Acute aortic aneurysm + malperfusion in hypertensive emergency (anti-impulse + emergent endovascular/open repair)
Phase E variant of cardio.hypertensive-emergency.core.v1 — narrowed to acute aortic aneurysm + branch malperfusion phenotype (rupture, contained leak, rapid expansion, or branch compromise) in the setting of severe HTN. Inherits HTN-emergency framework from parent; specializes for anti-impulse paradigm (HR <60 FIRST with esmolol/labetalol, THEN SBP 100-120 with nicardipine — ACC/AHA 2022 Class I + permissive hypotension until proximal control). AVOID isolated vasodilator + hydralazine + aggressive volume. Definitive repair: ENDOVASCULAR FIRST for descending thoracic (TEVAR per IRAD + INSTEAD-XL precedent), abdominal infrarenal (EVAR per UK Trial / EVAR-1 / DREAM / OVER), complex visceral (branched/fenestrated EVAR per SVS 2024 + ESVS 2024); OPEN REPAIR for ascending or complex anatomy not amenable to endovascular. Branch revascularization (covered stent for renal, mesenteric within 6h to prevent infarction, femoral-femoral bypass for lower extremity). CSF DRAINAGE protocol if T6-L1 endovascular for spinal cord protection (reduces paraplegia 5-10% to <2%). Massive transfusion protocol (1:1:1 PRBC:FFP:platelets per PROPPR PMID 25647203) if rupture. Lifelong BB + ARB + smoking cessation + statin + lifetime CTA surveillance + family screening. Severity triggers: free rupture with hemodynamic collapse (massive transfusion + permissive hypotension + emergent OR); mesenteric ischemia (revascularization within 6h); spinal cord ischemia post-T6-L1 endovascular (CSF drainage + MAP >85 + steroid pulse); concomitant dissection identified (route to dissection engines in parallel); refractory anti-impulse failure (escalate medical + expedited repair); endoleak with sac growth post-EVAR/TEVAR (re-intervention). Sibling differentiation: vs general HTN-emergency parent (anti-impulse + endovascular/open specialization); vs aortic dissection (different substrate + can coexist); vs aortic-dissection core engine (parallel run if both pathologies); vs vasc.aaa.v1 (acute vs chronic continuum, both run sequentially). Manifest pointer reuses cardio.hypertensive-emergency.core.v1 manifest. Design-brief pointer reuses parent. Status INTEGRATED. Authored 2026-05-15 by shard-06-cardio-acute (Wave 20 recovery batch).
Entry points (4)
- symptomSudden severe back, flank, or abdominal pain in patient with known AAA or thoracic aneurysm + severe HTN — suspect rapid expansion / contained rupture / branch malperfusionsevere_back_or_abdominal_pain_with_known_or_suspected_aneurysm
- imagingCTA showing aortic aneurysm (thoracic >5.5 cm or AAA >5.5 cm or rapidly expanding ≥5 mm/6mo) + intramural hematoma OR contained rupture OR branch malperfusion + severe HTNcta_with_aneurysm_above_threshold_with_intramural_hematoma_or_contained_rupture
- symptomPulsatile abdominal mass on exam + new severe abdominal/back pain + severe HTN — AAA expansion or rupture pathwaypulsatile_abdominal_mass_with_pain_and_htn
- symptomLimb ischemia, mesenteric ischemia, AKI, or paraplegia in patient with known aortic aneurysm + severe HTN — branch malperfusion pathwaylimb_or_organ_malperfusion_with_known_aneurysm_and_htn
Required inputs (15)
- agerequireddemographic • used at CONTEXTMost aneurysms occur >65 y; younger presentations suggest connective tissue disorder (Marfan, Loeys-Dietz, vEDS) — different surgical thresholds + genetic counseling
- sexrequireddemographic • used at CONTEXTAAA male predominance 4:1; female AAA ruptures at smaller diameters (rupture risk per cm equivalent; women often considered for repair at 5.0 vs 5.5 cm)
- sbprequiredvital • used at TREATMENTUniversally elevated in this presentation; goal SBP 100-120 within 20-30 min AFTER HR <60; permissive hypotension until proximal control
- hrrequiredvital • used at TREATMENTGOAL HR <60 FIRST before vasodilator — anti-impulse paradigm (ACC/AHA 2022 Class I PMID 36066317) — applies equally to dissection + aneurysm-with-malperfusion
- four_extremity_bp_and_pulse_examrequiredvital • used at INITIAL_WORKUPBranch malperfusion mapping: differential BP between arms suggests subclavian/innominate involvement; absent femoral/popliteal/DP/PT pulses suggest iliac/femoral compromise; pulse + capillary refill q15 min during pre-op stabilization
- known_aneurysm_history_and_surveillance_imagingrequiredhistory • used at CONTEXTPrior CTA / US findings establish baseline diameter; rapid expansion (≥5 mm/6mo or ≥10 mm/yr) is independent rupture risk per ACC/AHA 2022; prior repair history (anastomotic pseudoaneurysm pathway)
- connective_tissue_disorder_or_family_history_of_aneurysmrequiredhistory • used at CONTEXTMarfan, Loeys-Dietz, vEDS, Turner — different surgical thresholds (Marfan repair at 5.0 cm root, Loeys-Dietz at 4.0-4.5 cm); 1st-degree relative with AAA = 10-fold increased risk (USPSTF screening); informs genetic counseling
- smoking_history_pack_yearsrequiredhistory • used at CONTEXTStrongest modifiable AAA risk factor (smoking + male + age >65 = USPSTF AAA screening B); ongoing smoking accelerates expansion + repair complications
- pain_quality_and_location_and_migrationrequiredsymptom • used at ENTRYSudden severe sharp back/flank pain in AAA suggests expansion/rupture; abdominal pain with pulsatile mass = AAA pain; chest pain radiating to back = thoracic; tearing radiating migrating = consider concomitant dissection
- malperfusion_symptoms_organ_specificrequiredsymptom • used at BRANCHING_WORKUPRenal (flank pain, oliguria, hematuria); mesenteric (severe abdominal pain out of proportion to exam, lactate elevation); lower extremity (cold pulseless limb, pain); spinal (paraplegia, sensory loss) — drives revascularization priority
- creatinine_and_lactaterequiredlab • used at INITIAL_WORKUPRenal malperfusion screen (Cr trend); mesenteric ischemia screen (lactate elevation); contrast nephropathy baseline; AKI severity for endovascular planning
- troponinrequiredlab • used at INITIAL_WORKUPCoronary involvement (RCA dissection extension if ascending aneurysm with extension); demand ischemia from hypovolemia/HTN; type 2 MI risk
- cbc_inr_pt_ptt_fibrinogen_type_and_crossrequiredlab • used at INITIAL_WORKUPCoagulopathy screen (rupture-related consumption); type & cross 6+ units PRBC + FFP + platelets for massive transfusion protocol; hemoglobin trend for ongoing bleed
- cta_chest_abdomen_pelvis_definitiverequiredimaging • used at INITIAL_WORKUPDefinitive imaging: diameter, morphology (fusiform vs saccular), intramural hematoma, contained rupture, branch involvement, distance from key anatomy (renal arteries, celiac, SMA, iliac); ACC/AHA 2022 Class I; preferred over MRA in acute setting
- tte_for_ascending_extension_and_ai_and_tamponaderequiredimaging • used at INITIAL_WORKUPTTE rapid assessment of ascending aorta (extension), aortic insufficiency (proximal involvement), pericardial effusion (tamponade — suggests proximal extension to ascending), LV function
12-phase flow (10)
- 1FRAMEAcute aortic aneurysm + malperfusion in HTN crisis: aneurysm-driven (NOT dissection-driven) phenotype with rapid expansion / contained leak / branch compromise. Anti-impulse paradigm: HR <60 + dP/dt FIRST, THEN SBP 100-120 (permissive hypotension until proximal control). Endovascular FIRST for descending thoracic (TEVAR) + abdominal infrarenal (EVAR) per IRAD/SVS/ESVS 2024; OPEN REPAIR for ascending or complex anatomy. Branch malperfusion → urgent revascularization. CSF drainage if T6-L1 endovascular. Inherits parent HTN-emergency framework.inputs: sbp, hr, pain_quality_and_location_and_migrationadvance: aneurysm-with-malperfusion suspected + anti-impulse + endovascular vs open plan articulated
- 2ENTRYRecognize severe back/flank/abdominal pain + known/new aneurysm + severe HTN ± malperfusion; STAT CTA chest/abdomen/pelvis; activate vascular surgery + cardiothoracic surgery teams simultaneously; bedside echo for ascending extension + tamponade; type & cross 6+ units PRBC + FFP + plateletsinputs: age, sbp, hr, pain_quality_and_location_and_migration, four_extremity_bp_and_pulse_examadvance: workup launched + surgery teams notified + transfusion prepared
- 3CONTEXTKnown aneurysm history + surveillance imaging baseline + rapid-expansion screen; connective tissue disorder + family history; smoking history; prior aortic surgery; chronic HTN duration + medications; comorbidities (CAD, CKD, COPD — surgical risk)inputs: known_aneurysm_history_and_surveillance_imaging, connective_tissue_disorder_or_family_history_of_aneurysm, smoking_history_pack_yearsadvance: context complete + surgical risk stratified
- 4RED_FLAGSFree rupture (hemodynamic collapse, transport hypotension, hemoperitoneum/hemothorax on CTA — emergent OR); contained rupture (intramural hematoma, retroperitoneal hematoma — emergent endovascular vs open); critical branch malperfusion (mesenteric ischemia → urgent revascularization within 6 h to prevent infarction; spinal cord ischemia → immediate CSF drainage); ascending extension with AI/tamponade (emergent open repair); concomitant dissection identified (route to dissection dossier in addition)inputs: sbp, malperfusion_symptoms_organ_specificactions: htn_emergencyadvance: red flags screened + emergent surgical evaluation triggered
- 5INITIAL_WORKUPBMP, troponin, CBC, coags, fibrinogen, type & cross 6+ units, CTA chest/abdomen/pelvis (definitive — diameter, morphology, intramural hematoma, contained rupture, branch involvement); TTE (ascending extension, AI, tamponade); ECG; serial 4-extremity BP + pulse examinputs: creatinine_and_lactate, troponin, cbc_inr_pt_ptt_fibrinogen_type_and_cross, cta_chest_abdomen_pelvis_definitive, tte_for_ascending_extension_and_ai_and_tamponade, four_extremity_bp_and_pulse_examactions: panel.cardiac, panel.renal, panel.coagadvance: CTA confirms aneurysm + morphology + branch involvement documented
- 6BRANCHING_WORKUPAnatomy decision tree: Ascending aneurysm rupture/expansion → STAT cardiothoracic surgery (open repair); descending thoracic aneurysm rupture/expansion/malperfusion → TEVAR per IRAD + INSTEAD-XL precedent; abdominal aortic infrarenal aneurysm rupture/expansion → EVAR per UK Trial / EVAR-1 / DREAM / OVER; complex visceral aortic aneurysm with malperfusion → branched/fenestrated EVAR or hybrid open + endovascular per SVS 2024 + ESVS 2024; concomitant dissection → run cardio.aortic-dissection.core.v1 in parallel; serial CTA q6-24h to detect extension; CSF drainage protocol if T6-L1 endovascularinputs: cta_chest_abdomen_pelvis_definitive, malperfusion_symptoms_organ_specificactions: aortic_dissectionadvance: surgical/endovascular strategy decided + branch revascularization plan documented
- 7TREATMENTANTI-IMPULSE FIRST: esmolol 500 µg/kg IV bolus + 50-300 µg/kg/min infusion titrate to HR <60 within 5-10 min OR labetalol 20 mg IV q10 min up to 300 mg cumulative. AFTER HR <60: nicardipine 5 mg/h IV titrate to SBP 100-120 within 20-30 min (PERMISSIVE HYPOTENSION until proximal control). AVOID isolated vasodilator without prior BB. PAIN CONTROL with fentanyl 25-100 µg IV q5-10 min. MASSIVE TRANSFUSION PROTOCOL if rupture (1:1:1 PRBC:FFP:platelets). EMERGENT TEVAR/EVAR/open per anatomy. CSF drainage if T6-L1 endovascular for spinal cord protection. Branch revascularization (covered stent, bypass, fenestration) per malperfusion target.inputs: sbp, hr, creatinine_and_lactateadvance: HR <60 + SBP 100-120 achieved + pain controlled + endovascular/open repair underway
- 8DISPOSITIONAscending aneurysm rupture/expansion → emergent OR (CT surgery); descending TEVAR/EVAR → vascular IR/OR; uncomplicated stable aneurysm with controlled HTN → ICU medical management with arterial line + serial CTA; post-op → CICU/SICU for monitoring + spinal cord assessment if T6-L1advance: OR booking or ICU bed assigned + post-op care plan documented
- 9MONITORINGArterial line + q5-15 min BP + HR; serial CTA q6-24 h to detect extension or endoleak post-EVAR/TEVAR; daily exam for new pulse change, neuro deficit (spinal cord ischemia post-T6-L1 endovascular surveillance), abdominal pain (mesenteric ischemia recurrence); serial Cr + lactate for branch malperfusion + AKI; spinal cord protocol (CSF drainage to ICP <10 mmHg, MAP >85 if T6-L1)inputs: sbp, hr, creatinine_and_lactateactions: panel.renaladvance: BP/HR at target × 24-48 h + no extension on imaging + branch flow confirmed
- 10FOLLOWUPLifetime aortic surveillance: post-EVAR/TEVAR CTA at 1, 6, 12 mo then annually (endoleak surveillance); for unrepaired remaining aneurysm sites q6-12 mo per size; lifelong BB ± ARB to BP <130/80 (ACC/AHA 2022); smoking cessation (strongest modifiable progression risk); statin per ASCVD; genetic counseling if Marfan/Loeys-Dietz/vEDS; family screening per USPSTF (AAA in 1st-degree male >65 ever-smoker); cardiac surgery follow-up if open repairadvance: outpatient surveillance + BB + smoking cessation + family screening + genetic plan documented