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Left isomerism and complete atrioventricular block: a report of six cases
Insights
Left isomerism and complete atrioventricular (A-V) block are rare congenital conditions in children. This study highlights the severe cardiac anomalies and high mortality associated with these conditions, emphasizing the need for advanced cardiac care.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Electrophysiology
Background:
- Left isomerism is a rare congenital condition affecting cardiac and visceral அமைப்பு.
- Complete atrioventricular (A-V) block presents a significant challenge in pediatric cardiac care.
- The co-occurrence of left isomerism and complete A-V block is particularly rare and associated with complex cardiac anomalies.
Purpose of the Study:
- To describe the clinical presentation and outcomes of children with left isomerism and complete A-V block.
- To review the associated complex congenital heart diseases and anatomical abnormalities.
- To discuss the management and prognosis of this rare condition.
Main Methods:
- Case series presentation of six pediatric patients.
- Diagnostic methods included cardiac catheterization and angiocardiography.
- Assessment of associated cardiac and visceral anomalies.
Main Results:
- Six children (12 days to 13 years) diagnosed with left isomerism and complete A-V block.
- Common findings included interrupted inferior vena cava, common atrium, and other complex heart defects.
- Four patients had congenital complete A-V block; two developed it later in infancy.
- All patients required pacemaker implantation, and five died, indicating a high mortality rate.
Conclusions:
- Left isomerism with complete A-V block is a severe condition with complex cardiac malformations.
- High mortality underscores the critical nature of these combined congenital defects.
- Pacemaker implantation is necessary but does not alter the poor prognosis in most cases.
Abstract:
Six children, aged 12 days to 13 years, with left isomerism and complete atrioventricular (A-V) block are presented. In all six patients the diagnosis of left isomerism was suggested by an interrupted inferior vena cava found during cardiac catheterization and angiocardiography; four patients had complex heart disease consisting of endocardial cushion defect, five had a common atrium, three had pulmonary stenosis, three had patient ductus arteriosus and two had dextrocardia. Further anatomic abnormalities included situs inversus of the viscera (four patients) as well as partial malrotation of the bowel. Of the six patients, four had congenital complete A-V block, whereas the remaining two had A-V conduction disturbances documented during early infancy that progressed to complete A-V block later in life. All six patients required pacemaker implantation and five of the six patients died. This report discusses the clinical presentation of complete AV block and left isomerism and reviews the literature.