Functionally univentricular heart

Description

The term “functionally univentricular heart” describes a spectrum of congenital cardiovascular malformations in which the ventricular mass may not readily lend itself to partitioning that commits one ventricular pump to the systemic circulation, and another to the pulmonary circulation. Additional information: a heart may be functionally univentricular because of its anatomy or because of the lack of feasibility or lack of advisability of surgically partitioning the ventricular mass. Common lesions in this category typically include double inlet right ventricle (DIRV), double inlet left ventricle (DILV), tricuspid atresia, mitral atresia, and hypoplastic left heart syndrome. Other lesions which sometimes may be considered to be a functionally univentricular heart include complex forms of atrioventricular septal defect, double outlet right ventricle, congenitally corrected transposition, pulmonary atresia with intact ventricular septum, and other cardiovascular malformations. Specific diagnostic codes should be used whenever possible, and not the term “functionally univentricular heart”.

Exclusions from above levels

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

Child codes

LA89.0 – Double inlet atrioventricular connection
LA89.1 – Tricuspid atresia
LA89.2 – Mitral atresia
LA89.3 – Hypoplastic left heart syndrome
LA89.Y – Other specified functionally univentricular heart
LA89.Z – Functionally univentricular heart, unspecified

Sibling codes

LA80 – Anomalous position-orientation of heart
LA81 – Abnormal ventricular relationships
LA82 – Total mirror imagery
LA83 – Right isomerism
LA84 – Left isomerism
LA85 – Congenital anomaly of an atrioventricular or ventriculo-arterial connection
LA86 – Congenital anomaly of mediastinal vein
LA87 – Congenital anomaly of an atrioventricular valve or atrioventricular septum
LA88 – Congenital anomaly of a ventricle or the ventricular septum
LA8A – Congenital anomaly of a ventriculo-arterial valve or adjacent regions
LA8B – Congenital anomaly of great arteries including arterial duct
LA8C – Congenital anomaly of coronary artery
LA8D – Congenital pericardial anomaly
LA8E – Congenital anomaly of atrial septum
LA8F – Congenital anomaly of right atrium
LA8G – Congenital anomaly of left atrium
LA8Y – Other specified structural developmental anomaly of heart or great vessels
LA8Z – Structural developmental anomaly of heart or great vessels, unspecified

Foundation entity

Functionally univentricular heart – http://id.who.int/icd/entity/5417233

Part of: 20: Developmental anomalies