Clinical and cardiac characteristics of primary bilateral macronodular adrenal hyperplasia

Abstract

Background: Cardiovascular disease is the leading cause of death in Cushingžs 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) mg. 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.

Keywords

• primary bilateral macronodular adrenal hyperplasia
• pbmah
• cardiac
• echocardiogram
• steroid hormone