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LA8A.10 Pulmonary atresia with intact ventricular septum

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Pulmonary atresia with intact ventricular septum
• pulmonary valve atresia with intact ventricular septum

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