Relax and Take a Deep Breath: What s New in the Treatment of Pulmonary Hypertension
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1 Relax and Take a Deep Breath: What s New in the Treatment of Pulmonary Hypertension Nancy D. Bair, RN,MSN, CNS-BC Pulmonary Hypertension Mid-level Provider
2
3 History of PAH First recognized on clinical & pathological grounds in 1891 Named & chronicled in 1950 s through cardiac catheterization 1973 Geneva WHO PH classification-2 groups 1998 Evian World Health PH Symposium-5 groups 2013 Nice 5 th World Symposium
4 2013 Revised Nomenclature & Classification of PAH Pulmonary Arterial Hypertension- WHO Group I IPAH Heritable PAH Drugs and toxin induced APAH Collagen vascular disease Congenital systemic to pulmonary shunts Portal Hypertension HIV infection Shistosomiasis 1 Pulmonary veno-occlusive disease (PVOD) &/or pulmonary capillary hemangiomatosis (PCH) 1 Persistent pulmonary hypertension of the newborn
5 2013 Revised Nomenclature & Classification of PAH Pulmonary Venous Hypertension -WHO Group II Left heart systolic dysfunction Left heart diastolic dysfunction Valvular disease Congenital/acquired left heart inflow/outflow tract obstruction & congenital cardiomyopathy
6 2013 Revised Nomenclature & Classification of PAH Pulmonary hypertension due to lung diseases &/or hypoxia- WHO Group III COPD Interstitial lung disease Mixed obstructive and restrictive pattern Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental lung diseases
7 2013 Revised Nomenclature & Classification of PAH Chronic Thromboembolic PH (CTEPH)- WHO Group IV
8 2013 Revised Nomenclature & Classification of PAH Pulmonary hypertension with unclear multifactorial mechanisms-who Group V Hematologic Disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy Systemic disorders: sarcoidosis, pulmonary hystiocytosis, lymphangioleiomyomatosis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Other: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH
9 PAH Definition Mean Pulmonary Artery Pressure >25mm Hg at rest PCWP< 15mm Hg PVR > 3 Wood units
10 Pathophysiology of PAH: Pathways of Disease Copyright [2004] Massachusetts Medical Society. All rights reserved. 10 Humbert et al. N Engl J Med. 2004;351:
11 Imbalance of Vasoactive Mediators Prostacyclin-potent vasodilator & antiproliferative activity Nitric Oxide-potent vasodilator & antiproliferative activity Endothelin & Thromboxane- potent vasoconstrictor & proliferative activity
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13 Treatment of PAH No Cure at This Time Transplant and Calcium Channel Blockers only treatment 20 years ago Flolan, Tracleer,Veletri, Remodulin TM, Ventavis, Revatio TM, Letairis TM, Tyvaso TM Adcirca TM, Adempas, Opsumit Orenitram Other drugs in clinical trials in U.S. and abroad
14 Pathophysiology of PAH: Pathways of Disease Copyright [2004] Massachusetts Medical Society. All rights reserved. 14 Humbert et al. N Engl J Med. 2004;351:
15 Flolan, Veletri (epoprostenol) Remodulin (treprostinil) Potent vasodilator of pulmonary and systemic arterial beds Action: Vasodilator, Inhibits platelet aggregation and smooth muscle proliferation
16 IV therapy Flolan, Veletri, & Remodulin Side effects: Headache, jaw pain, flushing, nausea, diarrhea Long term indwelling catheter Need for ice to maintain solution- Flolan only Education about care of catheter Education about mixing and infusing the drug
17 Canè Crono Five
18 CADD MS-3 by Net
19
20 Inhaled Therapy Iloprost(Ventavis) Specialized nebulizer Frequent dosing (6-9 x/day) Similar side effect profile to other prostanoids
21 Tyvaso TM Inhaled prostacyclin 9 inhalations 4 times/day Handheld, battery-powered nebulizer
22 Orenitram (treprostinil) Oral doses are titrated 0.25 mg bid Side Effects: headache, nausea, diarrhea
23 Pathophysiology of PAH: Pathways of Disease Copyright [2004] Massachusetts Medical Society. All rights reserved. 23 Humbert et al. N Engl J Med. 2004;351:
24 Endothelin Receptor Antagonists (ETRA) ETRAs potential for teratogenicity and male infertility (Counseling, barrier contraception, monthly HCG) Liver dysfunction (LFTs q month-tracleer) Anemia (CBC q 3 month-tracleer)
25 Pathophysiology of PAH: Pathways of Disease Copyright [2004] Massachusetts Medical Society. All rights reserved. 25 Humbert et al. N Engl J Med. 2004;351:
26 Sildenafil and Tadalafil PDE-5 Inhibitors Side effects: headache, muscle aches, flushing
27 Adempas (riociquat) Soluble guanylate cyclase stimulator Vasodilator, anti-platelet, anti-fibrotic CTEPH (non-op) and PAH Cannot use w/ revatio or adcirca
28 Calcium Channel Blockers Vasodilation of smooth muscle in pulmonary arteries 10-20% responders Increases cardiac output Decreases mpap Requires a vasoreactive challenge Potential for severe adverse reactions systemic hypotension pulmonary edema right ventricular failure death
29 Updated Guidelines: Inadequate Clinical Response to Initial PAH Therapy Inadequate Clinical Response Sequential Combination Therapy ERAs Consider eligibility for lung transplantation Prostacyclin PDE-5i or sgcs Referral for lung transplantation Inadequate response on maximal therapy Balloon atrial septostomy Adapted from: Galie N, et al. J Am Coll Cardiol. 2013;62:D60-72.
30 Marvel at the beauty of winter!
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