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Original Investigation
April 2018

Association of Albuminuria With Major Adverse Outcomes in Adults With Congenital Heart DiseaseResults From the Boston Adult Congenital Heart Biobank

Author Affiliations
  • 1Department of Medicine, Brigham and Women’s Hospital, Boston, Massachusetts
  • 2Department of Cardiology, Boston Children’s Hospital, Boston, Massachusetts
  • 3Department of Cardiology, Nationwide Children’s Hospital, Columbus, Ohio
  • 4Department of Medicine, Ohio State University, Columbus
  • 5Division of Cardiovascular Medicine, Department of Medicine, University of California, San Diego
  • 6Department of Medicine, North Shore Medical Center, Salem, Massachusetts
JAMA Cardiol. 2018;3(4):308-316. doi:10.1001/jamacardio.2018.0125
Key Points

Question  How common is albuminuria, and does its presence predict prognosis in adults with congenital heart disease?

Findings  In this cohort study of 612 adult outpatients with congenital heart disease, albuminuria was present in 106 (17.3%) and was more prevalent among patients with cyanosis, Fontan circulation, systemic right ventricle, worse functional class, or greater disease complexity. Albuminuria was associated with increased risk for adverse outcomes in patients with biventricular circulation but not those with Fontan circulation.

Meaning  Albuminuria is prevalent and may provide an additional tool to identify increased risk in adults with biventricular congenital heart disease.


Importance  Albuminuria is associated with adverse outcomes in diverse groups of patients, but the importance of albuminuria in the emerging population of increasingly complex adults with congenital heart disease (ACHD) remains unknown.

Objective  To assess the prevalence, risk factors, and prognostic implications of albuminuria in ACHD.

Design, Setting, and Participants  This prospective study assessed a cohort of ambulatory patients aged 18 years and older who were examined at an ACHD referral center and enrolled in the Boston ACHD Biobank between May 17, 2012, to August 5, 2016. Albuminuria was defined as an urine albumin-to-creatinine (ACR) ratio of 30 mg/g or more.

Main Outcomes and Measures  Death or nonelective cardiovascular hospitalization, defined as overnight admission for heart failure, arrhythmia, thromboembolic events, cerebral hemorrhage, and/or disease-specific events.

Results  We measured the ACR of 612 adult patients with CHD (mean [SD] age, 38.6 [13.4] years; 308 [50.3%] women). Albuminuria was present in 106 people (17.3%) and was associated with older age (patients with ACR <30 mg/g: mean [SD]: 37.5 [13.2] years; vs patients with ACR ≥30 mg/g: 43.8 [13.1] years; P < .001), presence of diabetes mellitus (ACR <30 mg/g: 13 of 506 [2.6%]; vs ≥30 mg/g: 11 of 106 [10.4%]; P < .001), lower estimated glomerular filtration rate (ACR <30 mg/g: median [interquartile range (IQR)]: 103.3 [90.0-116.4] mL/min/1.73 m2; ACR ≥30 mg/g: 99.1 [78.8-108.7] mL/min/1.73 m2; P = .002), and cyanosis (ACR <30 mg/g: 23 of 506 [5.1%]; vs ACR ≥30 mg/g: 21 of 106 [22.6%]; P < .001). After a mean (SD) follow-up time of 270 (288) days, 17 patients (2.5%) died, while 68 (11.1%) either died or experienced overnight inpatient admission. Albuminuria predicted outcome, with 30 of 106 patients with albuminuria (28.3%) affected vs 38 of 506 patients without albuminuria (7.5%; hazard ratio [HR], 3.0; 95% CI, 1.9-4.9; P < .001). Albuminuria was also associated with increased mortality (11 of 106 [10.4%]; vs 6 of 506 [1.2%] in patients with and without albuminuria, respectively; HR, 6.4; 95% CI, 2.4-17.3; P < .001). Albuminuria was associated with the outcomes only in patients with a biventricular circulation (HR, 4.5; 95% CI, 2.5-8.0) and not those with single-ventricle circulation (HR, 1.0; 95% CI, 0.4-2.8; P = 0.01 compared with biventricular circulation group). Among 133 patients (21.7%) in NYHA functional class 2, albuminuria was strongly associated with death or nonelective hospitalization.

Conclusions and Relevance  Albuminuria is common and is associated with increased risk for adverse outcome in patients with ACHD with biventricular circulation. Albuminuria appears especially useful in stratifying risk in patients categorized as NYHA functional class 2.