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Spectrum of hearts with one underdeveloped and one dominant ventricle
Insights
This study introduces a new angiographic classification for complex heart defects involving unequal ventricles. The proposed system aids in diagnosing and surgically managing these conditions, emphasizing the importance of single ventricle function post-repair.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Complex congenital heart disease encompasses a spectrum of anomalies.
- Unequal ventricular development presents diagnostic and therapeutic challenges.
Purpose of the Study:
- To present an angiographic classification system for hearts with one underdeveloped and one dominant ventricle.
- To evaluate the utility of this system for diagnosis and surgical planning.
Main Methods:
- Retrospective analysis of 129 patients undergoing catheterization and cineangiography (1974-1983).
- Development of a five-group classification based on ventricular dominance and relationship.
- Assessment of associated intracardiac and extracardiac anomalies within each group.
Main Results:
- Five distinct groups were identified: dominant left ventricle (53%), dominant right ventricle (20%), dominant left ventricle with inversion (20%), dominant right ventricle with inversion (3%), and true single ventricle (5%).
- Physiological and surgical similarities were noted across groups, with single ventricle function post-repair being a critical factor.
Conclusions:
- The proposed angiographic grouping system offers advantages for the diagnostic and surgical approach to complex single ventricle physiology.
- Understanding the limitations of a single ventricle's stroke volume post-intervention is paramount.
Abstract:
Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983. The age at first catheterization ranged from one day to 24 years (mean 3.9 years). Male-female ratio was 2:1. Five groups of hearts (with their relative frequencies in the spectrum) were established: dominant left ventricle (53%); dominant right ventricle (20%), each with normally related chambers; dominant left ventricle (20%); dominant right ventricle (3%), each with ventricular inversion; true single ventricle (5%). The incidence of atrial anatomy, venous return, intracardiac connections and associated lesions within each group was assessed. From the standpoint of deranged physiology as well as surgical implications there are more similarities than differences among these hearts. The fact that one ventricle will not generate an adequate stroke volume after repair is overwhelmingly more important than most other considerations. For the diagnostic and surgical approach, we believe that the system offers many advantages.