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Area of Science:

  • Cardiology
  • Endocrinology
  • Oncology

Background:

  • Endocrine diseases, excluding hypothyroidism, hyperthyroidism, primary hyperaldosteronism, and PPGL, rarely cause acquired cardiomyopathy and heart failure.
  • PPGL are rare neuroendocrine tumors secreting catecholamines, with an incidence of 3-8 cases per million annually.
  • Catecholamine-induced cardiomyopathy (CICMP) affects 8-11% of PPGL patients, often presenting with hypertension (95% of PPGL patients, but only 65% with CICMP).

Purpose of the Study:

  • To review the pathophysiology, clinical features, and management of catecholamine-induced cardiomyopathies (CICMPs) in patients with phaeochromocytoma-paraganglioma (PPGL).
  • To highlight the importance of suspecting CICMP in non-ischemic cardiomyopathy and PPGL-associated Takotsubo cardiomyopathy (TCM) in acute coronary syndrome patients with blood pressure variability.

Main Methods:

  • Review of existing literature on PPGL, CICMP, and associated cardiac conditions.
  • Analysis of clinical presentations, diagnostic features, and therapeutic strategies for CICMP subtypes (DCM, HCM, TCM).

Main Results:

  • PPGL patients may develop dilated, hypertrophic, or Takotsubo cardiomyopathy due to endogenous catecholamine excess.
  • CICMPs share features like dramatic presentation, reversibility, ECG changes, mild biomarker elevation, and normal coronary arteries.
  • Classical PPGL symptoms (headache, sweating, palpitations) are infrequent (4%) in CICMP patients.

Conclusions:

  • CICMP should be considered in non-ischemic, non-valvular cardiomyopathy, even without overt catecholamine excess signs.
  • PPGL-associated TCM warrants suspicion in acute coronary syndrome patients with significant blood pressure lability and no obstructive coronary artery disease.