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Pulmonary Hypertension: The Other High Blood Pressure

Pulmonary Hypertension: The Other High Blood Pressure. Pediatric Grand Rounds November 2, 2011. Speaker Disclosures. Alan Harsch, MD – None Andrew Bensky, MD – None Kari Crawford Plant, CPNP-AC – Consultant for United Therapeutics. CMC Pediatric Pulmonary Hypertension Center.

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Pulmonary Hypertension: The Other High Blood Pressure

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  1. Pulmonary Hypertension: The Other High Blood Pressure Pediatric Grand Rounds November 2, 2011

  2. Speaker Disclosures • Alan Harsch, MD – None • Andrew Bensky, MD – None • Kari Crawford Plant, CPNP-AC – Consultant for United Therapeutics

  3. CMC Pediatric Pulmonary Hypertension Center Alan Harsch M.D. NorthEast Pediatric Pulmonology Jeff Gordon Children’s Hospital

  4. Introduction to the CMC Pediatric Pulmonary Hypertension Center • What is PPH? • What causes PPH? • How does PPH present? • How is it diagnosed? • How is it treated? • What is the purpose of a Pediatric Pulmonary Hypertension Center and why do we need one? • What are the outcomes?

  5. Pulmonary Arterial Hypertension • PAH is a disease of the small pulmonary arteries, characterized by vascular proliferation and remodeling • PAH results in a progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death • Over the past 20 years, treatment options have evolved to prolong survival and improve quality of life

  6. Pediatric Pulmonary Hypertension Clinic • Multidisciplinary clinical program to provide state of the art care to children with pulmonary hypertension • Collaborative effort between providers from Levine Children’s Hospital and Jeff Gordon Children’s Hospital • Cardiology: Sanger Heart and Vascular Institute • Pulmonary Medicine: NorthEast Pediatric Pulmonology

  7. Difficult Disease • Fatal disease but patients usually look healthy • No cure; treatment is aimed at arresting or slowing the progression of the disease • Treatment choices: • conventional therapy • oral or intravenous vasodilator therapy • lung transplantation • It is very difficult to judge whether treatment is helping • Patients are likely to deteriorate rapidly with little warning

  8. Pulmonary Arterial Hypertension • Definition: • mean pulmonary artery pressure of greater than 25 mm Hg at rest or 30 mm Hg during exercise • Pathophysiology • Classification

  9. Previous Nomenclature for PAH • Primary pulmonary hypertension (PPH) = no known cause • Secondary pulmonary hypertension = associated with other disease known to cause pulmonary hypertension

  10. Pulmonary arterial hypertension sporadic familial collagen vascular disease congenital systemic to pulmonary shunts portal hypertension HIV infection drugs and toxins persistent pulmonary hypertension of the newborn (PPHN) Pulmonary arterial hypertension with other associations disorders of the respiratory system or hypoxemia chronic thromboembolic disease direct disorders of the pulmonary vasculature Current Nomenclature

  11. Diagnosing Pulmonary Hypertension Andrew Bensky, MD Pediatric Cardiology SHVI

  12. Electrocardiography • Patients with PAH may have several findings consistent with the diagnosis • RVH • RAD • RV conduction delay • ECG findings are sensitive, but not specific

  13. Typical PAH ECG

  14. Echocardiography • Primary diagnostic test for evaluating PAH • Findings are specific and sensitive in patients with most patients with PAH • Non-invasive in most pediatric patients, though sedation may be required

  15. Estimating PA Pressures • Using Doppler measurement of flow velocities to estimate pressure differences • D P = 4 (Velocity in m/sec)2 • Direct systemic to pulmonary connections • PDA • VSD • Valve regurgitation • PI • TR

  16. Estimating RVSP by TR Velocity

  17. Estimating PA Diastolic Pressure

  18. Right to Left PDA

  19. Left to Right PDA

  20. Echo Signs of PAH • Right ventricular hypertrophy • Right ventricular dysfunction • Abnormal septal position • Circular LV- RVSP is less than ½ systemic • Flat septum – “D” shaped ventricles, RVSP is nearly systemic • Circular RV – Suprasystemic RVSP

  21. Normal Short Axis

  22. Flattened Septum

  23. Suprasystemic RVSP

  24. Echo Signs of Chronic PAH • RVH • RVE with reduced systolic function • Often associated with clinical signs and symptoms of cor pulmonale • Fatigue, dyspnea, cough, chest pain • Hepatomegaly, edema, crackles, JVD

  25. When Echo Isn’t Diagnostic • No intracardiac shunts • Inadequate valve insufficiency to measure regurgitant velocities • Normal septal position • Normal RV size and function • PA pressures may be elevated, but not be able to be estimated

  26. Cardiac Catheterization • Gold standard, but invasive, method of both measuring pressures and calculating resistance • Measurements are made at baseline, and repeated using pulmonary vasodilators such as oxygen and nitric oxide • Reactivity assessment can guide medical therapy and help assess status serially

  27. Catheterization Data GA, 21% FiO2 Qp = 4.82 L/min (11.21 L/min/m²) Qs = 1.68 L/min (3.90 L/min/m²) Rp = 6.02 units (2.59 units x m²) Rs = 25.05 units (10.77 units x m²) Qp/Qs = 2.88 : 1 | Rp/Rs = 0.24 100% FiO2 / 40ppm NO Qp = 7.82 L/min (18.18 L/min/m²) Qs = 1.75 L/min (4.08 L/min/m²) Rp = 2.69 units (1.15 units x m²) Rs = 25.09 units (10.79 units x m²) Qp/Qs = 4.46 : 1 | Rp/Rs = 0.11

  28. Medications Utilized for PAH Kari Crawford Plant, CPNP-AC

  29. PAH Treatments―a Historical Overview CCB, anticoagulation, digitalis, diuretics IV treprostinil Sildenafil Tadalafil Tyvaso SC treprostinil Ambrisentan Epoprostenol Iloprost Bosentan 2001 2009 <1995 1995 2002 2004 2005 2007 CCB, calcium channel blocker; IV, intravenous; PAH, pulmonary arterial hypertension; SC, subcutaneous. 30

  30. Conventional Therapies • Calcium Channel Blockers (CCB) – Help decrease blood pressure (Only appropriate for a small minority of patients demonstrating a favorable response to vasodilator testing at the time of heart catheterization.) • Digoxin • Diuretics • Oxygen • Warafin (Coumadin®) • Aspirin (www.phaorganization.org, 7/10)

  31. Oral Therapy Endothelin Receptor Antagonists (ERAs) help prevent blood vessels from narrowing. Ambrisentan (Letairis®) Bosentan (Tracleer®) Phosphodiesterase Inhibitors (PDE 5 Inhibitors) allow the lungs to produce more of its own natural vasodilators. Sildenafil (Revatio™) Tadalafil (Adcirca®) Inhaled Treatment Options, such as Prostacyclins, relieve shortness of breath. Iloprost (Ventavis®) Inhaled Treprostinil (Tyvaso™) New Oral/Inhaled Therapy

  32. Phosphodiesterase 5-Inhibitors Sildenafil Revatio Tadalafil Adcirca 20mg (1 tablet) TID 40mg (2 x 20mg tablets) QD pahdiseasestate_v.1 33 33

  33. Endothelin Receptor Antagonists Bosentan Tracleer Ambrisentan Letairis 62.5 mg 1 tablet BID 125 mg 5 mg 1 tablet QD 10 mg pahdiseasestate_v.1 34 34

  34. Prostanoids – Inhaled Iloprost Treprostinil OPTINEB®- I-neb® AAD® pahdiseasestate_v.1 36 36

  35. Intravenous Therapy • Intravenous Treatment Options • Intravenous Treatment Options open up the blood vessels and help ease symptoms of PH, including chest pain and shortness of breath. Treprostinil (Remodulin®)(available SQ) Epoprostenol (Flolan®)

  36. What is Infused Prostacyclin Therapy? Continuously infused prostacyclins are delivered in 1 of 2 ways: Through a small catheter right under the skin, generally in the abdominal area Subcutaneous or SQ Directly into a central vein in the chest using a surgically placed flexible catheter Intravenous or IV Continuous infusion is a method by which the drug is administered to the patient on a steady basis using a delivery pump and a catheter (tube). 38 pahdiseasestate_v.1

  37. What Should You Teach Familes? • Resources: • Medication pharmacy, clinic RNs, PHAssociation.org - Keep a log of medications, bring all medications to appointments, protect indwelling lines (staff unfamiliar with therapy should not draw from or manipulate gtt or line) - Have PAH clinic contact information with them at all times

  38. Specialty Pharmacy Service Offerings All 3rd party reimbursement management Assessment and evaluation Pre-teaching In home, hospital, or clinic Coordination of services Discharge planning support Hotline access Functions, limitations, service values 1-866-474-8326www.curascript.com 1-866-344-4874www.accredotx.com 1-877-242-2738www.caremark.com 40

  39. Wholesale Cost of Medications(Oral and Inhaled) • Revatio- $17,262 • Adcirca- $14,892 • Tracleer- $69,715 • Letairis- $69,302 • Ventavis- $142,350 • Tyvaso- $146,913 • These are wholesale prices for a one year supply and do not reflect any discounts to Medicare

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