Endocrine Abstracts

Introduction: Hypertension is a severe medical condition that significantly increases mortality and disability worldwide. Most hypertensive patients are classified with essential hypertension; however, secondary hypertension is present 5–10% of all cases. Aortic stenosis is a significant cause of secondary hypertension in children and adolescents. Middle-aortic syndrome constitutes 0.5%–2% of all cases of aortic stenosis. The stenosis involves the abdominal aorta and/or distal descending thoracic aorta and can include the renal and visceral branches.

Objective: We report a case of middle-aortic syndrome in a young adult with severe hypertension.

Results: The 21-year-old female patient was referred to our tertiary referral endocrine centre with resistant hypertension diagnosed at the age of 18 years. Her blood pressure exceeded normal ranges despite a triple combination of antihypertensive drugs (ACE inhibitor, calcium channel blocker and diuretics).

Physical examination revealed equally elevated blood pressure on both arms (180/100 mmHg) and significantly lower blood pressure values on both legs (150/80 mmHg). According to the 24-h blood pressure monitoring, median blood pressure was 155/80 mmHg (day time median: 137/67 mmHg, maximum systolic blood pressure 220 mmHg; night time median: 164/86 mmHg, maximum systolic blood pressure 175 mmHg). Hormonal investigations were performed after optimising antihypertensive treatment not affecting the renin–angiotensin–aldosterone system. The laboratory investigations confirmed hyperreninemic hyperaldosteronism: supine aldosterone level was 17.2 ng/dl (reference range: 0.7–15.0 ng/dl), plasma renin activity was 4.41 ng/ml per h (reference range: 0.20–2.80 ng/ml per hour). The patient was euthyroid and eucortisolemic. Both kidneys showed normal parenchymal function and ultrasound morphology. Abdominal and thoracic computed tomography–angiography revealed significant stenosis of thoracic aorta at the segment of the X–XI thoracic vertebrae. Percutaneous transluminal balloon angioplasty and stenting was performed with success as her blood pressure normalised after the intervention. Long – term blood pressure control could be achieved with low-dose doxazosin monotherapy.

Conclusion: Our case highlights that secondary hypertension should always be considered in young patients with hypertension. Middle-aortic syndrome is associated with high morbidity and mortality due to the increased risk of cardiovascular disorders. As conservative drug treatment is rarely effective vascular intervention is required to normalize blood pressure and prevent end-organ damage.