Related Experiment Videos
Atrial isomerism: a surgical experience
N Sinzobahamvya1, C Arenz, A M Brecher
1Department of Paediatric Cardio-thoracic Surgery, Johanniter Kinderklinik, Sankt Augustin, Germany.
Cardiovascular Surgery (London, England)
|August 3, 1999
Summary
Children with left atrial isomerism have treatable cardiac anomalies, with an 84% survival rate after surgical repair. Right atrial isomerism, however, presents complex congenital heart defects with a poorer prognosis and requires palliative care, showing a 49% survival rate.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Thoracic Surgery
Background:
- Atrial isomerism is a rare congenital condition affecting cardiac development.
- Most existing literature comprises autopsy or case reports, lacking comprehensive surgical outcome data.
Purpose of the Study:
- To review surgical outcomes for children with atrial isomerism.
- To analyze associated cardiac anomalies, surgical procedures, and survival rates in left and right atrial isomerism.
Main Methods:
- Retrospective review of 41 consecutive pediatric patients operated on between 1980 and 1996.
- Detailed analysis of cardiac diagnoses, surgical interventions (biventricular repair, Fontan circulation, palliative procedures), and patient outcomes.
Main Results:
- Left Atrial Isomerism (23 patients): Common diagnoses included interrupted inferior vena cava, atrioventricular septal defects, common atrium, and cor triatriatum sinistrum. 84% actuarial survival rate after repair and total cavopulmonary connection.
- Right Atrial Isomerism (18 patients): Predominant anomalies were pulmonary atresia/stenosis, discordant ventriculo-arterial connection, atrioventricular septal defects, single ventricle, and extracardiac total anomalous pulmonary venous drainage. 49% survival rate after repair and Fontan circulation.
- Surgical interventions varied, with biventricular repair feasible in some left isomerism cases, while right isomerism predominantly required staged palliation.
Conclusions:
- Cardiovascular malformations in left atrial isomerism are often amenable to successful surgical correction.
- Right atrial isomerism is associated with complex defects that typically preclude biventricular repair, necessitating staged palliation and carrying a significantly poorer prognosis.