Tetralogy of Fallot with absent pulmonary valve syndrome (Q46171): Difference between revisions
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Malformação cardiovascular congênita que é uma variante da tetralogia de Fallot em que a junção ventrículo-arterial do ventrículo direito com o tronco pulmonar apresenta uma valva atípica com folhetos (cúspides) ausentes ou rudimentares que não coaptam._x000D_ _x000D_ Informações adicionais: em sua forma usual ocorre dilatação do tronco pulmonar e das artérias pulmonares centrais direita e esquerda, que quando é extrema, está associada à arborização anormal de ramos lobares e segmentares da artéria pulmonar e à compressão da traqueia e brônquios-fonte, muitas vezes com traqueobroncomalácia. A consequência fisiológica é geralmente uma combinação de graus variáveis de estenose e regurgitação da valva pulmonar. | |||
| description / en | description / en | ||
A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt. Additional information: in its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia. The physiologic consequence is usually a combination of variable degrees of both stenosis and regurgitation of the pulmonary valve. | |||
Revision as of 14:34, 13 August 2026
A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt. Additional information: in its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia. The physiologic consequence is usually a combination of variable degrees of both stenosis and regurgitation of the pulmonary valve.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | LA88.20 |
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| English | Tetralogy of Fallot with absent pulmonary valve syndrome |
A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with absent or rudimentary leaflets (cusps) that do not coapt. Additional information: in its usual form there is dilatation of the pulmonary trunk and central right and left pulmonary arteries, which when extreme, is associated with abnormal arborization of lobar and segmental pulmonary artery branches and with compression of the trachea and mainstem bronchi, often with tracheobronchomalacia. The physiologic consequence is usually a combination of variable degrees of both stenosis and regurgitation of the pulmonary valve. |
