prognosi ipertensione polmonare arteriosa-pah prognosis
DESCRIPTION
prognosi ipertensione polmonare arteriosa - pah prognosisTRANSCRIPT
![Page 1: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/1.jpg)
Powerpoint Templates
Ipertensione Arteriosa Polmonare
Aspetti prognostici
Dip. Cardiovascolare e Toracico Direttore Dr. S.BaldiA.O.U. MolinetteTorino-Italy
Dr. D.Libertucci Dr. P.SolidoroS.C. Pneumologia
![Page 2: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/2.jpg)
Powerpoint Templates
Nella PAH definire la severità del quadro clinico è di fondamentale importanza
WHO-FC: Ruolo cardine per
definire la prognosi
valutare la risposta alla terapia
decidere la combinazione
The clinical assessment of the patient has a pivotal role in the choice of the initial treatment, the evaluation of the response to therapy, and the possibile
escalation of therapy if needed
ESC/ERS Guidelines Eur Heart J 2009; 30(20): 2493-2537
![Page 3: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/3.jpg)
Powerpoint Templates
6
2,5
0,5
0
1
2
3
4
5
6
Sopra
vviv
enza m
edia
(a
nni)
I I I I I IV
Classe funzionale
Storia naturale IPAH/ heritable PAH
D’Alonzo et al., Ann Intern Med, 1991; 115(5):343-349
L’ipertensione arteriosa polmonare ha una prognosi infausta: mediana di sopravvivenza dei pazienti in FC III =2.8 anni dalla diagnosi
La prognosi è correlata alla classe funzionale
Infatti
![Page 4: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/4.jpg)
Powerpoint Templates
Karyofillis et al., Hellenic J Cardiol 2009; 50(6):484-492 2009
Survival curve according to NYHA functional class
![Page 5: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/5.jpg)
Powerpoint Templates1. Kato I, et al. Cancer 2001; 92:2211-9.
2. D'Alonzo GE, et al. Ann Intern Med 1991; 115:343-9.3. Felker GM, et al. N Engl J Med 2000; 342:1077-84.
0 1 2 3 4 5 6
WHO class I – II IPAH2
WHO class III IPAH2
Advanced breast cancer1
Advanced colorectal cancer1
WHO Class IV IPAH2
Advanced lung cancer1
Survival (years)
Ischaemic cardiomyopathy3
6 months
2.6 years
4.9 years
7
Un piccolo ritardo nella diagnosi ha un impatto forte sulla prognosi
La prognosi della PAH è comparabile a quella di alcune neoplasie in stato avanzato
![Page 6: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/6.jpg)
Powerpoint Templates
La classe funzionale al momento della diagnosi ha un forte impatto sul decorso della PAH
Brenot F. Chest 1994; 105:33S-36S
WHO class III – IV (n = 75)
0 12 24 36 48 60 72 84 96
0
25
50
75
100
WHO class I – II (n = 30)
Time (months)
Su
rviv
al (%
)
Senza trattamento, la malattia progredisce anche nei pazienti oligosintomatici
![Page 7: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/7.jpg)
Powerpoint Templates
Miglioramenti nella FC = migliore prognosi
No. at risk
McLaughlin VV, et al. Circulation 2002; 106:1477-82.
1-year epoprostenol
102030466386112115
Time (months)
847260483624120
100
80
60
40
20
0
WHO I
WHO II
WHO III
WHO IV
Sitbon O, et al. J Am Coll Cardiol 2002; 40:780-8.
Su
rviv
al (%
)
Time (months)
FC after 3-months epoprostenol therapy
Su
rviv
al (%
)
p < 0.0001
0
20
40
60
80
100
0 12 24 36 48 60 72 84 96 108
WHO I or II(n = 91)
WHO III or IV(n = 75)
![Page 8: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/8.jpg)
Powerpoint Templates
Anche l’etiologia influenza la prognosi
McLaughlin et al., Chest 2004; 126:78S–92S
CHD congenital heart disease CVD collagen vascular disease HIV human immunodeficiency virus related IPAH idiopathic pulmonary arterial hypertension Portopulm Portopulmonary hypertension
A differenti etiologie corrispondono differenti percentuali di sopravvivenza
![Page 9: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/9.jpg)
Powerpoint Templates
Age, disease severity and aetiology are critical factors in determining clinical outcomes in patients with PAH. Treatment with bosentan improved survival outcomes in both
iPAH and SSc-PAH compared to historical controls.
Keogh et al., Intern Med J ; 2009
![Page 10: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/10.jpg)
Powerpoint Templates
1. Rubin, et al. New Engl J Med 2002; 346:896-903. 2. Badesch DB, et al. AHA 2006.
3. Gatzoulis M, et al. Int J Cardiol 2008; 127:27-32.
Baseline Baseline OLE End OLE
61
.3m
-10
10
30
50
70Ex-bosentan
(n =26)
Ex-placebo (n = 9)
33
.2m
Mean
6
-MW
D (
m)
OLE: Open label extension
BREATHE-11 and 1-year OLE2
Mean
6
-MW
D
(m)
-10
0
10
20
30
40
Ex-bosentan (n =133)
Ex-placebo (n = 61)
6.4
m3
4.1
m
Baseline OLE
End OLEBaseline
Il ritardo nella gestione terapeutica compromette la capacità di esercizio nel
periodo long-term
BREATHE-5 and 6-month OLE3
![Page 11: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/11.jpg)
Powerpoint Templates
Il miglioramento della prognosi è dovuto alla disponibiltà di farmaci specifici per la PAH
Sitbon O, et al. ERS 2007; Abstract 1593.
100
75
50
25
00 12 24 36 48 60
Tempo (mesi)
So
pra
vviv
enza
% Dopo il 2000 Post-ERA*
1992–1999 Pre-ERA
Prima del 1992 Pre-epoprostenolo
![Page 12: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/12.jpg)
Powerpoint Templates
The patients in the Chinese Registry were treated only with diuretics and anticoagulation. The 3 and 5-year survival rates in this registry were 38,9% and 20.8% respectively.The authors of the Chinese Registry attributed the poor survival to the lack of PAH-specific therapies in China during the study recruitment
Tenappan Int J Clin Pract 2009; 63 (Suppl 162):42-44
I farmaci che modificano i meccanismi patogentici hanno un ruolo cruciale
Non a caso
![Page 13: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/13.jpg)
Powerpoint Templates
Bosentan è l’unico trattamento che ha dimostrato di ritardare il peggioramento clinico in tre differenti trials
Study 351 Relative risk reduction
= 100%
BREATHE-1 Relative risk reduction
= 72%
EARLY Relative risk reduction
= 77%
No clinical worsening was reported in
patients given bosentan.
![Page 14: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/14.jpg)
Powerpoint Templates
Fattori prognostici
Galiè et al., ESC/ERS Guidelines Eur Heart J, 2009; 30:2493-2537
![Page 15: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/15.jpg)
Powerpoint Templates
SINTOMI
![Page 16: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/16.jpg)
Powerpoint Templates
ESERCIZIO/MARKERS
![Page 17: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/17.jpg)
Powerpoint Templates
CUORE/EMODINAMICA
![Page 18: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/18.jpg)
Powerpoint Templates
Dispnea Turgore delle giugulari Pulsazione delle giugulari Epatomegalia Edemi agli arti inferiori Edema periferico
1. Scompenso ventricolare destro
Fattori prognostici
Possibili segni e sintomi correlati
![Page 19: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/19.jpg)
Powerpoint Templates
2. Progressione dei sintomi
Fattori prognostici
sincope
dispnea affaticamento vertigini dolore toracico edema periferico
![Page 20: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/20.jpg)
Powerpoint Templates
3. Classe funzionale
Fattori prognostici
Mantenere il paziente in classe funzionale I-II
![Page 21: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/21.jpg)
Powerpoint Templates
3. 6MWD
Fattori prognostici
332 m: “storico” cut off correlato sopravvivenza
380 m: cut off che conferma efficacia trattamento
400 m: nuovo possibile obiettivo1
500 m: nuovo possibile obiettivo2
1Hoeper et al., Unpublished data 2Galiè Unpublished data
![Page 22: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/22.jpg)
Powerpoint Templates
3. Test cardio-polmonare: risultati
Fattori prognostici
Il consumo di ossigeno testimonia l’intensità dell’esercizio fisico che il paziente può eseguire
< 12 ml/min/kg prognosi peggiore
> 15 ml/min/kg prognosi migliore
![Page 23: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/23.jpg)
Powerpoint Templates
3. Markers PAH
Fattori prognostici
Mantenere il paziente in classe funzionale I-IIBNP < 150 pg/mL prognosi miglioreBNP > 180 pg/mL prognosi peggioreNT pro BNP < 1400 pg/mL prognosi migliore NT pro BNP> 1400 pg/mL prognosi peggioreNT pro BNP < 553 pg/mL prognosi migliore in pz PAH-SSC
BNP (B-type natriuretic peptide)Nt-proBNP (N-terminal fragment of pro- brain natriureticPeptide)
![Page 24: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/24.jpg)
Powerpoint Templates
4. Ecocardiografia
Fattori prognostici
Normalizzare Frazione di eiezione e indice cardiaco (TEI) TAPSEDimensione atrio destroIndice di eccentricità
Ridurre o eliminareEffusione pericardica
![Page 25: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/25.jpg)
Powerpoint Templates
TAPSE (Tricuspid annular plane systolic excursion)Movimento dell’anello tricuspidalico in fase di sistole
TAPSE (v.n.) = 16.3 ± 0.6 mm
Ridotto = Prognosi peggiore
TAPSE (v.n.) = 16.3 ± 0.6 mm
Ridotto = Prognosi peggiore
Il TAPSE misura lo spostamento sul piano longitudinale dell’annulus tricuspidalico. Il TAPSE riflette la funzionalità sistolica del ventricolo destro
Kaul S, et al. Am Heart J 1984; 107:526-31Kaul S, et al. Am Heart J 1984; 107:526-31
Ghio S et al. Am J Cardiol 2000;85:837-42Ghio S et al. Am J Cardiol 2000;85:837-42
![Page 26: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/26.jpg)
Powerpoint Templates
5. Emodinamica
Fattori prognostici
Pressione media in arteria polmonare Resistenze vascolari polmonari Indice cardiaco Pressione media in atrio destro
.
![Page 27: Prognosi Ipertensione Polmonare Arteriosa-PAH PROGNOSIS](https://reader035.vdocuments.mx/reader035/viewer/2022062614/546663b0af795969338b51b7/html5/thumbnails/27.jpg)
Powerpoint Templates
Obiettivo paziente stabile e soddisfacente
In particular, the patient is characterized:
absence of clinical signs of RV failure, stable WHO-FC I or II without syncope, 6 min walk distance 500 m (depending on the individual patient), peak VO2 >15 mL/min/kg, normal or nearnormal BNP/NT-proBNP plasma levels, no pericardial effusion, TAPSE > 2.0 cm, right atrial pressure < 8 mmHg, CI > 2.5 L/min/m
Patients in this condition should fulfil the majority of the findings listed in the ‘better prognosis’ column