ABSTRACT
Se presenta el caso de un lactante de dos meses con una cardiopatía congénita compleja del tipo coartación aórtica con comunicación interventricular asociada a un trastorno de migración celular cerebral. El manejo consistió en la corrección de la cardiopatía congénita en dos tiempos quirúrgicos sin haber profundizado en la evaluación neurológica preoperatoria. El paciente desarrolló múltiples complicaciones perioperatorias que incluyeron paro cardiaco en dos ocasiones, reconexiones consecutivas a circulación extracorpórea, tamponamiento cardiaco, quilotórax y choque séptico. Concomitante a las complicaciones postoperatorias, se realizó una evaluación neurológica secundaria bajo un protocolo de abordaje neurológico que consistió en electroencefalografía, resonancia nuclear magnética y tomografía por emisión de positrón (SPECT). De esta forma se detectó paquigiria con microgiria y se sospechó trastorno de migración celular cerebral. La evolución final fue hacia el deceso por falla multisistémica y la autopsia confirmó la patología neural, así como el pobre pronóstico para la función y la vida. De haberse considerado en el preoperatorio un estudio integral que incluyera el binomio corazón-cerebro, el planteamiento terapéutico podría haberse modificado.
A case of a two month infant with complex congenital heart disease (aortic coarctation with ventricular septal defect) associated to a cellular brain migration failure is presented. The management strategy consisted on the correction of congenital heart disease by means of a two-stage surgery without a further preoperative evaluation of the neurological status. The patient developed several perioperative complications such as two episodes of cardiac arrest, reconnection to cardiopulmonary bypass, cardiac tamponade, chilothorax and septic shock. A neurological protocol consisting in electroencephalography, brain magnetic resonance and Single Photon Emission Computed Tomography (SPECT) was practiced during the postoperative period, which detected microgyria with paquigyria and a cellular brain migration failure was suspected. The final outcome was death due to multisystemic failure and the autopsy confirmed the neurological disease, as well as poor life function prognosis. Should the heart-brain binomial had been considered in an integral preoperative evaluation, the therapeutical approach could have been modified.
Subject(s)
Humans , Infant , Male , Abnormalities, Multiple/diagnosis , Brain/abnormalities , Heart Defects, Congenital/therapy , Brain/pathology , Fatal OutcomeABSTRACT
Las malformaciones congénitas más frecuentes son las cardiopatías congénitas. La prevalencia reportada a nivel mundial va de 2.1 a 12.3 por 1000 recién nacidos. En nuestro país, se desconoce su prevalencia real; como causa de muerte infantil, se ubica en el sexto lugar en menores de un año y como la tercera causa en los niños entre uno y cuatro años; con base en la tasa de natalidad, se calcula que alrededor de 10 mil a 12 mil niños nacen con algún tipo de malformación cardiaca. Es de suma importancia conocer la magnitud del problema, identificar el número de niños que nacen cada año con una cardiopatía congénita y de manera desglosada por el tipo de la malformación; lo que permitiría determinar con mayor exactitud los recursos necesarios y planear su distribución. La regionalización tiene como objetivo la racionalización de los recursos con énfasis en servicios médicos muy especializados con la finalidad de lograr un mejor resultado para los pacientes. Por lo que, de manera simultánea a la creación de una base de datos fidedigna de las cardiopatías congénitas y, con base a la tasas de natalidad y mortalidad infantil secundaria a patología cardiovascular congénita y recursos en cada Estado, se debería proceder a regionalizar la atención. Lo anterior tendría diversos beneficios, ya que permitiría aumentar el número de casos atendidos, mejorar la calidad de la atención, aprovechar adecuadamente los recursos existentes y, seguramente, obtener una disminución de la mortalidad infantil.
Congenital cardiopathies are the most frequent congenital malformations. Reports of its prevalence around the world range from 2.1 to 12.3 for every 1000 newborns. Prevalence in our country remains unknown, but it probably occupies sixth place for mortality in infants less than a year old, and third place for mortality in those aged between 1 and 4 years. Based on birthrate, it is calculated that 10 to 12 000 infants in our country have some cardiac malformation. To understand the magnitude of the problem, it is important to identify the global number of newborns with some congenital cardiopathy each year and the type of malformation that they have, in order to determine the necessary resources and to plan their distribution. The main objective of regionalization is the justification of the resources with an emphasis in the specialized medical services to provide the best results for the patients. Hence, reason, based on the resources of each state, as well as their natality and infant mortality rates related to congenital cardiovascular pathology, we should proceed to regionalize the attention, and to simultaneously create a trustworthy database of the congenital cardiopathies. This should have many benefits, such as increase the number of total attended cases, improve the quality of attention, use appropriately the existent resources, and -surely- decrease the infant mortality.
Subject(s)
Humans , Infant, Newborn , Health Resources/organization & administration , Heart Defects, Congenital/therapy , Heart Defects, Congenital/epidemiology , Mexico , Risk AssessmentABSTRACT
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.
Subject(s)
Humans , Infant , Male , Pulmonary Veins/abnormalities , Tetralogy of Fallot , Abnormalities, Multiple , Abnormalities, Multiple , Cardiac Catheterization/methods , Pulmonary Veins , Pulmonary Veins , Tetralogy of FallotABSTRACT
Kawasaki's disease is by now the first cause of pediatric acquired cardiopathies in many countries, even more than rheumatic fever. Probably the most common complication of this disease is coronary affection, which often causes stenosis. Treatment of the acute and chronic coronary events in children is based on the knowledge acquired from the disease in adults. The increasing experience in pediatric patients with this pathology has led to better ways of handling and treating this disease.
Subject(s)
Adolescent , Humans , Male , Coronary Stenosis , Myocardial Revascularization , Mucocutaneous Lymph Node Syndrome , Coronary StenosisABSTRACT
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.