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Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital

Published online by Cambridge University Press:  13 December 2011

Gul H. Dadlani ,
Katherine Braley ,
Elimarys Perez-Colon ,
Gary Stapleton ,
Marguerite Crawford ,
Dima Turpin ,
Ivan Wilmot ,
Grace Freire ,
Jamie Decker  and
Richard Martinez
Gul H. Dadlani*
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America Department of Pediatrics, University of South Florida, Saint Petersburg and Tampa, Florida, United States of America
Katherine Braley
Affiliation:
Department of Pediatrics, University of South Florida, Saint Petersburg and Tampa, Florida, United States of America
Elimarys Perez-Colon
Affiliation:
Department of Pediatrics, University of South Florida, Saint Petersburg and Tampa, Florida, United States of America
Gary Stapleton
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Marguerite Crawford
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Dima Turpin
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Ivan Wilmot
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Grace Freire
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Jamie Decker
Affiliation:
Division of Pediatric Cardiology, All Children's Hospital Heart Institute, Saint Petersburg, Florida, United States of America
Richard Martinez
Affiliation:
Department of Pediatrics, University of South Florida, Saint Petersburg and Tampa, Florida, United States of America Division of Pediatric Cardiology, The Congenital Heart Institute of Florida (CHIF), Pediatric Cardiology Associates/Pediatrix Medical Group, All Children's Hospital, Saint Petersburg, Florida, United States of America
*
Correspondence to: Dr G. H. Dadlani, MD, All Children's Heart Institute, 2nd Floor Outpatient Care Center, 601, 5th Street South, Saint Petersburg, Florida 33701, United States of America. Tel: 727-767-3333; Fax: 727-767-8990; E-mail: Gul.dadlani@allkids.org
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Abstract

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

Keywords

Fontansingle ventriclefunctionally univentricular hearthypoplastic left heart syndromefollow-upadult congenital cardiac diseaseNorwood operation

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References

1.Washington, RL. Hypoplastic left heart syndrome – the rest of the story. J Pediatr 2008; 152: 456–457.Google Scholar
2.Jaquiss, R, Imamu, M. Single ventricle physiology: surgical options, indications and outcomes. Curr Opin Cardiol 2009; 24: 113–118.CrossRefGoogle Scholar
3.Driscol, DJ. Long term results of the Fontan operation. Pediatr Cardiol 2007; 28: 438–442.CrossRefGoogle Scholar
4.Atz, AM, Traviston, TG, McCrindle, BW, et al. Late status of Fontan patients with persistent surgical fenestration. J Am Coll Cardiol 2011; 57: 2437–2443.Google Scholar
5.Elmi, M, Hickey, EJ, Williams, WG, et al. Long-term tricuspid valve function after Norwood operation. J Thorac Cardiovasc Surg 2001, Epub June 22.Google Scholar
6.Gupta-Malhotra, M. An approach to imaging adult congenital heart disease: pitfalls and pearls. Methodist Debakey Cardiovasc J 2011; 7: 18–25.Google Scholar
7.Anderson, PA, Sleeper, LA, Mahony, L, et al. Contemporary outcomes after the Fontan procedure. J Am Coll Cardiol 2008; 52: 85–98.Google Scholar
8.Choung, YF, Reuny, DJ, Redington, AN. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 2000; 83: 420–424.Google Scholar
9.Hughes, ML, Tsang, VT, Kostolny, M, et al. Lessons from inter-stage cardiac magnetic resonance imaging in predicting survival for patients with hypoplastic left heart syndrome. Cardiol Young 2011; 31: 1–8.Google Scholar
10.Petko, C, Voges, I, Schlangen, J, et al. Comparison of right ventricular deformation and dyssynchrony in patients with different subtypes of hypoplastic left heart syndrome after Fontan surgery using two-dimensional speckle tracking. Cardiol Young 2011; 19: 1–7.Google Scholar
11.Menon, SC, Minich, LL, Casper, TC, et al. Regional myocardial dysfunction following Norwood with right ventricle to pulmonary artery conduit in patients with hypoplastic left heart syndrome. J Am Soc Echocardiogr 2011; 24: 826–833.CrossRefGoogle ScholarPubMed
12.Trivedi, B, Smith, PB, Barker, PC, et al. Arrhythmias in patients with hypoplastic left heart syndrome. Am Heart J 2011; 161: 138–144.CrossRefGoogle ScholarPubMed
13.Kaldararova, M, Balazova, E, Bordacova, L, et al. Arrhythmias in congenital heart defects. Bratisl Lek Listy 2007; 108: 14–19 (abstract).Google ScholarPubMed
14.Deal, BJ, Mavroudis, C, Backer, CL. Arrhythmia management in the Fontan patient. Pediatr Cardiol 2007; 28: 448–456.Google Scholar
15.Gersony, DR, Gersony, WM. Management of the postop Fontan patient. Progr Pediat Cardiol 2003; 17: 73–79.CrossRefGoogle Scholar
16.Khairy, P, Dore, A, Talajic, M, et al. Arrythmias in adult congenital heart disease. Expert Rev of Cardiovasc Ther 2006; 4: 83–95.Google Scholar
17.Agnoletti, G, Borghi, A, Vignati, G, Crupi, GC. Fontan conversion to total cavopulmonary connection and arrhythmia ablation. Heart 2003; 89: 193–198.CrossRefGoogle ScholarPubMed
18.Marino, BS. Outcomes after the Fontan procedure. Curr Opin Pediatr 2002; 152: 456–457.Google Scholar
19.Aboulhosn, J, Child, JS. The adult with a Fontan Operation. Curr Cardiol Rep 2007; 9: 331–335.CrossRefGoogle ScholarPubMed
20.Walker, HA, Gatzoulis, MA. Prophylactic anticoagulation following the Fontan operation. Heart 2005; 91: 854–856.CrossRefGoogle ScholarPubMed
21.Coon, PD, Rychik, J, Novello, RT, Ro, PS, Gaynor, JW, Spray, TL. Thrombus formation after the Fontan operation. Ann Thorac Surg 2001; 71: 1990–1994.CrossRefGoogle ScholarPubMed
22.Balling, G, Vegt, M, Kaemmerer, H, Eicken, A, Meisner, H, Hess, J. Intracardiac thrombus formation after the Fontan operation. J Thorac Cardiovasc Surg 2000; 119: 745–752.CrossRefGoogle ScholarPubMed
23.Kaulitz, R, Ziemer, G, Rauch, R, et al. Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis). J Thorac Cardiovasc Surg 2005; 129: 569–575.CrossRefGoogle ScholarPubMed
24.Odegard, KC, Zurakowski, D, DiNardo, JA, et al. Prosepective longitudinal study of coagulation profiles in children with hypoplastic left heart syndrome from stage 1 through Fontan completion. J Thoracic Cardiovasc Surg 2009; 137: 934–941.CrossRefGoogle Scholar