Quigg R J, Om A
Department of Internal Medicine, Medical College of Virginia, Richmond 23298.
J Heart Lung Transplant. 1994 May-Jun;13(3):525-32.
Whenever a patient is evaluated as a possible candidate for heart transplantation, potential causes of reversible cardiomyopathy must always be considered. Although rate, it is well-known that pheochromocytoma can result in a dilated cardiomyopathy, which can be partially or completely reversible. We report a case of a 33-year-old woman with heart failure that was caused by a severe dilated cardiomyopathy who was referred for urgent heart transplant evaluation. The diagnosis of bilateral adrenal pheochromocytomas was made, and within 3 weeks of medical therapy, left ventricular systolic dysfunction completely reversed, avoiding the need for heart transplantation. The patient later underwent successful adrenalectomy. Unique features of this case of pheochromocytoma-induced cardiomyopathy include (1) serial norepinephine measurements over 3 weeks documenting the efficacy of medical therapy, (2) unique cutaneous manifestations that resolved with medical therapy, and (3) familial multiple endocrine neoplasia syndrome with medullary carcinoma of the thyroid in three generations of this patient's family.
每当评估一名患者是否可能成为心脏移植的候选者时，必须始终考虑可逆性心肌病的潜在病因。尽管发生率较低，但众所周知，嗜铬细胞瘤可导致扩张型心肌病，这种心肌病部分或完全可逆。我们报告一例33岁因严重扩张型心肌病导致心力衰竭的女性患者，她被转诊进行紧急心脏移植评估。诊断为双侧肾上腺嗜铬细胞瘤，在药物治疗3周内，左心室收缩功能障碍完全逆转，从而避免了心脏移植的需要。该患者后来成功接受了肾上腺切除术。该例嗜铬细胞瘤诱发心肌病的独特特征包括：（1）连续3周测定去甲肾上腺素，记录了药物治疗的效果；（2）独特的皮肤表现经药物治疗后消退；（3）该患者家族三代患有家族性多发性内分泌肿瘤综合征伴甲状腺髓样癌。
