CONTACT PERSON PRESCRIBER PHONE PRESCRIBER FAX. PRESCRIBER ADDRESS CITY STATE ZIP Formulary Drug? Yes No

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1 ORAL DRUGS FOR PULMONARY ARTERIAL HYPERTENSION (PAH) BOSENTAN (Tracleer ), AMBRISENTAN (Letairis ), SILDENAFIL (Revatio ), TADALAFIL (Adcirca ), RIOCIGUAT (Adempas ), MACITENTAN (Opsumit ), TREPROSTINIL (Orenitram ), SELEXIPAG (Uptravi ) PRIOR REVIEW/CERTIFICATION FAXBACK FORM INCOMPLETE FORMS MAY DELAY PROCESSING ALL NC PROVIDERS MUST PROVIDE THEIR 5-DIGIT Blue Cross NC PROVIDER ID# BELOW PRESCRIBER NAME PRESCRIBER NPI [REQUIRED] Blue Cross NC PROV ID # / TAX ID [out of state] CONTACT PERSON PRESCRIBER PHONE PRESCRIBER FAX PRESCRIBER ADDRESS CITY STATE ZIP Formulary Drug? Yes No PATIENT NAME Blue Cross NC ID DATE OF BIRTH GENDER Please answer the following questions and provide diagnosis code: 1. Please check the medication requested: Adcirca Adempas Letairis Opsumit Orenitram Revatio suspension Revatio (brand) tablets Sildenafil tablets Tracleer Uptravi M F Diagnosis Code: 2. Will the requested medication be used for the treatment of pulmonary arterial hypertension (PAH/WHO Group 1 refer to pg 2 for definition)? yes No 3. Are the patient s symptoms Functional Class 2 through 4 (refer to pg 3)?.. Yes No 4. Is the patient currently stable on the requested medication?...yes No 5. Has the patient tried and failed, is intolerant to, or has a clinical contraindication to therapy with a calcium channel blocker (Amlodipine, Diltiazem or Nifedipine)?....Yes No 6. Has the patient undergone a vasoreactivity test?...yes No A. If Yes, did the patient demonstrate acute vasoreactivity?.... Yes No B. If No, does the patient have a contraindication to vasoreactivity testing?.. Yes No 7. FOR ADEMPAS ONLY Will the patient be using Adempas for treatment of Chronic Thromboembolic Pulmonary Hypertension (CTEPH/WHO Group 4)?...Yes No 8. FOR ORENITRAM AND UPTRAVI ONLY Has the patient remained symptomatic despite stable doses of an ERA (ex. Tracleer, Opsumit, Letairis) and/or a PDE5-1 (ex. Adcirca, Revatio)?.. Yes No Does the patient have a documented intolerance, FDA labeled contraindication, or hypersensitivity to BOTH an ERA (ex. Tracleer, Opsumit, Letairis) and a PDE5-1 (ex. Adcirca, Revatio).. Yes No Please certify the following by signing and dating below: I certify that I have been authorized to request prior review and certification for the above requested service(s). I further certify that my patient s medical records accurately reflect the information provided. I understand that Blue Cross NC may request medical records for this patient at any time in order to verify this information. I further understand that if Blue Cross NC determines this information is not reflected in my patient s medical records, Blue Cross NC may request a refund of any payments made and/or pursue any other remedies available. Prescriber s Signature (Required): Date: For Blue Cross NC members, fax form to Last Revision Date: September 2017 Page 1

2 WHO Groups Group 1- Pulmonary arterial hypertension (PAH) Idiopathic (IPAH) Heritable (HPAH) o Bone morphogenetic protein receptor type 2 (BMPR2) o Activin receptor-like kinase 1 gene (ALK1), endoglin (with or without haemorrhagic telangiectasia) o Unknown Drug- and toxin-induced Associated with (APAH): o Connective tissue diseases o Human immunodeficiency virus (HIV) infection o Portal hypertension o Congenital heart disease (CHD) o Schistosomiasis o Chronic haemolytic anaemia Persistent pulmonary hypertension of the newborn (PPHN) Group 1'- Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary haemangiomatosis (PCH) Group 2- Pulmonary hypertension due to left heart diseases Systolic dysfunction Diastolic dysfunction Valvular disease Group 3- Pulmonary hypertension due to lung diseases and/or hypoxemia Chronic obstructive pulmonary disease (COPD) Interstitial lung disease (ILD) Other pulmonary diseases with mixed restrictive and obstructive pattern Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental abnormalities Group 4- Chronic thromboembolic pulmonary hypertension (CTEPH) Group 5- PH with unclear multifactorial mechanisms Haematological disorders: myeloproliferative disorders, splenectomy Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis Last Revision Date: September 2017 Page 2

3 WHO Functional Class The World Health Organization (WHO) has developed a system to help doctors determine how limited a patient is in their ability to do the activities of daily living. In general, patients with more severe PH tend to have a higher functional class. Class I: Patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope. Class II: Patients with pulmonary hypertension resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope. Class III: Patients with pulmonary hypertension resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain, or near syncope. Class IV: Patients with pulmonary hypertension with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity. Non-Discrimination and Accessibility Notice Discrimination is Against the Law Blue Cross and Blue Shield of North Carolina ( Blue Cross NC ) complies with applicable Federal civil rights laws and does not discriminate on the basis of race, color, national origin, age, disability, or sex. Blue Cross NC does not exclude people or treat them differently because of race, color, national origin, age, disability, or sex. Blue Cross NC: Provides free aids and services to people with disabilities to communicate effectively with us, such as: - Qualified interpreters - Written information in other formats (large print, audio, accessible electronic formats, other formats) Provides free language services to people whose primary language is not English, such as: - Qualified interpreters - Information written in other languages Last Revision Date: September 2017 Page 3

4 If you need these services, contact Customer Service , TTY and TDD, call If you believe that Blue Cross NC has failed to provide these services or discriminated in another way on the basis of race, color, national origin, age, disability, or sex, you can file a grievance with: Blue Cross NC, PO Box 2291, Durham, NC 27702, Attention: Civil Rights Coordinator- Privacy, Ethics & Corporate Policy Office, Telephone , Fax , TTY You can file a grievance in person or by mail, fax, or . If you need help filing a grievance, Civil Rights Coordinator - Privacy, Ethics & Corporate Policy Office is available to help you. You can also file a civil rights complaint with the U.S. Department of Health and Human Services, Office for Civil Rights, electronically through the Office for Civil Rights Complaint Portal, available at or by mail or phone at: U.S. Department of Health and Human Services 200 Independence Avenue, SW Room 509F, HHH Building Washington, D.C , (TDD). Complaint forms are available at This Notice and/or attachments may have important information about your application or coverage through Blue Cross NC. Look for key dates. You may need to take action by certain deadlines to keep your health coverage or help with costs. You have the right to get this information and help in your language at no cost. Call Customer Service ATTENTION: If you speak another language, language assistance services, free of charge, are available to you. Call ATENCIÓN: Si habla español, tiene a su disposición servicios gratuitos de asistencia lingüística. Llame al 注意 : 如果您講廣東話或普通話, 您可以免費獲得語言援助服務 請致電 (TTY: ) CHÚ Ý: Nếu bạn nói Tiếng Việt, có các dịch vụ hỗ trợ ngôn ngữ miễn phí dành cho bạn. Gọi số 주의 : 한국어를사용하시는경우, 언어지원서비스를무료로이용하실수있습니다 (TTY: ) 번으로전화해주십시오. Last Revision Date: September 2017 Page 4

5 ATTENTION : Si vous parlez français, des services d'aide linguistique vous sont proposés gratuitement. Appelez le (ATS : ). ملحوظة: إذا كنت تتحدث اللغة العربية فإن خدمات المساعدة اللغوية تتوافر لك بالمجان. اتصل برقم المبرقة الكاتبة: LUS CEEV: Yog tias koj hais lus Hmoob, cov kev pab txog lus, muaj kev pab dawb rau koj. Hu rau ВНИМАНИЕ: Если вы говорите на русском языке, то вам доступны бесплатные услуги перевода. Звоните (телетайп: ). PAUNAWA: Kung nagsasalita ka ng Tagalog, maaari kang gumamit ng mga serbisyo ng tulong sa wika nang walang bayad. Tumawag sa (TTY: ). સ ચન : જ તમ ગ જર ત બ લત હ, ત નન:સ લ ક ભ ષ સહ ય સ વ ઓ તમ ર મ ટ ઉપલબ ધ છ. ફ ન કર ច ណ ប រស នបរ ប កអ នកន យ យជ ភ ស ខ ម រ បសវ កម ជ ន យខ នកភ ស ម ននតល ជ នសប ម រ ប កអ នកប យម នគ តថ ល ស ម ទ ន ក ទ នងត ម រយ បលម (TTY: ) ACHTUNG: Wenn Sie Deutsch sprechen, stehen Ihnen kostenlos sprachliche Hilfsdienstleistungen zur Verfügung. Rufnummer: (TTY: ). ध य न द : यदद आप द न द ब लत त आपक दलए म फ त म भ ष स यत स व ए उपलब ध (TTY: ) पर क ल कर ໂປດຊາບ: ຖ າວ າ ທ ານເວ າພາສາ ລາວ, ການບ ລການຊ ວຍເຫອດ ານພາສາ, ໂດຍບ ເສ ຽຄ າ, ແມ ນມ ພ ອມໃຫ ທ ານ. ໂທຣ 注意事項 : 日本語を話される場合 無料の言語支援をご利用いただけます (TTY: ) まで お電話にてご連絡ください Last Revision Date: September 2017 Page 5

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