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| Subsequent Aortic Arch and Left Ventricular Outflow Tract Procedures in Patients After Interrupted Aortic Arch Repair: A Multi-Institutional Study |
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Anusha Jegatheeswaran1, Marshall L. Jacobs2, Gary K. Lofland3, Jeffrey P. Jacobs4, Richard A. Jonas5, Brian W. McCrindle6, Eugene H. Blackstone7, Marco Ricci8, William G. Williams1, Christopher A. Caldarone1, William M. DeCampli9 1 Surgery, Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, ON, Canada; 2 Pediatric and Congenital Heart Surgery, Cleveland Clinic, Cleveland, OH; 3 Cardiothoracic Surgery, Children's Mercy Hospital, Kansas, MO; 4 Surgery, Division of Thoracic and Cardiovascular Surgery, The Congenital Heart Institute of Florida (CHIF), Cardiac Surgical Associates of Florida (CSAoF), University of South Florida (USF), All Children's Hospital, Children's Hospital of Tampa, Saint Petersburg and Tampa, FL; 5 Division of Cardiac Surgery, Center for Heart, Lung and Kidney Disease, Children's National Medical Center, Washington, DC; 6 Pediatrics, Division of Cardiology, Hospital for Sick Children, Toronto, ON, Canada; 7 Thoracic and Cardiovascular Surgery, Cleveland Clinic Foundation, Cleveland, OH; 8 Surgery, Division of Cardiothoracic Surgery, University of Miami Health Systems, Miller School of Medicine, Miami, FL; 9 Cardiothoracic Surgery, The Congenital Heart Institute at Arnold Palmer Hospital for Children, Orlando, FL Objective: Surgical management of interrupted aortic arch (IAA) requires aortic arch (AoA) repair and is often associated with multiple subsequent procedures (SPs) directed at the AoA and/or the left ventricular outflow tract (LVOT). We sought to determine time-dependent outcomes and freedom from these SPs after an index AoA repair, and factors associated with these SPs. Methods: 470 patients (pts) with IAA at 33 institutions from 1987-1997 were reviewed. “Time zero” was the date of index AoA repair. SPs were classified by type (catheter-based or surgical) and focus (AoA, LVOT, both, or neither). Competing risks and modulated renewal analysis were used to explore SPs of the AoA and LVOT. Results: 447 of the 470 pts had an index AoA repair (318 type B IAA), with concomitant repair of the LVOT in 39 pts. 287 pts had no AoA or LVOT SPs. 119 pts had 158 AoA SPs, 70 pts had 101 LVOT SPs, and 231 pts had 436 SPs of any type (136 catheter-based, 300 surgical). 15 years after index repair, competing risks analysis (AoA SPs, n=447) demonstrated that 32% had died, 29% had an AoA SP, and 39% remained alive without an AoA SP. Factors associated with a first AoA SP included index AoA repair with an unrepaired ventricular septal defect (VSD) or use of the left subclavian artery as a patch/conduit, and worse clinical status at the time of admission for index repair. Modulated renewal analysis demonstrated a lower risk of further repeated AoA SPs after the first AoA SP (Figure). 15 years after index repair, competing risks analysis (LVOT SPs, n=423) demonstrated that 33% had died, 18% had a LVOT SP, 48% remained alive and at risk, and 1% were alive but no longer at risk for an LVOT SP. Factors associated with a first LVOT SP included initial anatomic features (outlet VSD, smaller aortic valve, bicuspid aortic valve), concomitant procedures at index AoA repair (pulmonary artery (PA) banding, systemic to PA shunt, VSD repair not via right atrium), and undergoing a non-AoA procedure prior to the first LVOT SP. Modulated renewal analysis demonstrated a higher risk of further repeated LVOT SPs after the first LVOT SP (Figure). Conclusion: IAA repair is associated with a high prevalence of subsequent aortic arch and left ventricular outflow tract procedures. Anatomic and procedural factors are associated with increased risk. After the first subsequent procedure, the risk of further aortic arch procedures decreases, while the risk of further left ventricular outflow tract procedures increases. 
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