Pulmonary Arterial Hypertension (PAH) Targeted Therapy Cost Calculator

Pulmonary Arterial Hypertension (PAH) Targeted Therapy Cost Calculator helps estimate Korea-related chronic treatment, biologic drug, dialysis, obesity medication, and long-term management assumptions in English.

Health cost scenario inputs

Enter Korea-related chronic care, eldercare, therapy, procedure, fertility, diagnostic, or medical tourism assumptions. Results are simplified planning estimates.

Treatment cost over period

₩345,406,800

Monitoring or support cost

₩0

Increase reserve

₩34,540,680

Planning window cost

₩379,947,480

10 months

This English page explains Korea pulmonary arterial hypertension (PAH) targeted-therapy costs. PAH targeted drugs work through three pathways: endothelin receptor antagonists (ERA — bosentan, ambrisentan, macitentan, oral), the NO–cGMP pathway (the PDE5 inhibitor sildenafil, or the sGC stimulator riociguat; only one, since riociguat plus a PDE5 inhibitor is contraindicated), and prostacyclin-pathway agents (oral selexipag, inhaled iloprost, subcutaneous/IV treprostinil). As the disease progresses, therapy escalates from monotherapy to dual to triple combination, and the list drug cost climbs in steps — a triple oral regimen can run tens of millions of KRW a year. The key point is that idiopathic/primary PAH (ICD I27.0) is a registered rare-disease special-case condition (special mark V202), so the covered self-pay is 10% (not the ordinary 30% outpatient rate) for 5 years, and the annual out-of-pocket ceiling refunds covered self-pay above the income-tier cap (about KRW 900,000–8,430,000 in 2026). So even though adding a second or third drug multiplies the sticker price, the real annual out-of-pocket is effectively fixed at the income-tier ceiling once it is reached — the real barrier to escalating therapy is coverage/registration, not the drug price. Korea reimburses only sequential add-on combination (not upfront combination), escalating about every 3 months by WHO functional class and risk assessment, and selexipag is the only agent reimbursed up to triple therapy. Registered rare-disease patients also have no KRW 7,000,000 cap on the medical-expense tax credit (Income Tax Act Article 59-4), and the KDCA rare-disease medical-cost support program may cover the remainder for households under the income test (PAH is not one of the four special conditions, so the general rare-disease income limit applies). Tadalafil has no PAH coverage in Korea (sildenafil only) and epoprostenol is not marketed domestically. Drug prices are benefit-ceiling figures you should replace with the actual tariff. It is planning guidance based on 2026 Korean rules, not medical advice or an insurer decision.

Related calculators

What is pulmonary arterial hypertension (PAH) targeted therapy?

Pulmonary arterial hypertension (PAH) is a rare, progressive disease in which remodeling of the pulmonary artery walls raises pulmonary vascular resistance until the right ventricle fails.
Before modern drugs, average survival after diagnosis was only 2–3 years, but targeted therapies aimed at three disease pathways have transformed the outlook.

These drugs are mostly taken daily for life, and as the disease progresses therapy escalates from monotherapy to dual and triple combination (sequential add-on), so the drug cost climbs in steps.
This calculator sums the annual list cost of the drugs you pick across the three pathways, then applies the rare-disease copay special case (10%, special mark V202), the annual out-of-pocket ceiling, the medical-cost support program, and the medical-expense tax credit, on a 2026 basis.

Korea-based estimate. This calculator is built on Korean National Health Insurance rules for 2026 (rare-disease special-copay registration for idiopathic PAH, the annual out-of-pocket ceiling, and the KDCA rare-disease medical-cost support program). Drug prices are benefit-ceiling figures that change with formulation, generics, and price negotiation. It is an educational estimate, not medical advice, and does not replace diagnosis, treatment decisions, a hospital quote, or an insurer/benefit decision.

The three PAH pathways and their drugs

① Endothelin receptor antagonists (ERA — oral)

These oral drugs block the receptors (ETA/ETB) of endothelin-1, which drives vasoconstriction and vascular-wall proliferation.
Bosentan, ambrisentan, and macitentan are reimbursed in Korea.

  • • Bosentan (Tracleer): start 62.5 mg, then 125 mg twice daily after 4 weeks; needs liver-enzyme monitoring
  • • Ambrisentan (Volibris): 5–10 mg once daily; a selective ETA antagonist
  • • Macitentan (Opsumit): 10 mg once daily; a next-generation ERA (SERAPHIN trial), with a Korean generic now listed

② NO–cGMP pathway (PDE5 inhibitor / sGC stimulator — oral)

These oral drugs enhance the nitric oxide (NO)–cGMP pathway to dilate the pulmonary vessels.
You choose only one, not both.

  • • Sildenafil (Revatio): 20 mg three times daily; the PDE5 inhibitor reimbursed for PAH in Korea
  • • Riociguat (Adempas): a soluble guanylate cyclase (sGC) stimulator titrated from 1 mg up to 2.5 mg three times daily; also used for CTEPH
  • • ⚠️ Contraindication: riociguat and a PDE5 inhibitor (sildenafil/tadalafil) must never be combined (severe hypotension). Tadalafil has no PAH coverage in Korea

③ Prostacyclin pathway (oral, inhaled, infused)

These agents supplement the prostacyclin pathway (a potent vasodilator and antiplatelet), and their route of administration varies.
They are usually added for moderate-to-high-risk patients not controlled by the first two pathways.

  • • Selexipag (Uptravi, oral): an oral IP receptor agonist titrated from 200 µg to 1600 µg twice daily; the only agent reimbursed up to triple therapy in Korea
  • • Iloprost (Ventavis, inhaled): 2.5–5 µg inhaled 6–9 times a day, one ampoule per session, using a dedicated device
  • • Treprostinil (Remodulin, infused): continuous subcutaneous/IV infusion (ng/kg/min); the highest-cost option for high-risk patients, needing a dedicated pump and central line

Monotherapy → dual → triple, and sequential add-on

International guidelines (2022 ESC/ERS, the 2024 7th World Symposium) recommend initial dual oral therapy (ERA + PDE5 inhibitor) by risk, and initial triple therapy including IV prostacyclin for high-risk patients.
If low-risk status is not reached after 3–6 months, therapy is escalated.

But Korean coverage (a February 2022 notice revision) reimburses only “sequential add-on” combination.
Starting several drugs at once (upfront combination) is generally non-covered; instead, once WHO functional class and risk criteria are met, drugs are added step by step about every 3 months. Selexipag is essentially the only agent reimbursed up to triple therapy.

So this calculator’s tiers (monotherapy → dual → triple) map directly to Korea’s sequential add-on path.
The result screen shows how the list cost grows with each added drug — and what actually happens to your real out-of-pocket.

The sticker price multiplies — so why does the real cost stop at the ceiling?

The out-of-pocket ceiling applies on an annual basis.
However large a year’s covered out-of-pocket is, you pay only up to your income-decile cap and the rest is refunded.

Even at the 10% special-case rate, combination therapy runs to millions of KRW a year in covered out-of-pocket.
Once that exceeds the income-decile ceiling, the excess is refunded — so adding a second or third drug multiplies the sticker price, yet the real annual out-of-pocket is effectively fixed at the ceiling.

annual real out-of-pocket ≈ min(annual list cost × coinsurance, income-decile ceiling)
→ as tiers rise and the list cost grows, the real burden is fixed at the income-decile ceiling

For a decile-5 patient (annual cap about KRW 1.73 million), monotherapy or triple therapy converges to roughly KRW 1.73 million a year in real out-of-pocket once the cap is reached.
That is why this calculator headlines the real out-of-pocket, not the sticker price: the real barrier to escalating therapy is coverage/registration, not the drug price.

How the cost is calculated

1. Annual list cost per drug

drug annual cost = unit price × units per day × 365
regimen annual cost = Σ (selected drugs, 0–1 per pathway)

PAH drug prices vary with formulation, generic entry, and price-volume negotiation, so the defaults are benefit-ceiling figures at a standard daily dose that you can adjust per drug.
Inhaled and infused prostacyclins carry extra device, pump, and supply costs beyond the drug price.

2. Covered out-of-pocket and safety nets

covered out-of-pocket = annual list cost × coinsurance (10% or 30%)
− out-of-pocket ceiling refund (excess over the annual cap)
− rare-disease medical-cost support (if eligible)
− medical-expense tax credit (15% of the part above 3% of salary)
= real annual out-of-pocket

The three safety nets that cut your cost

① Rare-disease special copay (10% coinsurance)

Idiopathic/primary PAH is a registered rare-disease special-case condition — diagnosis code I27.0, special mark V202.
Once registered, the patient pays only 10% of covered charges (versus 30% outpatient without registration).

Basis: National Health Insurance Act Art. 44 and Enforcement Decree Art. 19(1) Table 2, and the Ministry of Health and Welfare Notice on Copayment Special Cases (No. 2026-101), Art. 5 and Table 4.
Registration lasts 5 years and can be renewed. (Eisenmenger complex/syndrome, I27.8, is separately coded V226.)

② Out-of-pocket ceiling (annual cap, retroactive refund)

When annual covered out-of-pocket exceeds the income-decile ceiling, the excess is refunded.
Even at 10%, high-cost combination therapy runs into the millions, so the ceiling is the key mechanism that caps the real burden.

For 2026 the income-decile annual ceiling ranges from about KRW 900,000 (decile 1) to KRW 8,430,000 (decile 10).
Because it applies per year, the real burden is fixed at the ceiling even as you escalate through the combination tiers.

③ Rare-disease medical-cost support

The KDCA rare-disease helpline runs a medical-cost support program that covers covered-care out-of-pocket for registered patients who meet income and asset tests.

PAH is not one of the four special conditions (hemophilia, Gaucher, Fabry, mucopolysaccharidosis), so the general rare-disease income limit applies (patient household under about 140% of median income).
If eligible, the remaining out-of-pocket after the special-case rate (and the ceiling) can also be supported; check eligibility at helpline.kdca.go.kr.

Frequently asked questions

Q. Is PAH covered by the special-case program?

A. Yes — idiopathic/primary PAH (code I27.0) is a rare-disease special-case condition with special mark V202.
Registration cuts the covered copay from 30% to 10% for 5 years (renewable).

Q. If I add a second or third drug, does my out-of-pocket rise proportionally?

A. The sticker price rises, but the real out-of-pocket does not rise much.
Because the annual ceiling refunds covered out-of-pocket above the income-decile cap, the real burden stays fixed at the ceiling.

Q. Why can’t I start combination therapy upfront in Korea?

A. Korean coverage reimburses only sequential add-on combination.
Rather than starting several drugs at once, drugs are added step by step about every 3 months once WHO functional class and risk criteria are met. Selexipag is the agent reimbursed up to triple therapy.

Q. Can I also claim the medical-expense tax credit?

A. With earned income you get a 15% credit on out-of-pocket above 3% of total salary.
For a registered rare/intractable-disease patient’s own expenses, the credit has no KRW 7 million cap.

Estimate your PAH targeted-therapy cost now

Enter the pathways, drugs, and coverage settings to see how far the combination drug cost falls after Korea’s safety nets, and to compare the real out-of-pocket from monotherapy through triple therapy.

This is a 2026 reference estimate; confirm actual prices, coverage criteria, and out-of-pocket with your hospital and the National Health Insurance Service.