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Martino CheliDottorato di ricerca in biologia e fisiopatologia cardiaca, vascolare, renale e metabolicaDipartimento di Medicina InternaUniversità degli Studi di Genova
Ipertensione arteriosa polmonare: percorso diagnostico e stratificazione prognostica
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Ipertensione polmonare e malattie del cuore sinistro
Fang J et al. J Heart Lung Transplant 2012. Guazzi M, Borlaug B. Circulation. 2012Fang J et al. J Heart Lung Transplant 2012. Guazzi M, Borlaug B. Circulation. 2012
• Può essere riscontrata in ogni patologia del cuore sinistro, principalmente HF (indipendentemente da EF) e valvulopatie
• Prevalenza elevata, aumenta con la severità della patologia
• Più sintomi, intolleranza allo sforzo e peggiore QoL
• Impatta negativamente sulla sopravvivenza
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Bursi F et al. JACC 2012; 59:222
• N=1049 patients with HF (incident and prevalent) with TR jet• 60% had EF > 45%
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Quando sospettare una ipertensione polmonare dovuta amalattia del cuore sinistro
Galiè et al. Eur Heart J 2009
Clinical features Echocardiography Interim evaluation
• Age > 65
• Elevated systolic BP
• Elevated pulsepressure
• Obesity, metabolic syndrome
• Coronary artery disease
• Diabetes mellitus
• Atrial fibrillation
• Left atrial enlargement
• Concentric remodelling of the LV
• Echocardiographic indicators of elevated LV filling pressure
• Symptomatic response to diuretics
• Exaggerated increase in BP during exercise
• Chest X-ray consistent with HF
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Ecocardiogramma - Pressioni
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Ecocardiogramma – Funzione sistolica
RV Fractional Area Change
TAPSE
TDI
S WaveTEI Index
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Ecocardiogramma
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Essere o non essere PAH???
110110
00 15 mmHg
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Classificazione emodinamicaGradiente transpolmonare vs diastolico
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DPG predicts survival in patients with increased TPG
Gerges C et al. CHEST 2012; in press
Patients undergoing RHCN= 3,107
TPG < 12 mmHg N= 604
Group 2 PHN= 1,094
TPG > 12 mmHg N= 490
DPG < 7 mmHg N= 311
DPG > 7 mmHg N= 179
Cut-off for DPG determined by ROC-curve analysis
• 35% of patients had PH due to LHD, 55% purely passive• 36% of patients with TPG > 12 also had a DPG > 7 mmHg
determined by ROC curve analysis (16% in total)
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DPG predicts survival in patients with increased TPG
Passive PH: TPG < 12 mmHg
OOPH: TPG > 12 mmHg
Gerges C et al. CHEST 2012; in press
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Ipertensione arteriosa polmonare: stratificazione prognostica
J. Hurdman, R. Condliffe, C. A. Elliot,
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Prognosi per sottogruppi
McLaughlin VV. Chest. 2004;126:78S–92S
J. Hurdman, R. Condliffe, C. A. Elliot,
J. Hurdman, R. Condliffe, C. A. Elliot et al. ERS Congress 2010
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Time
Fu
nct
ion
al a
bil
ity
afte
r 1st
inte
rven
tion
• Death is infrequently the 1st event in PAH
• Interventions are indicated late in the course of the disease
• Hospitalizations and clinical deterioration are likely to occur first
Typical time course of PAH in clinical practice
Clinical modifications
Death
2nd interventionHospitalization
Courtesy of J.L. Vachiery
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Definition of patient statusStable and satisfactory Meets majority of the criteria in the column
« better prognosis »
Stable and
not satisfactory
Has not reached the goals that were desirable
Unstable and deteriorating
Meet majority of the criteria in the column « worse prognosis »
Definition of inadequate responseInitial FC II or III Stable and not satisfactory
Unstable and deteriorating
Initial FC IV No rapid improvement to FC III or better
Stable and not satisfactory
Galiè N, et al. Eur Heart J 2009; 30:2493-537.
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Utilità dei diversi indicatori prognostici
Vachiery JL et al. Eur Respir Rev. 2012;21:40-7
Variable Baseline Follow upNYHA FC +++ +++
6MWD +++ +++
BNP/NT-ProBNP +++ ++
Uric Acid + ?
cTroponin-t + ?
Pericardial effusion +++ +
TAPSE ++ + ?
Tei index ++ + ?
mPAP - -
RAP ++ ++
CI +++ +++
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Inquadramento (mono o) multidimensionale
− La maggior parte degli strumenti di impiego clinico per valutare laprogressione di malattia è influenzata dal VDX
− Non esistono mezzi per determinare lo stato di attività della malattia a livello del letto vascolare polmonare
Strumenti & Variabili
− Nessuna variabile da sola è in grado di rilevare la progressione di malattia e di guidare le scelte cliniche
− Per quanto auspicabile, l’inquadramento multidimensionale non è stato ancora validato (score prognostico, equazione o formula magica)
Stratificazione prognostica - Punti critici
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− L’ipertensione arteriosa polmonare è una patologia rara, la cui diagnosi è clinica, emodinamica e di esclusione
− Una valutazione multiparametrica è fondamentaleper l’inquadramento ed il follow up
− Per quanto il danno sia primitivamente vascolare polmonare, è il ventricolo destro a determinare la prognosi
Conclusioni
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• BKU SLIDES
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Caratteristiche dei pazienti con PAH (42)Età alla diagnosi, aa (range) 61 (27-83)
Femmine, n (%) 27 (64%)
Multifattoriale (1 o più cause di PH) 16 (38%)
• PAH• Cardiopatia sinistra• Pneumopatia/ipossia• CTEPH• Multifattoriale
3 (7%)2 (5%)8 (19%)-2 (5%)
Decessi 11 (26%)
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Diagnosi finale WHO FC
I II III
1.1 Ipertensione arteriosa polmonare idiopatica 1.4.1.1 Associata con m. del tessuto connettivo (sclerodermia)1.4.2 Associata ad infezione HIV1.4.3 Associata ad ipertensione portale1.4.4 Associata a cardiopatia congenita
715389
11-24*
310243*
34121*
Tot 42 8 22 11
*1Pt. Missing FC
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Diagnosi finale WHO FC
I II III
1.1 Ipertensione arteriosa polmonare idiopatica 1.4.1.1 Associata con m. del tessuto connettivo (sclerodermia)1.4.2 Associata ad infezione HIV1.4.3 Associata ad ipertensione portale1.4.4 Associata a cardiopatia congenita
715389
11-24*
310243*
34121*
Tot 42 8 22 11
*1Pt. Missing FC
Diagnosi finale WHO FC
I II III
4. CTEPH 15 9 4
*2Pts. Missing FC
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Ipertensione arteriosa polmonare: Cateterismo cardiaco destro & 6MWT
• PAPm, media 43,5 +/-15 (25-95) mmHg• PVR, media 532 +/-320 (125-1225) dynes• CI 2,1 +/- 0,7 L/min/m2• RA, media 9,6 +/- 4 (2-22) mmHg
• 6MWT m 346 +/- 143• Pre Spo2 % 96 +/- 4,6• Post SpO2 % 90 +/- 7,9
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Ipertensione arteriosa polmonare: Terapia medica
PAH: 15/42 •12 AET; •1 IPDE5; •2 (AET + IPDE5);•1 (AET + IPDE5 + Prost.Inal)CTEPH: 7/15 •4 AET•3 AET + IPDE5 [2 + Prost Inal]•4 TEAP (-> 1AET; 1 in Trp di combinazione)
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Ipertensione arteriosa polmonare: Terapia medica
PH in ILD «out of proportion» (WHO III) 4/25pts•2 AET•2 IPDE5
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Ipertensione polmonare «out of proportion»
PH in ILD «out of proportion» (WHO III) 4/25pts•2 AT•2 IPDE
Mortalità: 52% (13/25)
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Ipertensione arteriosa polmonare: Terapia medica - AE
• 2 (7%) Reazioni allergiche cutanee (1AET; 1
AET e IPDE5)• Comune «intolleranza» [Cefalea, Capogiri,
Dispnea], richiesta interruzione in 5 (18%) pts
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Conclusioni
• L’ipertensione arteriosa polmonare è una patologia rara con prognosi severa
• Richiede una diagnosi tempestiva ed un trattamento mirato, nell’ottica di prevenire i fenomeni di remodeling vascolare
• Una strategia «attendista» non sempre paga!
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Ipertensione arteriosa polmonare: prognosi
McLaughlin VV. Chest. 2004;126:78S–92S
J. Hurdman, R. Condliffe, C. A. Elliot,
J. Hurdman, R. Condliffe, C. A. Elliot et al. ERS Congress 2010