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Chronic venous insufficiency prevalent in patients with Fontan physiology and congenital heart disease

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Patients with congenital heart disease and Fontan physiology had a high prevalence of chronic venous insufficiency in what researchers called the first assessment to document this correlation.

The U.S. researchers prospectively evaluated 159 patients (≥18 years) from seven adult congenital heart centers who had previously undergone Fontan procedures. They examined lower extremity chronic venous insufficiency (CVI), which was assigned clinical, etiological, anatomical and pathophysiological classification grades, and compared with age-matched and sex-matched controls.

CVI prevalence was greater in the Fontan population (60%; 95% CI, 52%-68%) than in the healthy controls (32%; 95% CI, 15%-54%). The prevalence of severe CVI (≥4 for all four grades) was also higher in the patients with Fontan (22%; 95% CI, 16%-29%) vs. the controls (0%; 95% CI, 0%-14%). Additionally, multivariate analysis revealed several factors independently linked with severe CVI: increased numbers of catheterizations with groin venous access, deep venous thrombosis and lower extremity itching.

“CVI is common in adult congenital heart patients with Fontan physiology and is often severe,” the researchers wrote. “The occurrence of CVI in other populations of adults with congenital heart disease, as well as the contribution of abnormal peripheral hemodynamic conditions in adult survivors with Fontan palliation to comorbidities, including thromboembolism and HF, and interventions to improve peripheral hemodynamics, requires further exploration.”

Ariane Marelli, MD, of McGill University’s Adult Unit for Congenital Heart Disease in Montreal, wrote in an accompanying editorial about what may differentiate the 22% of Fontan patients who exhibited severe CVI from those who did not.

“Perhaps the answer lies not only in the peripheral Fontan circulation but also in the function of the single ventricle,” she said. “It is remarkable to note that in the [Valente et al] study, the prevalence of CVI was not significantly different in Fontan patients and in those with tetralogy of Fallot repair known for right ventricular dysfunction. … If ventricular dysfunction is the final common pathway for CVI in both patient groups, the investigators may have uncovered an important finding applicable to a wide range of patients with congenital heart disease with right ventricular dysfunction.”

Valente AM. J Am Coll Cardiol. 2010;56:144-150.

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