Endocrine Abstracts

JOINT1798

Background: Large-volume hemoptysis due to a hypertensive crisis is a rare initial presentation of pheochromocytoma. We report a case of a postpartum patient who developed severe hypoxic respiratory failure necessitating ECMO support.

Clinical Case: A 34-year-old G2P2 woman presented to the emergency department (ED) on postpartum day 1 after an uncomplicated vaginal delivery with a one-day history of nausea, vomiting, headache and chest tightness. She had experienced similar but less severe symptoms following her first delivery three years earlier, which resolved within a few months. She was asymptomatic during her second pregnancy. In the ED, her systolic blood pressure (SBP) reached 200 mmHg. A CT pulmonary angiogram, ordered to assess for pulmonary embolism, incidentally revealed a large 18 cm left adrenal mass, highly suggestive of pheochromocytoma. Upon admission to ICU, she was started on doxazosin and IV phentolamine infusion. A 24-hour urine collection for metanephrines and catecholamines was sent. Labetalol was briefly introduced for persistent tachycardia but was discontinued due to extreme blood pressure fluctuations, with SBP exceeding 200 mmHg followed by profound hypotension. She subsequently developed hemoptysis and pulmonary edema, necessitating intubation. Worsening hypoxia and hypotension led to a pulseless electrical activity (PEA) arrest. Following resuscitation and return of spontaneous circulation (ROSC), VV-ECMO was planned for respiratory failure. However, she developed a narrow-complex SVT, triggering another PEA arrest. After ROSC, she was transitioned to V-A-V ECMO due to concern for catecholamine-induced arrhythmias. Doxazosin was increased to 8 mg BID alongside continued phentolamine infusion. Verapamil was added to manage her heart rate. By day 3, her hemodynamics stabilized, allowing for extubation and ECMO decannulation. Her 24-hour urine studies revealed norepinephrine 10,670 nmol/day, epinephrine 7,216 nmol/day, normetanephrine 3,153 nmol/day, and metanephrine 4,296 nmol/day. The remainder of her hospitalization was uneventful. She was discharged on doxazosin 12 mg BID and propranolol 20 mg QID. Adrenalectomy was deferred until six weeks postpartum to allow uterine involution and vascular stabilization. She underwent an uncomplicated laparoscopic left adrenalectomy. She returned five days postoperatively with pain, nausea, and vomiting, and imaging revealed a subtotal infarction of the left kidney. Despite this, renal function remained intact, and postoperative urine catecholamines and metanephrines normalized.

Conclusion: Severe hypoxemic respiratory failure requiring ECMO due to hemoptysis and pulmonary edema is a rare presentation of pheochromocytoma. This case highlights a rare pheochromocytoma crisis in which ECMO was required for respiratory failure rather than the more frequently reported cardiogenic shock.