Case Presentation: A 28-year-old man, recently experiencing homelessness, presented to the ER of an urban medical center with complaints of severe chest pain, shortness of breath, and lower extremity edema. For the past 5 days he did not take his outpatient medications including isosorbide, doxazosin, hydralazine, nifedipine, carvedilol, and furosemide prescribed by his nephrologist, who he sees for his advanced CKD stage 4.Vitals on admission: temperature 98.1 F, blood pressure 200/148 mmHg, heart rate 118 bpm, RR 36 rpm, SpO2 96%. He was diagnosed with acute decompensated heart failure, type 2 demand myocardial ischemia, and acute on chronic kidney disease. CTA Chest excluded aortic dissection. Echocardiogram found a large LV thrombus as well as a severely reduced function with an LVEF 25% with severe LV hypertrophy (LVH) and apical akinesis. BP became controlled after IV diuresis and with reintroduction of his home regimen. As part of his workup, plasma metanephrines were found to be elevated, an MRI confirmed a small left upper adrenal pheochromocytoma, and an MIBG (Metaiodobenzylguanidine) nuclear scan ruled out metastatic spread. Surgical intervention was postponed due to need for anticoagulation therapy. The patient was discharged with controlled BP, placement at a shelter, all his medications including a new prescription for warfarin, and appointments with primary care, endocrinology, surgery, and cardiology.
Discussion: This case demonstrates the long-term disastrous consequences of uncontrolled resistant hypertension (rHTN). He already had been dealing with advanced CKD and now was diagnosed with the classic signs of catecholamine cardiomyopathy with both LVH and akinesis predominantly in the apical segments and had a large LV thrombus. His recent experience of homelessness leading to decreased access to medications only worsened an already catastrophic situation in this young patient. Pheochromocytoma leads to rHTN through excessive catecholamine production. The first step is measuring plasma free metanephrines, which includes normetanephrine and metanephrine. A clonidine-suppression test can then be performed. CT or MRI adrenal imaging is then recommended and an MIBG scanning can be useful to rule out metastatic disease. (1, 2)Diagnosis of pheochromocytoma is frequently delayed, with an average gap of 3 years between initial symptoms to definitive diagnosis. (3) Given the grave consequences of this condition, it would behoove the clinician to rule out pheochromocytoma as part of rHTN workup. This patient also developed hypertrophic cardiomyopathy due to catecholamine released related to tachycardia induced cardiomyopathy, early resection is recommended in guidelines, and this may prevent further progression of cardiomyopathy. (4) Perioperative medical management approach includes starting beta-adrenergic blockers after alpha-adrenergic blockers to avoid hypertensive crises due to unopposed alpha-adrenergic receptors. (2)
Conclusions: Pheochromocytoma should be suspected in rHTN and complications like cardiomyopathy should be ruled out as part of work-up. Medical management before surgery should start with alpha-adrenergic blockers and then beta-adrenergic blockers. Early surgical intervention could have a role in cardiomyopathy evolution.