The diagnostic and prognostic value of brain natriuretic peptides in adults with a systemic morphologically right ventricle or Fontan-type circulation

Background In acquired heart disease, brain natriuretic peptide (BNP) and N-Terminal pro-brain natriuretic peptide (NT-proBNP) are increasingly used as diagnostic and prognostic markers. In adult congenital heart disease, the abnormal anatomy and physiology complicate assessment of cardiac function. We studied the clinical correlates of measurement of natriuretic peptides (NP) in adults with a right ventricle in the systemic position or with Fontan-type physiology. Methods A prospective longitudinal study (follow up time 23 ± 13 months, mean ± S.D.) was conducted in a specialised centre on 61 patients (age 26 ± 8 years; NYHA class 1.5 ± 0.6) including Senning/Mustard corrected transposition, congenitally corrected transposition and Fontan/total cavopulmonary connection. Plasma NP concentration was compared with NYHA class, exercise capacity and echocardiographically determined systemic systolic ventricular function. Results Neurohormone concentrations were generally elevated (mean = 290% of upper reference limit) and related to NYHA class (P < 0.001, NYHA I vs. II–IV). No clinically significant relationship to ventricular function or exercise capacity was found however. An NP measurement could not predict the future course of the disease in terms of functional status or ventricular function. Conclusion In contrast to patients with acquired heart disease, measurement of NP seems to have low clinical value in adults with a right ventricle in the systemic position or with Fontan-type physiology.