Case Report Percutaneous Closure of an Atrial Septal Defect in an Infant with Shone’s Syndrome Christopher J. Petit, 1 * MD, Henri Justino, 1 MD, and Charles D. Fraser, 2 MD 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 devel- oped pulmonary overcirculation following repair of coarctation of the aorta. The infant underwent cardiac catheterization, hemodynamic assessment, and successful percuta- neous 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. V C 2012 Wiley Periodicals, Inc. Key words: atrial septal defect; mitral stenosis; device; infant BACKGROUND Closure of the atrial septal defect (ASD) in infancy is rarely indicated [1]. However, in the presence of mi- tral valve disease, the ASD can become a source of significant pulmonary overcirculation [2]. Closure of the ASD in this setting, however, can result in signifi- cant left atrial hypertension, with resultant elevation of pulmonary artery pressures. We present a case wherein an infant with Shone’s syndrome and a parachute mi- tral valve underwent successful percutaneous device closure of the ASD. Closure of the defect in the cathe- terization laboratory allowed the measurement of left atrial pressure before and after closure of the ASD. The patient responded very well to the intervention and remains well following discharge home. The mitral valve annulus and left ventricular dimensions have increased since the procedure. To our knowledge, this is the first reported case of percutaneous closure of the ASD in an infant with Shone’s syndrome. CASE REPORT A 2.7-kg full-term newborn infant with Shone’s syn- drome, mitral valve stenosis, ventricular septal defect (VSD), and coarctation of the aorta was transferred to our hospital for surgical management. The patient was prostaglandin E 1 -dependent. The initial echocardiogram demonstrated a moderate-sized secundum atrial septal defect (ASD) with significant left-to-right shunting and right ventricular (RV) enlargement. The mitral valve was structurally abnormal, with a single papillary mus- cle, and an annular dimension Z score of À3.8. The left ventricle (LV) was hypoplastic, though it was apex-forming (Fig. 1). After a lengthy discussion of the various surgical options, including single ventricle palliation, the patient underwent surgical repair of the VSD and coarctation of the aorta. An attempt to patch-close the ASD with a fenestration resulted in 1 Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, , Houston, Texas 2 Congenital Heart Surgery, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas Conflicts of interest: Dr. Petit and Dr. Fraser have no conflicts to report. Dr. Justino is a physician proctor for Amplatzer Medical Devices (St Jude, MN). He has no financial interests in the com- pany. *Correspondence to: Christopher J. Petit, MD, Texas Children’s Hos- pital, 6621 Fannin St, MC 19345-C Houston, TX 77030. E-mail: petit@bcm.edu Received 28 March 2011; Revision accepted 15 August 2011 DOI 10.1002/ccd.23362 Published online in Wiley Online Library (wiley onlinelibrary.com) V C 2012 Wiley Periodicals, Inc. Catheterization and Cardiovascular Interventions 00:000–000 (2012)