TAKOTSUBO-LIKE CARDIOMYOPATHY IN A LARGE COHORT OF PATIENTS WITH PHEOCHROMOCYTOMA AND PARAGANGLIOMA.

OBJECTIVE: Pheochromocytoma (PHEO) and paraganglioma (PGL) (PPGL) may cause acute Takotsubo-like catecholamine cardiomyopathy (TLC). The objective of this study was to determine the prevalence and clinical presentation of TLC in a large cohort of patients with PPGL.

METHODS: We reviewed retrospectively the records of consecutive patients with PPGL investigated in our center from 1995 to 2016. We collected clinical and paraclinical data of patients that had TLC in this cohort. We performed a literature review of cases of Takotsubo cardiomyopathy related to PPGL described between 1990 and 2015.

RESULTS: Our cohort included 275 patients with PPGL. Acute TLC was found in 4 of 152 (2.6%) patients with secreting PPGL. There was no event recorded in 123 patients with unknown presurgical secretion (n = 51) or nonsecreting PPGL (n = 72). Four patients (44 to 79 years old) fulfilled the criteria for TLC, including 2 PHEO and 2 PGL patients. A precipitating stressor event was identified in 3 cases including surgery (n = 2) and upper respiratory tract infection. In all cases, the diagnosis of PPGL came after the cardiac event and following the investigation of a lesion incidentally found at imaging. Moreover, we identified in the literature 59 cases described in the last 25 years and analyzed this cohort together with our 4 new cases.

CONCLUSION: Acute TLC may be found in up to 3% of patients with secreting PPGL. Considering that the diagnosis of PPGL was performed following incidental finding of radiologic mass, the real prevalence of PPGL in TTC remains to be determined.

ABBREVIATIONS: ECG = electrocardiogram; LVEF = left ventricular ejection fraction; MIBG = metaiodobenzylguanidine; PGL = paraganglioma; PHEO = pheochromocytoma; PPGL = pheochromocytoma and paraganglioma; TLC = Takotsubo-like cardiomyopathy; TTC = Takotsubo cardiomyopathy; ULN = upper limit of normal.