Pheochromocytoma Is Characterized by Catecholamine-Mediated Myocarditis, Focal and Diffuse Myocardial Fibrosis, and Myocardial Dysfunction

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• 作者：Vanessa M. Ferreira ; SB ; MD ; DPhil^a ; ^{vanessa.ferreira@cardiov.ox.ac.uk" class="auth_mail" title="E-mail the corresponding author} ; Mafalda Marcelino ; MD^b ; Stefan K. Piechnik ; DSc ; PhD ; MScEE^a ; Claudia Marini ; MD^a ; Theodoros D. Karamitsos ; MD ; PhD^a ; Ntobeko A.B. Ntusi ; DPhil^a ; Jane M. Francis ; DCR(R) ; DNM^a ; Matthew D. Robson ; PhD^a ; J. Ranjit Arnold ; MBChB ; DPhil^a ; Radu Mihai ; MD ; PhD^c ; Julia D.J. Thomas ; MBBS ; PhD^d ; Maria Herincs ; MD^d ; Zaki K. Hassan-Smith ; MBBS ; BMedSci ; PhD^e ; ^f ; Andreas Greiser ; PhD^g ; Wiebke Arlt ; MD ; DSc^e ; ^f ; Má ; rta Korbonits ; MD ; PhD^d ; Niki Karavitaki ; MSc ; PhD^b ; ^e ; ^f ; Ashley B. Grossman ; BA ; BSc ; MD^b ; John A.H. Wass ; MD ; MA^b ; Stefan Neubauer ; MD^a
• 关键词：cardiac magnetic resonance ; catecholamine toxicity ; T1 mapping
• 刊名：Journal of the American College of Cardiology
• 出版年：2016
• 出版时间：24 May 2016
• 年：2016
• 卷：67
• 期：20
• 页码：2364-2374
• 全文大小：1927 K

文摘

Pheochromocytoma is associated with catecholamine-induced cardiac toxicity, but the extent and nature of cardiac involvement in clinical cohorts is not well-characterized.

Objectives

This study characterized the cardiac phenotype in patients with pheochromocytoma using cardiac magnetic resonance (CMR).

Methods

A total of 125 subjects were studied, including patients with newly diagnosed pheochromocytoma (n = 29), patients with previously surgically cured pheochromocytoma (n = 31), healthy control subjects (n = 51), and hypertensive control subjects (HTN) (n = 14), using CMR (1.5-T) cine, strain imaging by myocardial tagging, late gadolinium enhancement, and native T₁ mapping (Shortened Modified Look-Locker Inversion recovery [ShMOLLI]).

Results

Patients who were newly diagnosed with pheochromocytoma, compared with healthy and HTN control subjects, had impaired left ventricular (LV) ejection fraction (<56% in 38% of patients), peak systolic circumferential strain (p < 0.05), and diastolic strain rate (p < 0.05). They had higher myocardial T₁ (974 ± 25 ms, as compared with 954 ± 16 ms in healthy and 958 ± 23 ms in HTN subjects; p < 0.05), areas of myocarditis (median 22% LV with T₁ >990 ms, as compared with 1% in healthy and 2% in HTN subjects; p < 0.05), and focal fibrosis (59% had nonischemic late gadolinium enhancement, as compared with 14% in HTN subjects). Post-operatively, impaired LV ejection fraction typically normalized, but systolic and diastolic strain impairment persisted. Focal fibrosis (median 5% LV) and T₁ abnormalities (median 12% LV) remained, the latter of which may suggest some diffuse fibrosis. Previously cured patients demonstrated abnormal diastolic strain rate (p < 0.001), myocardial T₁ (median 12% LV), and small areas of focal fibrosis (median 1% LV). LV mass index was increased in HTN compared with healthy control subjects (p < 0.05), but not in the 2 pheochromocytoma groups.

Conclusions

This first systematic CMR study characterizing the cardiac phenotype in pheochromocytoma showed that cardiac involvement was frequent and, for some variables, persisted after curative surgery. These effects surpass those of hypertensive heart disease alone, supporting a direct role of catecholamine toxicity that may produce subtle but long-lasting myocardial alterations.