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J Am Coll Cardiol, 2007; 49:1806-1812, doi:10.1016/j.jacc.2007.01.071 (Published online 13 April 2007).
© 2007 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: CARDIAC SURGERY FOR CONGENITAL DISEASE

The Relationship Between Neo-Aortic Root Dilation, Insufficiency, and Reintervention Following the Ross Procedure in Infants, Children, and Young Adults

Sara K. Pasquali, MD*,||, Meryl S. Cohen, MD, FACC*,||, David Shera, ScD{dagger},||, Gil Wernovsky, MD, FACC*,||, Thomas L. Spray, MD{ddagger} and Bradley S. Marino, MD, MPP, MSCE, FACC*,§,||,#,*

* Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
{dagger} Division of Biostatistics and Epidemiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
{ddagger} Division of Cardiothoracic Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
§ Division of Critical Care Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
|| Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
# Department of Anesthesiology and Critical Care at The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.

Manuscript received September 25, 2006; revised manuscript received December 20, 2006, accepted January 9, 2007.

* Reprint requests and correspondence: Dr. Bradley S. Marino, Assistant Professor of Anesthesia and Pediatrics, University of Pennsylvania School of Medicine, The Children’s Hospital of Philadelphia, 7th Floor Main, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. (Email: marino{at}email.chop.edu).

Presented in part at the American Heart Association 2005 Scientific Session.

Objectives: The purpose of this study was to describe the relationship between neo-aortic root size, neo-aortic insufficiency (AI), and reintervention at mid-term follow-up.

Background: Data on neo-aortic valve function and growth after the Ross procedure in children are limited.

Methods: A total of 74 of 119 Ross patients from January 1995 to December 2003 had ≥2 follow-up echocardiograms at our institution and were included. Neo-aortic dimensions were converted to z-scores and modeled over time. Kaplan-Meier analysis was used to assess freedom from neo-aortic outcomes, and predictors were identified through multivariate analysis.

Results: Median age at Ross was 9 years (range 3 days to 34 years). Over 4.7 years (range 3 months to 9.3 years) follow-up, there was disproportionate enlargement of the neo-aortic root (z-score increase of 0.75/year [p < 0.0001]). Neo-AI progressed ≥1 grade in 36% of patients and ≥2 grades in 15%. Nine patients (12%) had neo-aortic reintervention at 2.0 years (range 1.1 to 9.5 years) after the Ross procedure owing to severe neo-AI (n = 7), neo-aortic root dilation (n = 1), and neo-aortic pseudoaneurysm (n = 1). At 6 years after the Ross procedure, freedom from neo-aortic reintervention was 88%. Freedom from neo-aortic root z-score >4 was only 3% and from moderate or greater neo-AI was 60%. Longer follow-up time was associated with neo-aortic root dilation (p < 0.0001). Prior ventricular septal defect (VSD) repair predicted neo-AI (p = 0.02) and reintervention (p = 0.03). Prior aortic valve replacement (p = 0.002) also predicted neo-AI. Neo-aortic root dilation was not associated with neo-AI or reintervention.

Conclusions: At mid-term follow-up after the Ross procedure, neo-aortic root size increases significantly out of proportion to somatic growth, and neo-AI is progressive. Prior VSD repair and aortic valve replacement were associated with neo-AI and reintervention.

Abbreviations and Acronyms
  AI = aortic insufficiency
  LV = left ventricular
  VSD = ventricular septal defect




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