12-phase flow (10)

1. 1FRAME
   Primary aldosteronism crisis = autonomous aldosterone secretion → mineralocorticoid receptor activation → Na+/H2O retention + K+ wasting + H+ secretion + RAAS suppression → severe HTN + hypokalemia + metabolic alkalosis. Most common curable secondary HTN (5-10% all HTN; up to 20% resistant HTN per PATHWAY-2). Pharmacology pivot: MRA FIRST (spironolactone or eplerenone) + K replacement + nicardipine acute; AVOID isolated thiazide acutely (worsens hypoK); biochemical workup requires aldosterone:renin ratio (ARR) AFTER K correction + off interfering drugs (ACEi/ARB/MRA × 4 wk; β-blocker × 2 wk acceptable bridge); confirmatory testing (saline suppression / fludrocortisone / captopril challenge / oral salt loading) before subtyping. SUBTYPING with adrenal vein sampling (AVS — gold standard for lateralization APA vs BAH) drives definitive: APA → laparoscopic unilateral adrenalectomy (curative HTN ~50%, biochemical cure ~95%); BAH → lifelong MRA (spironolactone first; eplerenone if gynecomastia). Route to parent engine for shared HTN-emergency arc; this dossier owns aldosterone-specific pharmacology + biochemical dx + AVS pathway + perioperative.
   inputs: sbp, dbp, potassium
   advance: PA phenotype identified (resistant HTN ± hypokalemia ± biochemistry positive)
2. 2ENTRY
   Recognize PA crisis (severe HTN + spontaneous hypokalemia + metabolic alkalosis + family hx OR resistant HTN OR adrenal incidentaloma); rapid biochemical screen (ARR after K correction); arterial line for severe crisis; ECG for hypokalemia changes
   inputs: age, sbp, potassium
   advance: IV access + cardiac monitor + K replacement initiated + ARR pending
3. 3CONTEXT
   HTN duration + resistance + medications + diuretic use (interferes with ARR); K supplements; family hx (FH-I/II/III/IV); early stroke <40 (FH-I glucocorticoid-remediable); OSA (overlap with PA); sodium intake; prior workup
   inputs: age
   advance: context complete
4. 4RED_FLAGS
   Concurrent severe hypokalemia <2.5 with arrhythmia (Wellens-like ECG, U waves, Torsades), stroke (chronic HTN + acute crisis), AKI (chronic HTN renal damage), MI (chronic HTN + LV hypertrophy + demand ischemia), HF/pulmonary edema (LV hypertrophy from chronic aldosterone), aortic dissection (chronic HTN), severe metabolic alkalosis with respiratory depression
   inputs: sbp, potassium
   actions: htn_emergency
   advance: RED flags screened + K replaced to ≥3.5 + MRA started
5. 5INITIAL_WORKUP
   CORRECT K TO ≥4 FIRST (low K falsely suppresses aldosterone); STOP interfering drugs × 4 wk if possible (MRA, ACEi/ARB, K-sparing diuretic); β-blocker bridge OK × 2 wk; doxazosin/verapamil/hydralazine acceptable for BP during washout. Then: AM seated × 30 min plasma aldosterone + plasma renin activity (or direct renin) + ARR; BMP + Mg + glucose + bicarbonate; ECG (hypokalemia U waves, QT, LV hypertrophy criteria); echo for LV mass + diastolic function; 24h urine sodium (must be >100 mEq for valid testing — confirms adequate Na intake); urinalysis
   inputs: plasma_aldosterone, plasma_renin_activity, aldosterone_renin_ratio, potassium, creatinine
   actions: panel.cardiac, panel.renal, panel.hormone
   advance: biochemistry sent (after K correction + drug washout) + crisis stabilized
6. 6BRANCHING_WORKUP
   After ARR positive (ARR >20-30 with aldosterone >15 ng/dL) → CONFIRMATORY TESTING (one of): saline suppression (2 L IV NS over 4 h, aldosterone >10 ng/dL post = positive); fludrocortisone suppression test (0.1 mg q6h × 4 d, aldosterone >6 ng/dL day 4 = positive); captopril challenge (50 mg PO, aldosterone fails to suppress >30% at 90 min = positive); oral salt loading + 24h urine aldosterone (>12 mcg/24h = positive). After confirmatory positive → SUBTYPING: CT adrenals (often equivocal — CT-AVS discordance 30-40%); ADRENAL VEIN SAMPLING (AVS — gold standard for lateralization, performed at high-volume center, ACTH stim improves accuracy, lateralization index >4 = unilateral disease eligible for adrenalectomy); GENETIC TESTING for FH if young/family-hx/early-stroke (KCNJ5 most common APA-associated somatic; FH-I glucocorticoid-remediable testable with dex suppression)
   inputs: confirmatory_test, ct_adrenals_thin_section
   advance: subtype determined (APA vs BAH) + surgical vs medical pathway selected
7. 7TREATMENT
   ACUTE CRISIS: nicardipine 5 mg/h IV titrate (predictable, safe in PA with chronic HTN renal involvement) + spironolactone 25-100 mg PO (or eplerenone 25-50 mg if gynecomastia concern) + aggressive K replacement (KCl IV 10-20 mEq/h via central line if K <3, oral KCl 40-80 mEq for milder); IV magnesium (Mg almost always coexisting low); AVOID isolated thiazide acutely (worsens hypoK); AVOID isolated ACEi/ARB until K stable. DEFINITIVE: APA (lateralized on AVS) → preop K + MRA optimization 2-4 wk → laparoscopic unilateral adrenalectomy (HTN cure ~50%, biochemical cure ~95%, K normalization expected post-op). BAH or non-lateralizing or surgical-decline → LIFELONG MRA: spironolactone 25-200 mg/d (titrate to BP normal + K normal; gynecomastia in 10% at high dose); eplerenone 25-100 mg BID (selective MR antagonist, less gynecomastia, less potent → higher doses); amiloride add-on if MRA insufficient (5-20 mg/d, ENaC blocker — K-sparing without MR antagonism). FH-I glucocorticoid-remediable → dexamethasone 0.5-2 mg/d at bedtime (suppresses ectopic CYP11B1-CYP11B2 chimeric gene). POSTOP: K normalizes within days → discontinue K supplements + MRA + ACEi/ARB; monitor for HYPERKALEMIA from hypoaldosterone state of contralateral suppressed adrenal (resolves over weeks-months); ongoing essential HTN may persist requiring ACEi/CCB.
   inputs: sbp, dbp, potassium, creatinine
   advance: BP at target (<160/100 acute, <130/80 chronic) + K ≥3.5 + MRA established + surgical vs medical pathway initiated
8. 8DISPOSITION
   ICU for severe crisis with arrhythmia, stroke, AKI, or pulmonary edema; step-down for stable on PO MRA; endocrine + endocrine surgery + interventional radiology (for AVS) consults; high-volume PA center referral for AVS + surgery (operator expertise critical for AVS success rate)
   advance: unit assigned + endocrine + IR + surgery consults booked + AVS scheduled
9. 9MONITORING
   Continuous ECG (hypokalemia Torsades risk); q4-6h K, Mg, glucose during acute crisis; daily K once on PO MRA (gynecomastia / hyperK risk); daily BP + weight; postop monitoring for HYPERKALEMIA (contralateral adrenal suppression → temporary hypoaldosterone state with hyperK risk especially with ongoing ACEi/MRA — typically discontinue these post-cure); aldosterone + ARR repeat at 6 wk (cure marker); annual cardiovascular risk reassessment + LVH regression on echo; bone density (chronic K wasting + secondary effects)
   inputs: potassium, creatinine
   actions: panel.cardiac
   advance: BP stable + K stable + postop biochemical cure trajectory documented
10. 10FOLLOWUP
    Postop: K + creatinine + aldosterone + renin at 1 wk + 6 wk; biochemical cure confirmed if aldosterone normalizes + renin recovers; HTN cure ~50% (rest have residual essential HTN — continue CCB/ACEi); BAH-medical: annual K + creatinine + BP; lifelong MRA titration; PA cardiovascular risk persists (LVH regression slow over years post-cure); annual ECG + echo for LVH; bone density q2-3 y; family screening if FH suspected (genetic counseling)
    advance: biochemical cure (APA) or stable medical regimen (BAH) + lifelong endocrine surveillance booked + family screening if syndromic