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cardio.hypertensive-emergency.pheochromocytoma-crisis.v1

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

cardiologyacuteadultacuteinpatienttransitionoutpatient
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Phase E variant of cardio.hypertensive-emergency.core.v1 — narrowed to pheochromocytoma / paraganglioma catecholamine crisis. Inherits HTN-emergency framework + workup arc from parent; specializes for alpha-FIRST pharmacology (phentolamine IV acute; phenoxybenzamine PO 7-14 d preop; β-blocker ONLY after ≥24-48 h alpha-blockade — NEVER monotherapy per Endocrine Society 2014 PMID 24893135; absolute contraindication). Volume repletion despite HTN (chronic alpha-mediated venoconstriction = volume-depleted; high-salt diet + IV fluids preop per Pacak NEJM 2007 PMID 17463337). Diagnosis: plasma free metanephrines (sens 99%) + 24h urine fractionated metanephrines + CT/MRI localization (Lenders JAMA 2002 PMID 12087161). Definitive: laparoscopic adrenalectomy after preop optimization. Lifelong annual metanephrine surveillance (10-15% recurrence/metastasis rate); genetic counseling if SDHx/MEN2/VHL/NF1 (30-40% genetic). AVOID dopamine, glucagon, metoclopramide, opioids — all catecholamine release triggers. Manifest pointer reuses cardio.hypertensive-emergency.core.v1 manifest. Design-brief pointer reuses parent (pheo-specific differences documented inline). Status INTEGRATED until terminology + RxNav-validated drug codes are reconciled. Authored 2026-05-14 by shard-06-cardio-acute as part of HTN emergency Phase E variant batch (wave 8).

Entry points (4)

  • symptom
    Episodic HTN crisis + headache + diaphoresis + palpitations + pallor (Lenders Lancet 2005 PMID 16125595)
    pheo_clinical_tetrad
  • history
    Adrenal incidentaloma on CT/MRI + HTN — biochemical screen for pheo (Endocrine Society 2014 PMID 24893135)
    adrenal_incidentaloma_with_HTN
  • lab_abnormality
    Plasma free metanephrines or 24h urine fractionated metanephrines >4× upper limit — strong pheo suggestion (Lenders JAMA 2002 PMID 12087161)
    elevated_metanephrines_screen
  • history
    MEN2A/2B, VHL, NF1, SDHx mutation — surveillance HTN crisis (Endocrine Society 2014)
    familial_pheo_syndrome

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Younger pheo (<40) suggests genetic syndrome (SDHB/SDHD); older incidentaloma more likely sporadic; age drives surgical risk
  • sbprequired
    vital • used at RED_FLAGS
    Defines crisis threshold (typically labile + episodic >220 in crisis); drives titration of phentolamine
  • dbprequired
    vital • used at RED_FLAGS
    Component of MAP; classic sustained DBP >120 in catecholamine excess
  • heart_raterequired
    vital • used at RED_FLAGS
    Catecholamine excess → tachy + reflex bradycardia (alpha-1 mediated reflex); HR pattern + pheo dx help drug sequencing
  • orthostatic_vitalsrequired
    vital • used at INITIAL_WORKUP
    Orthostatic hypotension despite supine HTN is CLASSIC pheo sign — chronic alpha-mediated venoconstriction + volume depletion
  • pheo_episode_patternrequired
    symptom • used at CONTEXT
    Paroxysmal pattern (5 min - 1 h episodes precipitated by exertion/anesthesia/abdominal pressure/tyramine) supports diagnosis vs sustained HTN of essential HTN
  • plasma_free_metanephrinesrequired
    lab • used at INITIAL_WORKUP
    Best initial test — sensitivity ~99% (Lenders JAMA 2002 PMID 12087161); supine sample preferred
  • urine_24h_fractionated_metanephrines
    lab • used at INITIAL_WORKUP
    24h urine fractionated metanephrines + catecholamines — high specificity (Endocrine Society 2014 PMID 24893135)
  • ct_or_mri_abdomen_pelvisrequired
    imaging • used at BRANCHING_WORKUP
    Localization after positive biochemistry — adrenal mass typical; if negative, look for paraganglioma in sympathetic chain (head/neck, thorax, abdomen, pelvis)
  • creatininerequired
    lab • used at INITIAL_WORKUP
    Renal function for IV phentolamine + perioperative planning
  • glucoserequired
    lab • used at INITIAL_WORKUP
    Catecholamine excess → glucose intolerance; resolves post-op

12-phase flow (10)

  1. 1FRAME
    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.
    inputs: sbp, dbp, heart_rate, pheo_episode_pattern
    advance: pheo phenotype identified (clinical tetrad or incidentaloma + biochemical screen positive)
  2. 2ENTRY
    Recognize tetrad (paroxysmal HTN + HA + diaphoresis + palpitations + pallor); episodic >220/120 in crisis with normal-low BP between episodes; 5 H rule: hypertension, headache, hyperhidrosis, palpitations (heart), hyperglycemia
    inputs: age, sbp, orthostatic_vitals
    advance: IV access + cardiac monitor + supine sample for metanephrines
  3. 3CONTEXT
    Family hx (MEN2/VHL/NF1/SDHx → 30-40% pheo are genetic per Endocrine Society 2014); precipitants (anesthesia, surgery, abdominal palpation, tyramine, MAOI, metoclopramide, glucagon, opioids); prior episodes
    inputs: age, pheo_episode_pattern
    advance: context complete
  4. 4RED_FLAGS
    Concurrent stroke (catecholamine HTN → ICH/ischemic), MI (catecholamine cardiomyopathy + coronary vasospasm), Takotsubo (pheo is classic precipitant), pulmonary edema (catecholamine cardiomyopathy), aortic dissection, hyperthermia (catecholamine surge); critical AVOID list — β-blocker monotherapy, dopamine, glucagon, metoclopramide, opioids
    inputs: sbp, pheo_episode_pattern
    actions: htn_emergency
    advance: RED flags screened + alpha-blockade started before any β-blocker
  5. 5INITIAL_WORKUP
    Plasma free metanephrines + 24h urine fractionated metanephrines + catecholamines; ECG (LV strain, ischemia, Takotsubo); echo (LV function + Takotsubo screening); BMP + glucose + Mg + troponin; CXR; chromogranin A (pheo marker)
    inputs: plasma_free_metanephrines, creatinine, glucose
    actions: panel.cardiac, panel.renal
    advance: biochemistry sent + crisis stabilized acutely
  6. 6BRANCHING_WORKUP
    After biochemistry positive (>4× ULN) → CT or MRI abd/pelvis for adrenal mass; if negative → MRI neck-chest-pelvis for paraganglioma; functional imaging — 123I-MIBG OR 68Ga-DOTATATE (preferred for SDHx + metastatic) per Endocrine Society 2014; genetic testing if young/multifocal/family hx
    inputs: ct_or_mri_abdomen_pelvis, urine_24h_fractionated_metanephrines
    advance: tumor localized + genetic workup booked
  7. 7TREATMENT
    ACUTE CRISIS: phentolamine 5 mg IV bolus q5 min titrate (pure alpha-blocker, fastest reversal of catecholamine HTN); nicardipine IV alternative; nitroprusside acceptable in crisis (short half-life, titratable); IV fluids (these patients are volume-depleted). After alpha-blockade established → β-blocker only if persistent tachy/AF (atenolol or propranolol AFTER 24-48 h alpha-blockade). PREOPERATIVE: phenoxybenzamine 10 mg PO BID titrate to BP normal + nasal stuffiness (alpha-blockade marker) + orthostasis × 7-14 d (Pacak NEJM 2007 PMID 17463337); high-salt diet + IV fluids preop to expand volume; after alpha-blockade established (≥48 h) → low-dose β-blocker if needed for HR control. Definitive: laparoscopic adrenalectomy (or open if large/extra-adrenal) after preop optimization.
    inputs: sbp, dbp, heart_rate, creatinine
    advance: BP at target with alpha-blockade established + surgical pathway initiated
  8. 8DISPOSITION
    ICU for q5-15 min BP + a-line; endocrine + endocrine surgery consults; preop alpha-blockade outpatient transition once stable; surgical referral to high-volume center
    advance: ICU bed assigned + endocrine + surgery consults booked
  9. 9MONITORING
    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
    inputs: sbp
    actions: panel.cardiac
    advance: BP stable on alpha-blockade + biochemistry trajectory documented
  10. 10FOLLOWUP
    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
    advance: postop biochemistry confirms cure + lifelong surveillance plan in place + genetic counseling booked