CLINICAL AND CARDIAC CHARACTERISTICS OF PRIMARY BILATERAL MACRONODULAR ADRENAL HYPERPLASIA.

Background: Cardiovascular disease is the leading cause of death in Cushing's syndrome (CS). Primary bilateral macro - nodular adrenal hyperplasia (PBMAH), is a rare cause of CS that is clinically distinct from the other common types of CS, but cardiac characteristics have been poorly studied. Methods: The clinical data, steroid hormones and echocardiographic variables of 17 patients with PBMAH were collected. Twenty-one CS patients with cortisol-producing adenoma (CPA) were collected as controls. The similarities and differences of clinical and cardiac features between the two groups were compared. Results: In the PBMAH group, the proportion of female was lower (35.30 vs 100.00%), the age was older (55.76 ± 2.42 years vs 39.57 ± 2.72 years), and the course of disease was longer -13.00 (5.00, 21.50) years vs 1.58 (1.00, 5.00) years'. The proportion of PBMAH patients with hypertension was higher than that of CPA patients (100.00% vs 61.90%). Plasma cortisol and 24h urinary free cortisol (24h UFC) were lower in PBMAH patients than that in CPA patients-morning cortisol 19.50 (15.35, 24.48) mg/dL vs 28.30 (22.88, 29.89) mg/dL, 24h UFC 106.20 (65.35, 156.58) vs 506.23 (292.53, 712.18) mg2. The PBMAH group had longer right ventricular diameter (24.06 ± 1.23 mm vs 20.48 ± 0.83 mm), left atrial diameter (39.41 ± 1.15 mm vs 32.86 ± 0.76 mm), and a higher rate of diastolic dysfunction (76.50% vs 38.10%) than the CPA group. Univariate regression analysis showed that combination of hypertension and duration of hypertension had significant effects on left atrial diameter (b=6.383, P=0.001; b = 0.276, P<0.001, respectively) and E/A ratios (b=-0.496, P< 0.001; b=0.022, P<0.001, respectively). Multivariate regression analysis showed that corticosterone level had a significant effect on systolic blood pressure (b=6.712, P=0.025). Conclusion: Comparing to the CPA, ventricular diastolic dysfunction was common in PBMAH patients under relatively lower cortisol level. PBMAH had high corticosterone levels which may play a role in the development of hypertension and further cardiac changes. [ABSTRACT FROM AUTHOR]