CC Pt is a 48 yo AA male who presents with SOB..

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Presentation transcript:

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 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 Lindau-hemangioblastomas (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, EF45-49%, 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%