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Does Aortic and Mitral Annular Hypoplasia Predict Need for Additional Cardiac Interventions In Infants After Isolated Aortic Arch Repair?

Friday, October 19, 2012: 2:00 PM
Room 275-277 (Morial Convention Center)
Jennifer M. Plymale, MD, Peter C. Frommelt, MD, Melodee Nugent, Pippa Simpson, PhD and Amanda J. Shillingford, MD, Pediatric Cardiology, Children's Hospital of Wisconsin Medical College of Wisconsin, Milwaukee, WI

Purpose: Significant aortic arch obstruction is often associated with hypoplasia of other left sided structures.  In these borderline left heart patients, surgical aortic arch repair (AAR) alone may not suffice due to the inadequacy of the other left heart structures, increasing the risk of morbidity and mortality.  We sought to identify preoperative echocardiographic features associated with the need for additional cardiac intervention (CI) on left heart structures or death within 1 year after isolated AAR in infants with small mitral (MV) and aortic valves (AV). 

Methods: The Herma Heart Center database was reviewed to identify infants ≤60 days of age who underwent isolated AAR from 1999-2010 and had AV and/or MV hypoplasia defined as annular Zscore ≤-2.  Preoperative echocardiograms were reviewed for AV, MV, tricuspid valve (TV) and aortic arch size and compared the need for CI and/or death during the 1styear of life.

Results: Seventy-eight patients met inclusion criteria.  Fifty-four (69%) survived AAR without further intervention in the first year.  Twenty-four (31%) patients required additional catheter or surgical interventions with 5(20%) deaths.  Six of the 24 (25%) failed biventricular physiology; 5 were converted to a Norwood (2 died), 1 required ECMO support after AAR and died.  Eighteen of the 24 (75%) patients maintained biventricular physiology but required 31interventions.  Interventions included 13 aortic outflow procedures (4 aortic valvuloplasties, 5 subaortic membrane resections, 4 Ross-Konno) with 1 death.  There were 9 additional aortic arch interventions and 9 MV procedures (8 valvuloplasties, 1 replacement) with 1 death.  The need for additional CI is associated with patients having both AV and MV Zscores ≤-2 (p=.013).  AV annulus <5mm, proximal transverse arch <3.5mm, and smaller MV to TV ratio also predict the need for additional CI.

Conclusion: In infants with aortic arch obstruction and small AV and/or MV size, additional CI after initial AAR is common during the 1styear of life.  Failure to tolerate biventricular physiology after isolated AAR is associated with significant morbidity and mortality. Progressive mitral and aortic outflow disease as well as recurrent arch obstruction necessitating additional procedures is frequently seen.  Infants with both AV and MV hypoplasia, AV <5mm, proximal transverse arch <3.5mm and smaller MV to TV ratio appear to be at highest risk for additional left heart interventions and should be considered for more aggressive initial surgical strategies.