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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
HIPERTENSIUNEA
PULMONARA (HTP)
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)
1. HTP arteriala
1.1. Idiopatica
1.2. Ereditara
1.3. Indusa de droguri si toxine
1.4. Asociata cu:
Boli de colagen
HIV
Hipertensiune portala
Boli cardiace congenitale
Schistosomiaza
Anemie hemolitica cronica
1.5. HTP persistenta la nou nascut
1 Boala venoocluziva pulmonara si/sau hemangiomatoza capilara pulmonara
CLASIFICAREA HTP
Dana Point, 2008
Normal
Flux crescut in lobii inferiori
Gravitatie
Presiuni diferite intra alveolare
Raport A/B = 1,2 : 1
CRESTEREA FLUXULUI PULMONAR
FLUX - VASE x 8 (rezerva) +
vase - flux + presiune
- presiune
Creste venos
Rx 1/3 ext. vascularizata
- Circ. Inf = circ. sup.
N a. pulm. desc. dr. = 10-15 mm la barbati si 9-14 mm la femei
Rx n HTP
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 n HTP
Mecanisme
Sechestrarea de lichid interstitial in lobii inferiori
Presiunea interstitiala
Complianta pulmonara
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
HTP IDIOPATICA
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
HTP IDIOPATICA
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
- Ciroza hepatica
- Istoric de tromboflebita superficiala
HTP IDIOPATICA
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
HTP IDIOPATICA
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)
HTP IDIOPATICA
HTP IDIOPATICA
DIAGNOSTIC DIFERENTIAL
HTP secundara (mai benigna si mai tratabila)
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 O2 nocturna, 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
TRATAMENTUL
MEDICAL
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 pts tolerate maximum dosage
Indicatii
Bolnavii cu ICC cl III IV, index cardiac < 2,1
l/min/m2 si/sau Sat O2 in 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
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
TRATAMENTUL
MEDICAL (PG)
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)
Prostanoid analogues
CTD= boala de tesut conjunctiv
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
Barst RJ N.Engl. J Med 1996;334:296-304
Badesch DB 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 inhalatory adm.(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
Phosphodiesterase inhibitors
Sildenafil ( REVATIO )
25 mg t.i.d.
Available in Romania
Humbert M N Engl J Med 2004;351: 142536.
Indicatii transplant
HTPI simptomatica, progresiva in ciuda tratamentului
medical optim, cu test de mers de 6 min < 400 m, cu
index cardiac < 2,1 l/min/m2 si/sau Sat O2 in artera
pulmonara < 63% si/sau PAD > 10 mm Hg sau PAP
m > 55 mmHg
TRATAMENTE
CHIRURGICALE
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 septostomy an option ?
Rarely (bridge to ) ; 5-15% mortality
CONCLUSIONS
PPHT pts to 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