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Page 1: HIPERTENSIUNEA PULMONARA

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

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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

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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

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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

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HIPERTENSIUNEA

PULMONARA (HTP)

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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

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VASOCONSTRICTIE

Hipoxia

Acidoza

Prostaglandine F2asi A2

HISTAMINA –H1

SEROTONINA ?

ANGIOTENSINA A2

ALTITUDINE

VASODILATATIE

Alcaloza

PROSTAGLANDINE I2si E

BLOCANTI a

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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

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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

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Anemiehemoliticacronica

1.5. HTP persistentala nounascut

1’ Boalavenoocluzivapulmonarasi/sauhemangiomatozacapilarapulmonara

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CLASIFICAREA HTPDana Point, 2008

2. HTP prinsuferintaventricululuistang

2.1. Disfunctiesistolica

2.2. Disfunctiediastolica

2.3. Bolivalvulare

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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

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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

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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)

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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

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FIZIOPATOLOGICE

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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

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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

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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

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Hypertension

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SINDROM

EISENMENGER

.Toatesunturilesistemico-pulmonarerezultanddin

maridefectecare ducla cresteride presiunein VD sila inversareasuntului(pulmonar-sistemic) sausuntbidirectional cu: cianoza, eritrocitozasimultiple

suferintede organ

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Eisenmenger syndrome

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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

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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-

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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),

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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

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immunohistochemistry (arrow).

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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).

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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

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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

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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

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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

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Rx înHTP

Mecanisme

.Sechestrarea de lichid interstitial in lobii inferiori

.

.Presiunea interstitiala .

.

.Complianta pulmonara .

.

.Fluxul spre lobii inferiori .

+

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.Spasm arterial

.Fluxul este redistribuit spre lobii superiori

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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

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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

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HIPOXIE. raspuns anormal . disfunctie endoteliala

Modificarea raportului EDRF -endoteline

Distrugeri de endoteliu

Tromboza

Vasoconstrictie . necroza fibrinoida

Leziuni plexiforme

HTPIDIOPATICA

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HTPIDIOPATICA

ASPECTE CLINICE

.Femei tinere

4 simptome principale

.Dispnee de efort

.Accentuarea zgomotului II

.Modificari Rx –cardiomegalie

-a. pulmonara proeminenta

.Modificari ECG : -HVD

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-deviatie axiala dr.

-HAD

Mai rar:

-Ameteli si sincope de efort

-Dureri toracice de efort

-Edeme

-Fenomene Raynaud

-Istoric de tromboflebita superficiala

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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

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.Ruptura de leziuni plexiforme

.Tromboze in situ

.Embolism pulmonar

DUREREA TORACICA

.Ischemia subendocardului VD

.Distensia a pulmonare

HTPIDIOPATICA

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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

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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

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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

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HTPIDIOPATICA

DIAGNOSTIC DIFERENTIAL

HTP secundara (mai benigna si mai tratabila)

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.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

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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

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< 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

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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

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-Digoxinul creste DC in administrarea acuta in HTPI, efectul sau in

administrarea cronica este discutabil

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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

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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

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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

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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

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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

Page 57: HIPERTENSIUNEA PULMONARA

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)

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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.

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Prostanoid analogues

CTD= boalade tesutconjunctiv

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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

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administered at ambient temperature

allow iv / subcutaneous /oral ( bid )

and inhalatoryadm.(6-9 d )

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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

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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)

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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 )

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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

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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

Page 67: HIPERTENSIUNEA PULMONARA

TRATAMENTUL

MEDICAL(IFD)

7.Inhibitoride fosfodiesteraza(Sildenafil)

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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.

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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

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vardenafil),

.impact on arterial oxygenation (improvement with sildenafil only).

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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

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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

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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

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Prostaglandine

+/-transplant

Warfarina + diuretic + digoxin

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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 ?

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Probably yes , but economically unwise

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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

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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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