Pulmonary atresia with intact ventricular septum (Q46177): Difference between revisions
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Malformação cardiovascular congênita em que normalmente existem grandes artérias alinhadas, nenhuma abertura entre o ventrículo morfologicamente direito e o tronco pulmonar e nenhuma comunicação no nível ventricular._x000D_ _x000D_ Informações adicionais: atresia pulmonar com septo ventricular íntegro é uma malformação congênita dependente do ducto que forma um espectro de lesões incluindo atresia da valva pulmonar, um grau variável de hipoplasia do ventrículo direito e da valva tricúspide e anomalias da circulação coronária. Uma circulação arterial coronária dependente do ventrículo direito está presente quando as fístulas da artéria coronária estão associadas a uma estenose proximal de artéria coronária. Anomalia de Ebstein associada da valva tricúspide pode estar presente. | |||
| description / en | description / en | ||
A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication. Additional information: pulmonary atresia with intact ventricular septum is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a varying degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation. A right ventricular dependent coronary artery circulation is present when coronary artery fistulas are associated with a proximal coronary artery stenosis. Associated Ebstein anomaly of the tricuspid valve can be present. | |||
Revision as of 14:35, 13 August 2026
A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication. Additional information: pulmonary atresia with intact ventricular septum is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a varying degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation. A right ventricular dependent coronary artery circulation is present when coronary artery fistulas are associated with a proximal coronary artery stenosis. Associated Ebstein anomaly of the tricuspid valve can be present.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | LA8A.10 |
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| English | Pulmonary atresia with intact ventricular septum |
A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication. Additional information: pulmonary atresia with intact ventricular septum is a duct-dependent congenital malformation that forms a spectrum of lesions including atresia of the pulmonary valve, a varying degree of right ventricle and tricuspid valve hypoplasia, and anomalies of the coronary circulation. A right ventricular dependent coronary artery circulation is present when coronary artery fistulas are associated with a proximal coronary artery stenosis. Associated Ebstein anomaly of the tricuspid valve can be present. |
