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(Circulation. 2004;110:2601-2608.)
© 2004 American Heart Association, Inc.

Congenital Heart Disease

Abnormalities of Neurohormonal and Cardiac Autonomic Nervous Activities Relate Poorly to Functional Status in Fontan Patients

Hideo Ohuchi, MD; Hisashi Takasugi, MD; Hiroyuki Ohashi, MD; Osamu Yamada, MD; Ken Watanabe, MD; Toshikatsu Yagihara, MD; Shigeyuki Echigo, MD

From the Departments of Pediatrics (H. Ohuchi, H.T., H. Ohashi, O.Y., K.W., S.E.) and Thoracic Surgery (T.Y.), National Cardiovascular Center, Osaka, Japan.

Correspondence to Hideo Ohuchi (Ouchi), MD, Physiological Imaging Laboratory, Mayo Clinic, Al2-404, 200 1st St SW, Rochester, MN 55905. E-mail ouchi.hideo{at}mayo.edu

Received October 23, 2003; revision received March 9, 2004; accepted March 11, 2004.

Background— Impaired cardiac autonomic nervous activities and increased neurohumoral activities (CANA, NHA) characterize Fontan patients. However, the clinical significance of these changes is not clearly understood. Our purpose was to clarify the clinical significance of the CANA and NHA in stable Fontan patients.

Methods and Results— We divided 22 atriopulmonary connection (APC) and 75 total cavopulmonary connection (TCPC) patients into 4 subgroups according to New York Heart Association (NYHA) class (1.8±0.6) and measured various CANA and NHA indices. All NHA indices were elevated in the symptomatic patients (P<0.001). Natriuretic peptides were higher in the APC than in the TCPC patients, and the hemodynamics showed no correlation with brain natriuretic peptide in the APC patients. Low arterial oxygen saturation and impaired hemodynamics greatly influenced all elevated NHA indices (P<0.01), except for plasma renin activity, in the TCPC patients. Impaired CANA indices did not relate to NYHA class, although surgeries were associated with lower heart rate variability. In addition to poor correlation between NHA and CANA, age and ventricular morphology had no impact on all CANA and NHA indices, except for high norepinephrine in right ventricular Fontan patients.

Conclusions— Although symptomatic Fontan patients exhibit higher NHA, CANA is not related to either NYHA class or NHA. APC itself is responsible for higher natriuretic peptides, and arterial oxygen desaturation has a great impact on elevated NHA in the TCPC patients. These characteristics of the NHA and CANA differ from those of heart failure patients with biventricular physiology.

Key Words: Fontan procedure • heart defects • nervous system, autonomic • hormones • heart failure

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