經尿道前列腺剜除手術中誘發嗜鉻性細胞瘤相關之章魚壺心肌症

謝宜穎¹、洪健華^1,2

¹台大醫院泌尿部²台灣大學醫學工程研究所

Pheochromocytoma-Related Takusubo Cardiomyopathy during Transurethral Enucleation of Prostate - A Case Report

Yi-Ying Hsieh¹、Jian-Hua Hong^1,3

¹Department of Urology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei,²Taiwan Institute of Biomedical Engineering, National Taiwan University, Taipei, Taiwan.

Abstract

Objective

Pheochromocytoma crisis (PCC) caused by acute catecholamine release from an adrenal pheochromocytoma or extra-adrenal paraganglioma may result in severely hypertensive crisis with multi-organ failure and cardiopulmonary collapse. PCC would be considered one of the differential diagnoses of secondary hypertension but can be difficult to diagnose because of a wide variety of clinical scenarios of catecholamine excess. We describe a case of PCC with an unusual presentation during transurethral enucleation of prostate (TUEP), which resulted in stress cardiomyopathy complicated with cardiogenic shock that resolved with stabilization on intra-aortic balloon pump (IABP), continuous venovenous hemofiltration (CVVH) and adrenalectomy.

Case Description

A 76-year-old man with a history of diabetes mellitus, hypertension, and prostate enlargement under medication control. He received TUEP for acute urinary retention. During operation, sudden onset of severe hypertension with chest tightness developed. Elevated cardiac enzyme was noticed and cardiac echo showed regional wall motion abnormalities as well as reduced ejection fraction (24.3%). IABP and vasopressor support with CVVH were applied for cardiogenic shock. Emergent coronary angiography revealed patent coronary arteries. Bilateral interarm blood pressure difference was also found, so abdominal and pelvis computer tomography was arranged to exclude aortic dissection, which revealed a 3.9-cm right adrenal tumor with wall enhancement. Pheochromocytoma-related takusubo cardiomyopathy was suspected. Normal catecholamine levels but elevated chormogranin A levels were reported. Preoperative preparations with Doxazosin and IV fluid hydration were initiated, and the patient smoothly underwent laparoscopic right adrenalectomy one month later. The pathology confirmed pheochromocytoma, pT1. Postoperatively, rapid clinical improvement was noted and he was weaned off respiratory and CVVH within 10 days and discharged 12 days after adrenalectomy. Follow-up cardiac echo showed improved EF (50.4%) and lab data showed normal level of urine catecholamine and Vanillylmandelic acid (VMA).

Conclusion

This case highlights diagnostic and management challenges associated with patients with PCC presenting with cardiogenic shock. Clinicians should be highly aware of suspicion for this elusive diagnosis and its potentially catastrophic complications. Early discovery of the disease and prompt intervention could lead to a full and quick recovery.