HIPERTENSIUNEA PULMONARA

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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 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

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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 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),

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A. Fibrotic, relatively paucicellularintima thickening (outlined by