Sujets)
Humains , Complexe d'Eisenmenger/classification , Complexe d'Eisenmenger/traitement médicamenteux , Complexe d'Eisenmenger/imagerie diagnostique , Bronchoscopie/méthodes , Cathétérisme cardiaque/méthodes , Électrocardiographie , Inhibiteurs de la phosphodiestérase-5/administration et posologie , Bosentan/administration et posologie , Cardiopathies congénitalesRésumé
Ausência de conexão atrioventricular à direita com o ventrículo dominante, morfologicamente direito, colocado à esquerda, em conexão com o átrio esquerdo, é uma rara situação. Os autores apresentam cinco casos com esta disposição morfológica, salientando os aspectos anatômicos e clínicos, bem como a importância da nomenclatura baseada na análise segmentar seqüencial.
Sujets)
Enfant , Enfant d'âge préscolaire , Nourrisson , Femelle , Humains , Adolescent , Atrium du coeur/malformations , Cardiopathies congénitales/diagnostic , Ventricules cardiaques/malformations , Atrium du coeur/chirurgie , Cardiopathies congénitales/chirurgie , Ventricules cardiaques/chirurgie , TerminologieRésumé
The authors present a case of a two-year-old boy with stridor since birth, and also recurrent episodes of pulmonary infections, due to mechanical compression of distal trachea by a left pulmonary artery sling. They discussed diagnostic approach, surgical management and review of the literature. To our knowledge, it is the first case of sling of the pulmonary artery reported in Brazil and the fifth in a latin language country.
Sujets)
Humains , Mâle , Enfant d'âge préscolaire , Artère pulmonaireRésumé
Rabdomiosarcoma primário do coraçäo obstruindo veias cavas, foi diagnosticado em uma criança de 10 anos de idade, ocm quadro clínico recente de três meses, caracterizado por comprometimento do estado geral, febre persistente e acentuado emagrecimento. Säo enfatizados os aspectos clínicos e os exames complementares desta rara afecçäo, que permitem o diagnóstico em vida, bem como a conduta terapêutica realizada e sua evoluçäo
The authors report a case of a child, 10 years old with a primary rhabdomyosarcoma of the heart that obstructed both caval veins. The clinical picture that lastedjust three months before the admission was characterized by persistent fever, accentuated loss of weight and a very deteriorated general aspect. We emphazise the clinical aspectos and other subsidiary elements that easily allowed to make the precise diagnosis in life as well as the therapeutic measures andfollow up, besides a review ofthe literature, where it was confirmed the rarity of this pathological entity.