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Thorac Cardiovasc Surg 2002; 50(5): 266-270
DOI: 10.1055/s-2002-34580
Original Cardiovascular
Original Paper
© Georg Thieme Verlag Stuttgart · New York

Borderline Hypoplastic Left Heart Malformations: Norwood Palliation or Two-Ventricle Repair?

S.  H.  Daebritz1 , A.  R.  Tiete1 , D.  Rassoulian1 , U.  Roemer2 , R.  Kozlik-Feldmann2 , J.  S.  Sachweh1 , H.  Netz2 , B.  Reichart1
  • 1Clinic of Cardiac Surgery, University Hospital Grosshadern, Munich
  • 2Department of Pediatric Cardiology and Intensive Care, University Hospital Grosshadern, Munich
Further Information

Publication History

Received February 5, 2002

Publication Date:
08 October 2002 (online)

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Abstract

Background: Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair. Methods: Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group I). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3). Results: Operative mortality in group I was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 ± 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20mmHg (patient 3). Clinical condition of all survivors in both groups is good. Conclusion: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

Key words

Cardiac surgery - Congenital heart disease - Hypoplastic left heart syndrome - Two-ventricle repair

References

  • 1 Kirklin J W, Barratt-Boyes B G. Cardiac Surgery.  New York, Churchill Livingstone. 1993;  2 21-26
    PubMedSearch in Google Scholar
  • 2 Jacobs M L, Rychnik J, Murphy J D, Nicolson S C, Steven J M, Norwood W I. Results of Norwood's Operation for Lesions other than Hypoplastic Left Heart Syndrome.  J Thorac Cardiovasc Surg. 1995;  110 1555-1562
    PubMedSearch in Google Scholar
  • 3 Kanter K R, Miller B E, Cuadrado A G, Vincent R N. Successful application of the Norwood procedure for infants without hypoplastic left heart syndrome.  Ann Thorac Surg. 1995;  59 301-304
    CrossrefPubMedSearch in Google Scholar
  • 4 Daebritz S H, Nollert G D, Zurakowski D, Khalil P N, Lang P, del Nido P J, Mayer J E , Jonas R A. Results of Norwood stage I operation: comparison of hypoplastic left heart syndrome with other malformations.  J Thorac Cardiovasc Surg. 2000;  119 (2) 358-367
    CrossrefPubMedSearch in Google Scholar
  • 5 Ohye R G, Kagisaki K, Lee L A, Mosca R S, Goldberg C S, Bove E L. Biventricular Repair for Aortic Atresia or Hypoplasia and Ventricular Septal Defect.  J Thorac Cardiovasc Surg. 1999;  118 648-654
    PubMedSearch in Google Scholar
  • 6 Akintuerk H, Michel-Behnke I, Valeske K, Mueller M, Thul J, Bauer J, Hagel K J, Kreuder J, Vogt P, Schranz D. Stenting of the arterial duct and banding of the pulmonary arteries: basis for combined Norwood stage I and II repair in hypoplastic left heart.  Circulation. 2002;  105 (9) 1099-1103
    CrossrefPubMedSearch in Google Scholar
  • 7 Tchervenkov C I, Tahta S A, Jutras L, Beland M J. Biventricular repair in neonates with hypoplastic left heart complex.  Ann Thorac Surg. 1998;  66 1350-1357
    CrossrefPubMedSearch in Google Scholar
  • 8 Tchervenkov C I, Jacobs M L, Tahta S A. Congenital Heart Surgery Nomenclature and Database Project: Hypoplastic Left Heart Syndrome.  Ann Thorac Surg. 2000;  69 170-179
    CrossrefPubMedSearch in Google Scholar
  • 9 Norwood W I, Kirklin J K, Sanders S. Hypoplastic Left Heart Syndrome: Experience With Palliative Surgery.  Am J Cardiol. 1980;  45 87-91
    CrossrefPubMedSearch in Google Scholar
  • 10 Bailey L, Concepcion W, Shattuck H, Huang L. Method of heart transplantation for treatment of hypoplastic left heart syndrome.  J Thorac Cardiovasc Surg. 1986;  92 (1) 1-5
    PubMedSearch in Google Scholar
  • 11 Lofland G K, McCrindle B W, Williams W G, Blackstone E H, Tchervenkov C I, Sittiwangkul R, Jonas R A. Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society
  • 12 Gentles T L, Mayer J E , Gauvreau K, Newburger J W, Lock J E, Kupferschmidt J P, Burnett J, Jonas R A, Castaneda A R, Wernovsky G. Fontan Operation in Five Hundred Consecutive Patients: Factors Influencing Early and Late Outcome.  J Thorac Cardiovasc Surg. 1997;  114 376-391
    PubMedSearch in Google Scholar
  • 13 Donnelly J P, Raffel D M, Shulkin B L, Corbett J R, Bove E L, Mosca R S, Kulik T J. Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography.  J Thorac Cardiovasc Surg. 1998;  116 (1) 182-183
    PubMedSearch in Google Scholar
  • 14 Bartram U, Grünefelder J, van Praagh R. Causes of Death After the Modified Norwood Procedure: A Study of 122 Postmortem Cases.  Ann Thorac Surg. 1997;  64 1795-8022
    CrossrefPubMedSearch in Google Scholar
  • 15 Mahle W T, Spray T L, Gaynor J W, Clark B J . Unexpected death after reconstructive surgery for hypoplastic left heart syndrome.  Ann Thorac Surg.. 2001;  71 (1) 61-65
    CrossrefPubMedSearch in Google Scholar
  • 16 Duffy C E, Muster A J, DeLeon S Y. et al . Successful surgical repair of aortic atresia associated with normal left ventricle.  J Am Coll Cardiol. 1983;  1 1503-1506
    PubMedSearch in Google Scholar
  • 17 Austin E H, Jonas R A;, Castaneda A R. Aortic atresia with normal left ventricle: single stage reapair in the neonate.  J Thorac Cardiovasc Surg. 1989;  97 392-395
    PubMedSearch in Google Scholar
  • 18 Francois K, Dollery C, Elliott M J. Aortic Atresia With Ventricular Septal Defect and Normal Left Ventricle: One-Stage Correction in the Neonate.  Ann Thorac Surg. 1994;  58 878-880
    PubMedSearch in Google Scholar
  • 19 Serraf A, Bruniaux J, Lebidois J, Lacour-Gayet F, Kachaner J, Planche C. Aortic Atresia With Normal Left Ventricle.  Ann Thorac Surg. 1991;  51 1017-1019
    PubMedSearch in Google Scholar
  • 20 Ohye R G, Kagisaki K, Lee L A, Mosca R S, Goldberg C S, Bove E L. Biventricular Repair for Aortic Atresia or Hypoplasia and Ventricular Septal Defect.  J Thorac Cardiovasc Surg. 1999;  118 648-654
    PubMedSearch in Google Scholar
  • 21 Serraf A, Piot J D, 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 (3) 827-834
    CrossrefPubMedSearch in Google Scholar
  • 22 Rhodes L A, Colan S D, Perry S B, Jonas R A, Sanders S P. Predictor of Survival in Neonates With Critical Aortic Stenosis.  Circulation. 1991;  84 2325-2335
    PubMedSearch in Google Scholar
  • 23 Leung M P, McKay R, Smith A, Anderson R H, Arnold R. Critical aortic stenosis in infancy-anatomical and echocardiographic substrates of successful open valvulotomy.  J Thorac Cardiovasc Surg. 1991;  101 526-535
    PubMedSearch in Google Scholar
  • 24 Nurozler F, Bradley S M. Considerations in biventricular repair after the Norwood procedure.  Eur J Cardiothorac Surg. 2000;  18 (5) 540-544
    CrossrefPubMedSearch in Google Scholar
  • 25 Foker J E, Berry J, Steinberger J, Krabill K, Moller J. The forward flow signal for growth of hypoplastic ventricles.  Circulation. 1997;  96 (8) 1-435
    PubMedSearch in Google Scholar

MD Sabine Daebritz

Department of Cardiac Surgery, University Hospital Grosshadern

Marchioninistr. 15

81377 Munich

Germany

Phone: ++49-89-7095-3941

Fax: ++49-89-7095-3943

Email: Sdaebritz@aol.com