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cardio.cardiogenic-shock.acromegaly-cardiomyopathy.v1

Cardiogenic shock — acromegaly-induced cardiomyopathy

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Phase E variant of cardio.cardiogenic-shock.core.v1 — narrowed to acromegaly-induced cardiomyopathy with cardiogenic shock per Endocrine Society 2014 Acromegaly Clinical Practice Guideline (Katznelson PMID 25356808) + Colao 2004 acromegaly cardiomyopathy review. Background: chronic GH/IGF-1 excess (>95% from GH-secreting pituitary adenoma) → biventricular concentric hypertrophy + diastolic dysfunction → eventual systolic dysfunction + arrhythmia + accelerated CAD + valvular disease (especially AR + MR); affects 30-40% of long-standing acromegaly per Colao 2004; cardiogenic shock is rare end-stage manifestation; concurrent OSA in ~70% (anatomical + central) exacerbates shock. Workup: IGF-1 (best screening — age-adjusted, >ULN suggests acromegaly); GH suppression test (oral glucose tolerance — failure to suppress GH <1 ng/mL after 75 g glucose = acromegaly per Endocrine Society 2014 PMID 25356808); pituitary MRI with contrast (locates adenoma; macro vs micro); cardiac MRI for biventricular hypertrophy + LGE pattern (subepicardial fibrosis characteristic); polysomnography for OSA; pituitary axis screen (TSH, ACTH/cortisol, prolactin, LH/FSH — RULE OUT adrenal insufficiency BEFORE any pituitary intervention); glucose/HbA1c (DM common — GH-induced insulin resistance); visual fields if macroadenoma. Treatment ACUTE (shock-focused): standard CS support per cardio.cardiogenic-shock.core.v1 — NE first-line per SOAP-II PMID 20200382, AVOID DOPAMINE (paradoxical somatotroph stimulation worsens GH); cautious volume (LV hypertrophy preload-sensitive); inotropes (milrinone or dobutamine per phenotype); SOMATOSTATIN ANALOG — octreotide IV 50 µg bolus then SC 100-500 µg q8h, OR octreotide LAR 20 mg IM monthly, OR lanreotide 120 mg SC q4 wk depot (reduces GH/IGF-1 within hours-days; may improve cardiac function over weeks per Colao 2004); PEGVISOMANT (GH-receptor antagonist) 10-30 mg SC daily for GH-resistant or intolerant per Trainer 2000 NEJM (normalizes IGF-1 in >90%); cabergoline (dopamine agonist) generally INEFFECTIVE in CS setting (long onset; only suppresses small adenomas); MCS bridge (Impella per DanGer Shock 2024 PMID 38587234, VA-ECMO if biventricular failure); CPAP/BiPAP for concurrent OSA; cath + PCI if ACS pattern (acromegaly accelerates CAD); AVOID β-blockers in active shock (hypertrophic LV depends on atrial kick). Definitive (after stabilization): TRANSSPHENOIDAL PITUITARY SURGERY first-line — remission rate 80-90% for microadenomas, 40-60% for macroadenomas per Endocrine Society 2014; STEREOTACTIC RADIOSURGERY (Gamma Knife) if surgery fails or adenoma residual; long-term somatostatin analog if surgery + radiation incomplete; pegvisomant if GH-resistant. AVOID: DOPAMINE infusion (worsens GH secretion paradoxically); over-volume resuscitation (LV hypertrophy preload-sensitive — small changes cause large pressure shifts); β-blockers in active shock (hypertrophic LV depends on atrial kick + may decompensate with negative inotropy). Outcomes: cardiomyopathy reversibility correlates with disease duration — early treatment may improve LVEF; late-stage fibrosis is irreversible; mortality dominated by cardiovascular disease (~60% per AcroQoL outcomes); cardiac mortality halved when IGF-1 normalized; lifelong cardiology + endocrinology + neurosurgery co-management. Status INTEGRATED until terminology + RxNav-validated drug codes are reconciled. Authored 2026-05-15 by shard-06-cardio-acute as Phase E wave 19 cardiogenic-shock rare-etiology variant.

Entry points (4)

  • symptom
    Cardiogenic shock (SBP <90 + lactate ≥2 + AKI / cool extremities) in patient with known acromegaly OR classic phenotype (frontal bossing, prognathism, enlarged hands/feet, soft-tissue swelling)
    shock_in_patient_with_known_acromegaly_or_phenotype
  • imaging
    Echo with biventricular concentric hypertrophy + LV systolic dysfunction (LVEF <35%) + shock physiology — acromegaly cardiomyopathy end-stage
    echo_biventricular_hypertrophy_with_systolic_dysfunction_and_shock
  • lab_abnormality
    Markedly elevated IGF-1 (age-adjusted >ULN) in shock patient with cardiomyopathy of unclear etiology — acromegaly screen
    elevated_igf1_in_shock_patient_without_known_diagnosis
  • history
    Known acromegaly patient (on or off somatostatin analog) with progressive HF now decompensated to shock — established acromegaly cardiomyopathy
    known_acromegaly_with_progressive_hf_now_decompensated

Required inputs (18)

  • agerequired
    demographic • used at CONTEXT
    IGF-1 reference ranges are age-adjusted; older patients have lower normals; transplant candidacy assessment
  • duration_of_acromegaly_or_phenotype_yearsrequired
    history • used at CONTEXT
    Cardiomyopathy reversibility correlates with disease duration; long-standing (>10 yr) acromegaly more likely to have irreversible fibrosis
  • prior_pituitary_surgery_or_radiation
    history • used at CONTEXT
    Documents prior treatments; informs current GH/IGF-1 status; may have hypopituitarism requiring replacement
  • concurrent_obstructive_sleep_apnearequired
    history • used at CONTEXT
    OSA in ~70% of acromegaly patients (anatomical + central); exacerbates HF and shock physiology; CPAP/BiPAP reduces RV strain
  • sbprequired
    vital • used at RED_FLAGS
    SCAI 2022 staging baseline; SBP <90 with end-organ hypoperfusion = SCAI C+
  • spo2required
    vital • used at RED_FLAGS
    Hypoxemia from pulmonary edema + concurrent OSA; guides NIPPV and CPAP/BiPAP strategy
  • hrrequired
    vital • used at CONTEXT
    Tachycardia + arrhythmia surveillance; AF and VT/VF more common in acromegaly cardiomyopathy
  • lactaterequired
    lab • used at RISK_STRATIFICATION
    SCAI 2022 staging; CardShock prognostication (Harjola PMID 26333869); ≥4 = SCAI D-E pattern
  • creatininerequired
    lab • used at CONTEXT
    Cardiorenal screen; pegvisomant + somatostatin analog dose adjustment if AKI; renal injury common in shock
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Rules out ischemic cardiomyopathy / accelerated CAD (acromegaly accelerates atherosclerosis); high values prompt cath
  • nt_probnprequired
    lab • used at INITIAL_WORKUP
    Markedly elevated in acromegaly cardiomyopathy with shock; trends response to therapy
  • igf1required
    lab • used at INITIAL_WORKUP
    Best screening test for acromegaly — reflects 24-h GH secretion; age-adjusted; elevated >ULN suggests acromegaly per Endocrine Society 2014 (PMID 25356808)
  • pituitary_axis_screenrequired
    lab • used at INITIAL_WORKUP
    TSH, ACTH/cortisol, prolactin, LH/FSH — panhypopituitarism may co-exist; hyponatremia from SIADH or adrenal insufficiency requires immediate replacement before any pituitary intervention
  • glucose_hba1crequired
    lab • used at CONTEXT
    DM common in acromegaly (GH-induced insulin resistance); dose-adjustment for SGLT2i; informs prognosis
  • echo_lvef_and_chamber_sizerequired
    imaging • used at INITIAL_WORKUP
    Biventricular concentric hypertrophy + LV systolic dysfunction + valvular disease (especially AR + MR) is characteristic; rules out other CS etiologies
  • pituitary_mri_with_contrastrequired
    imaging • used at BRANCHING_WORKUP
    Locates adenoma (macro >10 mm vs micro <10 mm); informs surgical approach; visual field assessment if macroadenoma (chiasm compression)
  • cardiac_mri_with_lge
    imaging • used at BRANCHING_WORKUP
    Subepicardial fibrosis pattern is characteristic of acromegaly cardiomyopathy; biventricular hypertrophy + late gadolinium enhancement; informs reversibility prognosis
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    LV hypertrophy voltage criteria; arrhythmia surveillance; rules out ischemia

12-phase flow (11)

  1. 1FRAME
    Acromegaly cardiomyopathy with cardiogenic shock = end-stage manifestation of GH/IGF-1 excess (~30-40% of long-standing acromegaly develops cardiomyopathy; rare progression to shock); biventricular hypertrophy + diastolic + eventual systolic dysfunction + arrhythmia + accelerated CAD + valvular disease pattern; concurrent OSA in ~70%; preload-sensitive LV hypertrophy demands cautious volume management
    inputs: echo_lvef_and_chamber_size, igf1, sbp
    advance: Acromegaly cardiomyopathy + SCAI C+ shock confirmed
  2. 2ENTRY
    CICU activation; advanced HF / MCS team mobilization; STAT endocrinology consult for acromegaly management; sleep medicine consult if OSA undiagnosed
    inputs: age, duration_of_acromegaly_or_phenotype_years
    advance: CICU + endocrinology + advanced HF team engaged
  3. 3CONTEXT
    Acromegaly duration, prior pituitary surgery / radiation, current somatostatin analog or pegvisomant therapy, concurrent OSA, DM, prior cath / known CAD, valvular history, hypopituitarism replacement (cortisol, thyroid, sex hormones)
    inputs: concurrent_obstructive_sleep_apnea, creatinine, glucose_hba1c, hr
    advance: Acromegaly + cardiac context complete
  4. 4RED_FLAGS
    Cardiogenic shock SCAI C+ → vasopressor + inotrope (AVOID dopamine); pituitary apoplexy (acute headache + vision loss + adrenal insufficiency from infarction of adenoma) → emergent neurosurgery + IV hydrocortisone 100 mg; severe OSA exacerbating shock → CPAP/BiPAP; somatostatin-induced bradycardia → reduce dose; refractory ventricular arrhythmia
    inputs: sbp, spo2
    actions: cardiogenic_shock
    advance: Red flags screened including pituitary apoplexy + adrenal insufficiency
  5. 5INITIAL_WORKUP
    Bedside echo (biventricular hypertrophy + LVEF + valvular); ECG (LVH voltage); STAT IGF-1; pituitary axis screen (TSH, ACTH/cortisol, prolactin, LH/FSH — RULE OUT adrenal insufficiency BEFORE any pituitary intervention); BMP; CBC; ABG; lactate; troponin; NT-proBNP; glucose/HbA1c; CXR
    inputs: echo_lvef_and_chamber_size, ecg, troponin, nt_probnp, lactate, igf1, pituitary_axis_screen
    actions: cardiogenic_shock, panel.cardiac, panel.renal, panel.abg
    advance: Workup obtained; SCAI stage assigned; adrenal insufficiency ruled out
  6. 6BRANCHING_WORKUP
    Pituitary MRI with contrast (locates adenoma); cardiac MRI with LGE for fibrosis pattern + reversibility prognosis; coronary angiography or CTA if ACS pattern (accelerated CAD common); polysomnography if OSA undiagnosed; visual fields if macroadenoma; GH suppression test (oral glucose tolerance) if IGF-1 borderline (after stabilization)
    inputs: pituitary_mri_with_contrast
    actions: acs_pathway
    advance: Pituitary lesion characterized + cardiac fibrosis pattern documented + CAD assessed
  7. 7RISK_STRATIFICATION
    SCAI 2022 staging (most acromegaly-CS = Stage C-D); CardShock score; cardiomyopathy reversibility prognosis based on duration + LGE pattern; transplant candidacy assessment if no recovery anticipated; Endocrine Society 2014 disease activity scoring (random GH, IGF-1) for acromegaly severity
    inputs: sbp, lactate
    advance: Risk stratified; transplant + MCS pathway + acromegaly disease activity assessed
  8. 8TREATMENT
    Standard CS support: NE first-line per SOAP-II (PMID 20200382); AVOID DOPAMINE (worsens GH secretion paradoxically); cautious volume (LV hypertrophy preload-sensitive); inotropes — milrinone or dobutamine per phenotype; SOMATOSTATIN ANALOG: octreotide IV 50 µg bolus then SC 100-500 µg q8h, OR octreotide LAR 20 mg IM monthly once stable, OR lanreotide 120 mg SC q4 wk depot — reduces GH/IGF-1 within hours-days, may improve cardiac function over weeks per Colao 2004; PEGVISOMANT (GH-receptor antagonist) 10-30 mg SC daily for GH-resistant or intolerant; cabergoline (dopamine agonist) generally INEFFECTIVE in CS setting (long onset; only suppresses small adenomas); MCS bridge (Impella, VA-ECMO) per DanGer Shock if refractory; CPAP/BiPAP for concurrent OSA; cath + PCI if ACS pattern; AVOID beta-blockers in active shock (hypertrophic LV depends on atrial kick)
    inputs: sbp, creatinine
    actions: protocol.cardiogenic_shock
    advance: CS regimen active + somatostatin analog initiated + dopamine avoided + cautious volume documented
  9. 9DISPOSITION
    CICU mandatory; transfer to advanced HF / MCS-capable + endocrinology / pituitary surgery–capable center if not already there; multidisciplinary team daily rounds (cardiology + endocrinology + neurosurgery + sleep medicine + nutrition)
    advance: CICU at appropriate-level center + MDT mobilized
  10. 10MONITORING
    Continuous telemetry, A-line, central line; lactate q1-2 h; UOP hourly; daily echo for cardiac recovery trajectory; daily IGF-1 trend (responsive in days); daily BMP / NT-proBNP / troponin / glucose; BP cuff in non-affected arm if obvious hand changes; CPAP compliance if OSA
    inputs: lactate, creatinine
    actions: panel.cardiac, panel.renal
    advance: Monitoring cadence + acromegaly biomarker surveillance documented
  11. 11FOLLOWUP
    Endocrinology + cardiology co-management; pituitary surgery evaluation (transsphenoidal first-line) once stable; stereotactic radiosurgery if surgery fails / residual; long-term somatostatin analog or pegvisomant if surgery + radiation incomplete; serial IGF-1 every 3 mo until normalized then every 6-12 mo; serial echo at 3 mo + 6 mo + 12 mo for LVEF recovery; ICD/WCD evaluation if LVEF <35% on full GDMT; advanced HF + transplant if no recovery; OSA management (CPAP); annual cardiac MRI for fibrosis surveillance; visual field testing if macroadenoma
    advance: Multidisciplinary follow-up + pituitary surgery + endocrine + transplant pathway booked