Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:69

Article

Atz, Andrew M.¹  Zak, Victor²  Mahony, Lynn³  Uzark, Karen⁴  D'agincourt, Nicholas²  Goldberg, David J.⁵  Williams, Richard V.⁶  Breitbart, Roger E.⁷  Colan, Steven D.⁷  Burns, Kristin M.⁸  Margossian, Renee⁷  Henderson, Heather T.⁹  Korsin, Rosalind¹⁰  Marino, Bradley S.¹¹  Daniels, Kaitlyn⁶  McCrindle, Brian W.¹² 

[1] Med Univ South Carolina, Charleston, SC 29425 USA

[2] New England Res Inst, 9 Galen St, Watertown, MA 02172 USA

[3] Univ Texas Southwestern Med Ctr Dallas, Dallas, TX 75390 USA

[4] Univ Michigan, Ann Arbor, MI 48109 USA

[5] Childrens Hosp Philadelphia, Philadelphia, PA 19104 USA

[6] Univ Utah, Salt Lake City, UT USA

[7] Boston Childrens Hosp, Boston, MA USA

[8] NHLBI, Bldg 10, Bethesda, MD 20892 USA

[9] Duke Univ, Med Ctr, Durham, NC USA

[10] Columbia Univ, Med Ctr, New York, NY USA

[11] Cincinnati Childrens Hosp Med Ctr, Cincinnati, OH 45229 USA

[12] Univ Toronto, Hosp Sick Children, Toronto, ON, Canada

关键词: adult congenital heart disease;    exercise;    Fontan procedure;    functional health status;    single ventricle;   

DOI  :  10.1016/j.jacc.2017.03.582

来源: Elsevier

【 摘 要 】

BACKGROUND Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. OBJECTIVES This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. METHODS Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 +/- 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. RESULTS From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 +/- 3.5 years of age. Among subjects with paired testing, the percent predictedmaximum oxygen uptake decreased (69 +/- 14% vs. 61 +/- 16%; p < 0.001; n = 95), ejection fraction decreased (58 +/- 11% vs. 55 +/- 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associatedwith poorer exercise performance (R-2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. CONCLUSIONS Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; (C) 2017 by the American College of Cardiology Foundation.

【 授权许可】

Free