Pulmonary hypertension

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Pulmonary hypertension

  1. 1. Pulmonary Artery Hypertension Natalie Bermudez, RN, BSN, MS Clinical Educator for Telemetry Telemetry Course
  2. 2. Statistics <ul><li>PH is rare disease with an incidence of about 2-3 per million per year and a prevalence of about 15 per million. </li></ul><ul><li>Women, usually age 30s to 40s, are almost three times as likely to present with idiopathic PAH than men. </li></ul>
  3. 3. Pulmonary Artery Pressures <ul><li>In a healthy adult, normal pulmonary artery pressure (PAP) ranges from 12 to 16 mmHg 3 (Vacca, 2009, p. 37) </li></ul><ul><li>For Pulmonary HTN </li></ul><ul><li>PAP > 25 mmHg at rest </li></ul><ul><li>PAP > 30 mmHg with activity </li></ul>
  4. 4. 4 Classes of PAH <ul><li>Class I – No symptom-induced limits on physical activity </li></ul><ul><li>Class II – Slight symptom-induced limits on physical activity </li></ul><ul><li>Class III – Marked symptom-induced limits on physical activity </li></ul><ul><li>Class IV – Right-sided heart failure with dyspnea and fatigue at rest, and an inability to perform any physical activity without symptoms </li></ul><ul><li>(Vacca, 2009, p. 38) </li></ul>
  5. 5. PAH Pathophysiology <ul><li>The abnormally high blood pressure is associated with changes in the small blood vessels (capillaries) in the lungs, resulting in an increased resistance to blood flowing through the vessels </li></ul>
  6. 6. PAH & the Endothelium <ul><li>Changes are due to weakening of the endothelial cells (lining of the vessels) which causes enlargement of the cells and narrowing of lumen leading to increased resistance </li></ul>
  7. 7. Types of PAH <ul><li>Idiopathic PAH (IPAH) </li></ul><ul><li>Familial PAH (FPAH) </li></ul><ul><li>Associated PAH (APAH) </li></ul>
  8. 8. Idiopathic PAH <ul><li>“ IPAH is diagnosed when the cause of PAH is undetermined to be unknown and all other causative conditions have been excluded.” </li></ul><ul><li>(Moser & Riegel, 2008, p. 1336) </li></ul>
  9. 9. Idiopathic PAH <ul><li>AKA Primary PAH </li></ul><ul><li>“ IPAH is mostly a disease of younger women, with female-to-male ratio of 2-3 to 1.” (Moser & Riegel, 2008, p. 1337) </li></ul><ul><li>Most common in women; ages 20 – 40 </li></ul><ul><li>Mortality rate is high </li></ul><ul><li>Fatalities occur within 3 – 4 years </li></ul>
  10. 10. Idiopathic PAH <ul><li>STATISTICS </li></ul><ul><li>“ IPAH has an estimated annual occurrence of 1 to 2 cases per million.” (Moser & Riegel, 2008, p. 1337) </li></ul>
  11. 11. Idiopathic PAH <ul><li>SURVIVAL RATES </li></ul><ul><li>The estimated median survival for untreated IPAH patients from diagnosis was 2.8 years, with group survival rates of: </li></ul><ul><li>68% at 1 year </li></ul><ul><li>48% at 3 years </li></ul><ul><li>34% at 5 years </li></ul><ul><li>(Moser & Riegel, 2008) </li></ul>
  12. 12. Idiopathic PAH <ul><li>PATHOPHYSIOLOGY: </li></ul><ul><li>Primary PAH </li></ul><ul><li>Lining of Pulmonary Arteries Thicken </li></ul><ul><li>Lumens Become Narrow </li></ul><ul><li>Impair Ability to Become Distended </li></ul><ul><li>Vascular Resistance is increased </li></ul>
  13. 13. Idiopathic PAH <ul><li>V/Q mismatch -> Hypoxemia </li></ul><ul><li>Vasoconstriction Occurs </li></ul><ul><li>Further Increases Vascular Resistance </li></ul>
  14. 14. Familial PAH <ul><li>Associated with a mutation of the bone morphogenetic protein receptor II gene (BMPR2) </li></ul><ul><li>Autosomal dominant with little penetrance </li></ul><ul><ul><li>Means that it does not manifest in all persons carrying the gene </li></ul></ul><ul><ul><li>Makes is difficult to trace the family history </li></ul></ul>
  15. 15. Familial PAH <ul><li>“ The incidence and prevalence is unknown because genetic investigation is not considered routine in the diagnosis and treatment of PAH” (Moser & Riegel, 2008, p. 1337) </li></ul>
  16. 16. Associated PAH <ul><li>Generally occurs later in life secondary to another disease process </li></ul><ul><ul><li>Connective tissue disease </li></ul></ul><ul><ul><li>Portal HTN </li></ul></ul><ul><ul><li>HIV infection </li></ul></ul><ul><ul><li>Drugs (Fen-Phen, cocaine) </li></ul></ul>
  17. 17. Associated PAH <ul><li>Other Causes </li></ul><ul><ul><li>Left heart disease/dysfunction </li></ul></ul><ul><ul><li>Respiratory disease or hypoxemia </li></ul></ul><ul><ul><li>Thromboembolic disease </li></ul></ul><ul><ul><li>Sarcoidosis, histiocytosis X, lymphangiomatosis, and compression of pulmonary vessels </li></ul></ul>
  18. 18. Associated PAH <ul><li>Secondary PAH </li></ul><ul><li>The most cause common is… </li></ul><ul><li>Left-Sided Heart Failure!!! </li></ul>
  19. 19. Associated PAH <ul><li>Connective Tissue Disease </li></ul><ul><ul><li>Scleroderma: affects 6% to 60% </li></ul></ul><ul><ul><li>Lupus: affects 4% to 14% </li></ul></ul><ul><li>Drug Use </li></ul><ul><ul><li>Amphetamines/Appetite suppressants: affects 25 to 50 cases per million annually </li></ul></ul><ul><li>HIV: incidence is 0.5% </li></ul><ul><ul><li>irrespective of viral load </li></ul></ul>
  20. 20. Associated PAH <ul><li>SURVIVAL STATISTICS </li></ul><ul><li>Congenital heart disease: 5 years </li></ul><ul><li>Portopulmonary HTN: 2 years </li></ul><ul><li>HIV: 1 to 2 years </li></ul><ul><li>Sickle Cell Disease: 1 to 2 years </li></ul><ul><li>Amphetamines/Appetite Suppressants: 2 to 3 years </li></ul><ul><li>(Moser & Riegel, 2008) </li></ul>
  21. 21. Associated PAH <ul><li>MORTALITY RATE VARIABLES </li></ul><ul><li>Of the Natural History of Untreated PAH </li></ul><ul><li>Mean PAP </li></ul><ul><li>Right Atrial Pressure </li></ul><ul><li>Cardiac Index </li></ul><ul><li>NYHA Functional class </li></ul><ul><ul><li>New York Heart Association classification of dyspnea </li></ul></ul><ul><li>(Moser & Riegel, 2008) </li></ul>
  22. 22. Associated PAH <ul><li>PATHOPHYSIOLOGY: </li></ul><ul><li>AKA Secondary PAH </li></ul><ul><li>Depends on Underlying Etiology </li></ul>
  23. 23. Pulmonary Artery Hypertension <ul><li>“ Patients with PAH benefit from oral, inhaled, or intravenous medications targeted to inhibit vascular growth and proliferation.” (Moser & Riegel, 2008, p. 1336) </li></ul>
  24. 24. Pulmonary Artery Hypertension <ul><li>If a patient does not receive treatment the following complications will occur: </li></ul>
  25. 25. Pulmonary Hypertension <ul><li>Hypertrophy of medial smooth muscle layer of the arterioles – worsens distensibility </li></ul>
  26. 26. Pulmonary Hypertension <ul><li>Increased pressure in the lungs spreads to right ventricle (which supplies the pulmonary artery) </li></ul>
  27. 27. Pulmonary Hypertension <ul><li>The ventricle becomes hypertrophic and eventually fails (Cor Pulmonale) </li></ul>
  28. 28. Pulmonary Hypertension <ul><li>Impaired distensibility due to hypertrophy can lead to arrhythmias </li></ul>
  29. 29. Pulmonary Hypertension <ul><li>SIGNS & SYMPTOMS: </li></ul>Early signs and symptoms of PAH/PH are similar to many other signs and symptoms of other disease processes May remain misdiagnosed until disease process has irreversibly progressed
  30. 30. Pulmonary Hypertension <ul><li>SIGNS & SYMPTOMS: </li></ul><ul><li>SOB/Tachypnea </li></ul><ul><li>Fatigue/Weakness </li></ul><ul><li>Nonproductive cough </li></ul><ul><li>Angina pectoris </li></ul><ul><li>Syncope </li></ul><ul><li>Peripheral edema </li></ul><ul><li>Hemoptysis (rarely) </li></ul><ul><li>Orthopnea or PND (with pulmonary venous hypertension) </li></ul><ul><li>Clubbing </li></ul><ul><li>Hepatojugular reflex </li></ul><ul><li>Loud P2 (pulmonic closure sound) </li></ul>
  31. 31. Pulmonary Hypertension <ul><li>CARDIAC SIGNS & SYMPTOMS: </li></ul><ul><li>Tachycardia </li></ul><ul><li>Decreased Peripheral B/P </li></ul><ul><li>S/S of Right-Sided HF (JVD, ascites) </li></ul><ul><li>Displaced PMI </li></ul>
  32. 32. Pulmonary Hypertension <ul><li>DIAGNOSTIC EVALUATION: </li></ul><ul><li>Echocardiography </li></ul><ul><ul><li>It is often the 1 st screening test for Dx of PAH </li></ul></ul><ul><ul><li>Estimates PAP </li></ul></ul><ul><ul><li>ventricular wall motion and possible valvular dysfunction/regurgitation (tricuspid); right atrial/ventricular enlargement </li></ul></ul><ul><li>Radionuclide Imaging </li></ul><ul><ul><li>abnormal right & left ventricular functioning </li></ul></ul>
  33. 33. Pulmonary Hypertension <ul><li>DIAGNOSTIC EVALUATION: </li></ul><ul><li>To RULE OUT Thromboembolic PAH: </li></ul><ul><li>Perfusion Lung Scanning -> multiple patchy and diffuse filling or normal results; inconsistent with findings associated with pulmonary embolism </li></ul>
  34. 34. Pulmonary Hypertension <ul><li>DIAGNOSTIC EVALUATION: </li></ul><ul><li>Pulmonary Angiography -> detect filling defects in pulmonary vasculature and if obstruction is operable </li></ul><ul><li>PA Catheterization -> ↑ PAP SBP > 30 </li></ul><ul><li>Pulmonary Function Studies -> ↓ Flow Rates, ↑ Residual Volume, ↓ Total Lung Capacity </li></ul>
  35. 35. Pulmonary Hypertension <ul><li>DIAGNOSTIC EVALUATION: </li></ul><ul><li>ABG’s -> hypoxemia (↓ PaO 2 ) </li></ul><ul><li>EKG Δ ’s </li></ul><ul><ul><li>RV hypertrophy is seen in 87% of patients w/ IPAH </li></ul></ul><ul><ul><li>Right axis deviation 79% of patients w/ IPAH </li></ul></ul><ul><ul><li>ST-segment depression and T-wave inversion in right precordial leads </li></ul></ul><ul><ul><li>RA enlargement </li></ul></ul><ul><ul><ul><li>Tall/Peaked P waves (known as Cor Pulmonale) in inferior leads (II, III, aV F ) </li></ul></ul></ul>
  36. 36. Treatment of PAH <ul><li>O 2 Therapy (hypoxemia): For all types of PAH </li></ul><ul><li>Fluid Restriction (↓ preload and ↓ workload) </li></ul><ul><li>Heart-Lung Transplant (irreversible Δ ’s) </li></ul><ul><li>Medication Regimen </li></ul>
  37. 37. Treatment of PAH <ul><li>Inotropics (ie, Digoxin) -> ↑ CO </li></ul><ul><li>Diuretics -> ↓ Intravascular & Venous Return </li></ul><ul><li>CCBs & other vasodilators -> ↓ Myocardial Workload and O2 consumption </li></ul><ul><li>Bronchodilators -> Relax smooth muscle and ↑ airway patency </li></ul><ul><li>β -blockers -> ↓ cardiac workload and improve oxygenation </li></ul><ul><li>Anticoagulant Therapy -> avoid blood clots </li></ul>
  38. 38. Common Medications Specifically for Treatment of IPAH <ul><li>Tracleer (bosentan) - PO </li></ul><ul><li>Revatio (sildenafil citrate) - PO </li></ul><ul><li>Flolan (epoprostenol) - IV </li></ul>
  39. 39. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>Most effective drug in treating IPAH </li></ul><ul><li>Very, very, very expensive!!! </li></ul><ul><li>Insurance will not cover unless ALL other meds have been proven to be ineffective </li></ul><ul><li>May take up to one month before insurance approves </li></ul>
  40. 40. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>Vasodilator </li></ul><ul><li>62.5 mg PO b.i.d. for 4 weeks, then </li></ul><ul><li>125 mg PO b.i.d. </li></ul><ul><li>May take 1 month to see results </li></ul>
  41. 41. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>Major Side Effects: </li></ul><ul><li>Liver Damage (hepatotoxicity) </li></ul><ul><li>Monitor LFT’s </li></ul><ul><li>Hypotension </li></ul><ul><li>Monitor B/P </li></ul>
  42. 42. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>Drug-Drug Interactions </li></ul><ul><li>Glyburide & Cyclosporine A </li></ul><ul><li>Cause retention of Tracleer resulting in increased risk of liver damage </li></ul>
  43. 43. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>Safe to Use With… </li></ul><ul><li>Coumadin (warfarin) </li></ul><ul><li>Lanoxin (digoxin) </li></ul><ul><li>Diuretics </li></ul><ul><li>Calcium Channel Blockers </li></ul>
  44. 44. Idiopathic PAH <ul><li>Tracleer (bosentan) </li></ul><ul><li>If interested in more info, visit the Tracleer website!!! </li></ul><ul><li>www.tracleer.com </li></ul>
  45. 45. Idiopathic PAH <ul><li>Revatio (sildenafil citrate) </li></ul><ul><ul><li>The FDA approved the main ingredient in Viagra (sildenafil citrate) June 7, 2005 under the name Revatio. </li></ul></ul><ul><ul><li>It dilates (relaxes) blood vessels and improves exercise ability in PH patients. </li></ul></ul>
  46. 46. Idiopathic PAH <ul><li>Revatio (sildenafil citrate) </li></ul><ul><ul><li>The approved dose for PH is 20 mg T.I.D. </li></ul></ul><ul><ul><li>(different than in Viagra – P.R.N.) </li></ul></ul><ul><ul><li>Just like Viagra, people taking nitrates (like nitroglycerin or Isordil [isosorbide dinitrate] ) should avoid Revatio. </li></ul></ul><ul><ul><li>Taking both can cause fatal blood pressure changes. </li></ul></ul>
  47. 47. Idiopathic PAH <ul><li>Flolan (epoprostenol) </li></ul><ul><ul><li>A prostacyclin (prostaglandin) </li></ul></ul><ul><ul><li>Flolan directly vasodilates (expands, relaxes) blood vessels. </li></ul></ul><ul><ul><li>Flolan also prevents blood cells from clumping together (antiplatelet agent). </li></ul></ul>
  48. 48. Pulmonary Hypertension <ul><li>Flolan (epoprostenol) </li></ul><ul><ul><li>It increases cardiac output and lowers afterload, improving PH symptoms, and it also improves survival time. </li></ul></ul><ul><ul><li>This prostacyclin is similar to the natural prostacyclin produced by the cells lining (endothelium) our blood vessels.       </li></ul></ul>
  49. 49. Pulmonary Hypertension <ul><li>Flolan (epoprostenol) </li></ul><ul><ul><li>Flolan is an IV drug which is only active in your blood for about 5 minutes, so you have to take it as a continuous IV infusion 24 hours a day. </li></ul></ul><ul><ul><li>The delivery system is complex and requires you to learn sterile preparation, operation of the pump, and care of the IV catheter. </li></ul></ul><ul><ul><li>Flolan must be kept cold after mixing but cannot be frozen. </li></ul></ul>
  50. 50. Pulmonary Hypertension <ul><li>Flolan (epoprostenol) </li></ul><ul><ul><li>VERY, VERY, VERY EXPENSIVE: </li></ul></ul><ul><ul><li>Costs anywhere from $25,000 a year and up. </li></ul></ul>
  51. 51. References <ul><li>Donofrio, J., Haworth, K, Schaeffer, L. & Thompson, G., (Eds.). (2005). Cardiovascular care made incredibly easy. Ambler, PA: Lippincott Williams & Wilkins. </li></ul><ul><li>Jacobs, M., & Meyer, T. (2006). The push is on pulmonary hypertension. Nursing Made Incredibly Easy, 4 (3), pp. 42-52. </li></ul><ul><li>McCance, K. L., & Huether, S. E. (2006). Pathophysiology: The biologic basis for disease in adults and children, (5 th ed.). Philadelphia, PA: Elsevier Mosby. </li></ul><ul><li>Moser, D. K., & Riegel, B. (2008). Cardiac nursing: A companion to braunwald’s heart disease. Saunders Elsevier: St. Louis, MO. </li></ul><ul><li>Smeltzer, S. C., Bare, B. G., Hinkle, J. L., & Cheever, K. H. (2008). Brunner and suddarth’s textbook of medical-surgical nursing, (11 th ed.). Philadelphia, PA: Lippincott Williams & Wilkins. </li></ul><ul><li>Vacca, V. M. (2009). On the alert for pulmonary artery hypertension. Nursing 2009, 39 (12), pp. 36-40. </li></ul><ul><li>Woods, S. L., Froelicher, E. S., Underhill Motzer, S., & Bridges, E. J. (2005). Cardiac nursing, (5 th ed.). Philadelphia, PA: Lippincott Williams & Wilkins. </li></ul>

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