HIPERTENSIUNEA PULMONARA

CIRCULATIA
PULMONARA NORMALA

.-oxigenareHb
.-filtru(particule,bacterii)
.-eliminareaCO2echilibruacido-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 NOendoteline

-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 (ISOPROTERENOL)

ACETILCOLINA (prin
EDRF)

HISTAMINA (prin H2?)

INDOMETACINcreste 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 exerciseinduced 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(pulmonarsistemic) 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 (35 oclock) 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 revascularized 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 nHTP

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 nHTP

Rx nHTP

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 nHTP

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

CTFa 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 of ptstolerate maximum dosage

TRATAMENTUL
MEDICAL(PG)

5. Prostaglandineleefectele 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 g/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 = 20g. 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): 211925

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: 142536.

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

F:\DCIM\101MSDCF\DSC02181.JPG