Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral artery

Description

A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which there is no direct communication between the right ventricle and the pulmonary arterial tree and there are collateral blood vessels between the systemic and pulmonary arteries.

Coding note

This morphological abnormality usually is an integral part of other congenital cardiovascular anomalies and does not need to be coded separately. It should be coded as secondary to an accompanying congenital cardiovascular anomaly if the left ventricular hypoplasia is not considered an integral and understood part of the primary congenital cardiovascular diagnosis such as hypoplastic left heart syndrome.

Exclusions from above levels

• http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism