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cardio.hypertensive-emergency.scleroderma-pulmonary-htn-crisis.v1

Pulmonary hypertension crisis in scleroderma (PAH-SSc WHO Group 1 — RV failure with shock)

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Pulmonary hypertension crisis in scleroderma (PAH-SSc WHO Group 1) = acute RV failure with low cardiac output + systemic hypoperfusion in patient with established or newly-diagnosed PAH-SSc. Pathophysiology: scleroderma vasculopathy → progressive pulmonary arterial remodeling (intimal proliferation, medial hypertrophy, plexiform lesions) → elevated PVR → RV pressure overload → RV-PA uncoupling → low cardiac output → cardiogenic shock. Crisis precipitants: infection, surgery, anesthesia, tachyarrhythmia (especially AFib/AFL), anemia, PE, abrupt PH-targeted therapy hold, systemic vasodilator administration. Pharmacology pivot: PULMONARY-SELECTIVE VASODILATORS — iNO 20-40 ppm + IV milrinone (negative-afterload inotrope reducing PVR + improving RV-PA coupling) + cautious diuresis (RV preload-dependent); INTRAVENOUS PROSTACYCLIN (epoprostenol IV 2 ng/kg/min titrate); PDE5i (sildenafil PO bridge); AVOID systemic vasodilators (NTG, hydralazine — worsen V/Q mismatch). RV-MCS (RVAD/VA-ECMO) bridge to lung transplant for refractory. Route to parent engine for shared HTN-emergency arc; this dossier owns the PAH-specific pharmacology + RV failure assessment + transplant pathway.

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PAH-SSc + acute RV failure phenotype identified + ICU triage initiated

Patient inputs (13)

Older PAH-SSc patients have worse prognosis; transplant eligibility cutoffs (typically <65-70 lung tx)

Limited cutaneous SSc + CREST highest PAH risk; long disease duration (>5 yr); anticentromere antibody association

BNP/NT-proBNP correlates with RV strain + prognosis; >300 BNP or >1400 NT-proBNP defines high-risk per ESC/ERS 2022

Troponin elevation = RV ischemia from supply-demand mismatch in pressure-overloaded RV; high-risk indicator

Cardiorenal — low cardiac output → AKI; drives diuretic + drug dosing; baseline for contrast nephropathy in workup

Echo: PASP elevation, RV dilation/dysfunction, septal flattening (D-shape LV), TAPSE <17 mm, IVC plethora, pericardial effusion (high-risk)

RV strain pattern (RAD, RVH, T-wave inversion V1-V3, S1Q3T3); arrhythmia detection

Systemic SBP — distinguishes from systemic HTN crisis; in PAH crisis SBP often LOW (cardiogenic shock from RV failure → low LV preload); SBP <90 + cool extremities = severe

HR >110 + low SBP suggests low-output state from RV failure; tachyarrhythmia (AFib, atrial flutter) common precipitant

Hypoxia from V/Q mismatch + low cardiac output; SpO2 <90 on room air requires supplemental O2 (PVR-reducing)

Elevated lactate = systemic hypoperfusion from RV-driven low CO; serial lactate tracks resuscitation

Confirms pre-capillary PAH: mPAP ≥20 mmHg + PVR >2 WU + PAWP ≤15 (ESC/ERS 2022 PMID 36215974); also vasoreactivity testing for IPAH (not for PAH-SSc — non-responder phenotype)

Differentiate WHO Group 1 PAH-SSc from WHO Group 3 (ILD-driven PH from SSc-ILD); honeycombing, ground-glass, mosaic perfusion patterns

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (4)

4 need judgement
  • informationallife_threateningpah_ssc_crisis_with_RV_failure_and_shock
    PAH-SSc crisis + cardiogenic shock from RV failure (SBP <90 + lactate ≥2 + cool extremities + oliguria + low CI on echo)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpah_targeted_therapy_adverse_event_severe
    Severe adverse event on PH-targeted therapy — ERA hepatotoxicity (LFT >3x ULN) OR IV prostacyclin line-related sepsis OR pump malfunction with abrupt cessation
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpah_ssc_with_concurrent_scleroderma_renal_crisis
    PAH-SSc crisis + concurrent new SBP ≥150 OR ≥30 mmHg above baseline + AKI + MAHA features — rare but documented overlap of two scleroderma vasculopathy emergencies
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepah_ssc_transplant_listing_decision
    PAH-SSc with REVEAL 2.0 high-risk despite max combination therapy — bilateral lung transplant listing decision point
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Pulmonary HTN crisis in scleroderma — pulmonary-selective vasodilators (iNO + milrinone + IV epoprostenol + PDE5i bridge); AVOID systemic vasodilators; RV-MCS bridge to lung transplant for refractory
axis: pah_ssc_crisis_pulmonary_selective_pharmacology
Selected axis "Pulmonary HTN crisis in scleroderma — pulmonary-selective vasodilators (iNO + milrinone + IV epoprostenol + PDE5i bridge); AVOID systemic vasodilators; RV-MCS bridge to lung transplant for refractory" by default fallback (first axis)
  • inhaled nitric oxide
    first line
    inhaled_pulmonary_vasodilator
    20-40 ppm via mask or ventilator • inhaled • continuous
    triggers: PAH_crisis_acute_RV_failure, PAH_crisis_with_hypoxia
    ESC/ERS 2022 PMID 36215974 — pulmonary-selective vasodilation without systemic SVR drop; rapid titration; ideal acute crisis bridge
    rxcui 7442
  • milrinone
    first line
    pde3_inhibitor_inodilator
    0.25-0.5 mcg/kg/min IV (no bolus to avoid SVR drop) • IV • continuous
    triggers: PAH_crisis_with_low_cardiac_output_RV_failure
    Negative-afterload inotrope reducing PVR + improving RV-PA coupling; ESC/ERS 2022 — milrinone preferred over dobutamine in RV failure (less tachycardia)
    rxcui 52769
  • epoprostenol (Flolan)
    first line
    IV_prostacyclin
    2 ng/kg/min IV continuous, titrate by 1-2 ng/kg/min q15-60 min as tolerated; typical maintenance 20-40 ng/kg/min • IV continuous (dedicated central line) • continuous 24/7 NEVER interrupt
    triggers: PAH_crisis_severe_or_high_risk_PAH-SSc
    ESC/ERS 2022 PMID 36215974 — IV epoprostenol most potent PVR reducer; mortality benefit in severe PAH; ABRUPT CESSATION = FATAL REBOUND (dedicated 24/7 infusion + backup pump mandatory)
    rxcui 8814
  • treprostinil
    second line
    prostacyclin_analog
    1.25 ng/kg/min IV/SC, titrate; or inhaled 18 mcg QID • IV/SC/inhaled • continuous IV/SC or QID inhaled
    triggers: PAH_crisis_alternative_to_epoprostenol, transition_from_inpatient_to_outpatient
    ESC/ERS 2022 — alternative to epoprostenol; longer half-life makes interruption less catastrophic; site reactions with SC
    rxcui 343048
  • sildenafil
    first line
    pde5_inhibitor
    20 mg PO TID (chronic) or 10 mg IV TID acute • PO/IV • TID
    triggers: PAH_chronic_or_acute_with_PO_tolerance
    ESC/ERS 2022 PMID 36215974 — PDE5i pulmonary-selective vasodilator; combination therapy backbone; avoid concurrent nitrate
    rxcui 136411
  • tadalafil
    second line
    pde5_inhibitor
    40 mg PO daily • PO • daily
    triggers: PAH_chronic_alternative_to_sildenafil
    ESC/ERS 2022 — once-daily PDE5i; AMBITION trial component PMID 26308684
    rxcui 358263
  • ambrisentan
    first line
    endothelin_receptor_antagonist_ETA_selective
    5 mg PO daily, titrate to 10 mg • PO • daily
    triggers: PAH_chronic_combination_therapy
    AMBITION trial PMID 26308684 — initial combination ambrisentan + tadalafil superior to monotherapy in PAH; LFT monitoring mandatory monthly
    rxcui 358274
  • macitentan
    first line
    endothelin_receptor_antagonist_dual
    10 mg PO daily • PO • daily
    triggers: PAH_chronic_combination_therapy
    SERAPHIN PMID 23984728 — macitentan reduces morbidity/mortality in PAH; less hepatotoxic than bosentan
    rxcui 1442132
  • selexipag
    add on
    oral_prostacyclin_receptor_agonist
    200 mcg PO BID, titrate by 200 mcg q3-7d to max 1600 mcg BID • PO • BID
    triggers: PAH_chronic_oral_prostacyclin_pathway
    GRIPHON PMID 26699168 — selexipag reduces morbidity in PAH; oral alternative to parenteral prostacyclin
    rxcui 1729002
  • riociguat
    second line
    soluble_guanylate_cyclase_stimulator
    1 mg PO TID, titrate to 2.5 mg PO TID • PO • TID
    triggers: PAH_PDE5i_intolerant, CTEPH_inoperable
    PATENT-1 PMID 23883378 — sGC stimulator; AVOID concurrent PDE5i (additive hypotension)
    rxcui 1439816
  • furosemide
    first line
    loop_diuretic
    20-40 mg IV initially (cautious; RV preload-dependent); reassess CVP/IVC • IV • BID-TID
    triggers: PAH_crisis_with_volume_overload
    Reduce RV preload + tricuspid regurgitation; CAUTIOUS — overdiuresis worsens cardiac output (RV preload-dependent)
    rxcui 4603
  • norepinephrine
    second line
    alpha_beta_adrenergic_vasopressor
    0.05-0.5 mcg/kg/min IV titrate to MAP ≥65 • IV continuous • continuous
    triggers: PAH_crisis_with_systemic_hypotension
    NE preserves RV coronary perfusion via systemic MAP; preferred over dopamine (less tachy/arrhythmia)
    rxcui 7512
  • AVOID systemic vasodilators (NTG, hydralazine, nitroprusside)
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: PAH_crisis_diagnosis
    ESC/ERS 2022 PMID 36215974 — systemic vasodilators worsen V/Q mismatch (vasodilate non-PAH territories) + drop SVR without pulmonary selectivity → catastrophic systemic hypotension
  • AVOID beta-blocker
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: PAH_crisis_diagnosis
    Negative inotropy in already-failing RV; if AFib rate control needed, prefer digoxin
  • AVOID positive-pressure ventilation if possible
    contraindication substitute
    do_not_use
    AVOID PEEP > 5; minimize tidal volumes • N/A • N/A
    triggers: PAH_crisis_diagnosis
    PEEP increases intrathoracic pressure → reduces RV preload → worsens cardiac output; if intubation needed use lowest PEEP + lung-protective ventilation
  • AVOID CCB unless documented +vasoreactivity
    contraindication substitute
    do_not_use
    AVOID • N/A • N/A
    triggers: PAH-SSc_diagnosis
    PAH-SSc rarely vasoreactive (<5%); CCB monotherapy without +RHC vasoreactivity test → harm; reserved only for confirmed responders
  • RV-MCS bridge to lung transplant
    rescue
    mechanical_circulatory_support
    RVAD or VA-ECMO per CT surgery • extracorporeal • continuous
    triggers: PAH_crisis_refractory_to_max_medical_therapy_with_transplant_listing
    ESC/ERS 2022 — RV-MCS (RVAD or VA-ECMO) bridge to bilateral lung transplant for refractory PAH; mortality benefit if appropriate candidate

outpatient playbook — drug actions (3)

  1. 1. lifelong triple combination
    rxcui 857304
    ERA + PDE5i + prostacyclin per regimen • PO + IV/SC • as scheduled
    trigger: Lifelong PAH-SSc
    ESC/ERS 2022
  2. 2. rheumatology-directed SSc disease modification (mycophenolate, etc.)
    Per rheumatology • PO • per regimen
    trigger: SSc disease activity
    EULAR 2024
  3. 3. AC for AFib only (not routine for PAH-SSc)
    rxcui 1364430
    Apixaban 5 mg BID • PO • BID
    trigger: AF + CHA2DS2-VASc ≥2
    ACC/AHA 2023 AF; routine warfarin in PAH-SSc not recommended ESC/ERS 2022

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Known PAH-SSc + acute progressive dyspnea + hypoxia + JVD + lower extremity edema + new oliguria — RV failure crisis (ESC/ERS 2022 PMID 36215974); Systemic sclerosis + new exertional dyspnea + reduced 6MWT distance + DLCO disproportionately low → urgent echo/RHC for PAH (DETECT algorithm); BNP >300 OR NT-proBNP >1400 + ECG RV strain pattern + elevated troponin (RV ischemia) — high-risk PAH crisis.

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Pulmonary hypertension crisis in scleroderma (PAH-SSc WHO Group 1 — RV failure with shock)** (cardio.hypertensive-emergency.scleroderma-pulmonary-htn-crisis.v1).
Scope: Pulmonary hypertension crisis in scleroderma (PAH-SSc WHO Group 1) = acute RV failure with low cardiac output + systemic hypoperfusion in patient with established or newly-diagnosed PAH-SSc. Pathophysiology: scleroderma vasculopathy → progressive pulmonary arterial remodeling (intimal proliferation, medial hypertrophy, plexiform lesions) → elevated PVR → RV pressure overload → RV-PA uncoupling → low cardiac output → cardiogenic shock. Crisis precipitants: infection, surgery, anesthesia, tachyarrhythmia (especially AFib/AFL), anemia, PE, abrupt PH-targeted therapy hold, systemic vasodilator administration. Pharmacology pivot: PULMONARY-SELECTIVE VASODILATORS — iNO 20-40 ppm + IV milrinone (negative-afterload inotrope reducing PVR + improving RV-PA coupling) + cautious diuresis (RV preload-dependent); INTRAVENOUS PROSTACYCLIN (epoprostenol IV 2 ng/kg/min titrate); PDE5i (sildenafil PO bridge); AVOID systemic vasodilators (NTG, hydralazine — worsen V/Q mismatch). RV-MCS (RVAD/VA-ECMO) bridge to lung transplant for refractory. Route to parent engine for shared HTN-emergency arc; this dossier owns the PAH-specific pharmacology + RV failure assessment + transplant pathway.

No severity triggers fired against current inputs.

Plan

Regimen axis: **Pulmonary HTN crisis in scleroderma — pulmonary-selective vasodilators (iNO + milrinone + IV epoprostenol + PDE5i bridge); AVOID systemic vasodilators; RV-MCS bridge to lung transplant for refractory**.
1. inhaled nitric oxide 20-40 ppm via mask or ventilator inhaled continuous (inhaled_pulmonary_vasodilator, first line) — ESC/ERS 2022 PMID 36215974 — pulmonary-selective vasodilation without systemic SVR drop; rapid titration; ideal acute crisis bridge
2. milrinone 0.25-0.5 mcg/kg/min IV (no bolus to avoid SVR drop) IV continuous (pde3_inhibitor_inodilator, first line) — Negative-afterload inotrope reducing PVR + improving RV-PA coupling; ESC/ERS 2022 — milrinone preferred over dobutamine in RV failure (less tachycardia)
3. epoprostenol (Flolan) 2 ng/kg/min IV continuous, titrate by 1-2 ng/kg/min q15-60 min as tolerated; typical maintenance 20-40 ng/kg/min IV continuous (dedicated central line) continuous 24/7 NEVER interrupt (IV_prostacyclin, first line) — ESC/ERS 2022 PMID 36215974 — IV epoprostenol most potent PVR reducer; mortality benefit in severe PAH; ABRUPT CESSATION = FATAL REBOUND (dedicated 24/7 infusion + backup pump mandatory)
4. treprostinil 1.25 ng/kg/min IV/SC, titrate; or inhaled 18 mcg QID IV/SC/inhaled continuous IV/SC or QID inhaled (prostacyclin_analog, second line) — ESC/ERS 2022 — alternative to epoprostenol; longer half-life makes interruption less catastrophic; site reactions with SC
5. sildenafil 20 mg PO TID (chronic) or 10 mg IV TID acute PO/IV TID (pde5_inhibitor, first line) — ESC/ERS 2022 PMID 36215974 — PDE5i pulmonary-selective vasodilator; combination therapy backbone; avoid concurrent nitrate
6. tadalafil 40 mg PO daily PO daily (pde5_inhibitor, second line) — ESC/ERS 2022 — once-daily PDE5i; AMBITION trial component PMID 26308684
7. ambrisentan 5 mg PO daily, titrate to 10 mg PO daily (endothelin_receptor_antagonist_ETA_selective, first line) — AMBITION trial PMID 26308684 — initial combination ambrisentan + tadalafil superior to monotherapy in PAH; LFT monitoring mandatory monthly
8. macitentan 10 mg PO daily PO daily (endothelin_receptor_antagonist_dual, first line) — SERAPHIN PMID 23984728 — macitentan reduces morbidity/mortality in PAH; less hepatotoxic than bosentan
9. selexipag 200 mcg PO BID, titrate by 200 mcg q3-7d to max 1600 mcg BID PO BID (oral_prostacyclin_receptor_agonist, add on) — GRIPHON PMID 26699168 — selexipag reduces morbidity in PAH; oral alternative to parenteral prostacyclin
10. riociguat 1 mg PO TID, titrate to 2.5 mg PO TID PO TID (soluble_guanylate_cyclase_stimulator, second line) — PATENT-1 PMID 23883378 — sGC stimulator; AVOID concurrent PDE5i (additive hypotension)
11. furosemide 20-40 mg IV initially (cautious; RV preload-dependent); reassess CVP/IVC IV BID-TID (loop_diuretic, first line) — Reduce RV preload + tricuspid regurgitation; CAUTIOUS — overdiuresis worsens cardiac output (RV preload-dependent)
12. norepinephrine 0.05-0.5 mcg/kg/min IV titrate to MAP ≥65 IV continuous continuous (alpha_beta_adrenergic_vasopressor, second line) — NE preserves RV coronary perfusion via systemic MAP; preferred over dopamine (less tachy/arrhythmia)
13. AVOID systemic vasodilators (NTG, hydralazine, nitroprusside) AVOID N/A N/A (do_not_use, contraindication substitute) — ESC/ERS 2022 PMID 36215974 — systemic vasodilators worsen V/Q mismatch (vasodilate non-PAH territories) + drop SVR without pulmonary selectivity → catastrophic systemic hypotension
14. AVOID beta-blocker AVOID N/A N/A (do_not_use, contraindication substitute) — Negative inotropy in already-failing RV; if AFib rate control needed, prefer digoxin
15. AVOID positive-pressure ventilation if possible AVOID PEEP > 5; minimize tidal volumes N/A N/A (do_not_use, contraindication substitute) — PEEP increases intrathoracic pressure → reduces RV preload → worsens cardiac output; if intubation needed use lowest PEEP + lung-protective ventilation
16. AVOID CCB unless documented +vasoreactivity AVOID N/A N/A (do_not_use, contraindication substitute) — PAH-SSc rarely vasoreactive (<5%); CCB monotherapy without +RHC vasoreactivity test → harm; reserved only for confirmed responders
17. RV-MCS bridge to lung transplant RVAD or VA-ECMO per CT surgery extracorporeal continuous (mechanical_circulatory_support, rescue) — ESC/ERS 2022 — RV-MCS (RVAD or VA-ECMO) bridge to bilateral lung transplant for refractory PAH; mortality benefit if appropriate candidate

Setting playbook (outpatient) — Lifelong PH center + rheumatology + lung transplant team — combination triple therapy maintenance, REVEAL 2.0 quarterly, transplant listing, palliative if refractory advanced disease
18. lifelong triple combination ERA + PDE5i + prostacyclin per regimen PO + IV/SC as scheduled — Lifelong PAH-SSc (ESC/ERS 2022)
19. rheumatology-directed SSc disease modification (mycophenolate, etc.) Per rheumatology PO per regimen — SSc disease activity (EULAR 2024)
20. AC for AFib only (not routine for PAH-SSc) Apixaban 5 mg BID PO BID — AF + CHA2DS2-VASc ≥2 (ACC/AHA 2023 AF; routine warfarin in PAH-SSc not recommended ESC/ERS 2022)

Non-pharmacologic actions:
- Quarterly PH center
- Annual lung transplant clinic until transplanted
- Rheumatology lifelong
- Annual flu + pneumococcal vaccine
- Cardiac rehab (PAH-modified low-intensity)
- AVOID pregnancy lifelong (high mortality unless transplanted)
- Palliative care if refractory advanced disease

AVOID / contraindication checks:
- Systemic_vasodilator_avoid_in_PAH_crisis (ESC/ERS 2022 PMID 36215974) — worsen V/Q + drop SVR
- Beta_blocker_avoid_in_failing_RV
- Positive_pressure_ventilation_minimize_PEEP_reduces_RV_preload
- CCB_avoid_unless_documented_+vasoreactivity_on_RHC
- Erectile_dysfunction_PDE5i_avoid_concurrent_with_PAH_PDE5i (additive hypotension)
- Nitrate_avoid_concurrent_with_PDE5i (severe hypotension)
- Abrupt_cessation_of_IV_epoprostenol_FATAL_REBOUND (dedicated 24/7 infusion + backup pump)
- Pregnancy_avoid_in_PAH_high_maternal_mortality
- Contrast_minimize_in_cardiorenal_PAH SSc

Monitoring

Regimen monitoring:
- arterial line and central line continuous hemodynamics
- continuous SpO2 and ECG
- q1-4h vitals and UOP
- q4-6h BMP lactate Mg
- daily BNP NTproBNP and troponin
- daily echo TAPSE RV size and function
- RHC q24-48h if catheter stable for hemodynamic optimization
- serial 6MWT once stable
- weekly transplant listing review
- monthly LFT during ERA therapy (ambrisentan/macitentan)
- quarterly REVEAL 2.0 risk reassessment

Setting (outpatient) monitoring:
- Quarterly clinical + BNP + 6MWT + REVEAL 2.0
- Annual echo + RHC if listed

Follow-up plan: Lifelong PH center + rheumatology + cardiology + transplant team coordination; combination triple therapy (ERA + PDE5i + prostacyclin) per AMBITION/SERAPHIN/GRIPHON; quarterly REVEAL 2.0 risk reassessment; pulmonary HTN registry; bilateral lung transplant listing for severe (FC III-IV despite max therapy); avoid pregnancy (high mortality in PAH); avoid systemic vasodilators; AVOID erectile dysfunction PDE5i if on PAH PDE5i (additive hypotension)
- Close-out criterion: lifelong PH center + transplant pathway + chronic PH-targeted therapy stable

Monitoring phase: A-line + central line for hemodynamics; continuous SpO2 + ECG; q1-4h vitals + UOP; q4-6h BMP + lactate; daily BNP/NT-proBNP + troponin + echo (TAPSE trajectory); RHC q24-48h if catheter-stable for hemodynamic optimization; serial 6MWT once stable; weekly transplant listing review

Disposition

Current setting: outpatient — Lifelong PH center + rheumatology + lung transplant team — combination triple therapy maintenance, REVEAL 2.0 quarterly, transplant listing, palliative if refractory advanced disease

Disposition criteria:
- Long-term continuation; cross-link to cardio.htn.core.v1 + rheumatology + lung transplant chronic management; cardio.hypertensive-emergency.scleroderma-renal-crisis.v1 if SRC overlap

Escalation triggers (move to higher acuity):
- REVEAL 2.0 worsening → escalate triple therapy + transplant listing acceleration
- New AKI / cardiorenal → nephrology
- Recurrent crisis → ED + ICU
- Family planning desire → high-risk OB + termination discussion

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] PAH-SSc crisis + cardiogenic shock from RV failure (SBP <90 + lactate ≥2 + cool extremities + oliguria + low CI on echo)
- [LIFE_THREATENING] Severe adverse event on PH-targeted therapy — ERA hepatotoxicity (LFT >3x ULN) OR IV prostacyclin line-related sepsis OR pump malfunction with abrupt cessation
- [LIFE_THREATENING] PAH-SSc crisis + concurrent new SBP ≥150 OR ≥30 mmHg above baseline + AKI + MAHA features — rare but documented overlap of two scleroderma vasculopathy emergencies

Citations

- ESC/ERS 2022 PH Guidelines (Humbert PMID 36215974) + AMBITION (Galiè NEJM 2015 PMID 26308684) + SERAPHIN (Pulido NEJM 2013 PMID 23984728) + GRIPHON (Sitbon NEJM 2015 PMID 26699168) + 2025 ACC/AHA HTN (Whelton) [PMID:36215974](https://pubmed.ncbi.nlm.nih.gov/36215974/)
- Cited evidence (PMID 26308684) [PMID:26308684](https://pubmed.ncbi.nlm.nih.gov/26308684/)
- Cited evidence (PMID 23984728) [PMID:23984728](https://pubmed.ncbi.nlm.nih.gov/23984728/)
- Cited evidence (PMID 26699168) [PMID:26699168](https://pubmed.ncbi.nlm.nih.gov/26699168/)
- Cited evidence (PMID 23883378) [PMID:23883378](https://pubmed.ncbi.nlm.nih.gov/23883378/)

Last reconciled with current guidelines: 2026-05-15.
References
  • ESC/ERS 2022 PH Guidelines (Humbert PMID 36215974) + AMBITION (Galiè NEJM 2015 PMID 26308684) + SERAPHIN (Pulido NEJM 2013 PMID 23984728) + GRIPHON (Sitbon NEJM 2015 PMID 26699168) + 2025 ACC/AHA HTN (Whelton) — PMID:36215974
  • Cited evidence (PMID 26308684) — PMID:26308684
  • Cited evidence (PMID 23984728) — PMID:23984728
  • Cited evidence (PMID 26699168) — PMID:26699168
  • Cited evidence (PMID 23883378) — PMID:23883378