Iranian Heart Journal

Iranian Heart Journal

Successful Hybrid Management of Ruptured Sinus of Valsalva Aneurysm With Associated Coarctation of the Aorta and Bicuspid Aortic Valve in a Pediatric Patient

Document Type : Case Report

Authors
Maryam Moradian 1
Hassan Birjandi 2
Mahmood Hosseinzadeh maleki 3
Mohammad Reza Naghibi 2
Elahe Heidari 2
1 Cardiovascular Research Center, Rajaie Cardiovascular Institute, Tehran, Iran.
2 Department of Pediatric Cardiology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IR Iran.
3 Department of Cardiac Surgery, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, IR Iran.
Abstract
Background: Congenital sinus of Valsalva aneurysm (SoVA) is a rare cardiac anomaly that predominantly affects the right coronary sinus. When ruptured, it can result in an aorto-cardiac shunt with hemodynamic consequences. Associated congenital lesions such as ventricular septal defect (VSD), coarctation of the aorta (CoA), bicuspid aortic valve, and patent ductus arteriosus may compound the clinical picture.
 
Case Presentation: A 12-year-old boy presented with acute chest pain, dyspnea, and palpitations. He was initially misdiagnosed with pneumonia and heart failure (New York Heart Association [NYHA] class III). Further evaluation revealed a congenital SoVA originating from the noncoronary cusp and rupturing into the right atrium (RA), producing a large left-to-right shunt. Transthoracic echocardiography demonstrated a windsock–like structure extending from the noncoronary sinus to the RA. Associated anomalies included severe discrete coarctation of the aorta.
 
Interventions: After stabilization with medical therapy, the patient underwent successful catheter-based CoA stenting. Because of the significant shunt and chamber dilation, open-heart surgery was subsequently performed.
 
Outcome: At postoperative follow-up, the patient reported no clinical symptoms. No residual coarctation or shunt was detected, and the boy remained hemodynamically stable.
 
Conclusions: This case underscores the importance of early recognition and comprehensive management of complex congenital cardiac anomalies, including SoVA, CoA, VSD, and valvular abnormalities. Multimodality imaging, catheter-based intervention, and surgical correction resulted in an excellent clinical outcome in this pediatric patient. (Iranian Heart Journal 2026; 27(1): 81-87)
Keywords
sinus of Valsalva
ventricular septal defect
coarctation of the aorta
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Iranian Heart Journal
Volume 27, Issue 1
Winter 2026
Pages 81-87