ABSTRACT
Introducción: la incidencia de dextrocardia como anomalía congénita es menor del 0.01% y la combinación con herniación intratorácica del hígado semejando una neoplasia benigna sin antecedente de trauma toracoabdominal abierto o contuso lo hace aún menos frecuente. Caso clínico: se presenta el caso de paciente femenina de 34 años de edad que consulta por dolor de espalda. Al examen físico, se auscultan ruidos cardíacos en el hemitórax derecho y la radiografía de tórax evidencia dextrocardia e imagen que semeja masa supra diafragmática derecha, la TAC trifásica confirma la presencia de protrusión de un segmento del hígado de forma redondeada a través de un defecto no abierto del hemidiafragma derecho. Su tratamiento ha sido conservador. Conclusión: la combinación de dextrocardia acompañada de herniación de una porción del hígado a través de un defecto del diafragma derecho es una asociación extremadamente rara y los reportes de caso publicados son escasos
Subject(s)
Humans , Female , Adult , Dextrocardia/epidemiology , Hernia, Diaphragmatic/epidemiology , Liver , Case Reports , Incidence , Diagnosis, DifferentialABSTRACT
OBJECTIVE: To expose our 26 year experience in clinical management, interventional catheterization and surgical treatment of patients younger than 18 years with scimitar syndrome at the National Cardiology Institute. MATERIAL AND METHODS: We reviewed retrospectively all patients with scimitar syndrome in infancy between 1984 and 2010. Patients were divided in two groups: younger an older than one year at the time of the diagnosis. Medical records were analized, as well as chest radiography, electrocardiogram, echocardiogram and helicoidal tomography. All therapeutic procedures performed and their outcomes were analized. RESULTS: We studied 22 patients with scimitar syndrome, 20 of them with associated congenital heart disease. Congestive heart failure (p > or = 0.0001) and severe pulmonary hypertension (p > or = 0.002) were more frequent in patients younger than one year. We documented dextroposition and right lung hypoplasia in 14 patients, and aorto-pulmonary collateral arteries in 15 of them. Pulmonary hypertension was an important mortality risk factor (p > or = 0.007). CONCLUSIONS: Congestive heart failure and pulmonary hypertension are more frequent in patients younger than one year, and the former is a mortality risk factor. It is important to suspect this congenital heart disease in infants with dextroposition and congestive heart failure in order to provide an opportune treatment.