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The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair. Pediatr Cardiol 2017 Aug;38(6):1296-1304

Date

07/06/2017

Pubmed ID

28676926

DOI

10.1007/s00246-017-1661-2

Scopus ID

2-s2.0-85021819800   5 Citations

Abstract

In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p < 0.001) and MV changed from -2.88 to -1.41 (p < 0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1 year survival is high, but the need for RI and the presence of residual lesions are common.

Author List

Plymale JM, Frommelt PC, Nugent M, Simpson P, Tweddell JS, Shillingford AJ

Authors

Peter C. Frommelt MD Professor in the Pediatrics department at Medical College of Wisconsin
Pippa M. Simpson PhD Chief, Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Aorta, Thoracic
Aortic Valve
Cardiac Surgical Procedures
Echocardiography
Echocardiography, Doppler
Female
Heart Ventricles
Humans
Hypoplastic Left Heart Syndrome
Infant
Infant, Newborn
Male
Mitral Valve
Prognosis
Regional Blood Flow
Reoperation
Retrospective Studies
Risk Assessment
jenkins-FCD Prod-399 190a069c593fb5498b7fcd942f44b7bc9cdc7ea1