Ipertensione Polmonare, quadri polmonari specifici associati

Quadri polmonari specifici associati ad ipertensione Polmonare F. Meloni Pavia, 21/05/2011

Sarcoidosis –associated PH (SAPH) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Prevalence of SAHP according to clinical stages From Palmero et al, 2010

SAHP: Diagnosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

SAHP workout algorithm From Palmero et al, 2010

SAHP: Therapy ,[object Object],[object Object],[object Object],[object Object]

SAHP: Prognosis ,[object Object],[object Object]

COPD and PH ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Cigarette smoke Inflammation Tissue distruction/remodeling Alveolar hypoxia Endothelial dysfunction Reduction of vascular bed Pulmonary artery modeling Pulm artery vasoconstriction Thrombosis Pulmonary hypertension

Disproportionate PH in COPD ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],LOW NUMBER OF Patients: prompt referral to specialized centres

CPFE ,[object Object],[object Object]

CFPE Definizione ,[object Object],[object Object]

QUADRO CLINICO ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

CARATTERISTICHE E MANIFESTAZIONI CLINICHE ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

QUADRO RADIOLOGICO HTRC ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

QUADRO RADIOLOGICO ,[object Object],[object Object],[object Object]

BAL ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Funzionalità Respiratoria ,[object Object],[object Object],[object Object]

Funzionalità Respiratoria ,[object Object],[object Object],[object Object],[object Object]

Ipertensione Polmonare ,[object Object],[object Object],[object Object],[object Object]

Quadro Istologico ,[object Object],[object Object],[object Object],[object Object]

Trattamento ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

OUTCOME ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Fisiopatologia della CPFE ,[object Object],[object Object],[object Object],[object Object]

Fisiopatologia della CPFE ,[object Object],[object Object],[object Object]

Possibile meccanismo patogenetico della CPFE ,[object Object],[object Object],[object Object]

Conclusioni ,[object Object],[object Object],[object Object],[object Object]

TGF-β e SMAD Pathway ,[object Object],[object Object],[object Object]

[object Object],[object Object],[object Object],[object Object]

Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome V. Cottin*, et al, ERJ, 2010 ,[object Object],[object Object],[object Object],[object Object],Sex male/female n 38/2 Age yrs 68.2¡8.9 (47.7–82.2) Current/ex/never-smokers n 2/37/1 NYHA class III 22 (55) NYHA class IV 12 (30) 6MWD m 244¡126 (78–689) 6MWT Sp,O2 at end of test 77¡10 (62–96) FVC % pred 86 % (46–116) FEV1 % pred 78 (38–114)

CFPE Delay in diagnosis ,[object Object]

Hemodynamic features of CFPE patients ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Cottin et al ERJ 2010 None of these variables significantly correlated with any PFT variable

Univariate analysis on variables influencing survival ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Cottin et al ERJ 2010

CFPE: Survival ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Ipertensione Polmonare, quadri polmonari specifici associati

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