Progression to accelerated hypertension in untreated primary aldosteronism

Abstract

Primary hyperaldosteronism accounts for less than 0.5% of cases of hypertension' and accelerated hypertension was a rare but recognised feature of the syndrome in early series. We report a case of untreated Conn's syndrome with accelerated hypertension leading to hypertensive cardiac failure and renal impairment.A 30-year-old Samoan man presented with a one year history of poorly controlled hypertension. Treatment with bendrofluazide, enalapril, and diltiazem had been unsuccessful; additionally he admitted to non-compliance. Blood pressure (BP) was 180/120 mmHg; fundoscopy showed arterio-venous nipping and silver wiring. Serum potassium was 3.0 mmol/L, and creatinine 0.10 mmol/L. Electrocardiogram showed left ventricular ( L v hypertrophy and chest X-ray showed cardiothoracic ratio of 0.49. Echocardiogram demonstrated increased LV size (end diastolic diameter 6.0 cm) and mass (262 gm), with normal LV function (fractional shortening 38%).Investigations excluded hypercortisolism, phaeochromocytoma, and renovascular disease. A random aldosterone: renin ratio was elevated at 223, with ambulant aldosterone 969 pmol/L (reference range[RR] 111-860 pmol/L) and renin 9 mU/L (RR 5-75 mUL) consistent with primary aldosteronism. A saline suppression test confirmed autonomous aldosterone secretion with basal recumbent level of 21 13 pmolL (RR 44-444 p m o l ) falling to only 899 pmol/L (RR <200 pmolL) after 2 litre of normal saline. Abdominal C T scan showed a 1.5X 1.5X2 cm low attenuation lesion in the left adrenal.Spironolactone 200 mg/day and verapamil 240 mg/ day were started and surgery recommended. However, the patient discharged himself from hospital and, despite repeated efforts, did not attend follow up.Twenty-one months later he presented, still untreated, with a two week history of dyspnoea and leg swelling. On examination BP was 260/140 mmHg and the jugular venous pressure was elevated to 15 cm with prominent V waves. The apex beat was displaced laterally and a summation gallop was audible. Marked peripheral oedema was present. Bi-basal chest dullness, inspiratory crackles and pulsatile hepatomegaly were present. Fundoscopy revealed arteriolar narrowing and hard exudates. Serum potassium was 2.9 mmol/L and creatinine 0.17 mmol/L. Electrocardiogram showed marked LV hypertrophy with a strain pattern. Chest Xray showed marked cardiomegaly (CTR 0.61) and pulmonary oedema. Initial management included nitrate infusion, spironolactone, felodipine, and frusemide. Echocardiography showed 4-chamber enlargement with severe concentric LV hypertrophy and dilatation, and a fractional shortening of 10%.Over the next six days he diuresed 13 kg. Serum potassium normalised with oral potassium chloride replacement and serum creatinine improved to 0.14 mmoVL. LV function had improved with fractional shortening of 17%. A computerised tomography scan confirmed the left adrenal lesion, unchanged in size. The patient agreed to surgery, and an uncomplicated laparoscopic left adrenalectomy was performed 15 days after admission; histology o...