ctRcuLATlA P.ULMONARA NORMALA 'oxigenare Hb .fi ltru (particule, bacterii) .

eliminarea CO, - eehilibru acido-bazic PLAMANUL

CIRCUL,ATIA PULMONARA singe venos
a.

qIRCULATIA BRON$CA singe arterial

1% DC t tur--

pulponari
*
Y

Capilare 'l

Vene pulmonare

*--- \

_ Capifare '+

a. brongice v

Vene sistemice -=t Sunt fizioloqic dr-stq (pand la 3O% din DCJ
- brongiectazii
- fibrozi

chistici

NORMAL

- boli congenitale - cardio-vasculare

PRES. A. PULMONARA - sist. 18 - 25 mmHg - diast. 6 - 10 mmHg - medie 12 - 16 mm Hg PRES. V. PULMONARE -2-10mmHg REZIST.VASC.PULM. = 1 ng din REZIST.SISTEMICA

HIPERTENSIUNEA PULMONARA {HTP

PRES.A.PULMONA - sist > 30 mmHg .reducerea calibrului vaselor pulmonare .cregterea fluxului

NORMAL A. SISTEMICE - media 20-25% din diam. vasului A. PULMONARE - media<10-5% din diam.vasului Arteriolele pufmonare 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. PUTMONARA - sist. 18 - 25 mmHg - diast. 6 - 10 mmHg - medie 12 - 16 mm Hg PRES. V. PULMONARE - 2 - 10

mmHg

'*

REZIST.VASC.PULM. = 1 110 din REZIST.SISTEMICA

H

TPERTE NS I UN EA

PRES.A.PULMONARA - sist > 30-35 mmHg - medie >20-25 mmHg - diast. >15 mmHg

IULMONA RA (HTP)

.reducerea calibrului vaselor pulmonare .cregterea fluxului

REACTIVITATEA VASCULARA PULMONARA'

HrPOXrfi <--> VASOCONSTRTCTTE PULMONARA
- histamina - receptori H,' vasculari
-

endoteliu - echilibru EDRF - endoteline

- pitrunderea Ca** in celula musculard netedd

HIPOXIA CRONTCA
1. Extensia musculaturii netede in peretele arterelor din periferia

pldminilor

2. ingrogarea peretilor arterelor musculare

3. Reducerea nr.arteretor - cregterea raportului alveole / artere VASOCONSTRIGTIE - Hipoxia

-

VASODILATATIE
- Alcaloza
- PROSTAGLANDINE lr gi E
Fzo

Acidoza
gi Az

- PROSTAGLANDINE
- HISTAM|NA - Hl
- SEROTONINA ? - ANG|OTENStNAz

- BLOCAN|I o
- STIMULARE B
(f

SoPRoTERENOL)

- ACETILCOLINA

(prin EDRF?)

. ALTITUDINE

- HISTAMINA (prin Hr?l INDOMETACINA - cregte rezistenfa

pulmonari
La 10000 m altitudine TA pulmonari medie = 25 mmHg in repaus
> 50 mmHg la efoft

HTP SECUNDARA

pufiaoNAR
A. CRE$TEREA pREStUNlt DTASTOLTCE
VENTRICULARE B. HIPERTENSIUNEA ATRtalA srArucA

c. oBSTRUcnA vENoasA PULMouanA

vnseutAR PULMorunn
A. BOLI ALE PARENCHIMULUI PULMONAR
B. SINDRO[ii EISENMENGER c, ALTE coNDrnr AsocrATE cu ScADEREA SEcTIUNII PATTJI-uI VAScULAR

MA PULMONARE

A. TROMBOEMBOLISMUL PUTMONAR B. srENozA DE A.puLMoNanA pERrFEnrcA c. ABSENTA coNGEbrrAlA pE A.puLmorunnA uNr sAU eiinrenniA

A. OBEZITATEA B. BOLI NEUROMUSCULARE C. BOLI ALE PERETELUI TORACIC

A. EDEMUL PULMONAR DE ALTITUDINE

B. TETRALOGIA FALLOT C. HEMOGTOBINOPATII D. PRorErNozA ALVEomnA E. ABUZDE DROGURI i.v. F. BOALA TAKAYASU

CRE$TEREA REZISTENTEI LA DRENAJUT VENOS putMoNAR

MECANISME PRESIUNE ATRTU STG. - T mmHg

Ir

(recrutare de vase) ,t 7 mmHg - cregte presiunea in a.pulmonari (ftuxul t_ ramane constant; gradientul rimdne constant) +
> 25 mmHg - cregtere dispropor,tionati de presiune in

I i Y
I

scade rezistenfa pulmonare

a.pulmonari (gradientul cregte; fluxul

constant sau scade) Variabilitatea reactivitatii vasculare pulmonare: Fcregte presiunea venoasi - distrugere sau inchidere de aeriene - hipoxie - cregte presiunea in a.pulmonari

cii

Fcregte presiunea venoasi - edem interstifial - rigidizarea vaselor - HTP

Fdrenajul limfatic cregte

Fstarea VD .normal

.hipertrofic .insuficient .miopatic (+VS)

.h
i

pope rt uzat

(i

nfa

rct)

Fvolumul sanguin pulmonar (depinde de debitul celor 2 ventriculi gi de distensibilitatea vaselor pulmonare )

MODIFIGARI ANATOMIGE

MODIFICART ANATOMICE celule endoteliale capilare umflate membrane bazale capilare ingrogate edem interstilial rupturi de membrane bazale - transsudare de eritrocite hemosiderozd alveole fibroase destindere de limfatice

CAUZE A. CRE$TERE.A PRES|UNil DTASTOLTCE VENTRTCULARE 1. prsFuNeTtE vs stsToLtcA - cregte volumul telesistolic - cregte presiunea telediastolicd - cregte pasiv presiunea in AS, venele pulmonare...
Boli valvulare ale inimii stingi Card!omiopatii 2. DISFUNGTIE VENTRICULARA DIASTOLICA - cre$te presiunea telediastolici - cregte pasiv presiunea in AS

' . . '

HTA BCI

. .

Hipertrofia eoncentrici Fibroza dituzd Cardiom iopatia restrictivi

3. PERtCARptTA CONSTRICTIVA

B. HIPERTENSIUNEA ATRIALA STAUCA

1. BOLILE VALVEI MITRALE 2. CORD TRIATRIATUM 3. MIXOM ATRIAL STG. SAU TROMBUS

c. oBSTRUCTTA VENOASA PULMONARA

1. STENOZA CONGENITAUA OE VENE PUTMONARE

2. BOALA VENOOCLUZIVA PULMONARA 3. FIBROZA UEOTASTTNALA

cRE$TEREA REZISTENTEI LA FLUX ir{ VASCULAR PULMONAR

parul

. . .

MECANISME Hipoxie - vasoconstricfie

Distrugere de vase (emfizem) Hipervdscoziiatea singelui prin hipoxie cronici

CAUZE
A. BOLI ALE PARENCHIMULUI PULMONAR 1. BOLI OBSTRUCTIVE CRONICE 2. BOLI RESTRICTIVE DE PLAMAN 3. BOLI DE COLAGEN: LED, PR, SCLERODERMTE 4, REACTil F|BROT|CE (HAMMAN-R|CH, HEMOSTDEROZA) 5. SARCOIDOZA 6. NEOPLASM
7. PNEUMONIE 8. REZECTII PULMONARE 9. HIPOP LAZ'E PULMONARA CONGENITALA B. S/wDROM EISENMENGER {defect septal atrial gi ventricular, patent ri l rnf r re, a rtarinrr sv, rc\ rvr

MODIFICARI ANATOMICE GR. | - hipeftrofia mediei arterelor mici musculare GR. ll - + proliferarea intimei GR. lll - + fibrozd concentrici cu obliterare de vase GR. lV - "leziuni plexiforme", dilata{ii, trombi GR. V - compiexe plexiforme, ieziuni angiomatoase gi carvenoase gi hialin izarea fibrozei i ntimale GR. Vl - aderitd necrozantd c. ALTE CONDTTE ASOCTATE CU SCADEREA SECT|UNIT qATULUT
VASCULAR
1.

crRozA HgpaTleA gt TRoMBozA DE VENA eORTA (meeanism

nutrifional)
2. PERSISTEruTA CIRCULATIET FETALE LA NOU NASCUT

922 153

tRete 53-3. DrAcNoslc sruDtEs

USEFUL FoR ELUctDATtNo cAUSES

oF puLMoNARy HypERTENstoN

POTENTIAL CAUSE OF PULMONARY HYPERTENSION Pulmonarrv thromboembolic disease

PuLnonary venous thrombosis or obstruction nance imaging Congenital intracardiac shunts Increased left atrial pressure secondarv to mitral or aortic vah'e disease, Ieft ventricular dvsfunction, or svstemic hvpertension Pulmonar-v ainvay disease (e.g., chronic bronchitis and emphvsema)

Transesophagleal echocardiographv Pulnronarv arter-v rvedge prpssure

rvith contrast

lo.ft_igl"-!. pressure (via -or patent foramen ovale) (>15 mm Hg and LYEDP) Respiratorl' function tests (FVC/FEV, chest x-ray)

H1'poxic pulmonary hvpertension associated rvith (1) impaired ventilation, either central (CNS) or peripheral Ichest n'all problems or upper ainvay obstructionJ and (2) residence at high altitude Interstitial lung disease, pneumoconioses, and fibrosis (e.9.. silicosis, rheumatoid disease, and sarcoidosis) Collagen-r'ascular disease (e.9., SLE, polvarteritis nodosa, sclerooermaJ

Sleep apnea studies and respiratory function

tests

-t
'-.
,

Chest .x-r.av, s.pirometrl, ancl carbon monoxide diffusion, highil;

Parasitic disease (schistosomiasis or filariasis) Cirrhosis rvith portal hypertension Peripheral pulmonarl' arterlr stenosis (ipcluding ease and fibrosing rrrecliastinitis)

" .; resolution chest computed tonrographv Serological and irnmunogenetic studies; skin, nruscle, o. otherl tissue biopsl'; esophageal motiiitv studies ,r Rectal biops'r', complement fixation, skin tests, blood smears :il Liver function tests, ultrasonographv, computed tornographv ..:i Selective puln:onary angiography or pressure gradienl ui ."tU*l
Erlthroclteniorphology,hemoglobinelectrophoresis',-.j
terizatior.r

svstem; FEYr = fofced expirator\ volunre irr 1 st-.con<i: IjVC = lbrced tital capacitr,: SLE : svstemic lupus erflhematosus. .'i It{odified from Weir EK: Diagnosis and managetnctit of prinrarl pulnronar-r'lrvperlcnsion. ,fn \Veir EK, Reeves ]T: Pulrnonarv Hvpertension. Mt Kisc6,i NY, Futura, 1984, p 141.

Sickle ceil disease CNS = central nervous

':;lai '-it:t\i

tReLe 53-4. DtAGNosrc cLAssrFtcATloN oF puLMoNARy HypERTENstoN

Interstitial lung disease
Sleep-disordered breathing Alveolar hr.'poventilation disorders Chronic exposure to high altitude
tr-eonatal lung
d isease

,,i.i"l

Pulmonary arterial hypertension Primary Sporadic

Familial
Secondary

Collagen-vascular disease Congenital s1'slenric-to-pulmonarv shltnts

P^rr.l hirhdrton<ini1

Alveolar-capillarv dvsplasia ['ulmonarv hl'pertension from chronic thrombotic and/or

Thrornboembolic obstruction of proximal pulmonary art Obstruction of distai pulmonarl' arteries Pulmonarv emboliim (thrombus. tumor, or-a and/or
fnrpion rnalpriall
d rsea se

Other

HiV infection
Drugs/toxins Anorexigens Other Persistent pulmonarv hypertensiorr of the nel'born Other Pulrnonarv venous hvpertension Left-sided atrial or ventricular heart disease Left-sided valvular heart disease Extrinsic compression of central pulmonar-"- r'eins Fibrosing mediasti nitis

In situ thrombosis
Sickle cell disease Pulruonarl' hvpertension caused b1' disorders directll'
.-,,1*^-^-.,,.^-^.,1^1,,- lature plr lmonar\' \'ascu a

Inflammatorv
Schistosomiasis Sa rcoi dosis

Adenopathy/tumors
Pulmonary venoocclusive disease Other

Other

Pulmonarl' capillarv

hemangiomatosis

Pulmonary hypertension associated rvith disorders of the respira^-,t /^- nvl)oremla rory s)'stem and/or L, Chronic obstructive pulmonarv disease From Rich S [ed): Priman'Pulmonuv Hlpertension: Erec:utile.summan from the \Vorld Si'mposiunt-PrimarY Fulmonarl
Available from the lVorld Health Organization f ia the lnternet (http://rlrlrr'.rlho,int/ncd/cvd/pph.html).

,;

RX IN HTP

Normal
Flux crescut in lobii inferiori Gravitafie Presiuni diferite intra alveotare Raport A/B = 1,2 : 1

CRE TEREA FLUXULUI PULMONAR FLUX - 0 VASE f x I (rezerva) +

c $vase.
.
Rx 113

prestune - |5L.llu*iune Cregte $ venos

ext.vas cularizatd Circ inf = circ.sup

N - 0 a.pulm.desc.dr, HTP arterial4

- 15 mm birbafi = I - 14 mm femei
10

vasoconstriefie periferici vasospasm ingrogarea peretelui vascular

scade circulatia (cregte transparenta) in 1/3 ext. vasele centrale elastice se firgesc calcificdri ale vaselor centrale

KY

HTP de origine venoasd

Pvenoasd>8-12mm

'
Rx

ff

uxul pulmonar este redistribuit spre lobii superiori

inversare a aspectului normal (cefatizarea fluxului arterial gi venos) P venoasd > 25 mm . Edem pulmonar
Mecanisme ' sechestrare de lichid interstifial in lobii inferioari

'

. . . . .

presiune interstifiali O
I

complian{a pulmonari
I

fluxul spre lcbii inferiori S

+ spasm arterial
\/

fluxul este redistribuit spre lobii superisri

\r