Contemporary outcomes after the Fontan procedure - A pediatric heart network multicenter study

被引：345

作者：

Anderson, Page A. W. ^{[1
]}

Sleeper, Lynn A. ^{[2
]}

Mahony, Lynn ^{[3
]}

Colan, Steven D. ^{[4
]}

Atz, Andrew M. ^{[5
]}

Breitbart, Roger E. ^{[4
]}

Gersony, Welton M. ^{[6
]}

Gallagher, Dianne ^{[2
]}

Geva, Tal ^{[4
]}

Margossian, Renee ^{[4
]}

McCrindle, Brian W. ^{[7
]}

Paridon, Stephen ^{[8
]}

Schwartz, Marcy ^{[4
]}

Stylianou, Mario ^{[9
]}

Williams, Richard V. ^{[10
]}

Clark, Bernard J., III ^{[8
]}

机构：

[1] Duke Univ, Med Ctr, Durham, NC 27710 USA

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

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

[4] Childrens Hosp, Boston, MA 02115 USA

[5] Med Univ S Carolina, Charleston, SC 29425 USA

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

[7] Hosp Sick Children, Toronto, ON M5G 1X8, Canada

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

[9] NHLBI, Bethesda, MD 20892 USA

[10] Primary Childrens Med Ctr, Salt Lake City, UT 84103 USA

来源：

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY | 2008年 / 52卷 / 02期

关键词：

D O I：

10.1016/j.jacc.2008.01.074

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Objectives We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP). Background The characteristics of contemporary Fontan survivors are not well described. Methods We enrolled 546 children (age 6 to 18 years, mean 11.9 years) and compared them within pre-specified anatomic and procedure subgroups. History and outcome measures were obtained within a 3-month period. Results Predominant ventricular morphology was 49% left ventricular (LV), 34% right ventricular (RV), and 19% mixed. Ejection fraction (EF) was normal for 73% of subjects; diastolic function grade was normal for 28%. Child Health Questionnaire mean summary scores were lower than for control subjects; however, over 80% of subjects were in the normal range. Brain natriuretic peptide concentration ranged from < 4 to 652 pg/ml (median 13 pg/ml). Mean percent predicted peak O-2 consumption was 65% and decreased with age. Ejection fraction and EF Z score were lowest, and semilunar and atrioventricular (AV) valve regurgitation were more prevalent in the RV subgroup. Older age at Fontan was associated with more severe AV valve regurgitation. Most outcomes were not associated with a superior cavopulmonary connection before Fontan. Conclusions Measures of ventricular systolic function and functional health status, although lower on average in the cohort compared with control subjects, were in the majority of subjects within 2 standard deviations of the mean for control subjects. Right ventricular morphology was associated with poorer ventricular and valvular function. Effective strategies to preserve ventricular and valvular function, particularly for patients with RV morphology, are needed.

引用

页码：85 / 98

页数：14

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