OBJECTIVE: Autonomic dysfunction is common in patients with cirrhosis of the liver, but more so in patients with decompensated state, and is associated with increased mortality. We evaluated the presence and extent of autonomic dysfunction in patients with extrahepatic portal venous obstruction (EHPVO) and noncirrhotic portal fibrosis (NCPF), diseases with relatively preserved liver functions. METHODS: Heart rate variability in response to standing, deep breathing, and Valsalva maneuver and blood pressure response to sustained handgrip and standing were studied in 18 patients with EHPVO (13 mol/L, 5 F, mean age 15.2 +/- 6 yr), 12 patients with NCPF (5 mol/L, 7 F, mean age 26.4 +/- 8 yr), 15 patients with cirrhosis (7 mol/L, 8 F, mean age 12.6 +/- 6 yr), and 17 healthy controls (11 mol/L, 6 F, mean age 18.6 +/- 3 yr). Time-domain parameters of heart rate variability on 24-h ambulatory monitoring were assessed in all the patients. RESULTS: Autonomic dysfunction was observed in 67% of EHPVO, 25% of NCPF, and 80% of cirrhotic subjects but none of the healthy controls (p < 0.05). Four of five time-domain heart rate variability indices showed significant abnormalities in patients with EHPVO (p < 0.05) and cirrhosis (p < 0.05), when compared with patients with NCPF and healthy controls. CONCLUSIONS: Autonomic dysfunction is frequently encountered in patients with EHPVO and cirrhosis, and the presence of autonomic dysfunction in patients with noncirrhotic portal hypertension suggests a primary role of portal hypertension per se in the dysfunction.
OBJECTIVE:Autonomic dysfunction is common in patients with cirrhosis of the liver, but more so in patients with decompensated state, and is associated with increased mortality. We evaluated the presence and extent of autonomic dysfunction in patients with extrahepatic portal venous obstruction (EHPVO) and noncirrhotic portal fibrosis (NCPF), diseases with relatively preserved liver functions. METHODS: Heart rate variability in response to standing, deep breathing, and Valsalva maneuver and blood pressure response to sustained handgrip and standing were studied in 18 patients with EHPVO (13 mol/L, 5 F, mean age 15.2 +/- 6 yr), 12 patients with NCPF (5 mol/L, 7 F, mean age 26.4 +/- 8 yr), 15 patients with cirrhosis (7 mol/L, 8 F, mean age 12.6 +/- 6 yr), and 17 healthy controls (11 mol/L, 6 F, mean age 18.6 +/- 3 yr). Time-domain parameters of heart rate variability on 24-h ambulatory monitoring were assessed in all the patients. RESULTS:Autonomic dysfunction was observed in 67% of EHPVO, 25% of NCPF, and 80% of cirrhotic subjects but none of the healthy controls (p < 0.05). Four of five time-domain heart rate variability indices showed significant abnormalities in patients with EHPVO (p < 0.05) and cirrhosis (p < 0.05), when compared with patients with NCPF and healthy controls. CONCLUSIONS:Autonomic dysfunction is frequently encountered in patients with EHPVO and cirrhosis, and the presence of autonomic dysfunction in patients with noncirrhotic portal hypertension suggests a primary role of portal hypertension per se in the dysfunction.