Endocrine Abstracts

JOINT1177

Background: Pheochromocytomas (PHEO) are rare neuroendocrine tumors that secrete excess catecholamines, leading to severe cardiovascular complications. While their typical symptoms include episodes of hypertension and tachycardia, their association with Takotsubo syndrome (TTS) is less commonly recognized, especially with reverse TTS, which is an even more rare clinical presentation of PHEO. TTS often resolves with supportive care, however when related to catecholamine excess from pheochromocytomas, can lead to recurrent episodes and long-term cardiovascular risks.

Objectives: This case report aims to increase awareness among clinicians regarding the causal association between Takotsubo cardiomyopathy and catecholamine secreting pheochromocytoma.

Case Presentation: A 40-year-old female patient with a history of reverse Takotsubo cardiomyopathy two years ago, currently on ongoing antihypertensive treatment, was admitted to the emergency department due to palpitations, chest pain, headache, and vomiting. Clinical evaluation revealed a hypertensive crisis (BP = 220/110 mmHg), sinus tachycardia (HR = 120 bpm) and ischemic changes on the ECG. Biochemical tests revealed elevated troponin levels, severe hyperglycemia and increased lactate in the blood gases without acidosis. Echocardiography showed hypokinesia of the left ventricle, leading to urgent coronary angiography, which was normal. The patient was admitted to the ICU and treated with a non-selective beta-blocker (labetalol) and intravenous nitrate, leading to significant improvement in the left ventricular echocardiographic findings, suggesting a possible reverse Takotsubo syndrome. Due to the clinical picture, the patient’s young age and medical history, pheochromocytoma was suspected.

Results: A computed tomography (CT) scan revealed a nodular mass in the left adrenal gland (3.1 x 2.7 cm) with heterogeneous contrast uptake. Hormonal screening confirmed elevated plasma catecholamines levels: metanephrine: 330 ng/l (normal <88), free normetanephrine: 1030 ng/l (normal <115), and free 3-methoxytyramine: 37 ng/l (normal <17.4). Staging with whole-body CT and Tecrotyde scan did not show secondary lesions or paragangiomas. The patient was prepared with alpha-blocker (doxasocin) for 15 days and underwent laparoscopic left adrenalectomy. Histopathological diagnosis confirmed pheochromocytoma (size =3 cm, PASS score= 1, Ki67 <1%, PT1NxMx). Genetic testing was negative. Postoperatively, hormonal screening was negative and imaging showed no recurrence. The patient remains asymptomatic without anthihypertensive medication.

Conclusion: The association between pheochromocytomas and Takotsubo syndrome underscores the importance of recognizing catecholamine-induced myocardial dysfunction in patients with acute chest pain and ECG abnormalities. Clinicians should suspect pheochromocytoma in unexplained TTS cases as early diagnosis and treatment are essential to prevent long-term cardiac damage. Further research is needed for optimal management of this potentially life-threatening complication.