Percutaneous closure of an atrial septal defect in an infant with Shone's syndrome
Christopher J Petit1, Henri Justino, Charles D Fraser
1Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children's Hospital, Houston, Texas 77030, USA. petit@bcm.edu
Insights
Closure of an atrial septal defect (ASD) in infants is rare but can be life-saving. This case study shows successful percutaneous ASD closure improved an infant with Shone's syndrome, enabling better left heart growth.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Interventional Cardiology
Background:
- Atrial septal defects (ASDs) are rarely closed in infants.
- Congenital mitral stenosis with ASD can cause infant congestive heart failure.
- Shone's syndrome involves multiple left-sided heart obstructions.
Observation:
- An infant with Shone's syndrome, left ventricular hypoplasia, and a large ASD developed pulmonary overcirculation post-coarctation repair.
- The infant underwent cardiac catheterization and hemodynamic assessment.
- Successful percutaneous closure of the ASD was performed.
Findings:
- The infant showed dramatic clinical improvement after ASD closure.
- Follow-up echocardiograms revealed growth in mitral valve annulus diameter and left ventricular dimensions.
- Percutaneous ASD closure led to immediate clinical improvement and enhanced left heart development.
Implications:
- Percutaneous ASD closure is a viable option for select infants with complex congenital heart disease.
- Early intervention can improve hemodynamics and promote growth of underdeveloped left heart structures.
- This approach may prevent long-term complications associated with untreated ASDs in this population.
Abstract:
Rarely is closure of the atrial septal defect indicated in infants. Yet, an atrial septal defect in the presence of congenital mitral stenosis may cause significant congestive heart failure in infancy. We present the case of an infant with Shone's syndrome, left ventricular hypoplasia, and a large ostium secundum atrial septal defect who developed pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percutaneous closure of the atrial septal defect. The patient improved dramatically following the intervention. Follow-up echocardiograms have demonstrated growth of mitral valve annulus diameter and left ventricular dimensions. Atrial septal defect closure in this small infant was associated with immediate improved clinical status, and improved growth of the left heart structures.
