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Mustard's operation for patients with ventriculoarterial concordance. Report of two cases and a cautionary tale
Insights
This study investigated complex congenital heart defects, specifically abnormal atrioventricular connections with ventriculoarterial concordance. Early diagnosis and surgical correction are crucial for survival in these rare pediatric cardiac cases.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Investigating complex congenital heart defects involving ventriculoarterial concordance and abnormal atrioventricular connections.
- Examining cases with total anomalous pulmonary venous connection and associated septal defects.
Observation:
- One patient with total anomalous pulmonary venous connection underwent repair but died due to missed atrioventricular discordance.
- Two patients with atrioventricular discordance and ventricular septal defects underwent successful Mustard's operation.
Findings:
- Delayed diagnosis of atrioventricular discordance led to a fatal outcome in one case.
- Mustard's operation provided survival for patients with situs solitus and left isomerism, despite complex cardiac anomalies.
- Spontaneous closure of a ventricular septal defect was observed in one patient with left isomerism.
Implications:
- Highlights the critical importance of accurately diagnosing atrioventricular discordance in complex congenital heart disease.
- Demonstrates the efficacy of Mustard's operation in selected cases of atrioventricular discordance.
- Underscores the need for comprehensive pre-operative assessment in pediatric cardiac surgery to prevent diagnostic errors.
Abstract:
Three patients with ventriculoarterial concordance and an abnormal atrioventricular connexion were investigated. One patient had total anomalous pulmonary venous connexion to a left vertical vein. In 1972 this was repaired and a ventricular septal defect was closed, but the presence of atrioventricular discordance was missed and the patient died. In the other two patients, Mustard's operation resulted in survival. One patient had situs solitus and atrioventricular discordance with an associated ventricular septal defect, which was closed. The other had left isomerism (quasi solitus) with an ambiguous atrioventricular connexion (quasi discordant). A previously noted ventricular septal defect had spontaneously closed.