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cardio.hypertensive-emergency.pheochromocytoma-crisis.v1PRODUCTION
cardio.hypertensive-emergency.pheochromocytoma-crisis.v1

Pheochromocytoma / paraganglioma crisis (catecholamine-driven episodic HTN crisis)

cardiologyacuteadult
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10/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Pheochromocytoma / paraganglioma crisis = paroxysmal catecholamine surge from chromaffin tumor → alpha-mediated severe HTN + reflex tachy + headache + diaphoresis + pallor + palpitations + glucose intolerance. Pharmacology pivot: ALPHA-BLOCKADE BEFORE β-blockade (NEVER β-blocker monotherapy — unopposed alpha precipitates severe HTN crisis); volume repletion despite HTN (these patients are chronically volume-depleted from alpha venoconstriction). Definitive: surgical resection after 7-14 d preop alpha-blockade. Route to parent engine for shared HTN-emergency arc; this dossier owns the catecholamine-specific pharmacology + biochemical dx + perioperative pathway.

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pheo phenotype identified (clinical tetrad or incidentaloma + biochemical screen positive)

Patient inputs (11)

Localization after positive biochemistry — adrenal mass typical; if negative, look for paraganglioma in sympathetic chain (head/neck, thorax, abdomen, pelvis)

Younger pheo (<40) suggests genetic syndrome (SDHB/SDHD); older incidentaloma more likely sporadic; age drives surgical risk

Paroxysmal pattern (5 min - 1 h episodes precipitated by exertion/anesthesia/abdominal pressure/tyramine) supports diagnosis vs sustained HTN of essential HTN

Orthostatic hypotension despite supine HTN is CLASSIC pheo sign — chronic alpha-mediated venoconstriction + volume depletion

Best initial test — sensitivity ~99% (Lenders JAMA 2002 PMID 12087161); supine sample preferred

Renal function for IV phentolamine + perioperative planning

Catecholamine excess → glucose intolerance; resolves post-op

Defines crisis threshold (typically labile + episodic >220 in crisis); drives titration of phentolamine

Component of MAP; classic sustained DBP >120 in catecholamine excess

Catecholamine excess → tachy + reflex bradycardia (alpha-1 mediated reflex); HR pattern + pheo dx help drug sequencing

24h urine fractionated metanephrines + catecholamines — high specificity (Endocrine Society 2014 PMID 24893135)

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Severity triggers (6)

6 need judgement
  • informationallife_threateningpheo_crisis_with_takotsubo_cardiomyopathy
    Pheo crisis + new severe LV apical ballooning + chest pain + minimal CAD on cath — Takotsubo precipitated by catecholamine surge
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpheo_crisis_after_inadvertent_beta_blocker
    SEVERE paradoxical HTN crisis after β-blocker administered without alpha-blockade — unopposed alpha vasoconstriction
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpheo_crisis_with_catecholamine_cardiomyopathy_and_shock
    Pheo crisis + acute LV failure + cardiogenic shock from prolonged catecholamine exposure → catecholamine cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpheo_crisis_intraoperative_severe_HTN
    Intraoperative severe HTN crisis during pheo resection (despite preop alpha-blockade) — tumor manipulation releases catecholamine surge
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepheo_postoperative_severe_hypotension
    Severe hypotension immediately post-pheo resection — chronic volume depletion + sudden catecholamine withdrawal → vasoplegia
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepheo_metastatic_or_malignant_disease
    Pheo with metastatic disease (bone, lung, liver, lymph nodes) on imaging — malignant pheo (10-17% of cases, higher in SDHB)
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Pheochromocytoma crisis — alpha-blockade FIRST (NEVER β-blocker monotherapy); volume repletion despite HTN; preop alpha-blockade 7-14 d before surgical resection
axis: pheochromocytoma_alpha_first_pharmacology
Selected axis "Pheochromocytoma crisis — alpha-blockade FIRST (NEVER β-blocker monotherapy); volume repletion despite HTN; preop alpha-blockade 7-14 d before surgical resection" by default fallback (first axis)
  • phentolamine
    first line
    alpha_adrenergic_blocker
    5 mg IV bolus q5-15 min titrate (max 15 mg single dose) • IV • PRN bolus or 0.5-1 mg/min infusion
    triggers: pheochromocytoma_crisis, acute_catecholamine_surge
    Endocrine Society 2014 PMID 24893135; Pacak NEJM 2007 PMID 17463337 — pure alpha-blocker fastest reversal of catecholamine HTN; rapid onset (1-2 min) + short half-life (~20 min)
    rxcui 8153
  • phenoxybenzamine
    first line
    noncompetitive_alpha_blocker
    10 mg PO BID, increase by 10-20 mg q2-3 d to BP normal + orthostasis + nasal stuffiness, typical max 80-100 mg/d • PO • BID
    triggers: preoperative_pheo_management
    Pacak NEJM 2007 PMID 17463337 — preop alpha-blockade × 7-14 d; titrate to nasal stuffiness (alpha-1 blockade marker), supine BP normal, mild orthostasis acceptable
    rxcui 8149
  • doxazosin
    second line
    selective_alpha1_blocker
    2 mg PO daily, titrate to 16 mg max • PO • daily
    triggers: preoperative_pheo_management, phenoxybenzamine_intolerant
    Endocrine Society 2014 — selective alpha-1 alternative to phenoxybenzamine; less reflex tachy; less postop hypotension; slightly less complete blockade
    rxcui 49276
  • nicardipine
    second line
    DHP_CCB
    5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/h • IV • continuous
    triggers: pheo_crisis_with_persistent_HTN_after_phentolamine, pheo_crisis_with_renal_impairment
    ACC/AHA 2025 — predictable titration; combine with phentolamine; CCB acceptable in pheo (no unopposed-alpha concern)
    rxcui 7396
  • nitroprusside
    add on
    arterial_venous_vasodilator
    0.25-3 mcg/kg/min IV titrate • IV • continuous
    triggers: refractory_pheo_crisis_short_term
    Acceptable in pheo crisis (no unopposed-alpha concern); short half-life allows tight control; AVOID >24-48h or eGFR <30 (cyanide risk)
    rxcui 7476
  • metoprolol
    comorbidity specific
    beta1_selective_blocker
    ONLY after ≥24-48 h alpha-blockade established; 25 mg PO BID titrate • PO • BID
    triggers: pheo_with_persistent_tachy_or_AF_AFTER_alpha_blockade
    Endocrine Society 2014 — β-blocker ONLY after alpha-blockade established; never alone; for HR control + arrhythmia prophylaxis preop
    rxcui 6918
  • propranolol
    comorbidity specific
    non_selective_beta_blocker
    ONLY after ≥24-48 h alpha-blockade; 10 mg PO TID titrate • PO • TID
    triggers: pheo_with_tachy_after_alpha_blockade
    Historical preferred BB in pheo (after alpha) per Bravo NEJM 1979 PMID 481031; never alone
    rxcui 8787
  • AVOID β-blocker monotherapy
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: pheochromocytoma_diagnosis_or_suspicion
    Endocrine Society 2014 PMID 24893135 — β-blocker without alpha-blockade causes UNOPPOSED ALPHA vasoconstriction → SEVERE paradoxical HTN crisis; absolute contraindication
  • AVOID dopamine, glucagon, metoclopramide, opioids
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: pheochromocytoma_diagnosis_or_suspicion
    These agents trigger catecholamine release from pheo → can precipitate crisis (Endocrine Society 2014 PMID 24893135)
  • IV fluids — high-salt diet preop + crystalloid intraop
    first line
    volume_expansion
    Liberal NaCl PO + IV NS 1-2 L preop • PO + IV • continuous
    triggers: pheochromocytoma_perioperative
    Pacak NEJM 2007 PMID 17463337 — chronic catecholamine venoconstriction = volume-depleted; preop volume expansion prevents postop hypotension after tumor removal

outpatient playbook — drug actions (1)

  1. 1. BP regimen if essential HTN persists
    rxcui 17767
    Amlodipine 5-10 ± lisinopril ± chlorthalidone • PO • daily
    trigger: Persistent HTN
    ACC/AHA 2025

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Episodic HTN crisis + headache + diaphoresis + palpitations + pallor (Lenders Lancet 2005 PMID 16125595); Adrenal incidentaloma on CT/MRI + HTN — biochemical screen for pheo (Endocrine Society 2014 PMID 24893135); Plasma free metanephrines or 24h urine fractionated metanephrines >4× upper limit — strong pheo suggestion (Lenders JAMA 2002 PMID 12087161).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Pheochromocytoma / paraganglioma crisis (catecholamine-driven episodic HTN crisis)** (cardio.hypertensive-emergency.pheochromocytoma-crisis.v1).
Scope: Pheochromocytoma / paraganglioma crisis = paroxysmal catecholamine surge from chromaffin tumor → alpha-mediated severe HTN + reflex tachy + headache + diaphoresis + pallor + palpitations + glucose intolerance. Pharmacology pivot: ALPHA-BLOCKADE BEFORE β-blockade (NEVER β-blocker monotherapy — unopposed alpha precipitates severe HTN crisis); volume repletion despite HTN (these patients are chronically volume-depleted from alpha venoconstriction). Definitive: surgical resection after 7-14 d preop alpha-blockade. Route to parent engine for shared HTN-emergency arc; this dossier owns the catecholamine-specific pharmacology + biochemical dx + perioperative pathway.

No severity triggers fired against current inputs.

Plan

Regimen axis: **Pheochromocytoma crisis — alpha-blockade FIRST (NEVER β-blocker monotherapy); volume repletion despite HTN; preop alpha-blockade 7-14 d before surgical resection**.
1. phentolamine 5 mg IV bolus q5-15 min titrate (max 15 mg single dose) IV PRN bolus or 0.5-1 mg/min infusion (alpha_adrenergic_blocker, first line) — Endocrine Society 2014 PMID 24893135; Pacak NEJM 2007 PMID 17463337 — pure alpha-blocker fastest reversal of catecholamine HTN; rapid onset (1-2 min) + short half-life (~20 min)
2. phenoxybenzamine 10 mg PO BID, increase by 10-20 mg q2-3 d to BP normal + orthostasis + nasal stuffiness, typical max 80-100 mg/d PO BID (noncompetitive_alpha_blocker, first line) — Pacak NEJM 2007 PMID 17463337 — preop alpha-blockade × 7-14 d; titrate to nasal stuffiness (alpha-1 blockade marker), supine BP normal, mild orthostasis acceptable
3. doxazosin 2 mg PO daily, titrate to 16 mg max PO daily (selective_alpha1_blocker, second line) — Endocrine Society 2014 — selective alpha-1 alternative to phenoxybenzamine; less reflex tachy; less postop hypotension; slightly less complete blockade
4. nicardipine 5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/h IV continuous (DHP_CCB, second line) — ACC/AHA 2025 — predictable titration; combine with phentolamine; CCB acceptable in pheo (no unopposed-alpha concern)
5. nitroprusside 0.25-3 mcg/kg/min IV titrate IV continuous (arterial_venous_vasodilator, add on) — Acceptable in pheo crisis (no unopposed-alpha concern); short half-life allows tight control; AVOID >24-48h or eGFR <30 (cyanide risk)
6. metoprolol ONLY after ≥24-48 h alpha-blockade established; 25 mg PO BID titrate PO BID (beta1_selective_blocker, comorbidity specific) — Endocrine Society 2014 — β-blocker ONLY after alpha-blockade established; never alone; for HR control + arrhythmia prophylaxis preop
7. propranolol ONLY after ≥24-48 h alpha-blockade; 10 mg PO TID titrate PO TID (non_selective_beta_blocker, comorbidity specific) — Historical preferred BB in pheo (after alpha) per Bravo NEJM 1979 PMID 481031; never alone
8. AVOID β-blocker monotherapy AVOID N/A N/A (do_not_use, contraindication substitute) — Endocrine Society 2014 PMID 24893135 — β-blocker without alpha-blockade causes UNOPPOSED ALPHA vasoconstriction → SEVERE paradoxical HTN crisis; absolute contraindication
9. AVOID dopamine, glucagon, metoclopramide, opioids AVOID N/A N/A (do_not_use, contraindication substitute) — These agents trigger catecholamine release from pheo → can precipitate crisis (Endocrine Society 2014 PMID 24893135)
10. IV fluids — high-salt diet preop + crystalloid intraop Liberal NaCl PO + IV NS 1-2 L preop PO + IV continuous (volume_expansion, first line) — Pacak NEJM 2007 PMID 17463337 — chronic catecholamine venoconstriction = volume-depleted; preop volume expansion prevents postop hypotension after tumor removal

Setting playbook (outpatient) — Lifelong endocrine surveillance — annual plasma metanephrines (10-15% recurrence/metastasis rate); genetic family screening; cardiac surveillance if catechol cardiomyopathy; second tumor screening if syndromic
11. BP regimen if essential HTN persists Amlodipine 5-10 ± lisinopril ± chlorthalidone PO daily — Persistent HTN (ACC/AHA 2025)

Non-pharmacologic actions:
- Annual endocrine visit
- Genetics counseling for family
- Patient education on recurrence symptoms
- Pre-surgical / pre-anesthesia alert wallet card

AVOID / contraindication checks:
- Beta_blocker_monotherapy_ABSOLUTE_CONTRAINDICATION (Endocrine Society 2014 PMID 24893135) — alpha blockade MUST be established first
- Dopamine_glucagon_metoclopramide_opioids_avoid_in_pheo (Endocrine Society 2014)
- Nitroprusside_avoid_eGFR_below_30_or_>24 48h (cyanide risk)
- Phenoxybenzamine_titrate_to_nasal_stuffiness_orthostasis (Pacak NEJM 2007)
- Volume_repletion_preop_NaCl_diet_plus_IV (chronic volume depletion from alpha venoconstriction)

Monitoring

Regimen monitoring:
- arterial line q5-15min BP during crisis (Endocrine Society 2014)
- continuous ECG (catecholamine arrhythmia + Takotsubo)
- q1h glucose during crisis (catecholamine glycogenolysis)
- q4-6h BMP with K attention (cathol-mediated K shifts)
- orthostatic vitals daily during alpha blockade (titrate to mild orthostasis)
- preop echo (Takotsubo + LV function)
- plasma metanephrines at 4-6 wk postop (confirm cure)
- lifelong annual metanephrines (10-15% recurrence/metastasis rate)

Setting (outpatient) monitoring:
- Annual metanephrines
- Annual BP + glucose
- Annual echo if cardiomyopathy
- Imaging q1-2 y if SDHx

Follow-up plan: Postop catecholamine + metanephrine repeat at 4-6 wk to confirm biochemical cure; lifelong annual surveillance metanephrines (10-15% recurrence/metastasis rate); genetic counseling + family screening if SDHx or syndromic; annual BP + glucose + cardiac assessment if catecholamine cardiomyopathy
- Close-out criterion: postop biochemistry confirms cure + lifelong surveillance plan in place + genetic counseling booked

Monitoring phase: A-line + q5-15 min BP during crisis; continuous ECG (catecholamine arrhythmia + ischemia + Takotsubo); q1h glucose (catecholamine glycogenolysis); q4-6h BMP; serial troponin if cardiomyopathy; postop catecholamine repeat at 4-6 wk to confirm cure

Disposition

Current setting: outpatient — Lifelong endocrine surveillance — annual plasma metanephrines (10-15% recurrence/metastasis rate); genetic family screening; cardiac surveillance if catechol cardiomyopathy; second tumor screening if syndromic

Disposition criteria:
- Long-term continuation; cross-link to endocrine surveillance + cardio.htn.core.v1 if persistent essential HTN

Escalation triggers (move to higher acuity):
- Recurrent symptoms → urgent metanephrines + imaging
- New mass on imaging → biopsy contraindicated; biochemical eval first

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Pheo crisis + new severe LV apical ballooning + chest pain + minimal CAD on cath — Takotsubo precipitated by catecholamine surge
- [LIFE_THREATENING] SEVERE paradoxical HTN crisis after β-blocker administered without alpha-blockade — unopposed alpha vasoconstriction
- [LIFE_THREATENING] Pheo crisis + acute LV failure + cardiogenic shock from prolonged catecholamine exposure → catecholamine cardiomyopathy

Citations

- Endocrine Society 2014 Pheochromocytoma & Paraganglioma CPG (Lenders JCEM 2014 PMID 24893135) + Lenders NEJM 2014 review (PMID 25006718) + 2025 ACC/AHA HTN (Whelton) [PMID:24893135](https://pubmed.ncbi.nlm.nih.gov/24893135/)
- Cited evidence (PMID 25006718) [PMID:25006718](https://pubmed.ncbi.nlm.nih.gov/25006718/)
- Cited evidence (PMID 12087161) [PMID:12087161](https://pubmed.ncbi.nlm.nih.gov/12087161/)
- Cited evidence (PMID 16125595) [PMID:16125595](https://pubmed.ncbi.nlm.nih.gov/16125595/)
- Cited evidence (PMID 17463337) [PMID:17463337](https://pubmed.ncbi.nlm.nih.gov/17463337/)

Last reconciled with current guidelines: 2026-05-14.
References
  • Endocrine Society 2014 Pheochromocytoma & Paraganglioma CPG (Lenders JCEM 2014 PMID 24893135) + Lenders NEJM 2014 review (PMID 25006718) + 2025 ACC/AHA HTN (Whelton) — PMID:24893135
  • Cited evidence (PMID 25006718) — PMID:25006718
  • Cited evidence (PMID 12087161) — PMID:12087161
  • Cited evidence (PMID 16125595) — PMID:16125595
  • Cited evidence (PMID 17463337) — PMID:17463337