Hypoplastic Left Ventricle: Definition, Morphology, and Classification of the Cardiac Phenotypes

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Morphology

Background:

• Previous work defined hypoplastic left heart syndrome (HLHS) using 2021 IPCCC and ICD-11.
• This study focuses on cardiac phenotypes associated with a hypoplastic left ventricle (HLV) potentially suitable for biventricular repair.

Purpose of the Study:

• To define, morphologically classify, and categorize four specific congenital cardiac malformations featuring a hypoplastic left ventricle.
• To provide a framework for evaluating suitability for biventricular repair in these conditions.

Main Methods:

• Utilized the 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and ICD-11 for classification.
• Described definitions and morphology of four distinct cardiac malformations associated with HLV.

Main Results:

• Detailed classification of hypoplastic left heart complex (HLHC).
• Characterization of critical aortic stenosis with left ventricular hypoplasia.
• Description of aortic atresia with ventricular septal defect.
• Analysis of hypoplastic left ventricle with unbalanced atrioventricular septal defect.

Conclusions:

• Established clear definitions, morphology, and classifications for four HLV-associated conditions.
• Aimed to improve diagnostic accuracy and guide treatment decisions, particularly regarding biventricular repair strategies.