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a:2:{s:4:"TEXT";s:94:"Guidelines for the management of patients with hypoplastic left heart syndrome (2017 revision)";s:4:"TYPE";s:4:"HTML";}

Authors: Russian Association of Cardiovascular Surgeons Working Group: Bockeria L.A., Berishvili D.O., Gorbachevsky S.V., Didyk V.P., Zelenikin M.M., Kalashnikov S.V., Kim A.I., Kokshenev I.V., Krupyanko S.M., Metlin S.N., Movsesyan R.R., Sabirov B.N., Tumanyan M.R., Shatalov K.V., Schmalts A.A., Yurlov I.A.

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Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, 121552, Russian Federation

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Heading: Clinical guidelines

UDC: 616.122-007.21-08-35

Link: Clinical Physiology of Blood Circulaiton. 2019; 1 (3): 27-35

Quote as: Bockeria L.A., Berishvili D.O., Gorbachevsky S.V., Didyk V.P., Zelenikin M.M., Kalashnikov S.V., Kim A.I., Kokshenev I.V., Krupyanko S.M., Metlin S.N., Movsesyan R.R., Sabirov B.N., Tumanyan M.R., Shatalov K.V., Schmalts A.A., Yurlov I.A. Guidelines for the management of patients with hypoplastic left heart syndrome (2017 revision). Cardiovascular Surgery News. 2019; 3 (1): 27–35 (in Russ.). DOI: 10.24022/2588-0284-2019-3-1-27-35

Keywords:  hypoplastic left heart syndrome left ventricular outflow tract obstruction univentricular hemodynamics hypoplastic left heart complex

Received / Accepted:  January 15, 2019 / March 11, 2019

Full text:  


Abstract

Hypoplastic left heart syndrome includes a wide range of malformations with varying degrees of hypoplasia as well as morphological disturbances of the structures of the left heart and aorta. Therefore, the degree of hypoplasia of the left heart structures determinates the severity of the patients and affects the choice of surgical treatment. However, it should be remembered that despite the anatomical features left heart hypoplasia syndrome is a critical congenital pathology which is characterized by early clinical manifestation and requires the urgent implementation of a number of stabilizing measures as well as the most rapid transfer of the patients to a specialized cardiac hospital.
These clinical guidelines are intended to adequately assess the severity of the newborns as well as to take adequate stabilizing measures in a timely manner which will further allow to provide specialized surgical treatment and facilitate the selection of optimal surgical tactics and as the final result to improve treatment outcomes and quality of life of the patients with this congenital heart disease.

References

Литература [References]

  1. Nicholls D.G., Ungerleider R.M., Spevak P.J. Hypoplastic left heart syndrome. In: Nichols D.G., Ungerleider R.M., Spevak P.J., Greeley W.J., Cameron D.E., Lappe D.G., Wedzel R.C. (Еds). Critical heart disease in infants and children. 2nd ed. Philadelphia: Mosby; 2006: 823–44.
  2. Lev M. Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes. Lab. Invest. 1952; 1: 61–70.
  3. Tchervenkov C.I., Jacobs J.P., Weinberg P.M., et al. The nomenclature, definition and classification of hypoplastic left heart syndrome. Cardiol. Young. 2006; 16: 339–68.
  4. Noonan J.A., Nadas A.S. The hypoplastic left heart syndrome. An analysis of 101 cases. Pediatr. Clin. North. Am. 1958; 5 (4): 1029–56.
  5. Kirklin J.W., Barratt-Boyes B.G. Aortic atresia and other forms of hypoplastic left heart physiology. In: Kirklin J.W., Barratt-Boyes B.G. (Eds). Cardiac surgery. Fourth edition. 2012: 1780–809.
  6. Mahowald J.M., Lucas R.V. Jr, Edwards J.E. Aortic valvular atresia. Associated cardiovascular anomalies. Pediatr. Cardiol. 1982; 2 (2): 99–105.
  7. Jonas R.A. Hypoplastic left heart syndrome. In: Jonas R.A. (Eds). Comprehensive surgical management of congenital heart disease. London: Hodder Education Publisher; 2004: 341–54.
  8. Izukawa T., Mullholland C., Rowe R.D. et al. Structural heart disease in newborn, changing profile: comparison of 1975 with 1965. Arch. Dis. Child. 1979; 54: 281–5.
  9. Scott D.J., Rigby M.L., Miller G.A.H., Shinebourne E.A. The presentation of symptomatic heart disease in infancy based on 10 years experience (1973–1982): implications for the provision of service. Br. Heart J. 1984, 52: 248–57.
  10. Бокерия Л.А., Шаталов К.В. Детская кардиохирургия: руководство для врачей. М.: НЦССХ им. А.Н. Бакулева. 2016; 684–700. [Bockeria L.A., Shatalov K.V. Children’s cardiac surgery: a guide for doctors. Moscow; 2016: 684–700 (in Russ.).]
  11. Hunter L.E., Simpson J.M. Prenatal screening for structural congenital heart disease. Nat. Rev. Cardiol. 2014; 11 (6): 323–34.
  12. Tchervenkov C.I., Tahta S.A., Jutras L.C., Béland M.J. Biventricular repair in neonates with hypoplastic left heart complex. Ann. Thorac. Surg. 1998; 66; 1350–7.
  13. Wernovsky G., Dominguez T.E., Gruber P.J., Anderson R.H. Hypoplasia of the left heart. In: Anderson R.H., Baker E.J., Penny D., Redington A.N., Rigby M.L., Wernovsky G. (Eds.). Paediatric cardiology. 3rd ed. Philadelphia: Churchill Livingstone; 2010: 625–45.
  14. Garcia R.U., Natarajan G., Walters H.L., Delius R.E., Aggarwal S. Acute kidney injury following first-stage palliation in hypoplastic left heart syndrome: hybrid versus Norwood palliation. Cardiol. Young. 2018; 28 (2): 261–8.
  15. Goldstein B.H., Goldstein S.L., Devarajan P., Zafar F., Kwiatkowski D.M., Marino B.S. et al. First-stage palliation strategy for univentricu- lar heart disease may impact risk for acute kidney injury. Cardiol. Young. 2018; 28 (1): 93–100.
  16. Mery C.M., Nieto R.M., De León L.E., Morris S.A. et al. The role of echocardiography and intracardiac exploration in the evaluation of can- didacy for biventricular repair in patients with borderline left heart structures. Ann. Thorac. Surg. 2017; 103 (3): 853–61.
  17. McElhinney D.B., Vogel M., Benson C.B., Marshall A.C., Wilkins-Haug L.E., Silva V., Tworetzky W. Assessment of left ventricular endocardial fibroelastosis in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome. Am. J. Cardiol. 2010; 106 (12): 1792–7.
  18. Mahle W.T., Weinberg P.M., Rychik J. Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants <1 year of age with left ventricular outflow obstruction? Am. J. Cardiol. 1998; 82 (1): 122–4.
  19. Kim H.J., Goo H.W., Park S.H., Yun T.J. Left ventricle volume measured by cardiac CT in an infant with a small left ventricle: a new and accurate method in determining unior biventricular repair. Pediatr. Radiol. 2013; 43 (2): 243–6.
  20. Dillman J.R., Dorfman A.L., Attili A.K., Agarwal P.P., Bell A., Mueller G.C., Hernandez R.J. Cardiovascular magnetic resonance imaging of hypoplastic left heart syndrome in children. Pediatr. Radiol. 2010; 40 (3): 261–74.
  21. Maller V.V., Cohen H.L. Neurosonography: assessing the premature infant. Pediatr. Radiol. 2017; 47 (9): 1031–45.
  22. Mitani Y., Takabayashi S., Sawada H., Ohashi H., Hayakawa H., Ikeyama Y. et al. Fate of the “opened” arterial duct: Lessons learned from bilateral pulmonary artery banding for hypoplastic left heart syndrome under the continuous infusion of prostaglandin E1. J. Thorac. Cardiovasc. Surg. 2007; 133 (6): 1653–4.
  23. Stieh J., Fischer G., Scheewe J., Uebing A., Dütschke P., Jung O. et al. Impact of preoperative treatment strategies on the early peri- operative outcome in neonates with hypoplastic left heart syndrome. J. Thorac. Cardiovasc. Surg. 2006; 131 (5): 1122–9.
  24. Barker G.M., Forbess J.M., Guleserian K.J., Nugent A.W. Optimization of preoperative status in hypoplastic left heart syndrome with intact atrial septum by left atrial decompression and bilateral pulmonary artery bands. Pediatr. Cardiol. 2014; 35 (3): 479–84.
  25. Gomide M., Furci B., Mimic B., Brown K.L., Hsia T.Y., Yates R. et al. Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants. J. Thorac. Cardiovasc. Surg. 2013; 146 (5): 1146–51.
  26. Theilen U., Shekerdemian L. The intensive care of infants with hypoplastic left heart syndrome. Arch. Dis. Child. Fetal. Neonatal. 2005; 90: 97–102.
  27. Feinstein J.A., Benson D.W., Dubin A.M., Cohen M.S., Maxey D.M., Mahle W.T. et al. Hypoplastic left heart syndrome: current considera- tions and expectations. J. Am. Coll. Cardiol. 2012; 59 (1 Suppl): S1–42.
  28. Nakano T., Kado H., Tatewaki H., Hinokiyama K. et al. Surgical strategy and the results for hypoplastic left heart syndrome. Kyobu Geka. 2014; 67 (4): 299–304.
  29. Maeda Y., Yamagishi M., Miyazaki T., Yamamoto Y., Taniguchi S., Kanzaki T. et al. The treatment strategy for hypoplastic left heart syndrome. Kyobu Geka. 2014; 67 (4): 305–9.
  30. Sano S., Ishino K., Kawada M., Arai S., Kasahara S., Asai T. et al. Right ventriclepulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J. Thorac. Cardiovasc. Surg. 2003; 126 (2): 504–9.
  31. Bacha E.A., Daves S., Hardin J., Abdulla R.I., Anderson J., Kahana M. et al. Single-ventricle palliation for high-risk neonates: the emer- gence of an alternative hybrid stage I strategy. J. Thorac. Cardiovasc. Surg. 2006; 131 (1): 163–71.
  32. Caldarone C.A., Benson L., Holtby H., Li J., Redington A.N., Van Arsdell G.S. Initial experience with hybrid palliation for neonates with single-ventricle physiology. Ann. Thorac. Surg. 2007; 84 (4): 1294–300.
  33. Galantowicz M., Cheatham J.P., Phillips A., Cua C.L., Hoffman T.M., Hill S.L., Rodeman R. Hybrid approach for hypoplastic left heart syn- drome: intermediate results after the learning curve. Ann. Thorac. Surg. 2008; 85 (6): 2063–70.
  34. Galantowicz M., Cheatham J.P. Lessons learned from the development of a new hybrid strategy for the management of hypoplastic left heart syndrome. Pediatr. Cardiol. 2005; 26: 190–9. Erratum in: Pediatr. Cardiol. 2005; 26: 307.
  35. Hill S.L., Galantowicz M., Cheatham J.P. Emerging strategies in the treatment of HLHS: combined transcatheter & surgical techniques. Pediatr. Cardiol. Today. 2003; 1: 1–4.
  36. Almond C.D., Thiagarajan R.R., Piercey G.E., Blume E.D., Bastardi H.J., Fynn-Thompson F., Singh T.P. Waiting list mortality among children listed for heart transplantation in the United States. Circulation. 2009; 119: 717–27.
  37. Boucek R.J., Chrisant M. Cardiac transplantation for hypoplastic left heart syndrome. Cardiol. Young. 2004; 14: 83–7.
  38. Kirk R., Edwards L.B., Kucheryavaya A.Y. et al. The Registry of the International Society for Heart and Lung Transplantation: thirteenth official pediatric heart transplantation report – 2010. J. Heart. Lung. Transplant. 2010; 29: 1119–28.
  39. Alsoufi B., Deshpande S., McCracken C., Kogon B., Vincent R., Mahle W., Kanter K. Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies. Eur. J. Cardiothorac. Surg. 2015; 48 (5): 792–8.
  40. Yasui H., Kado H., Nakano E. et al. Primary repair of interrupted aortic arch and severe aortic stenosis in neonates. J. Thorac. Cardiovasc. Surg. 1987; 93: 539–45.
  41. Бокерия Л.А., Подзолков В.П., Барышникова И.Ю., Симогаева Е.Б., Беришвили Д.О. Двухжелудочковая реконструкция после гиб- ридного I этапа при обструктивных поражениях левого сердца. Детские болезни сердца и сосудов. 2013; 4: 32–6. [Bockeria L.A., Podzolkov V.P., Baryshnikova I.Yu., Simogaeva E.B., Berishvili D.O. Dual ventricular reconstruction after the hybrid stage I with obstructive lesions of the left heart. Detskie Bolezni Serdtsa (Children's Diseases Heart and Vascular Diseases). 2013; 4: 32–6 (in Russ.).]
  42. Калашников С.В., Беришвили Д.О. Влияние гипоплазии левых отделов сердца на возможность двухжелудочковой коррекции сложных врожденных пороков сердца периода новорожденности. Детские болезни сердца и сосудов. 2015; 4: 47–57. [Kalashnikov S.V., Berishvili D.O. Influence of hypoplastic left heart syndrome on the possibilities of biventricular correction of the critical congenital heart diseases. Detskie Bolezni Serdtsa i Sosudov (Children's Diseases Heart and Vascular Diseases). 2015; 4: 47–57 (in Russ.).]
  43. Avitabile C.M., Mercer-Rosa L., Ravishankar C., Rome J.J., Gaynor J.W., Spray T.L., Cohen M.S. Experience with biventricular intervention for neonates with mitral valve abnormalities in the setting of critical left-side heart obstruction. Ann. Thorac. Surg. 2015; 99 (3): 877–83.
  44. Kanter K.R., Kirshbom P.M., Kogon B.E. Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles. Ann. Thorac. Surg. 2012; 93 (6): 1999–2005.
  45. Raat H., Bonsel G.J., Essink-Bot M.L., Landgraf J.M., Gemke R.J. Reliability and validity of comprehensive health status measures in children: The Child Health Questionnaire in relation to the Health Utilities Index. J. Clin. Epidemiol. 2002; 55: 67–76.
  46. Martin B.J., De Villiers J.I., Joffe A.R., Bond G.Y., Acton B.V. et. al. Hypoplastic left heart syndrome is not associated with worse clinical or neurodevelopmental outcomes than other cardiac pathologies after the Norwood-Sano operation. Pediatr. Cardiol. 2017; 38 (5): 922–31.
  47. Knirsch W., Liamlahi R., Dave H., Kretschmar O., Latal B. Neurodevelopmental outcome of children with hypoplastic left heart syndrome at one and four years of age comparing hybrid and Norwood procedure. Ann. Thorac. Cardiovasc. Surg. 2016; 22 (6): 375–7.
  48. Rogers B.T., Msall M.E., Buck G.M. et al. Neurodevelopmental outcome of infants with hypoplastic left heart syndrome. J. Pediatr. 1995; 126: 496–8.
  49. Kern J.H., Hinton V.J., Nereo N.E., Hayes C.J., Gersony W.M. Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics. 1998; 102: 1148–52.

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