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Pulmonary arterial hypertension (PAH): 국가별 선택지

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선택지 정리됨희귀·유전 질환최종 확인 2026.06

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임상시험 검색어

Pulmonary arterial hypertension (PAH) clinical trial

국가별 선택지

국가별 치료 선택지

공식 규제·평가 기관 출처를 바탕으로 한 국가별 승인·접근 상태입니다. 무엇이 어디에 존재하는지를 보여줄 뿐, 추천이 아닙니다.

United States

  • sapropterin dihydrochloride (Kuvan); pegvaliase-pqpz (Palynziq); sepiapterin (Sephience)[1]FDA-approvedPAH deficiency; BH4 responsiveness / sepiapterin responsiveness as described in labels; Kuvan and Sephience label contexts specify adjunct use with Phe-restricted diet and response-based evaluation; Palynziq label specifies uncontrolled blood Phe greater than 600 micromol/L on existing management. · Response to Kuvan and Sephience cannot be assumed from genotype alone in the FDA labels. Palynziq has boxed warning/REMS risk language and the fetched FDA label is adult-only despite a separate 2026 manufacturer approval announcement for adolescent expansion. Confidence/conflicts: High for FDA label claims; medium for Palynziq adolescent expansion until an updated FDA label is fetched.
  • sapropterin dihydrochloride (Kuvan); pegvaliase-pqpz (Palynziq); sepiapterin (Sephience)[1]FDA-approvedPAH deficiency; BH4 responsiveness / sepiapterin responsiveness as described in labels; Kuvan and Sephience label contexts specify adjunct use with Phe-restricted diet and response-based evaluation; Palynziq label specifies uncontrolled blood Phe greater than 600 micromol/L on existing management. · Response to Kuvan and Sephience cannot be assumed from genotype alone in the FDA labels. Palynziq has boxed warning/REMS risk language and the fetched FDA label is adult-only despite a separate 2026 manufacturer approval announcement for adolescent expansion. Confidence/conflicts: High for FDA label claims; medium for Palynziq adolescent expansion until an updated FDA label is fetched.
  • sotatercept-csrk (Winrevair); macitentan/tadalafil (Opsynvi); selexipag (Uptravi); riociguat (Adempas); treprostinil inhalation powder or solution (Tyvaso DPI / Tyvaso)[2]FDA-approvednot biomarker-defined; WHO group and functional class context; Adult PAH disease-targeted therapy across activin signaling inhibition, endothelin/PDE5 combination, prostacyclin pathway agonist, soluble guanylate cyclase stimulation, and inhaled prostacyclin mimetic pathways. · Labels do not rank therapies or replace specialist risk stratification. Pregnancy/embryo-fetal toxicity, REMS, hypotension, bleeding, pulmonary veno-occlusive disease, liver issues, route/device burden, and drug-interaction caveats vary by product. PAH must be distinguished from other pulmonary hypertension groups, especially CTEPH and PH due to left-heart or lung disease. Confidence/conflicts: High for the listed U.S. label/indication claims; no conflict identified.
  • sotatercept-csrk (Winrevair); macitentan/tadalafil (Opsynvi); selexipag (Uptravi); riociguat (Adempas); treprostinil inhalation powder or solution (Tyvaso DPI / Tyvaso)[2]FDA-approvednot biomarker-defined; WHO group and functional class context; Adult PAH disease-targeted therapy across activin signaling inhibition, endothelin/PDE5 combination, prostacyclin pathway agonist, soluble guanylate cyclase stimulation, and inhaled prostacyclin mimetic pathways. · Labels do not rank therapies or replace specialist risk stratification. Pregnancy/embryo-fetal toxicity, REMS, hypotension, bleeding, pulmonary veno-occlusive disease, liver issues, route/device burden, and drug-interaction caveats vary by product. PAH must be distinguished from other pulmonary hypertension groups, especially CTEPH and PH due to left-heart or lung disease. Confidence/conflicts: High for the listed U.S. label/indication claims; no conflict identified.

Australia

  • ambrisentan; bosentan; epoprostenol; iloprost; macitentan; macitentan/tadalafil (Opsynvi); riociguat; selexipag; sildenafil; tadalafil[3]Approvednot biomarker-defined; WHO functional class and specialist-confirmed PAH access context; Australian PBS PAH access framework for monotherapy, dual therapy, and triple therapy; WHO FC III/IV combination-therapy definitions are described in the source. · The PBS pathway is not a treatment recommendation or sequencing rule for an individual person. It requires PBS eligibility, restriction criteria, S100 hospital arrangements, and completion by a physician with PAH expertise. Confidence/conflicts: High for Australian PBS access framework and listed agent classes; exact Schedule restrictions for each product remain source-pending. No conflict identified. Availability/reimbursement outside the approving regulator not established.
  • ambrisentan; bosentan; epoprostenol; iloprost; macitentan; macitentan/tadalafil (Opsynvi); riociguat; selexipag; sildenafil; tadalafil[3]Approvednot biomarker-defined; WHO functional class and specialist-confirmed PAH access context; Australian PBS PAH access framework for monotherapy, dual therapy, and triple therapy; WHO FC III/IV combination-therapy definitions are described in the source. · The PBS pathway is not a treatment recommendation or sequencing rule for an individual person. It requires PBS eligibility, restriction criteria, S100 hospital arrangements, and completion by a physician with PAH expertise. Confidence/conflicts: High for Australian PBS access framework and listed agent classes; exact Schedule restrictions for each product remain source-pending. No conflict identified. Availability/reimbursement outside the approving regulator not established.
  • macitentan + tadalafil (Opsynvi)[4]TGA-registered (Australia)adult maintenance/substitution treatment for PAH WHO Group 1 FC II-III in Australia, as stated by the product information. · The product information warns that the AusPAR-approved PI may have been superseded and points to the TGA site for the most recent PI. This entry records the fetched AusPAR attachment, not current PBS subsidy. Confidence/conflicts: Medium-high. The fetched PI attachment itself notes it may have been superseded; current PI/PBS status remains a follow-up. Availability/reimbursement outside the approving regulator not established.
  • macitentan + tadalafil (Opsynvi)[4]TGA-registered (Australia)adult maintenance/substitution treatment for PAH WHO Group 1 FC II-III in Australia, as stated by the product information. · The product information warns that the AusPAR-approved PI may have been superseded and points to the TGA site for the most recent PI. This entry records the fetched AusPAR attachment, not current PBS subsidy. Confidence/conflicts: Medium-high. The fetched PI attachment itself notes it may have been superseded; current PI/PBS status remains a follow-up. Availability/reimbursement outside the approving regulator not established.
  • sapropterin dihydrochloride (Kuvan)[5]TGA-registered (Australia)PAH deficiency; BH4-responsive phenotype; sapropterin-responsive PKU or BH4-deficiency HPA in Australia. · The PBAC source is a reimbursement appraisal and historic listing discussion, not a current individual access rule. Response testing and current subsidy criteria need confirmation with Australian metabolic specialists and current PBS/ARTG materials. Confidence/conflicts: Medium-high for Australian registration/ARTG context; current access criteria remain source-pending. No conflict identified.
  • selexipag (Uptravi)[6]Approvednot biomarker-defined; Australian PBS combination-therapy access context for PAH involving selexipag. · The 2021 media release documents listing history; current eligibility depends on the live PBS Schedule and Services Australia authority process. This finding does not establish suitability for monotherapy or any non-PAH pulmonary hypertension group. Confidence/conflicts: Medium-high for PBS listing/access context; current item-level criteria require live Schedule verification. No conflict identified. Availability/reimbursement outside the approving regulator not established.
  • selexipag (Uptravi)[6]Approvednot biomarker-defined; Australian PBS combination-therapy access context for PAH involving selexipag. · The 2021 media release documents listing history; current eligibility depends on the live PBS Schedule and Services Australia authority process. This finding does not establish suitability for monotherapy or any non-PAH pulmonary hypertension group. Confidence/conflicts: Medium-high for PBS listing/access context; current item-level criteria require live Schedule verification. No conflict identified. Availability/reimbursement outside the approving regulator not established.
  • sotatercept (Winrevair)[7]TGA-registered (Australia)adult PAH WHO FC II or III in combination with standard therapy, as stated by the TGA AusPAR. · The AusPAR states Winrevair is included in Australia's Black Triangle Scheme and records monitoring-related dosage-modification discussion for haemoglobin increase or platelet-count decrease. This entry records regulator status, not PBS subsidy or eligibility. Confidence/conflicts: High for Australian regulatory status; no conflict identified. Availability/reimbursement outside the approving regulator not established.
  • sotatercept (Winrevair)[7]TGA-registered (Australia)adult PAH WHO FC II or III in combination with standard therapy, as stated by the TGA AusPAR. · The AusPAR states Winrevair is included in Australia's Black Triangle Scheme and records monitoring-related dosage-modification discussion for haemoglobin increase or platelet-count decrease. This entry records regulator status, not PBS subsidy or eligibility. Confidence/conflicts: High for Australian regulatory status; no conflict identified. Availability/reimbursement outside the approving regulator not established.

Thailand

  • macitentan (Opsumit)[8]Approvedlong-term treatment of WHO Functional Class II to III PAH; source states Opsumit is effective as monotherapy or with phosphodiesterase-5 inhibitors or inhaled/oral prostanoids. · Treatment should only be initiated by a physician experienced in PAH. The label lists pregnancy and women of child-bearing potential not using reliable contraception as contraindications, warns not to initiate with aminotransferases above 3 times upper normal level, and notes monitoring considerations for liver enzymes, hemoglobin, PVOD/pulmonary edema, renal impairment, and drug interactions. This entry does not establish Thai reimbursement, specialist-center availability, or pediatric use under age 12. Confidence/conflicts: High for Thai NDI Opsumit PAH label and authorization details; no conflict identified. Reimbursement/access remains unresolved.
  • selexipag (Uptravi)[9]ApprovedPAH WHO Functional Class II, III, or IV symptoms across the etiologic categories listed in the source; treatment initiation and monitoring by a physician experienced in PAH. · The source states dose titration is individualized to the highest tolerated dose and notes adverse reactions reflecting the mode of action, interruption/restart considerations, CYP2C8 inhibitor interactions, hepatic impairment limitations, severe coronary/unstable angina and recent myocardial infarction contraindications, and that pediatric safety and efficacy are not established with administration in pediatric population not recommended. This entry does not establish Thai reimbursement or specialist-center availability. Confidence/conflicts: High for Thai NDI Uptravi PAH label content; no conflict identified. Reimbursement/access remains unresolved.

Canada

  • epoprostenol; treprostinil; bosentan; ambrisentan; macitentan; sildenafil; tadalafil; riociguat[10]Approvednot biomarker-defined; WHO FC II-III or III-IV product-context distinctions in CADTH report; Canadian class-level policy/recommendations context for advanced PAH therapy and supportive therapy. · This is a 2015 CADTH policy report and predates newer PAH therapies such as selexipag, macitentan/tadalafil, and sotatercept. Use it as class-framework history, not a current complete label or formulary list. Confidence/conflicts: Medium-high for 2015 Canadian class framework; not current-complete for newer agents. No direct conflict, but temporal limitation is material. Availability/reimbursement outside the approving regulator not established.
  • epoprostenol; treprostinil; bosentan; ambrisentan; macitentan; sildenafil; tadalafil; riociguat[10]Approvednot biomarker-defined; WHO FC II-III or III-IV product-context distinctions in CADTH report; Canadian class-level policy/recommendations context for advanced PAH therapy and supportive therapy. · This is a 2015 CADTH policy report and predates newer PAH therapies such as selexipag, macitentan/tadalafil, and sotatercept. Use it as class-framework history, not a current complete label or formulary list. Confidence/conflicts: Medium-high for 2015 Canadian class framework; not current-complete for newer agents. No direct conflict, but temporal limitation is material. Availability/reimbursement outside the approving regulator not established.
  • macitentan + tadalafil (Opsynvi)[11]Approvedlong-term treatment/substitution context for adult PAH WHO Group 1 FC II-III in Canada, as stated by the Canadian product monograph. · The monograph states pediatric safety and efficacy are not established and Health Canada has not authorized pediatric use. It includes pregnancy and nitrate/riociguat contraindication caveats; this entry is not a dosing or eligibility recommendation. Confidence/conflicts: High for Canadian label status; no conflict identified. Availability/reimbursement outside the approving regulator not established.
  • macitentan + tadalafil (Opsynvi)[11]Approvedlong-term treatment/substitution context for adult PAH WHO Group 1 FC II-III in Canada, as stated by the Canadian product monograph. · The monograph states pediatric safety and efficacy are not established and Health Canada has not authorized pediatric use. It includes pregnancy and nitrate/riociguat contraindication caveats; this entry is not a dosing or eligibility recommendation. Confidence/conflicts: High for Canadian label status; no conflict identified. Availability/reimbursement outside the approving regulator not established.
  • sotatercept (Winrevair)[12]Health Canada approvedadd-on to standard PAH therapy in adult WHO Group 1 PAH; product monograph includes FC II-IV while SBD records FC II-III. · The fetched Canadian sources differ in functional-class wording: the current product monograph includes FC IV, while the SBD records FC II or III. This scope difference should be checked against the live Drug Product Database and payer criteria. Health Canada has not authorized pediatric use. Confidence/conflicts: Medium-high because Canadian functional-class wording differs between product monograph and SBD; both are recorded with attribution. Availability/reimbursement outside the approving regulator not established.
  • sotatercept (Winrevair)[12]Health Canada approvedadd-on to standard PAH therapy in adult WHO Group 1 PAH; product monograph includes FC II-IV while SBD records FC II-III. · The fetched Canadian sources differ in functional-class wording: the current product monograph includes FC IV, while the SBD records FC II or III. This scope difference should be checked against the live Drug Product Database and payer criteria. Health Canada has not authorized pediatric use. Confidence/conflicts: Medium-high because Canadian functional-class wording differs between product monograph and SBD; both are recorded with attribution. Availability/reimbursement outside the approving regulator not established.
  • sotatercept (Winrevair)[13]ApprovedCanadian public-drug-plan reimbursement recommendation for add-on sotatercept in WHO Group 1 PAH, FC II or III. · CADTH is a reimbursement recommendation, not a prescribing label; it states conditions and cost considerations and does not establish individual access or provincial implementation. Confidence/conflicts: High for CADTH recommendation context; payer implementation remains jurisdiction-specific.
  • sotatercept (Winrevair)[13]ApprovedCanadian public-drug-plan reimbursement recommendation for add-on sotatercept in WHO Group 1 PAH, FC II or III. · CADTH is a reimbursement recommendation, not a prescribing label; it states conditions and cost considerations and does not establish individual access or provincial implementation. Confidence/conflicts: High for CADTH recommendation context; payer implementation remains jurisdiction-specific.
  • specialist diagnostic framework; echocardiography, cardiac MRI, right-heart catheterization context[14]Standard option (per Canadian Cardiovascular Society / Canadian Thoracic Society)not biomarker-defined; Canadian specialist diagnostic and classification framework before selecting PAH-targeted therapy. · This is not a drug label or payer source. It is included to prevent conflating PAH with other pulmonary hypertension groups such as left-heart disease, lung disease/hypoxia, or CTEPH. Confidence/conflicts: High for Canadian diagnostic/classification framework. No conflict identified.

출처

  1. U.S. Food and Drug Administration — official drug label · official drug label
  2. U.S. Food and Drug Administration — official drug label · official drug label
  3. Services Australia — PBS complex drugs program guidance · PBS complex drugs program guidance
  4. Therapeutic Goods Administration (TGA) / Janssen-Cilag product information — AusPAR product-information attachment · AusPAR product-information attachment
  5. Therapeutic Goods Administration (TGA) — Australian Public Assessment Report · Australian Public Assessment Report
  6. Australian Government Department of Health, Disability and Ageing — ministerial PBS listing announcement · ministerial PBS listing announcement
  7. Therapeutic Goods Administration (TGA) — Australian Public Assessment Report · Australian Public Assessment Report
  8. Thai National Drug Information / Thai FDA-MOPH — physician label PDF / regulator drug-information repository · physician label PDF / regulator drug-information repository
  9. Thai National Drug Information / Thai FDA-MOPH — hybrid product information PDF / regulator drug-information repository · hybrid product information PDF / regulator drug-information repository
  10. CADTH / Canada's Drug Agency — recommendations report / comparative efficacy-safety-cost-effectiveness · recommendations report / comparative efficacy-safety-cost-effectiveness
  11. Janssen Canada / Health Canada product monograph format — official product monograph · official product monograph
  12. Merck Canada / Health Canada product monograph format — official product monograph · official product monograph
  13. CADTH via NCBI Bookshelf — reimbursement recommendation / health technology review · reimbursement recommendation / health technology review
  14. Canadian Cardiovascular Society / Canadian Thoracic Society — professional society position statement · professional society position statement

위 내용은 공식 규제·접근 상태일 뿐, 의학적 조언이나 추천이 아니고, 적격성을 판단하지도 않습니다. 어떤 선택지가 적합한지는 환자의 상황과 종양내과 팀에 달려 있습니다. 규제 상태는 바뀔 수 있으니 표시된 출처에서 확인하세요. 임상 세부 내용은 영문이 정본입니다. 최종 확인 2026-06-12.