Ειδικές θεραπείες σε µη-αρτηριακή πνευµονική υπέρταση, πότε; - Στέλλα Μπρίλη Α Πανεπιστηµιακή Καρδιολογική Κλινική Ιπποκράτειο Νοσοκοµείο Αθηνών

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1 Ειδικές θεραπείες σε µη-αρτηριακή πνευµονική υπέρταση, πότε; - Στέλλα Μπρίλη Α Πανεπιστηµιακή Καρδιολογική Κλινική Ιπποκράτειο Νοσοκοµείο Αθηνών

2 . Updated Clinical Classification of Pulmonary Hypertension Updated Clinical (Dana Classification Point, 2008) of Pulmonary Hypertension (Dana Point, 2008) 1Idiopathic PAH1.2Heritable1.2.1BMPR21.2.2ALK1, endoglin (with or without PAH1.2Heritable1.2.1BMPR21.2.2ALK1, endoglin (with or without hereditary hemorrhagic telangiectasia)1.2.3unknown1.3drug- and toxininduced1.4associated with1.4.1connective tissue diseases1.4.2hiv infection1.4.3portal anemia1.5persistent pulmonary hypertension of the newborn1 Pulmonary venoocclusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) 2Pulmonary hypertension owing to left heart disease 2.1Systolic dysfunction2.2diastolic dysfunction2.3valvular disease 3Pulmonary hypertension owing to lung diseases and/or hypoxia 3.1Chronic obstructive pulmonary disease3.2interstitial lung disease3.3other pulmonar diseases with mixed restrictive and obstructive pattern3.4sleep-disordered altitude3.7developmental abnormalities (CTEPH) 5Pulmonary hypertension with unclear multifactorial disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis5.3metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders5.4others:

3 Severe Emphysema Am J Respir Crit Care Med Vol 166. pp , 2002

4 EUROPEAN RESPIRATORY JOURNAL 2008

5 Pulmonary hypertension in COPD The image cannot be displayed. Your computer may not have enough memory to open the image, or the image may have been corrupted. Restart your computer, and then open the file again. If the red x still appears, you may have to delete the image and then insert it again. EUROPEAN RESPIRATORY JOURNAL

6 EUROPEAN RESPIRATORY JOURNAL 2008

7 AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE 2005

8 AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE 2005

9 Working Group Recommendations for PH in Chronic Lung Disease (COPD, ILD, and Other Forms) Profound hypoxemia, hyperventilation, or low DLCO values can be indicators of PH. Doppler echocardiography remains the most useful noninvasive tool for assessing the presence of PH in patients with chronic lung disease. Biomarkers such as BNP or NT-proBNP need to be further evaluated. They seem to be useful screening tools for the presence of PH in patients with chronic lung disease.

10 Any pulmonary vasodilator has the potential to worsen gas exchange in patients with chronic lung disease, and the effects of these drugs may vary substantially depending on whether the underlying disease has obstructive or restrictive features. Short-term studies have been performed in ILD patients with sildenafil, bosentan, and inhaled iloprost, and these drugs had no adverse effects on oxygenation. In contrast, unpublished data suggest that sildenafil may worsen oxygenation in patients with COPD and PH and it has been shown that sildenafil can inhibit hypoxic pulmonary vasoconstriction. Much more evidence is needed before the use of PH targeted drugs can be recommended for certain subpopulations of patients with chronic lung disease.

11 Treatment of PH in chronic lung disease The underlying lung disease should be optimally treated according to the respective guidelines, including the use of long-term oxygen therapy in patients with chronic hypoxemia. There is no sufficient evidence that the drugs currently used for PAH are safe and effective in patients with PH associated with chronic lung disease. Patients with PH and chronic lung disease should be treated in the setting of clinical trials whenever possible.

12 Treatment of PH in chronic lung disease PH in various lung diseases should be studied separately, because patients with COPD and PH may respond differently to medical therapy than patients with ILD and PH. Registries are needed to obtain data from patients with very rare conditions. The use of drugs currently approved for PAH in patients with chronic lung disease is not recommended until further data are available.

13 Chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH results from obstruction of the pulmonary vascular bed by nonresolving thromboemboli. CTEPH differs from PAH by its major vessel involvement of the vascular remodeling process, which can be approached surgically by pulmonary endarterectomy (PEA).

14 Chronic thromboembolic pulmonary hypertension (CTEPH).

15 Treatment of CTEPH Patients with CTEPH should receive lifelong anticoagulation adjusted to a target international normalized ratio between 2.0 and 3.0 to prevent recurrence of thromboembolic events. Surgical PEA is the preferred treatment of CTEPH because it is potentially curative.

16 Treatment of CTEPH Patients with predominantly peripheral (i.e., inoperable) disease may be candidates for medical therapy and should be considered for enrollment in clinical trials whenever possible. In severely compromised patients with surgically accessible disease but for whom surgery must be delayed, pre-operative medical therapy with prostanoids, ERAs, or PDE-5 inhibitors may be used to improve hemodynamics and clinical performance before surgery, but this approach needs to be adjusted with the responsible surgeon.

17 Treatment of CTEPH

18 PH Associated With LHD LHD is one of the most common causes of PH. It may be caused by chronic heart failure attributable to LV dysfunction of systolic or diastolic origin or by valvular diseases, predominantly mitral valve disorders. Pulmonary hypertension and RV dysfunction carry a poor prognosis for patients with chronic heart failure. The pathogenesis of PH in LHD is complex. There is a passive (pulmonary venous) component in response to increased left atrial pressure. In some patients, a superimposed active component caused by pulmonary arterial vasoconstriction and vascular remodeling may lead to a further increase in PAP. Features suggestive of LV diastolic dysfunction or diastolic left heart failure include an older age, atrial fibrillation, and an enlarged left atrium.

19 Diagnosis and assessment of PH associated with LHD

20 Treatment of PH associated with LHD Optimize LV filling pressures In valvular heart disease such as mitral stenosis, post-operative reduction of PCWP Results of recent small studies suggest that sildenafil may improve exercise capacity and quality of life in patients with PH caused by LHD. In patients with elevated PCWP and an elevated transpulmonary gradient, reduction of LV afterload with nipride or other vasodilators is useful to identify an active pulmonary arterial component of PH.

21 Working Group Recommendations for PH Associated With LHD Treatment of LHD according to established criteria is the basis for a successful approach to patients with LHD and PH. Patients with end-stage heart failure and PH may be candidates for heart transplantation (in milder, largely reversible forms of PH) or heart lung transplantation (if PH is severe and fixed). The use of prostanoids, ERAs, and PDE-5 inhibitors in patients with LHD and PH is not recommended until robust data from long-term clinical trials are available.

22 Treatment of PH associated with LHD Circulation January 2/9, 2007

23 Treatment of PH associated with LHD Circulation October 2, 2007

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