James T N, Kamb M L, Sandberg G A, Silver R M, Kilbourne E M
University of Texas Medical Branch, Galveston.
Ann Intern Med. 1991 Jul 15;115(2):102-10. doi: 10.7326/0003-4819-115-2-102.
To examine the hearts of individuals who died from the eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan, with particular attention paid to the coronary arteries, the neural structures, and the conduction system of the heart because of reported terminal disturbances of cardiac rhythm and conduction.
Three hearts fixed in neutral formalin and well preserved with all the relevant areas of conduction system intact.
Light microscopic examination of subserial sections of the sinus node, atrioventricular node and His bundle, coronary chemoreceptor and regional nerves, ganglia, and small coronary arteries. Routine stains used were Goldner trichrome and Verhoeff-van Gieson.
Arterial abnormalities were numerous and primarily of two types: focal fibromuscular dysplasia causing moderate to severe narrowing, as well as endarteritis and panarteritis. Extensive examples of neuritis and ganglionitis were present throughout the heart, including the conduction system, where arterial abnormalities were also abundant. In the coronary chemoreceptor there were both old and new lesions comprising focal inflammation with degeneration as well as older areas of fibrotic destruction. Within the sinus node, areas of dense fibrosis replaced all nodal tissue. These abnormalities were similar in nature and extent in all three hearts.
The pathologic lesions present in the coronary arteries, neural structures, and conduction system of the heart in patients who died from the eosinophilia-myalgia syndrome provide a suitable anatomic substrate for substantial cardiac electrical instability, including the occurrence of sudden death. In cases of unexplained cardiac electrical instability or sudden unexpected death an inquiry should be made about previous use of L-tryptophan. In patients with the eosinophilia-myalgia syndrome, the possibility of cardiac electrical instability should be considered as part of long-range clinical management.
研究因摄入L-色氨酸导致嗜酸性粒细胞增多性肌痛综合征而死亡者的心脏，特别关注冠状动脉、神经结构和心脏传导系统，因为有报道称其存在终末期心律失常和传导紊乱。
三颗用中性福尔马林固定且保存完好的心脏，所有相关传导系统区域均完整无损。
对窦房结、房室结、希氏束、冠状动脉化学感受器以及局部神经、神经节和小冠状动脉的亚连续切片进行光学显微镜检查。使用的常规染色方法为戈德纳三色染色法和韦尔霍夫-范吉森染色法。
动脉异常众多，主要有两种类型：局灶性纤维肌发育不良导致中度至重度狭窄，以及动脉内膜炎和全动脉炎。整个心脏，包括传导系统，均存在广泛的神经炎和神经节炎实例，而传导系统中动脉异常也很常见。冠状动脉化学感受器既有新旧病变，包括局灶性炎症伴变性以及较陈旧的纤维化破坏区域。在窦房结内，致密纤维化区域取代了所有结组织。所有三颗心脏的这些异常在性质和程度上均相似。
死于嗜酸性粒细胞增多性肌痛综合征患者的冠状动脉、神经结构和心脏传导系统中存在的病理病变为严重的心脏电不稳定，包括猝死的发生，提供了合适的解剖学基础。对于不明原因的心脏电不稳定或意外猝死病例，应询问其既往是否使用过L-色氨酸。对于嗜酸性粒细胞增多性肌痛综合征患者，应将心脏电不稳定的可能性视为长期临床管理的一部分。
