Secreting Metastatic Pheochromocytoma: Next Step When Alpha-Adrenergic Blockade Fails to Meet Blood Pressure and Heart Rate Goals
Clinical Scenario
This protocol addresses patients with hormonally functional secreting metastatic pheochromocytoma or paraganglioma (mPPGL) characterised by high catecholamine production. High catecholamine production is associated with hypertension, diaphoresis, headaches, and palpitations, as well as orthostatic hypotension, hyperglycemia, and anxiety. All such patients are recommended to undergo preoperative or preprocedural blockade before surgery or most systemic therapies to prevent periprocedural cardiovascular complications.
Prior Treatment & Why This Protocol Is Indicated
First-line perioperative management with alpha-adrenergic receptor blockade — titrated over 7 to 14 days before the procedure, with beta-adrenergic blocker added to control tachycardia — did not achieve the required targets: blood pressure below 130/80 mmHg while seated and above 90 mmHg systolic while standing, with heart rate below 90 bpm pre-procedure and 70–80 bpm for long-term control. Failure to reach these goals on first-line blockade is the indication for escalation to this next-line protocol.
Next-Line Approach (Partial — Full Protocol Behind the Link)
When first-line alpha-blockade is insufficient, second-line antihypertensive agents from specific drug classes may be added to bring blood pressure and heart rate within target range. The complete selection, sequencing, and clinical criteria are detailed in the full protocol.
Treatment Goals
Target blood pressure <130/80 mmHg while seated and >90 mmHg systolic while standing; target heart rate <90 bpm before procedures and 70–80 bpm for long-term control.
References
DOI: 10.1097/MPA.0000000000001792
- Given this knowledge, we recommend that all patients with a hormonally functional secreting mPPGL undergo preoperative or preprocedural blockade for 7 to 14 days before surgery/procedure and for most localized and systemic therapies to prevent periprocedural cardiovascular complications.
- High catecholamine production is associated with hypertension, diaphoresis, headaches, and palpitations among other symptoms and signs such as orthostatic hypotension, hyperglycemia, and anxiety.
- Nondihydropyridine calcium channel blockers (eg, amlodipine, nifedipine) are most commonly used as second-line agents (Table 5).
- In addition, angiotensin-converting enzyme (ACE) inhibitors (eg, benazepril, enalapril, lisinopril, ramipril) and angiotensin receptor blockers (ARBs) (eg, candesartan, irbesartan, losartan, olmesartan, valsartan) may help to improve BP in patients with PPGL.
- There is no evidence from randomized controlled trials to determine the optimal target BP, but based on retrospective studies and expert experience, the goal should be a target BP of at least less than 130/80 mm Hg while seated and greater than 90 mm Hg systolic while standing with a target heart rate of less than 90 bpm before procedures and closer to 70-80 bpm, if possible, for long term control.
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