ISH AHA-2 A CASE OF CHRONIC INDOLENT PHEOCHROMOCYTOMA WHICH CAUSED MEDICALLY-CONTROLLED HYPERTENSION BUT TREATMENT-RESISTANT DIABETES MELLITUS


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Abstract

A 47-year-old woman was admitted via emergency department due to dyspnea NYHA Fc II-III aggravated for 2 months after upper respiratory infection. Her height and body weight were 161 cm / 67 kg. Initial vital signs were 110/70 mmHg - 112 BPM - 24/min - 36.5°C. Chest PA showed cardiomegaly and pulmonary congestion (Figure 1). B-natriuretic peptide level was markedly increased (2002 pg/mL, normal range ≤ 100 pg/mL). The echocardiographic examination showed severely dilated LV cavity (61/72 mm) and severe LV systolic dysfunction (EF 28%) with normal left ventricular wall thickness (9/11 mm) (Figure 2). She was initially treated with dobutamine and parenteral diuretics. After hemodynamic stabilization with body weight reduction of 4 kg with heart failure medication with diuretics, ACE inhibitors and beta blocker (Carvedilol 3.125 mg bid), she was discharged. At the time of discharge, her blood pressure was 90/74 mmHg and pulse rate was 107 BPM.After 2 years of follow up, her left ventricular systolic function was completely normalized (24/46/73%). Left ventricular wall thickness showed mild hypertrophy (11/12 mm) but apical segments showed akinesia. (Figure 3 ECG and chest PA, Figure 4 Echocardiography). Her blood pressure was recovered to the normal range of 132/70 mmHg and pulse rate was 72 BPM. Her heart failure medication was carvedilol (6.25 mg twice daily) and losartan (100 mg once daily)After 1 year of follow up (Year 3), her blood pressure increased to hypertensive range (150/100 mmHg) and pulse rate was 84 BPM. Nifedipine GITS 30 mg was added to the heart failure medication. Diabetes mellitus was newly developed. Despite diet control, her blood glucose levels were continuously increased (HbA1C levels 7.2%), therefore, oral hypoglycemic agents were intensified with metformin and sitagliptin. As heart failure signs disappeared and glycemic control was difficult, beta blocker was discontinued and antihypertensive regimen was changed to singly pill combination of valsartan 160 mg / amlodipine 5 mg. During follow up, blood pressure control was marginal (136/84 mmHg) and pulse rate was high (86 BPM), so low dose pure beta-1 antagonist (bisoprolol 2.5 mg once daily) was added. Then her blood pressure and pulse rate remained stable (102/70 – 122/80, 72 – 84 BPM)However, for two years’ follow up (Year 5), her glycemic levels were continuously aggravated (HbA1C levels 9.3%) despite intensive oral glycemic agent treatment (full dose metformin + sitagliptin + sulfonylurea). Blood pressure and pulse rate were continuously increased (152/88 mmHg, 104 BPM). Indapamide SR 1.5 mg once daily was added and beta blocker dose was increased (Carvedilol 25 mg twice daily). Echocardiographic evaluation showed normal left ventricular size and systolic function (25/47 mm, 72%). Left ventricular wall thickness (8/7 mm) and wall motion were normalized (Figure 5 and 6).After 2 year of follow up (Year 8), insulin treatment was started in endocrinology department because of failure of glycemic control with oral hypoglycemic agents (HbA1C 9.4% with full dose metformin + sitagliptin + sulfonylurea + pioglitazone). As blood pressure control was fair (114/80 mmHg, 84 BPM), carvedilol was discontinued due to hyperglycemia. Then blood pressure and pulse rate were increased to 144/86 mmHg -103 BPM.Incidentally, she was examined abdominal CT due to microscopic hematuria (3+) and found 7.0 x 6.9 cm sized lobulating mass in right adrenal gland suggesting pheochromocytoma. 24 hour urine metanephrine (> 20000 pg/mL, normal range 90 – 930 pg/mL) and normetanephrine (16877 pg/mL, normal range 100 – 230 pg/mL) levels were markedly elevated. Plasma renin activity (26.7 ng/mL/hr, normal range 1 - 2.5 ng/mL/hr) and aldosterone level (23.5 ng/dL, normal range 3 - 16.0) were also elevated. MRI showed 7.1 x 6.7 cm mass in right adrenal gland with good enhancement, internal cystic change and suspicious focal hemorrhage, consistent with pheochromocytoma (Figure 7).After sympathetic blockade with alpha agonist (Doxazosin), the mass was removed by unilateral adrenalectomy. Pathologic evaluated showed 6.5 × 6.0 × 5.5 cm of pheochromocytoma with moderate risk of malignancy (Figure 8 - 11).After surgical removal of pheochromocytoma (Year 9), diabetes mellitus was completely disappeared and blood pressure was controlled with single antihypertensive medication (Valsartan 80 mg once daily, 110/67mmHg – 90 BPM).

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