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Late outcomes in children with Shone’s complex: a single-centre, 20-year experience

Published online by Cambridge University Press:  26 July 2016

George T. Nicholson
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, United States of America
Michael S. Kelleman
Affiliation:
Department of Pediatrics, Division of Biostatistics, Children’s Healthcare of Atlanta, Atlanta, Georgia, United States of America
Caridad M. De la Uz
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas, United States of America
Ricardo H. Pignatelli
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas, United States of America
Nancy A. Ayres
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas, United States of America
Christopher J. Petit*
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, United States of America
*
Correspondence to: C. J. Petit, MD, Department of Pediatrics, Division of Pediatric Cardiology, Children’s Healthcare of Atlanta, Emory University School of Medicine, 1405 Clifton Rd NE, Atlanta, GA 30322-1062, United States of America. Tel: 404 785 1796; Fax: 404 785 0029; E-mail: cjpetit@emory.edu

Abstract

Objective

Shone’s syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone’s syndrome and the factors associated with left heart growth.

Methods

All patients diagnosed with Shone’s syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed.

Results

A total of 121 patients with Shone’s syndrome presented at a median age of 28 days (0–17.3 years) and were followed-up for 7.2 years (0.01–35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time.

Conclusions

Shone’s syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.

Type
Original Articles
Copyright
© Cambridge University Press 2016 

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