CC • Pt is a 48 yo AA male who presents with SOB. HPI • SOB has been progressive over the last couple of weeks. Patient has had orthopnea, no dyspnea on exertion, cough productive of white sputum. Patient denies fever,admits wheezing, denies pedal edema, denies palpitations, denies chest pain or pressure. PMHx • Pt has had 16 year Hx of HTN, refractory to multiple medications including, Ace-I, HCTZ, BB and CCB. • BPH FHx • Diabetes in Father • Father died of MI SHx • Patient has a Hx of cocaine use that ended 2 years ago. • Patient currently smokes 1 ppd ROS • Claims weight loss of 10 lbs over 1 month • Constipation for 2 weeks, only broken miralax, several days ago Medications and Allergies • • • • Clonidine Metoprolol HCTZ Amlodipine • PCN-allergy of rash PE • Afebrile, HR 111, BP 203/139, RR 18, SAO2 99% on RA • A large non-obese AA man AAOX3 • Coughing, speaking complete sentences, in NAD, mild dyspnea on minimal exertion • No visible JVD, no pedal edema, • Taccycardic s1s2, with s4 gallop, no murmur, no rub • Good inspiratory effort with reduced breath sounds on posterior lung fields, no crackles Labs Na-134 K-3.9 Cl-95 CO2-23 BUN-13 Cr-1.1 Glu-103 BNP-4054 WBC-12.4 Hg-11.7 Hct-33.8 Plat-287 Troponin=0.06, CKMB=9.0, CK=522 CT chest- no PE, b/l pleural effusions, L heterogenous mass w/ internal calcification from adrenal gland SHOW IMAGE Differential? • • • • • • • • • Essential Hypertension Renal Cell Carcinoma Cocaine induced HTN Thyrotoxicosis Systolic CHF Diastolic CHF COPD exacerbation Pheochromocytoma Post-renal ARF with fluid overload Pheochromocytoma • Definition: a catecholamine secreting tumor derived from chromaffin cells in adrenal medulla. • Epidemiology: no gender difference, 0.05% of population, incidence at is highest at 30-40 yo. • Associated with MEN II: Medullary carcinoma, 1 Hyperparathroidism, Pheochromocytoma, (RET gene) • Associated with Von Hippel Lindauhemangioblastomas (CNS), retinal angiomas, renal cysts, clear cell renal carcinoma, pancreatic cysts, papillary cystadenoma of epididymus, endolymphatic sac tumors (Von Hippel Lindau gene) Symptoms: hypertension 55%, headache 80%, palpitations 70%, hyperhydrosis 60% Signs of heart failure, high BP, sinus taccycardia s4 gallop, pulmonary edema, MR, LVH w/ lateral PMI. 24 hour collection for metanephrines: 100% sensitive Patient’s: Normetanephrine 20,154 (649) Metanephrine 105 (203) Total Metanephrine 20,259 (739) Plasma [normetanephrine>2.5 pmol/ml] or [metanephrine>1.4 pmol/ml] 100% specific We decided to wait for biopsy, also 100% specific • Scintigraphy w/ 131 I-MIBG (NE analog) targets to adrenergic tissue • Clonidine suppression test- clonidine should no suppress the plasma levels of NE • Treatment requires phenoxybenzamine, B blocker, metyrosine and IV rehydration 14 days prior to surgery • Surgical Resection Cardiac Reflections • Echo 3/2: LV diastolic dysfunction, EF4549%, LV relaxation abnormality. • Techitium 99 M Stress test 3/4: LVEF estimated at 29%, global hypokinesis, and inhomogenous trace uptake, compatible with dilated cardiomyopathy. • Cardiac Catheterization: moderately severe RCA 70%, LV 30%