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cardio.achd-coarctation.chronic.v1

Adult aortic coarctation (native + repaired, chronic ACHD)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

Adult aortic coarctation — intervention at peak-to-peak ≥20 mmHg (or significant narrowing + HTN/LVH); lifelong aggressive HTN control even after successful repair (residual HTN drives premature CV death); repair-site/ascending-aorta + one-time intracranial-aneurysm surveillance; BAV/aortopathy co-management. Manifest points at existing sibling cardio.valvular_disease.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (intervention + lifelong-HTN axis + workups + calculators + panels), test_files, 3-PMID evidence object (high-confidence guideline citations only), chronic phases all present. Antihypertensive RxCUIs RxNav-validated 2026-05-16 (losartan 52175, metoprolol 855332, amlodipine 17767, chlorthalidone 2409, labetalol 202693); stent/surgery/screening non_pharm; SNOMED deferred. 9 trigger/special-pop branches: significant-coarctation intervention, residual-HTN lifelong, repair-site aneurysm, BAV-aortopathy, intracranial-aneurysm, re-coarctation, pregnancy, premature-CV-prevention, CKD.

Entry points (5)

  • vital_abnormality
    Upper-extremity HTN with arm-leg BP gradient / radio-femoral delay
    upper_limb_htn_arm_leg_gradient
  • imaging
    Aortic narrowing on echo/CTA/MRA (native or re-coarctation)
    aortic_narrowing_imaging
  • history
    Repaired coarctation — surveillance visit
    repaired_coarctation_surveillance
  • imaging
    Repair-site aneurysm / pseudoaneurysm on imaging
    repair_site_aneurysm
  • history
    Associated bicuspid aortic valve / ascending aortopathy
    bicuspid_aortic_valve

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Adult intervention modality (stent) + lifelong surveillance
  • native_vs_repairedrequired
    history • used at FRAME
    Native vs repaired (re-coarctation/aneurysm) — different surveillance + intervention
  • arm_leg_bp_gradientrequired
    vital • used at INITIAL_WORKUP
    Resting gradient + exercise HTN — hemodynamic significance
  • peak_to_peak_gradientrequired
    imaging • used at RISK_STRATIFICATION
    Peak-to-peak ≥20 mmHg = intervention threshold
  • aortic_anatomyrequired
    imaging • used at BRANCHING_WORKUP
    Discrete vs long-segment + repair-site aneurysm — modality (stent vs surgery)
  • resting_and_exercise_bprequired
    vital • used at TREATMENT
    Residual/exercise HTN — drives lifelong aggressive control
  • bav_ascending_aorta
    history • used at CONTEXT
    BAV (50–85%) + ascending aortopathy co-management
  • intracranial_aneurysm_screen
    imaging • used at BRANCHING_WORKUP
    One-time intracranial MRA berry-aneurysm screening
  • nyha_class
    symptom • used at RISK_STRATIFICATION
    Symptomatic significance + LV burden
  • creatininerequired
    lab • used at TREATMENT
    Contrast for CTA/MRA; antihypertensive dosing
  • pregnancy_status
    demographic • used at CONTEXT
    mWHO risk; dissection/HTN risk; avoid ACEi/ARB → labetalol

12-phase flow (12)

  1. 1FRAME
    Native vs repaired coarctation; BAV/ascending-aorta involvement
    inputs: native_vs_repaired
    advance: coarctation context framed
  2. 2ENTRY
    Upper-limb HTN/gradient, aortic narrowing, repaired surveillance, repair-site aneurysm, BAV
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Repair type/era, BAV, aneurysm history, pregnancy
    inputs: bav_ascending_aorta, pregnancy_status
    advance: context complete
  4. 4RED_FLAGS
    Aortic dissection, repair-site aneurysm rupture/expansion, malignant HTN
    inputs: repair_site_aneurysm
    actions: cardiogenic_shock
    advance: no red flags or routed to emergency pathway
  5. 5INITIAL_WORKUP
    4-limb BP, echo (gradient, LVH, BAV), ECG (LVH)
    inputs: arm_leg_bp_gradient
    actions: panel.cardiac
    advance: gradient + LV burden documented
  6. 6BRANCHING_WORKUP
    CTA/MRA aorta (coarctation + repair site + ascending aorta), exercise BP, one-time intracranial MRA screen
    inputs: aortic_anatomy, peak_to_peak_gradient, intracranial_aneurysm_screen
    actions: preop_cardiac
    advance: anatomy + gradient + cerebral screen resolved
  7. 7DIFFERENTIAL
    Significant vs non-significant; native vs re-coarctation vs repair-site pseudoaneurysm
    inputs: peak_to_peak_gradient, aortic_anatomy
    advance: significance + lesion type assigned
  8. 8RISK_STRATIFICATION
    Peak-to-peak ≥20 mmHg, OR <20 with significant anatomic narrowing + HTN/LVH/collaterals → intervention
    inputs: peak_to_peak_gradient, nyha_class
    advance: intervention decision assigned
  9. 9TREATMENT
    Stent (adult discrete) or surgical (long-segment/aneurysm) intervention; lifelong aggressive HTN control; BAV/ascending-aorta surveillance; intracranial aneurysm management
    inputs: resting_and_exercise_bp, creatinine, aortic_anatomy
    advance: intervention + HTN-control + surveillance plan documented
  10. 10DISPOSITION
    ACHD centre + structural/aortic referral
    inputs: nyha_class
    actions: preop_cardiac
    advance: referral plan set
  11. 11MONITORING
    BP (incl. exercise), repair-site + ascending-aorta CTA/MRA surveillance
    inputs: resting_and_exercise_bp, aortic_anatomy
    actions: panel.cardiac
    advance: surveillance cadence documented
  12. 12FOLLOWUP
    Lifelong ACHD; pregnancy + transition planning; CV-prevention (premature CAD/stroke)
    inputs: pregnancy_status
    advance: lifelong ACHD + prevention plan documented