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. Author manuscript; available in PMC: 2020 Sep 10.
Published in final edited form as: Heart. 2020 May 22;106(16):1202–1210. doi: 10.1136/heartjnl-2020-316540

Table 1.

Biochemical phenotypes, associated clinical presentations and therapeutic options of paragangliomas

Biochemical phenotype Typical signs and symptoms Therapeutic options
Noradrenergic (norepinephrine/normetanephrine) Sustained hypertension, constipation, diaphoresis, headache, nervousness/anxiety, nausea/vomiting, paleness, organ ischaemia
  1. α-adrenoceptor blockage followed by 3-adrenoceptor blockage.
    • Long-acting non-competitive α-adrenoceptor blockers*†
    • Phenoxybenzamine
    • -Short-acting a-adrenoceptor blockers *‡
    • Prazosin, doxazosin, terazosin
    • Cardioselective β-adrenoceptor blockers
    • Atenolol, metoprolol, bisoprolol, nebivolol

  2. Calcium channel blockers, especially in slightly hypertensive or normotensive patients or as an add-on therapy for optimal blood pressure control.

  3. Metyrosine (catecholamine synthesis inhibitor) typically used as an add-on drug, especially in patients with high catecholamine levels usually associated with extensive tumour burden such as metastatic disease.

  4. Ivabradine (If current inhibitor) in severe and refractory catecholamine-induced tachycardia.
Adrenergic (epinephrine/metanephrine) Episodic hypertension, episodic palpitations/tachycardia; headache, nervousness/anxiety, hyperglycaemic, hyperlipidaemia, anxiety, diaphoresis, rarely flushing episodes
Dopaminergic Asymptomatic, hypotension, diarrhoea (only if dopamine levels are very high) Patients presenting with hypotension should have adequate volume repletion including 1–2 L of 0.9% normal saline on the day before surgery (to prevent postsurgical hypotension).
Biochemically silent (not producing any catecholamines) Asymptomatic/non-specific symptoms None.
*

Mild orthostatic hypotension is the most common side effect, which can be minimised by starting the medications at a low dose at night and titrate to blood pressure as a patient tolerates.

†

Potential risk of causing postoperative hypotension.

‡

Typically used in normotensive and borderline hypertension patients and on the day of the surgical resection or the night before surgery (long-acting α-adrenoceptor blockers are not typically used on the day of surgery to avoid postoperative hypotension).