HIPERTENSIUNEA PULMONARA

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HIPERTENSIUNEA PULMONARA. CIRCULATIA PULMONARA NORMALA. PLAMANUL. - oxigenare Hb - filtru (particule, bacterii) - eliminarea CO2 – echilibru acido-bazic CIRCULATIA CIRCULATIA PULMONARABRONSICA Sange venossange arterial a. pulmonara a. b ronsice - PowerPoint PPT Presentation

Text of HIPERTENSIUNEA PULMONARA

  • - oxigenare Hb- filtru (particule, bacterii)- eliminarea CO2 echilibru acido-bazic

    CIRCULATIA CIRCULATIA PULMONARABRONSICASange venossange arteriala. pulmonaraa. bronsice CapilareCapilareVene pulmonare Vene sistemice

    CIRCULATIA PULMONARA NORMALA

    PLAMANUL

  • Sunt fiziologic dr-stg (pana la 30% din DC) - bronsiectazii - fibroza chistica - boli congenitale cardio-vasculareCIRCULATIA BRONSICA

  • NORMALA SISTEMICE media 20-25% din diam. vasuluiA. PULMONARE - media < 10- 5% din diam. vasuluiArteriolele pulmonare nu au tunica medie si nu contribuie la rezistenta vasculara

    VD fluxul coronarian cel mai mare in sistola - depinde de gradientul pres. pulm. aortaPres. VD creste gradientul scade fluxul coronar drept scade ischemie VD

    HIPERTENSIUNEA PULMONARA (HTP)

  • NORMALPRES. A. PULMONARA - sist. 18-25 mm Hg - diast. 6-10 mm Hg - medie 12-16 mm HgPRES V. PULMONARE 2-10 mm HgREZIST. 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 HgReducerea calibrului vaselor pulmonareCresterea fluxului

    HIPERTENSIUNEA PULMONARA (HTP)

  • HIPOXIA VASOCONSTRICTIE PULMONARA-histamina receptori H1 vasculari- endoteliu - echilibru NO endoteline- patrunderea Ca 2+ in celula musculara neteda

    HIPOXIA CRONICAExtensia musculaturii netede in peretele arterelor din periferia plamanilorIngrosarea peretilor arterelor musculareReducerea nr. arterelor cresterea raportului alveole/artere

    REACTIVITATEA VASCULARA PULMONARA

  • VASOCONSTRICTIEHipoxiaAcidozaProstaglandine F2 si A2HISTAMINA H1SEROTONINA ?ANGIOTENSINA A2ALTITUDINEVASODILATATIEAlcalozaPROSTAGLANDINE I2 si EBLOCANTI STIMULARE (ISOPROTERENOL)ACETILCOLINA (prin EDRF)HISTAMINA (prin H2 ?)INDOMETACIN creste rezistenta pulmonaraLa 10 000 m altitudineTA pulmonara medie = 25 mm Hg in repaus > 50 mm Hg in efort

  • HTP arteriala1.1. Idiopatica1.2. Ereditara1.3. Indusa de droguri si toxine1.4. Asociata cu:Boli de colagenHIVHipertensiune portalaBoli cardiace congenitaleSchistosomiazaAnemie hemolitica cronica1.5. HTP persistenta la nou nascut1 Boala venoocluziva pulmonara si/sau hemangiomatoza capilara pulmonara

    CLASIFICAREA HTPDana Point, 2008

  • 2. HTP prin suferinta ventriculului stang2.1. Disfunctie sistolica2.2. Disfunctie diastolica2.3. Boli valvulareCLASIFICAREA HTPDana Point, 2008

  • 3. HTP prin boli pulmonare sau hipoxie3.1. BPOC3.2. Boli interstitiale3.3. Alte boli cu restrictie si obstructie3.4. Apneea de somn3.5. Boli cu hipoventilatie alveolara3.6. Expunerea cronica la mare altitudine3.7. Anomalii de dezvoltare fizicaCLASIFICAREA HTPDana Point, 2008

  • 4. HTP prin tromboembolism

    5. HTP prin factori multipli neclari5.1. Boli hematologice: mieloproliferare, hipersplenism5.2. Boli sistemice: sarcoidoza, histiocitoza pulmonara cu celule Langerhans5.3. Boli metabolice: B. Gaucher, glicogenoze, disfunctii tiroidiene5.4. Altele: obstructii tumorale, mediastinita fibrozanta,, IRC sau dializaCLASIFICAREA HTPDana Point, 2008

  • In suferinta inimii stangiPRESIUNE 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)CATEVA MECANISME FIZIOPATOLOGICE

  • Variabilitatea reactivitatii vasculare pulmonare:creste presiunea venoasa distrugere sau inchidere de cai aeriene hipoxie creste presiunea in a. pulmonaracreste presiunea venoasa edem interstitial rigidizarea vaselor HTP drenajul limfatic crestestarea VD normalhipertroficinsuficientmiopatic (+ VS)hipoperfuzat (infarct) Volumul sanguin pulmonar (depinde de debitul celor 2 ventriculi si de distensibilitatea vaselor pulmonare)CATEVA MECANISME FIZIOPATOLOGICE

  • Celule endoteliale capilare umflateMembrane bazale capilare ingrosateEdem interstitialRupturi de membrane bazale transudare de eritrocite hemosiderozaAlveole fibroaseDestindere de limfaticeMODIFICARI ANATOMICE

  • TABLE 73-2 --Clues for Interpretation of Diagnostic Tests for Pulmonary Hypertension

    TestNotable FindingsChest x-rayEnlargement of central pulmonary arteries reflects level of PA pressure and duration.ElectrocardiographyRight axis deviation and precordial T wave abnormalities are early signs.Pulmonary function testsElevated pulmonary artery pressure causes restrictive physiology.Perfusion lung scanNonsegmental perfusion abnormalities can occur from severe pulmonary vascular disease.Chest computed tomography scanMinor interstitial changes may reflect diffuse disease; mosaic perfusion pattern indicates thromboembolism and/or left heart failure.EchocardiographyRight ventricular enlargement will parallel the severity of the pulmonary hypertension.Contrast echocardiographyMinor right to left shunting rarely produces hypoxemia.Doppler echocardiographyThis is too unreliable for following serial measurements to monitor therapy.Exercise testingThis is very helpful to assess the efficacy of therapy. Severe exercise-induced hypoxemia should cause consideration of a right-to-left shunt.

  • Toate sunturile sistemico-pulmonare rezultand din mari defecte care duc la cresteri de presiune in VD si la inversarea suntului (pulmonar-sistemic) sau sunt bidirectional cu: cianoza, eritrocitoza si multiple suferinte de organSINDROM EISENMENGER

  • GR. I : hipertrofia mediei artereor mici musculareGR. II : + proliferarea intimeiGR. III: + fibroza concentrica cu obliterare de vaseGR. IV: leziuni plexiforme, dilatatii, trombiGR. V: complexe plexiforme, leziuni angiomatoase si cavernoase si hialinizaea fibrozei intimaleGR. VI: arterita necrozanta

    MODIFICARI ANATOMICE

  • 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). Plexiform lesion (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 plexiform lesion, outlined by arrowheads. Note perilesional inflammatory infiltrate (arrow). D. High magnification histology of plexiform lesions shown slit-like vascular channels lined by hyperchromatic and cuboidal endothelial cells. Cells in the core do not display distinct cytoplamic borders. E. Low magnification immunohistology with 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 plexiform lesion (arrow), and dilated/angiomatoid lesions (between arrowheads). F. High magnification immunohistology of cellular plexiform lesion stained with Factor VIII related antigen (arrowheads). G and H. Histological identification of plexiform and 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/angiomatoid lesions with Factor VIII related antigen immunohistochemistry. J. Endothelial cells in plexiform lesion 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),

  • A. Fibrotic, relatively paucicellular intima 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 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 plexiform lesions, highlighted by smooth muscle cell actin immunohistochemistry (arrow).

  • 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 septal thickening with engorged capillaries (arrowhead). B. Marked vein thickening with intimal projection probably representing organized thrombus (arrow). Alveolar hemorrhage and septal thickening are highlighted with arrowhead. C and D. Movat stained pulmonary vein showing arterialization pattern with internal and external elastic layers (arrow). The