嗜鉻細胞瘤相關之章魚壺心肌病變：病例報告

李琇碧、江怡德

衛生福利部雙和醫院 泌尿科

Pheochromocytoma related Takotsubo cardiomyopathy: a case report

Siou-bi Li, Yi-Te Chiang 

Department of Urology, Shuang Ho hospital, Taipei Medical University, Taipei, Taiwan

Introduction: 

Pheochromocytoma often arises from adrenal medulla, with a prevalence of less than 0.2% among hypertensive patients. Common symptoms include episodic headache, sweating, and tachycardia. This report discusses a 71-year-old female presenting with suspicious of Takotsubo cardiomyopathy associated with severe systolic heart failure. Further investigation led to a diagnosis of pheochromocytoma, confirmed by histopathology after adrenectomy. 

Case description: 

A 71-year-old female without prior significant medical history presented to emergent department with shortness of breath and chest tightness. On arrival, her blood pressure was 162/121 mmHg and heart rate was 140 bpm. Her respiratory rate was 22 breaths per minute without desaturation. The patient reported no fever, chills, vomiting, or abdominal pain. Physical examination was unremarkable. Laboratory results showed elevated white blood cells (20.23 x 10³/μl), BUN/creatinine (37/1.33 mg/dl), CKMB (35.2 ng/ml), NT-proBNP (5880 pg/ml), troponin I (7341 pg/ml), and CK (320 U/L). Electrocardiography indicated ST elevation in the precordial leads, raising suspicion of acute myocardial infarction. Emergent coronary angiography (CAG) revealed mild stenosis in the mid-left anterior descending (LAD) and right coronary (RCA) arteries (40-50%). Ventriculography and echocardiography revealed severe systolic heart failure (left ventricular ejection fraction 28.8%), along with left ventricular stunning and outflow tract obstruction, suggesting Takotsubo cardiomyopathy. After initial management, due to labile blood pressure (SBP fluctuating from 70mmHg to 240mmHg) despite medication adjustment during hospitalization, endocrine-related laboratory measurement was done, which showed elevation of 24-hour urinary catecholamine (1094 ug/24hr) and vanillylmandelic acid (VMA) (46.88 mg/24hr) levels. Thus adrenal gland related disease was suspected, compatible with the following computed tomography showing a 3.7x4.7x4.3 cm left adrenal gland mass with areas of necrosis. To maintain hemodynamic balance and also for preoperative preparation, the patient received fluid supplement and medication including Doxazosin then Diltiazem for at least 7 days and Bisoprolol for at least 2 days. Left laparoscopic adrenalectomy was done. After operation, histopathology confirmed pheochromocytoma, with tumor invasion extending through the adrenal capsule into periadrenal adipose tissue. The patient was later discharged under stable postoperative status and controlled blood pressure. During follow up, echocardiogram showed improved systolic heart function with normal motion of the left ventricle. 

Discussion: 

Takotsubo cardiomyopathy, more commonly seen in postmenopausal women, may be triggered by a catecholamine surge associated with pheochromocytoma. Although the pathophysiology remains unclear, mechanisms may involve microvascular dysfunction, transient vasospasm, increased ventricular afterload, and catecholamine-induced myocyte injury. Based on current consensus, pheochromocytomas are seen as all malignancy but with 10% risk of metastasis. Most cases are intra-adrenal, with familial cases having higher risk to involve bilateral adrenal glands. Clinical suspicion of pheochromocytoma is warranted in patients with long-standing hypertension and cardiovascular symptoms. Initial measurement includes 24-hour urinary fractionated catecholamines and metanephrines or plasma metanephrines, followed by imaging if biochemical markers are elevated or if clinical suspicion is high. In this case, timely surgical intervention after adequate preoperative preparation resulted in a favorable outcome, highlighting the importance of early diagnosis and intervention. 

Conclusion: 

Pheochromocytoma is clinically significant and can present with cardiovascular manifestations like Takotsubo cardiomyopathy. Comprehensive preoperative management, including alpha and beta blockade and imaging studies, is essential for optimizing outcomes. This case highlights the critical role of vigilance in diagnosing pheochromocytoma in patients with atypical cardiovascular presentations.