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[Single ventricle and related anomalies. Anatomic findings in 30 cases. Diagnostic and therapeutic implications
Insights
This study analyzes 30 single ventricle cases, revealing common L-bulboventricular loops and frequent obstructive outflow tract anomalies. Accurate preoperative assessment is crucial for surgical planning in complex congenital heart disease.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Anatomy
Context:
- Single ventricle and related anomalies represent complex congenital heart defects.
- Understanding the detailed anatomical variations is essential for diagnosis and treatment.
- Previous classifications and criteria guide case selection for this study.
Purpose:
- To investigate the anatomical characteristics of single ventricle and related anomalies.
- To classify cases based on atrioventricular canal expansion and ventricular septal hypoplasia.
- To identify key anatomical features influencing surgical management.
Summary:
- 30 cases of single ventricle anomalies were analyzed and categorized into 6 groups.
- An L-bulboventricular loop was observed in 43.47% of cases, primarily in groups with defective atrioventricular canal expansion.
- Obstructive outflow tract anomalies were prevalent (66.6%), as was a small left atrioventricular orifice (56.66%).
Impact:
- Findings highlight the frequent occurrence of specific anatomical variations like L-bulboventricular loops and outflow tract obstructions.
- The study underscores the necessity of detailed preoperative anatomical evaluation for effective surgical planning.
- This research contributes to a better understanding of single ventricle pathophysiology and guides clinical decision-making.
Abstract:
Anatomic characteristics are studied in 30 cases of single ventricle and related anomalies. Cases are distributed into 6 groups according to: a) the derangement in the expansión of the atrioventricular canal towards the "bulbus cordis", which may be either defective (groups I, II and III) or excessive (groups IV and V), and b) the hypoplastic nature of the ventricular septum (group VI). The most important conclusions are: 1) An L-bulboventricular loop was found in 43.47% of the entire material; all of them pertained to groups I, II and III. 2) A normal position of the great arteries was uncommon, being encountered in 13.4% of the cases. An aorta situated anteriorly and to the left was present in 30.43% of the cases included in groups IV and V. 3) Obstructive anomalies of either outflow tract were discovered in 66.6% of the entire material, regardless of the type of bulboventricular loop. 4) The left atrioventricular orifice was found to be abnormally small in 56.66% of the total case material and in 88.23% of the cases pertaining to groups I, II and III. 5) A mitral atresia was encountered in two cases pertaining to groups I and II. The criteria which have been used to admit cases with this association of anomalies have been established in previous publications by one of the authors of the present study. 6) The changes in the spacial orientation of the leaflets of the right atrioventricular orifice in cases with an L-bulboventricular loop are attributed to the rotation of the heart around its longitudinal axis which takes place in cases with levocardia and mesocardia. 7) A preoperative study as accurate as possible is necessary in order to establish a rational approach to the surgical management of these patients.