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HIPERTENSIUNEA PULMONARA
CIRCULATIA
PULMONARA NORMALA
.-oxigenareHb
.-filtru(particule,bacterii)
.-eliminareaCO2–echilibruacido-bazic
CIRCULATIA CIRCULATIA
PULMONARABRONSICA
Sange venossange arterial
a. pulmonaraa. bronsice
CapilareCapilare
Vene pulmonare Vene sistemice
PLAMANUL
CIRCULATIA BRONSICA
.Sunt fiziologic dr-stg
(pana la 30% din DC)
-bronsiectazii
-fibroza chistica
-boli congenitale
cardio-vasculare
HIPERTENSIUNEA
PULMONARA (HTP)
NORMAL
A SISTEMICE –media 20-25% din diam. vasului
A. PULMONARE -media < 10-5% din diam. vasului
Arteriolele pulmonare nu au tunica medie si nu contribuie
la rezistenta vasculara
VD –fluxul coronarian cel mai mare in sistola
-depinde de gradientul pres. pulm. –aorta
Pres. VD creste –gradientul scade –fluxul coronar drept
scade –ischemie VD
NORMAL
PRES. A. PULMONARA -sist. 18-25 mm Hg
-diast. 6-10 mm Hg
-medie 12-16 mm Hg
PRES V. PULMONARE –2-10 mm Hg
REZIST. VASC. PULM. = 1/10 din REZIST. SISTEMICA
HIPERTENSIUNEA PULMONARA (HTP)
PRES. A. PULMONARA -sist. > 30-35 mm Hg
-medie > 20-25 mm Hg
-diast. > 15 mm Hg
.Reducerea calibrului vaselor pulmonare
.Cresterea fluxului
HIPERTENSIUNEA
PULMONARA (HTP)
REACTIVITATEA
VASCULARA PULMONARA
HIPOXIA VASOCONSTRICTIE PULMONARA
-histamina –receptori H1vasculari
-endoteliu -echilibru NO–endoteline
-patrunderea Ca 2+in celula musculara neteda
HIPOXIA CRONICA
1.Extensia musculaturii netede in peretele arterelor din
periferia plamanilor
2.Ingrosarea peretilor arterelor musculare
3.Reducerea nr. arterelor –cresterea raportului alveole/artere
VASOCONSTRICTIE
Hipoxia
Acidoza
Prostaglandine F2asi A2
HISTAMINA –H1
SEROTONINA ?
ANGIOTENSINA A2
ALTITUDINE
VASODILATATIE
Alcaloza
PROSTAGLANDINE I2si E
BLOCANTI a
STIMULARE ß(ISOPROTERENOL)
ACETILCOLINA (prin
EDRF)
HISTAMINA (prin H2?)
INDOMETACIN–creste rezistentapulmonara
La 10 000 m altitudine
TA pulmonara medie = 25 mm Hg in repaus
> 50 mm Hgin efort
CLASIFICAREA HTPDana Point, 2008
1.HTP arteriala
1.1. Idiopatica
1.2. Ereditara
1.3. Indusade drogurisitoxine
1.4. Asociatacu:
Bolide colagen
HIV
Hipertensiuneportala
Bolicardiacecongenitale
Schistosomiaza
Anemiehemoliticacronica
1.5. HTP persistentala nounascut
1’ Boalavenoocluzivapulmonarasi/sauhemangiomatozacapilarapulmonara
CLASIFICAREA HTPDana Point, 2008
2. HTP prinsuferintaventricululuistang
2.1. Disfunctiesistolica
2.2. Disfunctiediastolica
2.3. Bolivalvulare
CLASIFICAREA HTPDana Point, 2008
3. HTP prinbolipulmonaresauhipoxie
3.1. BPOC
3.2. Boliinterstitiale
3.3. Altebolicu restrictiesiobstructie
3.4. Apneeade somn
3.5. Bolicu hipoventilatiealveolara
3.6. Expunereacronicala mare altitudine
3.7. Anomaliide dezvoltarefizica
CLASIFICAREA HTPDana Point, 2008
4. HTP printromboembolism
5. HTP prinfactorimultiplineclari
5.1. Bolihematologice: mieloproliferare, hipersplenism
5.2. Bolisistemice: sarcoidoza, histiocitozapulmonaracu celuleLangerhans
5.3. Bolimetabolice: B. Gaucher, glicogenoze, disfunctiitiroidiene
5.4. Altele: obstructiitumorale, mediastinitafibrozanta,,
IRC saudializa
CATEVA MECANISME
FIZIOPATOLOGICE
In suferintainimiistangi
PRESIUNE ATRIU STG =7 mm Hg
scade rezistenta pulmonara (recrutare de vase)
>7 mmHg –creste presiunea in a. pulmonara (fluxul ramane
constant; gradientul ramane constant)
> 25 mmHg –crestere disproportionata de presiune in a.
pulmonara (gradientul creste; fluxul constant sau scade)
Variabilitatea reactivitatii vasculare pulmonare:
.creste presiunea venoasa –distrugere sau inchidere de cai
aeriene –hipoxie –creste presiunea in a. pulmonara
.creste presiunea venoasa –edem interstitial –rigidizarea
vaselor –HTP
.drenajul limfatic creste
.starea VD
.normal
.hipertrofic
.insuficient
.miopatic (+ VS)
.hipoperfuzat (infarct)
.Volumul sanguin pulmonar (depinde de debitul celor 2
ventriculi si de distensibilitatea vaselor pulmonare)
CATEVA MECANISME
FIZIOPATOLOGICE
MODIFICARI ANATOMICE
-Celule endoteliale capilare umflate
-Membrane bazale capilare ingrosate
-Edem interstitial
-Rupturi de membrane bazale –transudare de eritrocite –
hemosideroza
-Alveole fibroase
-Destindere de limfatice
Test
Notable Findings
Chest x-ray
Enlargement of central pulmonary arteries reflects level of PA pressure
and duration.
Electrocardiography
Right axis deviation and precordial T wave abnormalities are early signs.
Pulmonary function tests
Elevated pulmonary artery pressure causes restrictive physiology.
Perfusion lung scan
Nonsegmental perfusion abnormalities can occur from severe pulmonary
vascular disease.
Chest computed tomography scan
Minor interstitial changes may reflect diffuse disease; mosaic perfusion
pattern indicates thromboembolism and/or left heart failure.
Echocardiography
Right ventricular enlargement will parallel the severity of the pulmonary
hypertension.
Contrast echocardiography
Minor right to left shunting rarely produces hypoxemia.
Doppler echocardiography
This is too unreliable for following serial measurements to monitor
therapy.
Exercise testing
This is very helpful to assess the efficacy of therapy. Severe exercise-
induced hypoxemia should cause consideration of a right-to-left shunt.
TABLE 73-2--Clues for Interpretation of Diagnostic Tests for Pulmonary
Hypertension
SINDROM
EISENMENGER
.Toatesunturilesistemico-pulmonarerezultanddin
maridefectecare ducla cresteride presiunein VD sila inversareasuntului(pulmonar-sistemic) sausuntbidirectional cu: cianoza, eritrocitozasimultiple
suferintede organ
Eisenmenger syndrome
MODIFICARI ANATOMICE
GR. I : hipertrofia mediei artereor mici musculare
GR. II : + proliferarea intimei
GR. III: + fibroza concentrica cu obliterare de vase
GR. IV: “leziuni plexiforme”, dilatatii, trombi
GR. V: complexe plexiforme, leziuni angiomatoase si cavernoase si hialinizaea
fibrozei intimale
GR. VI: arterita necrozanta
An external file that holds a picture, illustration, etc.
Object name is nihms20458f1.jpg Object name is nihms20458f1.jpg
Histopathology of endothelial cell lesions in
IPAH. A. Pulmonary artery showing medial
hypertrophy and lined by a single layer of
endothelial cells, as outlined by Factor VIII
related antigen immunostaining(arrow).
Plexiformlesion (outlined by the rim of
arrowheads) with the proximal vascular arterial
segment with marked intimal and medial
thickening by smooth muscle cells (arrow). Note
the proliferation of endothelial cells with the
outer edge (3–5 o’clock) occupied by dilated
blood vessel-like structures. C. Cross section of
a plexiformlesion, outlined by arrowheads. Note
perilesionalinflammatory infiltrate (arrow). D.
High magnification histology of plexiformlesions
shown slit-like vascular channels lined by
hyperchromaticand cuboidal endothelial cells.
Cells in the core do not display distinct
cytoplamicborders. E. Low magnification
immunohistologywith Factor VIII related antigen
immunohistochemistry of different endothelial
cell based vascular lesions. This area has re-
vascularized lesions (possibly an organized
thrombus), with well-formed and distinct small
capillaries/vessels (arrowhead), a plexiformlesion (arrow), and dilated/angiomatoidlesions
(between arrowheads). F. High magnification
immunohistologyof cellular plexiformlesion
stained with Factor VIII related antigen
(arrowheads). G and H. Histological
identification of plexiformand dilation lesions (G)
is markedly improved by Factor VIII related
antigen immunohistochemistry (H)
(arrowheads), while the parent vessel (arrow)
shows mild medial remodeling. I. Highlight of
vascular dilation/angiomatoidlesions with Factor
VIII related antigen immunohistochemistry. J.
Endothelial cells in plexiformlesion is
highlighted by CD34 immunohisochemistry(arrowheads). Proximal pulmonary artery with
marked intima and medial thickening is
highlighted by the arrow. K and I. Endothelial
cells are highlighted by CD31
immunohistochemistyr(arrowheads). Note that
capillary endothelial cells express CD31 as well
(arrow in I),
An external file that holds a picture, illustration, etc.
Object name is nihms20458f3.jpg Object name is nihms20458f3.jpg
A. Fibrotic, relatively paucicellularintima thickening (outlined by
arrowheads) in a pulmonary artery
with the media highlighted with the
arrow. B. Marked intima remodeling
with almost complete obliteration by
fibrous tissue with a marked
intravascular and perivascular
inflammatory infiltrate (arrows). C.
Smooth muscle cell hypertrophy, with
prominent thickening of medial layer
(arrow). D. Highlight of medial
hypertrophy with smooth muscle a
actin immunohistochemistry. E.
Markedly remodeled pulmonary artery
with endothelial cell layer highlighted
by Factor VIII related antigen
immunohistochemistry. Note that the
intima and medial smooth muscle
cells are negative for Factor VIII
related antigen reactivity. F. Ingrowth
of smooth muscle cells in a plexiformlesions, highlighted by smooth
muscle cell a actin
immunohistochemistry (arrow).
An external file that holds a picture, illustration, etc.
Object name is nihms20458f5.jpg Object name is nihms20458f5.jpg
Veno-occlusive PH. A. Low-power histological view of thickened pulmonary veins running into the lung parenchyma from the
pleural surface (left edge) (arrows). Note marked vein wall thickening and decreased lumen. Adjacent alveoli are filled with blood
and show septalthickening with engorged capillaries (arrowhead). B. Marked vein thickening with intimal projection probably
representing organized thrombus (arrow). Alveolar hemorrhage and septalthickening are highlighted with arrowhead. C and D.
Movatstained pulmonary vein showing arterialization pattern with internal and external elastic layers (arrow). The vein shows
marked intima thickening with organized thrombus (arrowheads).
Rx înHTP
Normal
.Flux crescut in lobii inferiori
Gravitatie
Presiuni diferite intra –alveolare
Raport A/B = 1,2 : 1
CRESTEREA FLUXULUI PULMONAR
FLUX -FVASE x 8 (rezerva) +
.Fvase -flux + presiune
-presiune
.Creste Fvenos
Rx –1/3 ext. vascularizata
-Circ. Inf = circ. sup.
N –Fa. pulm. desc. dr. = 10-15 mm la barbati si 9-14 mm la femei
HTP arteriala
-vasoconstrictie periferica
-vasospasm
-ingrosarea peretelui vascular
Rx
-scade circulatia (creste transparenta) in 1/3 ext.
-vasele centrale elastice se largesc
-calcificari ale vaselor centrale
Rx înHTP
Rx înHTP
HTP de origine venoasa
P venoasa > 8 –12 mm Hg
.fluxul pulmonar este redistribuit spre lobii superiori
Rx –inversare a aspectului normal (cefalizarea fluxului arterial si venos)
P venoasa > 25 mm Hg
.Edem pulmonar
Rx înHTP
Mecanisme
.Sechestrarea de lichid interstitial in lobii inferiori
.
.Presiunea interstitiala .
.
.Complianta pulmonara .
.
.Fluxul spre lobii inferiori .
+
.Spasm arterial
.Fluxul este redistribuit spre lobii superiori
HTPIDIOPATICA
ETIOLOGIE
.Embolism pulmonar recurent, asimptomatic
.Embolism amniotic
.Tromboza in situ,tulburari de coagulare si fibrinoliza ,contraceptive
.Vasoconstrictie cronica . necroza fibrinoida . leziuni plexiforme
.Vasculita generala cu fenomen Raynaud
.Hipersensibilitate la droguri
.Ingestia de fumarat de aminorex (anorexigen)
.Hormoni feminini
HTPIDIOPATICA
MODIFICARI HISTOLOGICE
.Ingrosarea intimala a a. mici si arteriole cu fibroza in
“foi de ceapa”
.Ingrosarea mediei a. musculare si muscularizarea
arteriolelor
.Arterita necrozanta cu necroza fibrinoida
.Leziuni plexiforme . arteriopatie pulmonara
plexogenica . umbre vasculare . reducerea patului
vascular
HIPOXIE. raspuns anormal . disfunctie endoteliala
Modificarea raportului EDRF -endoteline
Distrugeri de endoteliu
Tromboza
Vasoconstrictie . necroza fibrinoida
Leziuni plexiforme
HTPIDIOPATICA
HTPIDIOPATICA
ASPECTE CLINICE
.Femei tinere
4 simptome principale
.Dispnee de efort
.Accentuarea zgomotului II
.Modificari Rx –cardiomegalie
-a. pulmonara proeminenta
.Modificari ECG : -HVD
-deviatie axiala dr.
-HAD
Mai rar:
-Ameteli si sincope de efort
-Dureri toracice de efort
-Edeme
-Fenomene Raynaud
-Istoric de tromboflebita superficiala
PROGNOSTIC
.Prost (supravietuire peste 5 ani –21%)
.Anticoagulantele imbunatatesc prognosticul
MOARTEA
.Insuficienta cardiaca congestiva
.Pneumonie
.Moarte subita
.Moarte la cateterism
.Disectie de a pulmonara
HEMOPTIZIA IN STADII TARDIVE
.Ruptura de leziuni plexiforme
.Tromboze in situ
.Embolism pulmonar
DUREREA TORACICA
.Ischemia subendocardului VD
.Distensia a pulmonare
HTPIDIOPATICA
SEMNE CLINICE
.Zgomot II intarit la pulmonara
.Suflu sistolic la pulmonara
.Semne de insuficienta cardiaca dreapta
.Semne de regurgitare triscuspidiana
.Cianoza -tardiv prin deschidere de foramen ovale
.Paralizie de recurent (rara)
HTPIDIOPATICA
HTPIDIOPATICA
LABORATOR -Uneori defecte de coagulare si fibrinoliza
ECG: HVD, HAD
Rx:largirea a pulmonare; marirea atriului si ventriculului dr
CT–Fa pulmonare
TESTE FUNCTIONALE -Disfunctie restrictiva
ECHOCARDIOGRAFIA
Marirea atriului si ventriculului drept
Cavitati stangi normale
Ingrosarea septului
Regurgitare tricuspidiana si prolaps de valva mitrala secundare dilatatiei
de VD
HTPIDIOPATICA
SCINTIGRAFIA PULMONARA-normala
In stadii avansate poate fi daunatoare –trasorul legat de albumina –
procoagulant
CATETERISMUL CARDIAC SI ANGIOGRAFIA
.RISCANTE
.Presiunea in VD egala sau chiar mai mare decit presiunea sistemica
.Rezistenta vasculara pulmonara depaseste uneori pe cea sistemica
.Uneori foramen ovale este patent
.Presiunile stg. sunt normale sau mici, dar uneori greu de determinat
BIOPSIA PULMONARA
HTPIDIOPATICA
DIAGNOSTIC DIFERENTIAL
HTP secundara (mai benigna si mai tratabila)
.In sala de cateterism cardiac
.PAPm dupa administrarea de NO inhalator (sau adenozina iv,
epoprostenol iv sau inhalator)
Test + = reducerea cu 20% a PAPm sau a rezistentei vasculare
pulmonare –bolnavul va primi vasodilatator indelungat
TESTUL
VASODILATATOR
PROGNOSTICUL
Supravietuirea medie in HTP netratata= 2,8 ani
Factori de prognostic:
Varsta< 14 ani sau 65 ani –prognostic prost
Clasa NYHA:I –II: supravietuire 6 ani in medie
III: supravietuire 2,5 ani in medie
IV: supravietuire 0,5 ani in medie
Scaderea capacitatii de efort
Sincopa
Hemoptizie
Semne de insuficienta ventriculara dreapta
O2 in a pulmonara> 63 –55% supravietuire la 5 ani
< 63 –17% supravietuire la 5 ani
Indexul cardiac< 2,1 l/min/m2 supravietuire medie 17 luni
Presiunea in AD< 10 mmHg -supravietuire 4 ani in medie
> 20 mmHg -supravietuire medie o luna
Test de vasodilatatienegativ
TRATAMENTUL
MEDICAL
1. Anticoagulantele
Warfarina dubleaza supravietuirea in HPP
Indicatiile anticoagularii permanente: toti pacientii cu HTPI
Tromboembolismul pulmonar (INR = 2-3)
HTP secundare, daca nu exista contraindicatii
2. Oxigenoterapia
Se recomanda monitorizarea Sat O2nocturna, Sat O2< 90% in aerul
atmosferic corectabila la administrarea de O2, indica oxigenoterapia
nocturna
3. Tratamentul insuficientei ventriculare drepte:
-Diuretice
-Digoxinul creste DC in administrarea acuta in HTPI, efectul sau in
administrarea cronica este discutabil
4. Tratamentul vasodilatator
Antagonistii de Ca (diltiazem sau nifedipina):
.HTP de tip arterial cu test vasodilatator pozitiv
.CI in : HTP venoasa (precipita EPA)
HTP hipoxica din bolile cronice pulmonare cu Sat O2 in sangele
venos < 63% (agraveaza hipoxemia)
PAD > 10 mm Hg
Index cardiac < 2,1 l/min/m2
TRATAMENTUL
MEDICAL
Responders: Ca.-blockers
.(if bradicardic)
Nifedipine:120 -240 mg
.(if tahicardic)
Diltiazem240-720 mg
Begin low dosage , increase weekly
Less than ½ of ptstolerate maximum dosage
TRATAMENTUL
MEDICAL(PG)
5. Prostaglandinele–efectele pozitive ale tratamentului
indelungat (min 3 luni)
Reducerea rezistentei vasculare pulmonare
Cresterea indexului cardiac
Dublarea supravietuirii la 5 ani
Reducerea riscului si ameliorarea evolutiei dupa transplantul
pulmonar
TRATAMENTUL
MEDICAL
Indicatii
.Bolnavii cu ICC cl III –IV, index cardiac < 2,1
l/min/m2si/sau Sat O2in artera pulmonara < 63%
si/sau PAD > 10 mmHg, indiferent de testul
vasodilatator
.Toti bolnavii care nu raspund la tratamentul medical
conventional, in asteptarea transplantului pulmonar
TRATAMENTUL
MEDICAL(PG)
Efecte adverse:
.Diaree, dureri abdominale, cefalee, flush, artralgii,
dureri musculare
.Ascita, edem pumonar (prin cresterea permeabilitatii
vasculare)
.Toleranta ce necesita cresterea dozelor
.Rebound al HTP la intreruperea brusca a
tratamentului
.Infectii de cateter
Preparate folosite:
Epoprostenol= PGI2 iv. Se incepe cu 2 ng/kc/min in pev continua si se
creste doza dupa o saptamana pana la doza maxima tolerata de
pacient
Iloprost= analog al epoprostenolului, mai puternic iv (pev continua)
sau in aerosoli, 6-9 inhalatii/zi (50 -200 µg/zi)
Trepostenil(UT –15) este analog de PGI2. Doza initiala este de 1,25
ng/kc/min si se creste cu 1,25 ng/kc/min la 7 zile pana la 9,3
ng/kc/min
TRATAMENTUL
MEDICAL(PG)
TRATAMENTUL
MEDICAL(PG)
.Beraprost:derivat stabil de PGI2, poate fi adminisrat
p.o. 1 tb = 20µg. Se incepe cu 1 tb/zi si dupa 6
saptamani de titrare, se ajunge la 6 tb/zi (studiul
ALPHABET). Rezultate bune in HTPI,
neconcludente in HTP sec. Se pare ca nu este eficient
in stadiile avansate de boala.
Prostanoid analogues
CTD= boalade tesutconjunctiv
Epoprostenol
short HL, temp.-dependent , i-v (infusion pump ) , local facilities
2-4ng/kg/min ..20-40 ( tolerance , rebound , adverse reactions: common)
>100.000 $ /year
Rubin LJ Ann. Intern.Med. 1990;112:485-92
BarstRJ N.Engl. J Med 1996;334:296-304
BadeschDB Ann. Intern.Med. 2000;132:425-34
3 month results: indic. surv/altern
Conversion to oral agents ??
Treprostenil
sufficient chemical stability to be
administered at ambient temperature
allow iv / subcutaneous /oral ( bid )
and inhalatoryadm.(6-9 d )
Beraprost
Orally :40-80microg qid/zi
efficacy does not appear to be sustained
with extended duration of therapy
Iloprost
Inhalations 6-12 times/d
(20-40 microg/d.)
Advant: selective to pulm.circ.
J Am CollCardiol. 2003 Jun 18;41(12): 2119–25
6. Antagonistii receptorilor de endotelina
Bosentan(ET1RA) po. Doze: 62,5 mg de 2 ori/zi, pana la 125 mg
de 2 ori/zi, timp de 16 saptamani.
TRATAMENTUL
MEDICAL(ARE)
Endothelin receptor antagonists
Type A receptor:
vasoconstriction, proliferation, fibrosis.
Type B receptor (endotelial):
increases the synthesis of nitric oxide
( vasodilation )
Type B receptor (SMC):
activates aldosterone,
thromboxane, norepinephrine.
( vasoconstriction )
Bosentan( Tracleer) available in Romania
dual ETA and ETB-receptor antagonist
125 mg bid BREATHE-1
BREATHE-3 (children )
10% liver enzimes> BREATHE -5
EARLY
Sitaxsentan
6500 times greater affinity for the ETA STRIDE I and II
Chest ,2008; 134(4): 775 -782.
100-300 mg od 2004 : Level of evidence : B
Incl.HTP in congenitals/CTD aprovedin Europe
Warfarineinterference
AmbrisentanARIES-2 . Am J RespirCirtCare Med. 2006;173:
lower incidence of liver enzyme abnormality Ann. Pharmacother,2008; 42(11):
1653
absence of significant drug interactions -
2004 : Level of evidence : C
TRATAMENTUL
MEDICAL(IFD)
7.Inhibitoride fosfodiesteraza(Sildenafil)
Phosphodiesteraseinhibitors
Sildenafil ( REVATIO )
25 mg t.i.d.
Available in Romania
25 mg t.i.d.
Available in Romania
HumbertM N EnglJ Med 2004;351: 1425–36.
Type 5 Phosphodiesterase inhibitors
.VardenafilHCL( Levitra ) Br J Pharmacol., 154(4):787-96. 2008 Apr
21
.Tadalafil( Cialis )
longer half-life (17.50 hours )
marketing approval began in late 2008
J Am CollCardiol, 2004; 44:1488-1496
Circulation. 2004;110:3149-3155
The three PDE5 inhibitors differ markedly in :
.kinetics of pulmonary vasorelaxation(most rapid effect by vardenafil)
.selectivity for the pulmonary circulation (sildenafil and tadalafil, but
not
vardenafil),
.impact on arterial oxygenation (improvement with sildenafil only).
TRATAMENTE
CHIRURGICALE
.Septostomie atriala.Procedeu paleativ ce scade presiunea
in inima dreapta.
.Indicatii:
.HTP severa, care nu raspunde la prostaglandine
.Se exclud pacientii cu Insuf Ventr dr severa, disfunctie de VS sau
in stare critica
.Trombendarterectomie
.Transplantpulmonar
TRATAMENTE
CHIRURGICALE
Indicatiitransplant
HTPIsimptomatica, progresiva in ciuda tratamentului
medical optim, cu test de mers de 6 min < 400 m, cu
index cardiac < 2,1 l/min/m2si/sau Sat O2in artera
pulmonara < 63% si/sau PAD > 10 mm Hg sau PAP
m > 55 mmHg
Test vasodilatator acut
Raspuns +
Sv O2>63%, IC > 2,1
Raspuns -
NYHA I/II, Sv O2>63%,
IC > 2,1
NYHA III/IV, Sv O2<63%,
IC < 2,1
Blocanti de Ca
Nu raspund la tratament
Prostaglandine
+/-transplant
Warfarina + diuretic + digoxin
Frequently asked
questions
.At which level of pulm.pressure should we
begin pharmacological treatment in sec. PHT ?
Adapted to etiology ! Unknown borderline !
.Is it harmful to use CCB in nonresponders?
Yes , at least for high doses
ACCP Gd.: Level of evidence: expert opinion; benefit: substantial;
grade of recommendation: E/A.
.Would it be better to use the more active drugs
for responders also ?
Probably yes , but economically unwise
Frequently asked
questions
.How useful is multiple drug therapy
Which order of introduction /doses ?
BREATHE -2
.Is atrial septostomyan option ?
Rarely (bridge to… ) ; 5-15% mortality
CONCLUSIONS
.PPHT ptsto be treated in dedicated centers
.New therapies available ; debate on results
.Combination therapy in developpement
.Rapid change of recomandations/guidelines
.Cost –effectiveness analysis vital
.Hard end points-including mortality may be influenced
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