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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 ,
Michael S. Kelleman ,
Caridad M. De la Uz ,
Ricardo H. Pignatelli ,
Nancy A. Ayres  and
Christopher J. Petit
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: [email protected]
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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.

Keywords

CHDcomplex CHDShone’s complex
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 4 , May 2017 , pp. 697 - 705
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Copyright
© Cambridge University Press 2016 

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References

1. Shone, JD, Sellers, RD, Anderson, RC, Adams, P Jr, Lillehei, CW, Edwards, JE. The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 1963; 11: 714–725.Google Scholar
2. Tchervenkov, CI, Tahta, SA, Jutras, LC, Beland, MJ. Biventricular repair in neonates with hypoplastic left heart complex. Ann Thorac Surg 1998; 66: 1350–1357.Google Scholar
3. Bolling, SF, Iannettoni, MD, Dick, M 2nd, Rosenthal, A, Bove, EL. Shone’s anomaly: operative results and late outcome. Ann Thorac Surg 1990; 49: 887–893.CrossRefGoogle ScholarPubMed
4. St Louis, JD, Bannan, MM, Lutin, WA, Wiles, HB. Surgical strategies and outcomes in patients with shone complex: a retrospective review. Ann Thorac Surg 2007; 84: 1357–1362; discussion 1362–1353.CrossRefGoogle ScholarPubMed
5. Brauner, RA, Laks, H, Drinkwater, DC Jr, Scholl, F, McCaffery, S. Multiple left heart obstructions (Shone’s anomaly) with mitral valve involvement: long-term surgical outcome. Ann Thorac Surg 1997; 64: 721–729.Google Scholar
6. Brown, JW, Ruzmetov, M, Vijay, P, et al. Operative results and outcomes in children with Shone’s anomaly. Ann Thorac Surg 2005; 79: 1358–1365.CrossRefGoogle ScholarPubMed
7. Malhotra, SP, Lacour-Gayet, F, Campbell, DN, et al. Outcomes of reparative and transplantation strategies for multilevel left heart obstructions with mitral stenosis. Ann Thorac Surg 2008; 86: 1305–1309; discussion 1310.Google Scholar
8. Ikemba, CM, Eidem, BW, Fraley, JK, et al. Mitral valve morphology and morbidity/mortality in Shone’s complex. Am J Cardiol 2005; 95: 541–543.Google Scholar
9. Petit, CJ, Ing, FF, Mattamal, R, Pignatelli, RH, Mullins, CE, Justino, H. Diminished left ventricular function is associated with poor mid-term outcomes in neonates after balloon aortic valvuloplasty. Catheter Cardiovasc Interv 2012; 80: 1190–1199.Google Scholar
10. Han, RK, Gurofsky, RC, Lee, KJ, et al. Outcome and growth potential of left heart structures after neonatal intervention for aortic valve stenosis. J Am Coll Cardiol 2007; 50: 2406–2414.Google Scholar
11. Minich, LL, Tani, LY, Hawkins, JA, Shaddy, RE. Possibility of postnatal left ventricular growth in selected infants with non-apex-forming left ventricles. Am Heart J 1997; 133: 570–574.Google Scholar
12. Page, DA, Levine, MM. Left ventricular growth in a patient with critical coarctation of the aorta and hypoplastic left ventricle. Pediatr Cardiol 1995; 16: 176–178.Google Scholar
13. Petit, CJ, Justino, H, Fraser, CD. Percutaneous closure of an atrial septal defect in an infant with Shone’s syndrome. Catheter Cardiovasc Interv 2012; 80: 188–191.CrossRefGoogle Scholar
14. Sluysmans, T, Colan, SD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol 2005; 99: 445–457.Google Scholar
15. Rhodes, LA, Colan, SD, Perry, SB, Jonas, RA, Sanders, SP. Predictors of survival in neonates with critical aortic stenosis. Circulation 1991; 84: 2325–2335.Google Scholar
16. Schwartz, ML, Gauvreau, K, Geva, T. Predictors of outcome of biventricular repair in infants with multiple left heart obstructive lesions. Circulation 2001; 104: 682–687.CrossRefGoogle ScholarPubMed