This handout is for pulmonary hypertension crisis in scleroderma (pah-ssc who group 1 — rv failure with shock). Your care team identified this based on: known pah-ssc + acute progressive dyspnea + hypoxia + jvd + lower extremity edema + new oliguria — rv failure crisis (esc/ers 2022 pmid 36215974).
Other reasons your team may use this plan: 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; pah-ssc + syncope or near-syncope on exertion → high-risk rv failure prognostic indicator (esc/ers 2022 high-risk reveal/compera).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| inhaled nitric oxide | 20-40 ppm via mask or ventilator | inhaled | continuous | ESC/ERS 2022 PMID 36215974 — pulmonary-selective vasodilation without systemic SVR drop; rapid titration; ideal acute crisis bridge |
| milrinone | 0.25-0.5 mcg/kg/min IV (no bolus to avoid SVR drop) | IV | continuous | Negative-afterload inotrope reducing PVR + improving RV-PA coupling; ESC/ERS 2022 — milrinone preferred over dobutamine in RV failure (less tachycardia) |
| 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 | 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) |
| treprostinil | 1.25 ng/kg/min IV/SC, titrate; or inhaled 18 mcg QID | IV/SC/inhaled | continuous IV/SC or QID inhaled | ESC/ERS 2022 — alternative to epoprostenol; longer half-life makes interruption less catastrophic; site reactions with SC |
| sildenafil | 20 mg PO TID (chronic) or 10 mg IV TID acute | PO/IV | TID | ESC/ERS 2022 PMID 36215974 — PDE5i pulmonary-selective vasodilator; combination therapy backbone; avoid concurrent nitrate |
| tadalafil | 40 mg PO daily | PO | daily | ESC/ERS 2022 — once-daily PDE5i; AMBITION trial component PMID 26308684 |
| ambrisentan | 5 mg PO daily, titrate to 10 mg | PO | daily | AMBITION trial PMID 26308684 — initial combination ambrisentan + tadalafil superior to monotherapy in PAH; LFT monitoring mandatory monthly |
| macitentan | 10 mg PO daily | PO | daily | SERAPHIN PMID 23984728 — macitentan reduces morbidity/mortality in PAH; less hepatotoxic than bosentan |
| selexipag | 200 mcg PO BID, titrate by 200 mcg q3-7d to max 1600 mcg BID | PO | BID | GRIPHON PMID 26699168 — selexipag reduces morbidity in PAH; oral alternative to parenteral prostacyclin |
| riociguat | 1 mg PO TID, titrate to 2.5 mg PO TID | PO | TID | PATENT-1 PMID 23883378 — sGC stimulator; AVOID concurrent PDE5i (additive hypotension) |
| furosemide | 20-40 mg IV initially (cautious; RV preload-dependent); reassess CVP/IVC | IV | BID-TID | Reduce RV preload + tricuspid regurgitation; CAUTIOUS — overdiuresis worsens cardiac output (RV preload-dependent) |
| norepinephrine | 0.05-0.5 mcg/kg/min IV titrate to MAP ≥65 | IV continuous | continuous | NE preserves RV coronary perfusion via systemic MAP; preferred over dopamine (less tachy/arrhythmia) |
| AVOID systemic vasodilators (NTG, hydralazine, nitroprusside) | AVOID | N/A | N/A | 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 | AVOID | N/A | N/A | Negative inotropy in already-failing RV; if AFib rate control needed, prefer digoxin |
| AVOID positive-pressure ventilation if possible | AVOID PEEP > 5; minimize tidal volumes | N/A | N/A | PEEP increases intrathoracic pressure → reduces RV preload → worsens cardiac output; if intubation needed use lowest PEEP + lung-protective ventilation |
| AVOID CCB unless documented +vasoreactivity | AVOID | N/A | N/A | PAH-SSc rarely vasoreactive (<5%); CCB monotherapy without +RHC vasoreactivity test → harm; reserved only for confirmed responders |
| RV-MCS bridge to lung transplant | RVAD or VA-ECMO per CT surgery | extracorporeal | continuous | ESC/ERS 2022 — RV-MCS (RVAD or VA-ECMO) bridge to bilateral lung transplant for refractory PAH; mortality benefit if appropriate candidate |
Plan: 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
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
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)
Guideline: 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)