Butt Khurram, Ali Saeed, Sattar Zeeshan, Ur Rahman Asad, Burt Jeremy R
Internal Medicine, Florida Hospital-Orlando, Orlando, USA.
Internal Medicine, Khyber Teaching Hospital, Peshawar, PAK.
Cureus. 2018 Nov 28;10(11):e3646. doi: 10.7759/cureus.3646.
Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium. Different theories have been postulated for myocardial stunning in patients with pheochromocytoma that include microvascular spasm, impaired fatty acid metabolism, increased production of oxygen-derived free radicals and dynamic left ventricular mid-cavity obstruction. QT interval prolongation is seen in 16% to 35% of patients with pheochromocytoma. Takotsubo cardiomyopathy (TS) is now being increasingly identified and it may be responsible for up to 40% of cases of acute catecholamine cardiomyopathy. These manifestations may sometimes precede or cloud the typical triad of a headache, sweating, and tachycardia. We herein present a case of a 42-year-old female with a unique combination of QT prolongation, torsades de pointes, and TS caused by pheochromocytoma in the background of NF1. All these complications are potentially reversible with the removal of the underlying adrenal tumor, underscoring the importance of a high suspicion for pheochromocytoma in patients with NF1.
嗜铬细胞瘤是0.1%的高血压病例的潜在病因。然而,它可能存在于高达5.7%的1型神经纤维瘤病(NF1)患者中。嗜铬细胞瘤固有的儿茶酚胺爆发对心肌的机械和电活动有显著影响。对于嗜铬细胞瘤患者的心肌顿抑,已经提出了不同的理论,包括微血管痉挛、脂肪酸代谢受损、氧自由基产生增加和动态左心室中腔梗阻。16%至35%的嗜铬细胞瘤患者可见QT间期延长。应激性心肌病(TS)现在越来越多地被发现,它可能占急性儿茶酚胺心肌病病例的40%。这些表现有时可能先于典型的头痛、出汗和心动过速三联征出现或使其变得模糊。我们在此报告一例42岁女性,在NF1背景下,由嗜铬细胞瘤引起的QT延长、尖端扭转型室速和TS的独特组合。随着潜在肾上腺肿瘤的切除,所有这些并发症都有可能逆转,这突出了对NF1患者高度怀疑嗜铬细胞瘤的重要性。