PULMONARY HYPERTENSION. Charles A. Thompson, M.D., FACC, FSCAI Cardiovascular Institute of the South Zachary, Louisiana

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1 PULMONARY HYPERTENSION Charles A. Thompson, M.D., FACC, FSCAI Cardiovascular Institute of the South Zachary, Louisiana

2 What is Pulmonary Hypertension? What is normal? Pulmonary artery systolic pressure (PAP) mmhg Mean Pulmonary artery systolic pressure (mpap) 15 mmhg Pulmonary Capillary wedge pressure (PCWP) 6-9 mmhg Mean PCWP 7 mmhg Pulmonary vascular resistance (PVR) less than 3 Woods u

3 Pulmonary Hypertension (PH) or Pulmonary Artery Hypertension (PAH) PHà à à Elevation of PAP without regard to other hemodynamic measurement or cause of the elevated pressure (General name of all forms of elevated PAP) PAHà à à mpap greater than 25 mmhg with PCWP less than 15 mmhg and PVR greater than 3 Woods units (Specific to elevated PAP due to diseased pulmonary arteries) Revised WHO classification adopted from the 5th World Symposium on Pulmonary Hypertension in Nice, France in 2013 of pulmonary hypertension

4 Revised WHO Classification Group 1 Group 1 (Elevated PAP with normal PCWP) Pulmonary Arterial Hypertension 1.1 Idiopathic (IPAH) 1.2 Familial (FPAH) morphogenic protein receptor-2 (BMPR2) 1.3 Associated with (APAH) Connective tissue disorder Congenital systemic-to-pulmonary shunts Portal hypertension HIV infection Drugs and toxins Other (thyroid disorders, glycogen storage disease, Gaucher s, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy

5 Revised WHO Classification Group 1 (con t) Group 1 (con t) Pulmonary Arterial Hypertension Associated with significant venous or capillary involvement Pulmonary veno-occlusive disease (PVOD) Pulmonary capillary hemangiomatosis (PCH) Persistent pulmonary hypertension of the newborn

6 Revised WHO Classification Group 2 Group 2 (Elevated PAP and PCWP) Pulmonary Hypertension due to pulmonary venous hypertension (due to left heart disease) Left-sided atrial or ventricular disease Left-sided valvular disease

7 Revised WHO Classification Group 3 Group 3 (Elevated PAP with chronic hypoxia) Pulmonary Hypertension associated with lung diseases or hypoxemia COPD Interstitial lung disease Sleep disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude Developmental abnormalities

8 Revised WHO Classification Group 4 Group 4 (Elevated PAP with documented PA obstruction) Pulmonary Hypertension due to chronic thrombotic and /or embolic disease (CTEPH) Thromboembolic obstruction of proximal pulmonary arteries Thromboembolic obstruction of distal pulmonary arteries Nonthrombotic pulmonary embolism (tumor, parasites, foreign material)

9 Group 5 Revised WHO Classification Group 5 Miscellaneous (Elevated PAP where causal relationship is suspected but not understood) Sarcoidosis Histiocytosis X Lymphangiomatosis Compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)

10 Early Nonspecific Fatigue Dyspnea Symptoms Often dismissed as due to age/ deconditioning/other medical problems Late/Severe Right Heart Failure -Ascites Chest Pain Syncope Anorexia 10

11

12 Treatment Based on Group of pulmonary hypertension Primary Therapy all groups (with rare exceptions) Advance Therapy Only for Group 1 (PAH) with Class II, III, IV functional capacity and on Primary Therapy

13 Primary Therapy Treat the underlying cause Cardiac valvular disease CHF COPD PTE Left to right shunts Connective tissue disease

14 Primary Therapy Diuretics useful in all Groups of PH including the subgroup of PAH Reduction of pulmonary congestion Reduction of hepatic congestion Caution Excess reduction of right ventricular preload Electrolyte imbalance Metabolic alkalosis

15 Primary Therapy Oxygen Primary treatment for Group 3 (Lung disease/hypoxemia) No long term data in other groups Caution with oxygen driven respiratory mechanisms Not significantly helpful in right to left shunt states

16 Primary Therapy Digoxin Improves RV ejection once RV failure develops Drug of choice for supraventricular tachycardia EXCEPT for MAT (?verapamil??diltiazem?)

17 Primary Therapy Calcium Blockers nifedipine, diltiazem, amlodipine?verapamil should be avoided (ACCF/AHA 2009) Group 1 (PAH) unlikely to respond Requires invasive testing mpap drop 10 mmhg and at least equal to or less than 40 mmhg Discontinue if Class I or II functional class not reached

18 Primary Therapy Anticoagulation---warfarin with INR goal of 2-3 Indicated with survival benefit in Group I (PAH) and Group 4 (Chronic thromboembolic) Thought to be helpful but without supportive data in all other Groups Frequently indicated due to the nature of underlying cause of PH

19 Primary Therapy Exercise Beneficial in PAH and PH patients Improves functional class Improves 6 minute walk test time Improves peak oxygen consumption Does not improve hemodynamics Skeletal muscle training seems to provide the improvement

20 Advanced Therapy Only effective treatment for Group 1 (PAH) patients Rarely useful for Group 2 (Pulmonary venous HTN) after Primary therapy Contraindicated and not FDA approved for Group 3 (Pulmonary hypoxemia) Limited data for use in Group 4 (Embolic) and Group 5 (Other/uncertain) Requires close/frequent invasive monitoring of hemodynamic response Should probably only be provided in PAH referral centers

21 Advanced Therapy Prostanoids Treats the underproduction of prostacyclin I2 (vasodilator with antiproliferative effects) due to the lack of prostacyclin syntase Epoprostenol----intravenous Treprostinil----subcutaneous Iloprost----inhaled Short half-lifeà dose every 4 hours Irritating to skin and veins Cough, flushing, headache

22 Advanced Therapy Endothelin Receptor Antagonists Endothelin produces smooth muscle vasoconstriction Bosentan oral twice a day Sitaxsentan oral once a day Ambrisentan---in phase 2 study Monthly liver function check Monthly blood count

23 Advanced Therapy Phosphodiesterase Inhibitors Promote vasodilation via nitric oxide Sildenafil--approved under the Trade name Revatio for PH (Trade name Viagra for Erectile dysfunction) Tadalafil---approved under the Trade name Adcirca for PH (Trade name Cialis for Erectile dysfunction)

24 Advanced Therapy Combined heart and lung transplant Right ventricular assist devices Support groups

25 Effectiveness confirmed to justify continuation of any treatment Six minute unencouraged walk test Right heart catherization---usually reserved if Advanced Therapy utilized

26 PULMONARY HYPERTENSION Charles A. Thompson, M.D., FACC, FSCAI Cardiovascular Institute of the South Zachary, Louisiana

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