y largo plazo.

Abstract Introduction: The anomalous origin of one of the branches of the pulmonary artery from the ascending aorta is rare. Objective: To identify the clinical and surgical characteristics of the patients undergoing reimplantation of the affected branch. Method: An observational, cross-sectional, descriptive, retrospective and retrolective study was performed; patients diagnosed with anomalous origin of one of the branches of the pulmonary artery and treated by corrective surgery at the Instituto Nacional de Cardiología Ignacio Chavez, in the period from January 1, 2003 to January 31, 2019, were included. It was extracted from the files: demographic characteristics, surgical antecedents, diagnoses, echocardiographic and tomographic reports, surgical technique and post-surgical status. Results: Nine patients underwent correction surgery, with an average age of 2 ± 2 years, 11.4 ± 1.5 kg and height 82 ± 15 cm; 67% were male. The most frequent affected branch was the right, 68% originated directly from the aorta and 42% from a patent ductus arteriosus. The correction with extracorporeal circulation had an average aortic cross-clamp of 35 minutes and circulatory support of 45 minutes, the direct or synthetic implant was used. The complications were ventricular failure, bleeding, arrhythmias and nosocomial pneumonia. Mortality was 11%. Conclusions: Surgical treatment for reimplantation of the anomalous branch of the pulmonary artery is the procedure of choice, which with surgical and subsequent care has a good prognosis in the medium and long term.

Tratamiento quirúrgico de la coartación aórtica. Resultados a largo plazo en el Instituto Nacional de Cardiología / Sugical treatment of aortic coarctation. Long-term results at National Institute of Cardiology of Mexico

OBJECTIVES: Evaluate long-term evolution of patients submitted to surgery for coarctation of the aorta. Compare event free survival in younger vs older patients at the time of surgical correction. METHODS: We reviewed the clinical records of patients operated for coarctation of the aorta from January 1 1980 to December 31 1994. The mean follow-up ranged from 9 to 23 years (mean 10.9 y). Final events registered were recoarctation, death, systemic hypertension, endocarditis, stroke, aneurisms. RESULTS: Two hundred and sixteen patients were found with mean age 13 +/- 12 y male gender was most frequent (61%). Recoarctation was found in 13 patients (6.02%), persistent hypertension in 14.1%. Event free survival at 10 years was 86.2%. In patients less than 10 y was 89% vs 80.2% in older patients. Hypertension free survival in patients less than 10 y was 98.3% vs 80.1% in older patients (p < 0.001). CONCLUSIONS: Our data confirms that surgical treatment for coarctation of the aorta is associated with low morbidity and mortality at long-term with reduced rate of recoarctation (7%). Early correction (< 10 y) is associated with a better long term survival. Hypertension and use of pharmacologic treatment are reduced after surgery and persist in the long-term evolution.

Possible relationship between neurocysticercosis and hematological malignancies

Background. Previous studies have shown an increased frequency of chromosomal abnormalities in lymphocytes from animals and humans with cysticercosis. Some reports have suggested an association between neurocysticercosis and cancer. Methods. We designed a mortality rate study from the autopsy files of the Department of pathology at the General Hospital of Mexico. A total of 1,271 autopsy files were reviewed. All files in which a malignant neoplasia was found during autopsy were selected a cases. Autopsies in which no malignant disease was found were used as controls. The odds ratio was determined between the frequency of neurocysticercosis in patients with any malignant neoplasia and that of the controls. Results. Neurocysticercosis was more frequent in cases with malignant hematological diseases (MHD), than in controls (p= 0.01). The odds ratio for this association was 3.54, with 95 percent confidence interval from 1.17-9-79. Conclusions. Most human cancers arise from the interaction of a multiplicity of factors, indluding xenobiotics and endogenous constituents. Therefore, while it will be difficult to demostrate that neurocysticercosis is a causal agent of malignant hematological diseases (MHD), it should be considered as a potential risk factor for cancer induction in countries where cysticercosis remains a public health problem