Hypoplastic Left Heart Syndrome Current Considerations and Expectations

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:59

Article

Feinstein, Jeffrey A.¹  Benson, D. Woodrow³  Dubin, Anne M.¹  Cohen, Meryl S.⁵  Maxey, Dawn M.¹  Mahle, William T.⁶  Pahl, Elfriede⁷  Villafane, Juan⁹  Bhatt, Ami B.¹⁰  Peng, Lynn F.¹  Johnson, Beth Ann³  Marsden, Alison L.²  Daniels, Curt J.⁴  Rudd, Nancy A.¹¹  Caldarone, Christopher A.¹²  Mussatto, Kathleen A.¹¹  Morales, David L.¹³  Ivy, D. Dunbar¹⁴  Gaynor, J. William⁴  Tweddell, James S.¹¹  Deal, Barbara J.⁸  Furck, Anke K.¹⁵  Rosenthal, Geoffrey L.¹⁶  Ohye, Richard G.¹⁷  Ghanayem, Nancy S.¹¹  Cheatham, John P.⁴  Tworetzky, Wayne¹⁰  Martin, Gerard R.¹⁸ 

[1] Stanford Univ, Sch Med, Dept Pediat, Lucile Salter Packard Childrens Hosp, Palo Alto, CA 94304 USA

[2] Univ Calif San Diego, Dept Mech & Aerosp Engn, San Diego, CA 92103 USA

[3] Univ Cincinnati, Coll Med, Dept Pediat, Cincinnati Childrens Hosp,Med Ctr, Cincinnati, OH USA

[4] Ohio State Univ, Coll Med, Nationwide Childrens Hosp, Dept Pediat, Columbus, OH 43210 USA

[5] Univ Penn, Childrens Hosp Philadelphia, Sch Med, Dept Pediat, Philadelphia, PA 19104 USA

[6] Emory Univ, Sch Med, Dept Pediat, Atlanta, GA USA

[7] Childrens Mem Hosp, Dept Pediat, Feinberg NW Sch Med, Chicago, IL 60614 USA

[8] Northwestern Univ, Dept Pediat, Feinberg Sch Med, Chicago, IL 60611 USA

[9] Univ Kentucky, Dept Pediat, Louisville, KY USA

[10] Harvard Univ, Childrens Hosp, Sch Med, Dept Cardiol, Boston, MA 02115 USA

[11] Childrens Hosp Wisconsin, Med Coll Wisconsin, Dept Pediat, Madison, WI USA

[12] Univ Toronto, Hosp Sick Children, Sch Med, Dept Surg,Div Cardiac Surg, Toronto, ON M5G 1X8, Canada

[13] Texas Childrens Hosp, Baylor Coll Med, Dept Pediat, Houston, TX 77030 USA

[14] Univ Colorado, Dept Pediat, Denver Sch Med, Childrens Hosp, Denver, CO 80202 USA

[15] Royal Brompton & Harefield NHS Fdn Trust, Dept Pediat, London, England

[16] Univ Maryland, Sch Med, Dept Pediat, Baltimore, MD 21201 USA

[17] Univ Michigan Hlth Syst, CS Mott Childrens Hosp, Dept Surg, Ann Arbor, MI USA

[18] Childrens Natl Med Ctr, Div Cardiol, Washington, DC 20010 USA

关键词: congenital heart defects;    Fontan procedure;    Glenn procedure;    hypoplastic left heart syndrome;    Norwood procedure;   

DOI  :  10.1016/j.jacc.2011.09.022

来源: Elsevier

【 摘 要 】

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

【 授权许可】

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