Abstract
F. Iqbal: None. A. Altaf: None. A. Kassai: None. Introduction: Cushing syndrome (CS), characterized by endogenous hypercortisolism is associated with increased risk of cardiometabolic disease including myocardial infarction, stroke, hypertension, hyperlipidaemia, obesity and diabetes. According to one study, those with active CS have a 7-fold increased mortality rate with the main causes of death being infections, cardiovascular disease, and pulmonary embolism. We present a case of CS presenting with hypertensive crisis and DeBakey Type 1 aortic dissection. Case: 51-year-old female presented with sudden onset chest pain radiating to back and jaw followed by syncope. At presentation, blood pressure was 145/60 mm of Hg in the right arm and 90/40 mm of Hg in left arm. CT angiogram showed an aneurysmal ascending aorta, measuring 5.0 cm with a dissection extending from the aortic root to just proximal to the renal arteries (DeBakey Type 1). She underwent ascending aortic replacement with a 20 mm straight tube graft and aortic valve resuspension. She represented 8 weeks later with severe back and left shoulder pain. Admission vitals notable for BP 188/98 mm of Hg. CT-angiogram did not show acute lesions or pseudoaneurysm but was notable for a lipid poor (24 HU) 1.7 cm left adrenal mass. Assessment by Endocrinology team revealed acanthosis nigricans and central obesity. She reported being diagnosed with hypertension at 49 years of age and pre-diabetes several years prior. Her BP control in the preceding two years had been sub-optimal in spite of using multiple agents. Cortisol was unsuppressed at 27.7 mcg/dL following 1 mg-overnight dexamethasone suppression test. ACTH (non-paired) was 9 pg/mL and DHEAS 14 mcg/dL (Females: 19-237 mcg/dL). Three mid-night salivary cortisol’s were elevated at 1.28 mcg/dL, 0.42 mcg/dL and 0.49 mcg/dL (Range: 10Pm-1 AM: < or = 0.09 mcg/dL). Two 24-hour urine free cortisol collections were elevated at 243 mcg/24hr and 157.8 mcg/24 hr respectively (range: 4.0-50.0 mcg/24 hr). She underwent laparoscopic left adrenalectomy and histopathology confirmed adrenocortical adenoma. On recent assessment, over 1 year since left adrenalectomy, her BP is well controlled on irbesartan and carvedilol, and HbA1C improved from 6.7% to 5.7%. Conclusion: While the association of CS with cardiovascular disease is well-established, the incidence of aortic dissection remains rare, and that of DeBakey Type 1 is even rarer. Our literature search revealed only a handful of case reports with the majority being Type B Stanford aortic dissections. Mechanisms which lead to dissecting aortic aneurysm in patients with CS remain unclear although severity of hypertension is a key factor. The successful clinical outcome of our case with extensive, potentially life-threatening dissection highlights the importance of timely intervention and recognition of CS as a risk factor. Sunday, June 2, 2024