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Treatment of ILD and Pulmonary Hypertension

Treatment of ILD and Pulmonary Hypertension. Levent Tabak, M.D. Istanbul Medical Faculty. Pulmon ary H ypertension (PH). DEFINITION Mean pulmonary artery pressure ~14 mmHg) Pulmonary hypertension; During resting > 25 mm Hg During exercise > 30 mm Hg. P H severity.

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Treatment of ILD and Pulmonary Hypertension

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  1. Treatment of ILD and Pulmonary Hypertension Levent Tabak, M.D. Istanbul Medical Faculty

  2. Pulmonary Hypertension(PH) DEFINITION • Mean pulmonary artery pressure ~14 mmHg) • Pulmonary hypertension; • During resting > 25 mm Hg • During exercise > 30 mm Hg PHseverity Mild26 - 35 mmHg Moderate36 - 45 mmHg Severe> 45 mmHg

  3. 1.Pulmonary arterial hypertension 1.Idıopathic 2.Familial 3.Secondary a)Collagen vascular disease b)Congenital systemic to pulmonary shunt c)Portal hypertension d)HIV infection e)Drugs and toxins f) Other(Thyroid, glycogen storage disease splenectomy, nyeloproliferative disor) 4.Venous or capillary involvement a)Pulmonary veno-oclusive disease b)Pulmonary capillary hemangiomatozis 2. Pulmonary venous hypertension 1.Left sided atrial/ventricular heart dise 2.Left sided valvular heart disease 3.PH associated with hypoxemia 1. COPD 2. Interstitial lung diseases 3. Sleep disorders 4. Alveolar hypoventilation 5. High altitude 6. Developmental abnormalities 4.PH due to chronic thrombotic/embolic disease 1.Obstruction of proximal pulmonary arteries 2.Obstruction of distal pulmonary arteries 3.Non-thrombotic pulmonary embolism 5.Miscellaneous a)Sarcoidosis b)Histiocytosis XSarkoidoz c)Lymphangiomatosis d)Compression of pulmonary vessels PULMONARY HYPERTENSIONVenice 2003

  4. Clinics • Fatique • Dyspnea • Chest pain • Syncop • Hemoptysis The interval between disease onset and symptoms is around 3 years

  5. Diagnosis of PH PH Detection Chest Radiography ECG TT Echocardiography PH Class Identification Pulmonary Function Test V/Q scan HRCT Spiral CT Pulmonary Angiography PAH evaluation Blood Tests&Immunology HIV test Abdominal USG 6 min Walk Test, Max VO2 Right Heart Cath+Vasoreactivity

  6. Chest X-ray • May helpfull only in 30-40% of patients! • Dilated central pulmonary arteries • Reduced peripheral vascularization (Tree in bud)

  7. ECG • Right atrial dilatation • Right ventricular hypertrophy • RBBB (55% sensitivity, 70% specifity)

  8. ECHOCARDIOGRAPHY Bestnon-invasivediagnostic test. • Right ventricular and atrial dilation • Tricuspid/pulmonary valveregurgitation • Non-invasive measurement of pulmonary arterial pressure

  9. Diagnosis of PH PH Detection Chest Radiography ECG TT Echocardiography PH Class Identification Pulmonary Function Test V/Q scan HRCT Spiral CT Pulmonary Angiography PAH evaluation Blood Tests&Immunology HIV test Abdominal USG 6 min Walk Test, Max VO2 Right Heart Cath+Vasoreactivity

  10. Pulmonary Function tests & Blood Gases • PAH patients usually have mild to moderate reduced lung volumes and decreased DLCO • PaO2 is normal or slightly low and PaCO2 is decreased due to alveolar hyperventilaton • PaCO2 is increased in COPD as a cause of hypoxic PH together with obstruction • A decrease in lung volumes and DLCO indicate ILD

  11. V/Q Lung Scan • In PAH V/Q scans may be entirely normal • V/Q scanning showed sensitivity of 90-100% with spesifity of 94-100% for distinguishing between IPAH and CTEPH • V/Q scans are similar in veno-occlusive disease and CTEPH, HRCT is reguired for differentiation

  12. Chest CT/MRI • Right ventricular morphology • Right atrial morphology • Pulmonary artery morphology • Right ventricular function *The value of serial CT/MRI scans in following the course of patients is not established *CT; evaluate the lung parenchyma

  13. Pulmanaryangiography

  14. Diagnosis of PH PH Detection Chest Radiography ECG TT Echocardiography PH Class Identification Pulmonary Function Test V/Q scan HRCT Spiral CT Pulmonary Angiography PAH evaluation Blood Tests&Immunology HIV test Abdominal USG 6 min Walk Test, Max VO2 Right Heart Cath+Vasoreactivity

  15. Exercise testing • A six-minute walk test or a cardiopulmonary exercise test is recommended • Exercise test best characterise the functional impairment of patients with PPH and their response to therapy

  16. Right Cath+Vasodilator Testing • Patients with idiopathic pulmonary arterial hypertension (IPAH) should undergo acute vasoreactivity testing using a short-acting agent such as intravenous (IV) epoprostenol, adenosine, or inhaled nitric oxide (NO). • Level of evidence: fair; benefit: substantial; grade of recommendation: A. • Patients with pulmonary arterial hypertension (PAH) associated with underlying processes, such as scleroderma or congenital heart disease, should undergo acute vasoreactivity testing. • Level of evidence: expert opinion; benefit: small/weak; grade of recommendation: E/C.

  17. PAH Treatment • General • Oral anticoagulant • Diuretics-venesection • Oxygen • Digoksin • VAZODİLATÖR TEDAVİ • Calciun Channel Blockers • Endothelin antagonists • Bosentan…Sitaxsenten/Ambrisentan • Prostacyclins • Epoprostenol, Treprostinil, Iloprost, Oral beraprost • PDE-5 inhibitors (c-GMP elevators) • Sildenafil • SURGICAL INTERVENTIONS • Atrial septostomY • Lung transplantation

  18. Anticoagulation • Microthrombosis of the small vessels is often seen in PPH • Fibrin degradation products have a proliferative effect on the vessels • Patients treated with coumadin have a better prognosis • Targed INR value in IPAH patients; • North America 1.5-2.5 • Europe; 2-3 *Patients with IPAH should receive anticoagulation with warfarin. Level of evidence: fair; benefit: intermediate; grade of recommendation: B. **In patients with PAH occurring in association with other underlying processes, such as scleroderma or congenital heart disease, anticoagulation should be considered. Level of evidence: expert opinion; benefit: small/weak; recommendation: E/C. *Patients with IPAH should receive anticoagulation with warfarin. Level of evidence: fair; benefit: intermediate; grade of recommendation: B. **In patients with PAH occurring in association with other underlying processes, such as scleroderma or congenital heart disease, anticoagulation should be considered. Level of evidence: expert opinion; benefit: small/weak; recommendation: E/C.

  19. Diuretics • Prevent sodium and water retension • Great impact on physical capacity and quality of life in many patients with right heart failure • Drug and dose;……….expertise • Follow up;……………. Electrolits, renal function

  20. Venesection • Polycytemia increases the viscosity of the blood and therefore increases vascular resistance (HCT>60%) • Benefial in COPD patients • Decompensation in Eisenmenger

  21. Oxygen Therapy • Oxygen treatment decreases pulmonary artery pressure • Recommended when PaO2< 65 mmHg, or oxygen saturation < 85% • It does rarely work in IPAH patients • O2 therapy is beneficial for right ventricle and respiratory muscles In patients with PAH, supplemental oxygen should be used as necessary to maintain oxygen saturations at >90% at all times. Level of evidence: expert opinion; benefit: substantial; recommendation: E/A. In patients with PAH, supplemental oxygen should be used as necessary to maintain oxygen saturations at >90% at all times. Level of evidence: expert opinion; benefit: substantial; recommendation: E/A.

  22. Digitalis&Dobutamine • Depression of myocardial contractility is the primary event in the progression of right heart failure, inotropic agents have been used. • Short-term IV administration of digoxin produces increase in cardiac output and a significant reduction in circulating norepinephrine levels • End stage PAH patients treated with dobutamine in most centres • Long-term treatment?

  23. Calcium Channel Blockers • Smooth muscle hyperthrophy • Vasoconstriction • Voltage-dependent potassium channels • Rich ve ark.NEJM,1992 Only in patients with vasodilator testing (+) Side effects should be followed closely • Nifedipin (in case of bradicardia) • Diltizem (in case of tachycardia) • ***Verapamil : negative inotropic effects!!! A/F!

  24. Calcium Channel Blockers • Beginning with low doses are recommende; • Nifedipine 30 mg 2x1 • Diltiazem 60 mg 3x1 • Doses increased gradually in weeks up to maxium tolerated level; • Nifedipine…> 120-240 mg/day • Diltiazem….-> 240-720 mg/day *Systemic hypotension and peripheral edema of lower extremities are dose limiting factors.

  25. Pulmonary Arterial Hypertension(New treatments) Endothelin Hypoxia NO Prostacyclin C-GMP Vasodilation Vasoconstruction

  26. New Agents • Prostacyclins • Epoprostenol • Treprostinil • Iloprost • Beraprost • Endothelin Receptor Antagonists • Nonselective Endothelin Receptor Blockade: (Bosentan) • Specific A-receptor Blockers. (Sitaxentan, Ambrisentan) • Phosphodiesterase 5 Inhibitors; • Sildenafil

  27. PAH and IPF survival • The median length of survival has been estimated to be between 2.5 and 5 years after the onset of respiratory symptoms or diagnosis • 1-year survival rate; • 45% in patients with IPF and PAP> 50 mmH • 83% in patients with IPF and PAP< 50 mm Hg • Therefore, there is often a short window to enable the study of pulmonary hypertension in this population

  28. Chest X-ray • Patients with a suggestion of pulmonary hypertension on chest radiography have a poorer prognosis than those without pulmonary artery enlargement

  29. Echocardiography • Echocardiography is the most common noninvasive test used to screen for pulmonary hypertension. Unfortunately, in patients with advanced lung disease, the sensitivity and specificity of echo-cardiography are imperfect: • sensitivity; 85% • specificity; 55% • positive predictive; 52% • Negative predictive; 87%

  30. V/Q Lung Scan • In PAH V/Q scans may be entirely normal • V/Q scanning showed sensitivity of 90-100% with spesifity of 94-100% for distinguishing between IPAH and CTEPH • V/Q scans are similar in veno-occlusive disease and CTEPH, HRCT is reguired for differentiation • In parenchymal lung disease the perfusion defects are matched by ventilation defects

  31. PAH-PFT relationship • FVC is 50 to 80% of that predicted; • pulmonary hypertension may occur with exercise • FVC < 50% or DLCO < 45% : • pulmonary hypertension may occur at rest

  32. ILD-PH Treatment • Limited data suggest that the treatment of pulmonary hypertension in ILD is beneficial. • There are some theoretic risks to vasodilation in patients with ILD. • pulmonary artery vasodilation leads to improved blood flow into areas of fibrotic lung, then worsening of ventilation perfusion mismatch may result • Patients receiving intravenous prostacyclin experienced decreased arterial oxygen tension, largely because of an increase in shunt fraction. By contrast, inhaled nitric oxide and sildenafil maintained ventilation perfusion matching and decreased pulmonary vascular resistance without a decrease in arterial oxygen tension. • Ghofrani HA, Wiedemann R, Rose F, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet 2002; 360:895-900

  33. Therapy for PH in ILD • Pulmonary hypertension is common in advanced ILD and correlates with poor outcome. • Therapy that targets pulmonary hypertension has been initiated in small clinical trials. • Larger randomized controlled trials will be needed to establish that therapy of pulmonary hypertension is both efficacious and safe for patients with ILD

  34. PAH Treatment-summary • General • Oral anticoagulant • Diuretics-venesection + Physiotherapy • Oxygen Control of infection • Digoksin • VAZODİLATÖR TEDAVİ • Calciun Channel Blockers • Endothelin antagonists • Bosentan…Sitaxsenten/Ambrisentan • Prostacyclins • Epoprostenol, Treprostinil, Iloprost, Oral beraprost • PDE-5 inhibitors (c-GMP elevators) • Sildenafil • SURGICAL INTERVENTIONS • Atrial septostomY • Lung transplantation ?

  35. References • Medical Therapy For Pulmonary Arterial Hypertension ACCP Evidence-Based Clinical Practice Guidelines David B. Badesch, Steve H. Abman, Gregory S. Ahearn, Robyn J. Barst, Douglas C. McCrory, MHSc; Gerald Simonneau,Vallerie V. McLaughlin Chest 2004;126;35-62 • Guidelines on diagnosis and treatment of pulmonary arterial hypertension Nazzarone Galie, Adam Torbicki, Robyn Barst, Philippe Dartevelle, Shelia Haworth, Tim Higenbottam, Horst olschewki, Andrew Peacock, Guiseppe Pietra, Lewis J Rubin European Heart Journal 2004; 25: 2243-2278

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