ABSTRACT
El corazón univentricular es una cardiopatía congénita poco frecuente. Constituye un 2% del total de las cardiopatías congénitas. Se caracteriza por la ausencia de un ventrículo cardíaco, generalmente el derecho, por lo que este ventrículo único recibe toda la sangre tanto la que procede de los pulmones como la que llega sin oxigenar del organismo. Además, debe impulsar la sangre hacia el pulmón y hacia todo el cuerpo, por lo que realiza un doble trabajo. En el 80% de los casos se asocia a una estenosis pulmonar (estrechez de la válvula que comunica el ventrículo con la salida de la arteria pulmonar) por lo que el flujo que va a los pulmones es menor, y se oxigena menos sangre. Esto se traduce clínicamente en la presencia de cianosis en estos pacientes, aunque permite una disminución en el trabajo del ventrículo. El diagnóstico prenatal es posible gracias a la ecografía fetal que presenta una sensibilidad del 30-36%. Dentro de las cardiopatías congénitas existen cuadros complejos como el corazón univentricular de escasa incidencia pero de mal pronóstico, el cual puede mejorarse si se realiza un diagnóstico prenatal óptimo que permita entre otros una atención y tratamiento adecuados
Univentricular heart is a rare congenital malformation, constituting 2% of all congenital heart diseases. This malformation is characterized by the absence of a heart ventricle, usually the right ventricle, and consequently the single ventricle receives all the blood coming from the lungs, as well as the blood reaching this ventricle without oxygenating the body. Moreover, the single ventricle must pump blood to the lungs and to the entire body, thus performing a double duty. In 80% of patients, univentricular heart is associated with pulmonary stenosis (narrowing of the valve that connects the ventricle to the pulmonary artery output) and consequently less blood flows to the lungs and less blood is oxygenated. Clinically, this reduction results in the presence of cyanosis, while at the same time decreasing ventricular work. Fetal ultrasound has allowed prenatal diagnosis with a sensitivity of 30-36%. Congenital heart diseases include complex entities such as univentricular heart, which has a low incidence but poor prognosis. However, prognosis can be improved by early prenatal diagnosis, which allows optimal care and treatment
Subject(s)
Humans , Female , Pregnancy , Adult , Heart Ventricles/abnormalities , Pulmonary Valve Stenosis/epidemiology , Heart Defects, Congenital/diagnosis , Prenatal Diagnosis/methods , Ultrasonography, PrenatalABSTRACT
Se presenta el caso clínico de una paciente de sexo femenino, de 39 años de edad, la cual cursó con cardiopatía reumática crónica inactiva con secuelas valvulares del tipo de la estenosis mitral y doble lesión aórtica con predominio de la estenosis, insuficiencia tricúspide funcional e hipertensión arterial pulmonar, cardiopatía clasificada por la New York Heart Association como clase iv , con asociación de embarazo de 16 semanas, presentando trombo en la aurícula izquierda. Fue tratada mediante comisurotomía mitral cerrada y pérdida del embarazo a las 20,2 semanas. Se efectúa revisión de la literatura médica acerca de cirugía valvular durante el embarazo, haciendo hincapié en el tratamiento quirúrgico así como en las posibles repercusiones para el binomio (AU)
We present the case of a 39-year-old woman with inactive rheumatic heart disease and valvular sequelae consisting of mitral stenosis, double aortic lesion with a predominance of stenosis, functional tricuspid insufficiency, and pulmonary hypertension. The patient had New York Heart Association heart failure class IV associated with a 16-week pregnancy and a thrombus in the left atrium. Treatment consisted of closed mitral commissurotomy and abortion at 20.2 weeks of pregnancy. We provide a review of the literature on valvular surgery during pregnancy, with emphasis on surgical treatment and the possible repercussions to mother and fetus (AU)
Subject(s)
Humans , Female , Adult , Rheumatic Heart Disease/surgery , Pregnancy Complications, Cardiovascular/surgery , Maternal Age , Pregnancy, High-Risk , Fetal Death/etiologyABSTRACT
OBJECTIVE: To establish distribution frequency and demographic characteristics of salivary gland tumours (SGT) in order to identify possible risk profiles. DESIGN OF STUDY: The present report constitutes an eight year retrospective study (January 2000-August 2007). The archives of the Clinical and Experimental Pathology Laboratory (Graduate and Research Division, Dental School, National Autonomous University of Mexico) as well as archives of the Surgical Pathology Service (General Hospital, Mexico City) were subject to revision in order to select all cases where SGT tumour diagnoses were emitted. Age and gender of patients as well as SGT topography were obtained from medical records. Selected cases were classified according to location of the lesion, histological lineage and biological behaviour. RESULTS: 360 cases of SGT were included, 227 (67%) cases were benign tumours, while 83 cases (23%) were malignant tumours. SGT were most frequent in women with ages ranging from their 3rd to 5th decades of life. 275 tumours were located in major salivary glands, 78.9% of them were identified in the parotid gland. The most frequent location of tumours arising from minor salivary glands (33 cases, 38%) was found in the palatine glands. Tumours of epithelial lineage were the predominant histological type. The most frequent benign tumours were pleomorphic adenomas (86.1%) and papillary cystadenoma lymphomatosum (7.3%). The most frequent malignant tumours were adenoid cystic carcinomas (25%) and mucoepidermoid carcinomas (23.6%). CONCLUSIONS: Salivary gland tumours in Mexican population appear principally in major salivary glands of women in their 3rd to 5th decade of life.
Subject(s)
Salivary Gland Neoplasms/epidemiology , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Mexico/epidemiology , Middle Aged , Retrospective Studies , Risk Factors , Young AdultABSTRACT
El síndrome de poiquilodermia congénita también denominado síndrome de Rothmund-Thompson es una rara genodermatosis de carácter autosómico recesivo por mutación del gen RECQ4 del cromosoma 8. Tiene una gama de manifestaciones clínicas las que destacan: poiquilodermia, alteraciones esqueléticas, hipotiroidismo e hipogonadismo y cataratas juveniles. Se han documentado 200 casos de esta entidad a nivel mundial, su asociación al embarazo nunca se ha documentado. Se presenta el caso clínico de síndrome de Rothmund en paciente gestante a término. Se hace revisión del tema en la literatura mundial (AU)
Congenital poikiloderma syndrome, also called Rothmund-Thomson syndrome, is a rare autosomal recessive genodermatosis due to a RECQ4 gene mutation on chromosome 8. This syndrome has a range of clinical manifestations that include poikiloderma, skeletal changes, hypothyroidism and hypogonadism and juvenile cataracts. Although 200 cases of this disease have been documented worldwide, its association with pregnancy has not been previously reported. We report a case of Rothmund-Thomson syndrome in a pregnant patient at term and review the world literature on the topic (AU)
