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Biventricular repair in the management of hypoplastic left heart syndrome

  • Victor O. Morell (a1), James A. Quintessenza (a1) and Jeffrey P. Jacobs (a1)

Extract

Hypoplastic left heart syndrome is the term introduced by Noonan and Nadas1 to describe a spectrum of cardiac anomalies characterized by varying degrees of significant underdevelopment of the left heart and aortic arch. These cardiac anomalies include mitral valvar disease, left ventricular hypoplasia, aortic stenosis at subvalvar, valvar, and supravalvar levels, hypoplasia of the ascending aorta and aortic arch, and aortic coarctation. Although descriptive, hypoplastic left heart syndrome suffers from being a very unspecific term, since it encompasses multiple degrees and combinations of abnormalities involving the left sided structures. In an attempt to provide gradings of severity, Kirklin and Barratt-Boyes2 categorized these patients falling into the syndrome into four classes, according to whether obstruction was found at one, two, or more levels, or whether there is aortic atresia. Then, in 1998, Tchervenkov and colleagues3 introduced the term hypoplastic left heart complex to describe a set of patients falling within the spectrum of hypoplasia of the left heart, but in the absence of intrinsic aortic or mitral valvar stenosis, this concept subsequently being endorsed by the International Committee established by the Society of Thoracic Surgeons, together with the European Association of Cardiothoracic Surgery, to rationalize the approach to nomenclature and databases.4 The analysis offered by Tchervenkov et al.3 showed that, in certain circumstances, there are potentially patients considered to have hypoplasia of the left heart who might be candidates for biventricular repair. If this is the case, then it is important to establish how this subset can be recognized, and how they are best treated.

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Corresponding author

Correspondence to: Victor Morell MD, Chief, Pediatric Cardiac Surgery, Congenital Heart Institute of Florida, Tampa Children's Hospital, Cardiac Surgical Associates, 3001 West Martin Luther King Blvd., Suit 102, Tampa, Florida 33607, USA. Tel: 813 554 8343; E-mail: vmorell@heartsurgery-csa.com; Web: http://www.heartsurgery-csa.com

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References

Noonan JA, Nadas AS. The hypoplastic left heart syndrome. An analysis of 101 cases. Pediatr Clin North Am 1958; 5: 10291056.
Kirklin JW, Barratt-Boyes BG. Coarctation of the aorta and interrupted aortic arch. In: Kirklin JW, Barratt-Boyes BG (eds). Cardiac Surgery. Churchill Livingstone, New York, 1993, pp 12691270.
Tchervenkov CI, Tahta SA, Jutras LC, Beland MJ. Biventricular repair in neonates with hypoplastic left heart complex. Ann Thorac Surg 1998; 66: 13501357.
Tchervenkov CI, Jacobs ML, Tahta SA. Congenital heart surgery nomenclature and database project: hypoplastic left heart syndrome. Ann Thorac Surg 2000; 69: S170S179.
Rhodes LA, Colan SD, Perry SB, Jonas RA, Sanders SP. Predictors of survival in neonates with critical aortic stenosis. Circulation 1991; 84: 23252335.
Gundry SR, Behrendt DM. Prognostic factors in valvotomy for critical aortic stenosis in infancy. J Thorac Cardiovasc Surg 1986; 92: 747754.
Parsons MK, Moreau GA, Graham TP Jr, Johns JA, Boucek RJ Jr. Echocardiographic estimation of critical left ventricular size in infants with isolated aortic valve stenosis. J Am Coll Cardiol 1991; 18: 10491055.
Hammon JW Jr, Lupinetti FM, Maples MD, Merrill WH, First WH, Graham TP Jr, Bender HW Jr. Predictors of operative mortality in critical valvular aortic stenosis presenting in infancy. Ann Thorac Surg 1988; 45: 537540.
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: 570574.
Tani LY, Minich LL, Pagotto LT, Shaddy RE, McGough EC, Hawkins JA. Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable? J Thorac Cardiovasc Surg 1999; 118: 8186.
Alboliras ET, Mavroudis C, Pahl E, Gidding SS, Backer CL, Rocchini AP. Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta. Ann Thorac Surg 1999; 68: 549555.
Serraf A, Piot JD, Bonnet N, Lacour-Gayet F, Touchot A, Bruniaux J, Belli E, Galletti L, Planche C. Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle. J Am Coll Cardiol 1999; 33: 827834.
Tchervenkov CI. Two-ventricle repair for Hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Annual 2001; 4: 8393.
Yasui H, Kado H, Nakano E, Yonenaga K, Mitani A, Tomita Y, Iwao H, Yoshii K, Mizoguchi Y, Sunagawa H. Primary repair of interrupted aortic arch and severe aortic stenosis in neonates. J Thorac Cardiovasc Surg 1987; 93: 539545.
Pearl JM, Cripe LW, Manning PB. Biventricular repair after Norwood palliation. Ann Thorac Surg 2003; 75: 132137.
Lofland GK, McCrindle BW, Williams WG, Blackstone EH, Tchervenkov CI, Sittiwangkul R, Jonas RA. Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons' Society. J Thorac Cardiovasc Surg 2001; 121: 1027.

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