Patient handout

Idiopathic pulmonary arterial hypertension (Group 1 PAH)

PRODUCTION

1. Your condition

This handout is for idiopathic pulmonary arterial hypertension (group 1 pah). Your care team identified this based on: progressive exertional dyspnea unexplained by left hd or lung disease (esc/ers 2022).

Other reasons your team may use this plan: exertional syncope / presyncope — high-risk pah presentation (esc/ers 2022); right hf signs — jvd, peripheral edema, ascites, hepatic congestion (esc/ers 2022); echo with rvsp >=40 mmhg, dilated rv, septal flattening, tapse <=17 mm (esc/ers 2022 humbert).

2. Your medications

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
macitentan	10 mg	PO	once daily	SERAPHIN — reduced morbidity/mortality endpoint; once-daily; less hepatotoxic than bosentan
ambrisentan	5 mg	PO	once daily	AMBITION dual upfront with tadalafil
tadalafil	20 mg	PO	once daily	PHIRST + AMBITION; once-daily; no nitrate use
sildenafil	20 mg	PO	TID	SUPER-1; less convenient than tadalafil; no nitrate use

Plan: PAH-specific therapy by ESC/ERS 4-strata risk score

3. Your action plan

Use these zones to know what to do based on how you feel.

GREEN — Stable — at baseline 6MWD, no new dyspnea, weight steady

If you have:

No new shortness of breath at rest or on usual activity (ESC/ERS 2022)
No new ankle / leg swelling, abdominal fullness, or weight gain (ESC/ERS 2022)
Daily home weight within 1 kg of baseline (ESC/ERS 2022)
oxygen level (SpO₂) >=92% on baseline O2 (ESC/ERS 2022)

Do this:

Take all PAH meds exactly as prescribed (NEVER stop IV/SC prostacyclin abruptly — life-threatening rebound) (ESC/ERS 2022)
Sodium <2 g/day; fluids per plan (ESC/ERS 2022)
Daily weight at the same time, after voiding (ESC/ERS 2022)
Continue prescribed exercise plan (ESC/ERS 2022)
Keep all PAH centre appointments (ESC/ERS 2022)

YELLOW — Caution — new RV strain or congestion

If you have:

Weight gain >2 kg in 3 days (ESC/ERS 2022)
New ankle / leg swelling, abdominal fullness, or RUQ discomfort (ESC/ERS 2022)
New shortness of breath on lower-than-usual activity (ESC/ERS 2022)
New palpitations or dizziness (ESC/ERS 2022)
Pump alarms or site pain (SC) (ESC/ERS 2022)

Do this:

Take prescribed rescue diuretic dose (ESC/ERS 2022)
Tighten sodium intake (ESC/ERS 2022)
Check pump function, site, and infusion line if applicable (ESC/ERS 2022)
Call PAH centre within 24 h (ESC/ERS 2022)

Call your provider if:

No improvement after 24-48 h of rescue diuretic (ESC/ERS 2022)
Pump malfunction or line concern (any time) (ESC/ERS 2022)
New oxygen requirement above baseline (ESC/ERS 2022)

RED — Medical alert — RV failure or pump failure

If you have:

Severe shortness of breath at rest, cannot lie flat (ESC/ERS 2022)
Fainting, near-fainting, or chest pain (ESC/ERS 2022)
New confusion or extreme drowsiness (ESC/ERS 2022)
Pump stopped / line disconnected (IV/SC prostacyclin) (ESC/ERS 2022)
Hemoptysis (ESC/ERS 2022)
BP <90 systolic with cool extremities (ESC/ERS 2022)

Do this:

Call 911 / emergency services immediately (ESC/ERS 2022)
For IV prostacyclin pump failure establish backup line/pump or send EMS to PAH centre directly (ESC/ERS 2022)
Sit upright (ESC/ERS 2022)
Do NOT lie flat (ESC/ERS 2022)
Bring medication list and PAH centre contact (ESC/ERS 2022)

Call your provider if:

Any red zone symptom — ED + page PAH centre on the way (ESC/ERS 2022)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

WHO FC IV (symptoms at rest) OR exertional syncope(life-threatening)
RV failure — JVD elevated, ascites, hepatic congestion, hypotension, oliguria, lactate elevation (ESC/ERS 2022 Humbert)(life-threatening)
Positive pregnancy test in PAH patient (ESC/ERS 2022 — Class III contraindication)(life-threatening)
AST/ALT ≥3× ULN on bosentan / macitentan / ambrisentan
IV prostacyclin pump failure or line disconnection(life-threatening)
New or enlarging pericardial effusion on echo

5. Follow-up

PAH-specific exercise rehab; pneumococcal/influenza/COVID vaccinations; contraception counselling; mental-health support

6. Sources

Guideline: 2022 ESC/ERS Pulmonary Hypertension Guidelines + 2026 ATS/CHEST/PHA PAH Update