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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) Study

Anne Marie Valente, MD^_*,,_*, Ami B. Bhatt, MD^_*,, Stephen Cook, MD, Michael G. Earing, MD, Deborah R. Gersony, MD^||, Jamil Aboulhosn, MD^¶, Alexander R. Opotowsky, MD, MPH^#, George Lui, MD^||, Michelle Gurvitz, MD, MS^_**, Dionne Graham, PhD^_*, Susan M. Fernandes, PA-C^_*, Paul Khairy, MD, PhD^_*, Gary Webb, MD^#, Marie Gerhard-Herman, MD, Michael J. Landzberg, MD^_*, AARCC (Alliance for Adult Research in Congenital Cardiology) Investigators

^_* Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts
Division of Cardiology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts
Ohio State University, Columbus, Ohio
Medical College of Wisconsin, Milwaukee, Wisconsin
^|| Columbia University, New York, New York
^¶ University of California, Los Angeles, Los Angeles, California
^# University of Pennsylvania, Philadelphia, Pennsylvania
^_** University of Washington, Seattle, Washington

Manuscript received September 16, 2009; revised manuscript received February 5, 2010, accepted February 15, 2010.

^_* Reprint requests and correspondence: Dr. Anne Marie Valente, Boston Adult Congenital Heart Program, Department of Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, Massachusetts 02115 (Email: anne.valente{at}cardio.chboston.org).

Objectives: The objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.

Background: As the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.

Methods: A total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.

Results: The prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade 4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.

Conclusions: CVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

Key Words: Fontan • venous insufficiency • adult congenital heart disease

Abbreviations and Acronyms   CEAP = clinical, etiological, anatomical, and pathophysiological   CI = confidence interval   CVI = chronic venous insufficiency   TOF = tetralogy of Fallot

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