嗜铬细胞瘤相关心脏受累:一项119例嗜铬细胞瘤患者的回顾性研究

Cardiac Involvement in Patients with Pheochromocytoma and Paraganglioma:A Retrospective Study of 119 Cases

目的通过回顾性队列探究嗜铬细胞瘤患者出现心脏受累的发生率和表现形式。方法连续入选2018年01月—2020年12月行外科手术且病理诊断的嗜铬细胞瘤或副神经节瘤患者,描述心脏受累的发生率及表现形式,比较心脏受累组和未受累组在基线资料、辅助检查和病理结果上的差别。结果研究期间内有119例患者诊断为嗜铬细胞瘤或副神经节瘤,男性占53.8%,中位年龄为49岁。共33例(27.7%)患者出现了心脏受累,其中14例(11.8%)出现了症状性心脏受累,包括11例(9.2%)应激性心肌病(4例为反复应激性心肌病),2例(1.7%)心力衰竭,1例(0.8%)儿茶酚胺性心肌病,剩余19例(16.0%)患者出现了高敏肌钙蛋白T或NT-proBNP增高或心超异常。和未受累组比较,心脏受累组的胸闷(48.5%比4.7%,P<0.001)、典型三联征(27.3%比11.6%,P=0.037)发生率高,且肿瘤更大(6.4 cm比4.3 cm,P=0.001),出血或坏死的病理表现更多(40.6%比14.6%,P=0.003)。结论约1/4的嗜铬细胞瘤患者出现心脏受累,每9例中就会有1例症状性心脏受累,其中以应激性心肌病多见,提示在临床工作中,对于无诱因的特别是反复发病的应激性心肌病患者需要警惕嗜铬细胞瘤的可能性。

Objective To explore the incidence and manifestations of cardiac involvement in a large retrospective cohort of patients with pheochromocytomas or paragangliomas(PPGLs).Methods Clinical data of consecutive patients diagnosed with PPGLs by histopathology and admitted between January 2018 and December 2020(n=119)were documented.The incidence and manifestations of cardiac involvement in the included patients were investigated.Moreover,comparisons were conducted between patients with and without cardiac involvement.Results 119 patients were identified with PPGLs in the study period,of whom 53.8%were male and median age was 49 years old.33 patients(27.7%)fulfilled the criteria for cardiac involvement,of whom 14(11.8%)cases presented with symptomatic involvement,including 11(9.2%)with Takotsubo syndrome(4 cases with recurrent episodes),2(1.7%)with heart failure,and finally 1(0.8%)with catecholamine-induced cardiomyopathy.Among 33 patients with cardiac involvement,19(16.0%)cases were identified with subclinical myocardial injuries.Compared with those without cardiac involvement(n=86),patients with cardiac involvement were more likely to show dyspnea(48.5%vs 4.7%,P<0.001),classic triad(27.3%vs 11.6%,P=0.037),hemorrhage/necrosis(40.6%vs 14.6%,P=0.003)on histology,and had larger maximal tumor diameter(6.4 cm vs 4.3 cm,P=0.001).Conclusion About one quarter of PPGL patients displayed cardiac involvement,and one in every nine showed symptomatic cardiac involvement,in whom the cases with Takotsubo syndrome were dominant.The diagnosis of PPGLs should be considered in patients displaying Takotsubo syndrome without precipitating factors,especially in those experiencing recurrent episodes.