보험인정기준 상세내용

■ 고시 개정 전체내용

1. 허가사항 범위 내에서 [일반원칙] 폐동맥고혈압약제 “세부사항” 및 아래와 같은 기준으로 투여 시 요양급여를 인정하며, 동 인정기준 이외에는 약값 전액을 환자가 부담토록 함.

- 아 래 -

가. 대상환자

WHO 기능분류 단계 Ⅱ, Ⅲ에 해당하는 폐동맥 고혈압(WHO Group Ⅰ) 환자 중 아래 질환으로 진단이 확인된 환자

1) 특발성 폐동맥 고혈압(Idiopathic pulmonary arterial hypertension)

2) 가족성 폐동맥 고혈압(Familial pulmonary arterial hypertension)

3) 교원성혈관질환과 관련된 폐동맥 고혈압(Pulmonary arterial hypertension associated with collagen vascular disease)

4) 선천성 심장질환과 관련된 폐동맥 고혈압(Pulmonary arterial hypertension associated with congenital heart diseases)

5) 문맥고혈압이 동반된 폐동맥 고혈압(Pulmonary arterial hypertension associated with portal hypertension)



나. 치료 효과에 대해 정기적인 평가가 이루어져야 하며, 허가사항 중 주의사항(약물상호작용, 부작용 등)을 고려하여 투여하여야 함.



2. ‘신생아지속성 폐고혈압(Persistent pulmonary hypertension of the newborn, PPHN)’에 대하여 단독요법으로 투여 시 요양급여를 인정함.

※ 단, ‘환자 상태가 급속도로 악화되고, 동 약제만으로는 임상적 반응이 충분하지 않은 경우’ 에는 iloprost 흡입액과 병용을 투여소견서 첨부 시 사례별로 인정함.



3. 금기환자

1) 이 약의 성분에 과민반응을 보이는 환자

2) 어떠한 형태의 유기 질산염 제제 (니트로글리세린, 질산이소소르비드, 아밀나이트레이트, 니트로프루싯나트륨)라도 정기적으로 혹은 간헐적으로 복용하는 환자

3) 중증 간부전환자

4) 저혈압 (혈압 90/50mmHg 미만) 또는 조절되지 않는 고혈압 환자 (휴식시 수축기 혈압 170mmHg 초과, 휴식기 이완기 혈압 100mmHg 초과)

5) 지난 6개월 이내 심근경색, 뇌졸중, 생명을 위협하는 부정맥 등이 있었던 환자

6) 색소성 망막염(retinitis pigmentosa) 환자 (이들 환자 중 일부는 망막 포스포디에스테라제의 유전성질환을 가짐)

7) 이전의 PDE5 저해제 복용 여부와 관계없이, 비동맥전방허혈성시신경증(Non-arteritic anterior ischemic optic neuropathy, NAION)으로 인해 한쪽 눈의 시력이 손실된 환자





■ 고시 개정 고시번호(시행일자)

고시 제2019-93호(2019.6.7.)



■ 고시 개정 사유

투여 요법(단독요법 및 병용요법)의 [일반원칙]화.



■ 관련문헌 등

· Pharmacotherapy: A Pathophysiologic Approach, 10e > Chapter 28: Pulmonary Arterial Hypertension

· Current Diagnosis & Treatment: Pediatrics, 24e(2018)

· Pediatric Critical Care. 5e(2017)

· Nelson Textbook of Pediatircs. 20e(2016)

· Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 8e (2016)

· Pediatric Pulmonary Hypertension (AHA/ATS Guideline 2015)

· Persistent Pulmonary Hypertension of the Newborn. NeoReviews an official journal of the American Academy of Pediatrics. 2015

· Kelly LE et al. Sildenafil for pulmonary hypertension in neonates. cochrane review. 4 August 2017.

· Baquero H et al. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded study. Pediatrics. 2006 Apr;117(4):1077-83.

· Al Omar S et al. Effect of early adjunctive use of oral sildenafil and inhaled nitric oxide on the outcome of pulmonary hypertension in newborn infants. A feasibility study. J Neonatal Perinatal Med. 2016 Sep 16;9(3):251-9

· Uslu S et al. A comparison of magnesium sulphate and sildenafil in the treatment of the newborns with persistent pulmonary hypertension: a randomized controlled trial. J Trop Pediatr. 2011 Aug;57(4):245-50.

· Vargas-Origel et al. The use of sildenafil in persistent pulmonary hypertension of the newborn. Am J Perinatol. 2010 Mar;27(3):225-30.

· Mulligan C et al. Inhaled iloprost for the control of acute pulmonary hypertension in children: a systematic review. Pediatr Crit Care Med. 2012 Jul;13(4):472-80.

· Kahveci H et al. Oral sildenafil and inhaled iloprost in the treatment of pulmonary hypertension of the newborn. Pediatr Pulmonol. 2014 Dec;49(12):1205-13.

· Kiran More et al. Endothelin receptor antagonists for persistent pulmonary hypertension in term and late preterm infants. cochrane Intervention Review. 2016

· Steinhorn RH et al. Bosentan as Adjunctive Therapy for Persistent Pulmonary Hypertension of the Newborn: Results of the Randomized Multicenter Placebo-Controlled Exploratory Trial. J Pediatr. 2016 Oct;177:90-96.e3

· 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

· Simonneau G et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002 Mar 15;165(6):800-4.

· Pulido T et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369:809？-818.

· Coghlan JG et al. Targeting the Prostacyclin Pathway with Selexipag in Patients with Pulmonary Arterial Hypertension Receiving Double Combination Therapy: Insights from the Randomized Controlled GRIPHON Study. Am J Cardiovasc Drugs. 2018 Feb;18(1):37-47.

· Hoeper MM et al. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2006 Oct;28(4):691-4.

· McLaughlin VV et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006 Dec 1;174(11):1257-63.

· Han X et al. Treatment of Pulmonary Arterial Hypertension Using Initial Combination Therapy of Bosentan and Iloprost. Respir Care. 2017 Apr;62(4):489-496.

· Strange G et al. Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand. Heart Lung Circ. 2017 Sep 20. pii: S1443-9506(17)31394-X.

· Sitbon O et al. Initial dual oral combination therapy in pulmonary arterial hypertension. Eur Respir J. 2016 Jun;47(6):1727-36.

· Nicole F. Ruopp, et al. Gaps and Controversies of New Treatment Recommendations in Recent Pulmonary Hypertension Guidelines: What We Know and What We Don’t. Advances in Pulmonary Hypertension.(2017)

· William Hopkins, et al. Treatment of pulmonary hypertension in adults, UpToDate Sep 26, 2018.

· Clinical Commissioning Policy Proposition: Selexipag for treating pulmonary arterial hypertension (adults). Prepared by the National Institute for Health and Care Excellence (NICE) Commissioning Support Programme. 2018

· Drugs for Pulmonary Arterial Hypertension: Comparative Efficacy, Safety, and Cost-Effectiveness ―Recommendations Report, March 2015

· Pharmaceutical Benefits Scheme. Post-market Review of Medicines to treat Pulmonary Arterial Hypertension, May 2018



■ 변경 전 고시번호(시행일자)

고시 제2019-21호(2019.2.1.)