Diagnosis And

Treatment Of Pulmonary
Hypertension
Aisyah Cholifaur R.
Alfiani Rahmi M.
Fatimah Zahra
 Definisi
× tekanan abnormal yang meningkat dalam
pembuluh darah paru.
× Penyebabnya terjadi peningkatan :
 resistensi pembuluh darah paru (PVR)
 aliran darah paru
 tekanan vena paru
 etiologi

Hipertensi Tromboemboli
Penyakit pada Idiopatik /
Arteri Pulmonal Penyakit paru kronis
jantung kiri multifaktorial
(PAH) (CTEPH)
 Penyakit paru

Area yang terkena Tidak bisa kirim O2 Arteriol paru Tapi kalau masalah
penyakit paru ke darah balik vasokonstriksi meluas

↑ resistensi
Daerah vaskuler paru
RV ↑ tekanan HP
vasokonstriksi >> Kerja RV mompa
jadi berat

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 Penyakit Jantung Kiri

Darah akan
Peningkatan
kembari ke
valvular disfx tekanan di
vena dan
pulmonal
kapiler paru

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 Tromboemboli kronis (CTEPH)

Blood cloting Emboli paru Blok p.d paru

Konstriksi
↑ resistensi Pelepasan H
arteriol
aliran darah dan serotonin
pulmonal
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 Hipertensi Arteri Pulmonal (PAH)

Congenital heart
Konstriksi ar. Paru
defect
Mengeluarkan :
endotelin 1
serotonin
thromboxane
Gangguan Sel endotel lining
Hipertrofi otot polos
connective tissue a. paru rusak
Nekan prod :
NO
Prosta cyclin

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 Gejala klinis

Nyeri
Lelah sinkop
dada

Edema
palpitasi
perifer
 ECG

× An ECG is required screening and follow-up.

× An increased RV mass is reflected by higher R waves in V1, V2, I, II, III,
and aVF, and deeper S waves in I, aVL, and V3–V6.
× A discordant repolarisation pattern may be an expression of RV strain or
ischaemia, especially when this is present in leads V1, V2, II, III, and aVF.

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 IMAGING

× Imaging is important in the initial confirmation of elevated right sided

pressures.
× Cardiac MRI (CMRI) is the gold standard in the assessment of RV
volumes, morphology, and function.
× Both CT angiography and high resolution CT enable characterization of
the pulmonary vasculature and lung parenchyma.
× CT may also provide more insight regarding the underlying cause of PH.
Dilatation of the main pulmonary artery on CT indicates the presence of
PH.

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 ECHOCARDIOGRAPHY

× Most widely available imaging test.

× The guidelines define the presence of PH, based on echocardiographic
measurements, as:
× unlikely when the velocity of the TR jet is ≤2.8 m/s or the estimated
SPAP is ≤36 mm Hg;
× possible if the TR jet is between 2.9–3.4 ms/s (SPAP 37–50 mm Hg)
or if there are other signs of PH such as RV dilatation and
hypertrophy;
× very likely if the TR jet is >3.4 ms/s (SPAP >50 mm Hg).

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× An abnormal motion of the interventricular
septum in the direction of the LV during systole
 the D-shape of the LV. Expressed by the
eccentricity index, where a normal value is 1
and in the case of increased RV pressure >1

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 RIGHT HEART CATHETERISATION

× RHC  diagnosis, evaluation of the effect of PH specific treatment or as

confirmation in the case of clinical deterioration.
× RHC  hemodynamics  important in the final diagnosis, classification
and assessment of the prognosis of PH.
× RHC vasoreactivity testing can be performed and shunting may be
detected.
× Vasoreactivity  the contribution of the increased vascular tone. The
results  implications for the treatment of PAH.
× A positive test may benefit from treatment with calcium channel
blockers.

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× Vasoreactivity testing  idiopathic PAH,
hereditable PAH, and PAH associated with
drugs or toxins.
× Vasodilators that can be used for vasoreactivity
testing are nitric oxide (NO), epoprostenol,
iloprost, adenosine, and sildenafil

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TREATMENT
Lifestyle

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 Supportive therapy

× PAH  coagulopathiesincreased risk of

thrombosis oral anticoagulation should be
considered in PAH
× CTEPH receive livelong anticoagulation therapy
× Diuretics  right side decompensation ↓ fluid
overload
× Digoxin  as antiarrhythmic drug maintanance
sinus arrhytmic to PH patien is important too.
× AF ↑ mortality and onset of SVT clinical
deterioration and/or RV failure
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× The use of long term oxygen therapy  hypoxic
patients  vasoconstriction may be reduced

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 PH spesific therapy

× Prostacyclin analogues are potent pulmonary

vasodilators are recommended in patients with
PAH in NYHA functional class III and IV
× Epoprostenol short acting pulmonary
vasodilator It was shown to improve exercise
capacity, quality of life and survival in patients
with idiopathic PAH and other forms of PAH.
× Epoprostenol  intravenous adverse effect

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× ERAs(Endothelin receptor antagonis)  Oral
× Vasoconstriction in the pulmonary vasculature
by binding two endothelin receptors, subtypes A
and B.
× ERAs  bind both receptors or one selective
receptor
× ERAs are recommended in PAH patients in NYHA
functional class II and III
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 ERAs

Drug positive negative

Bosentan improve functional class, increase in hepatic Inhibitor two
haemodynamics, and Aminotransferases in ∼10% of subtype A and B
Time to clinical worsening. patients Monthly control of liver
enzymes

Ambrisentan Reduces the risk However,monthly Inhibitor selective

Of elevation in hepatic Testing of liver enzymes is required subtype A
enzymes in patients
Taking ambrisentan
Macitentan Probably has no effect on New ERAs
liver enzymes.In addition, it
has been shown to reduce
morbidity andmortality in
PAH

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× phosphodiesterase-5 inhibitors (Sildenafil and
Tadalafil) inhibit the cyclic guanosine mono
Phosphate (cGMP) degrading enzyme
phosphodiesterase type 5 and cause
vasodilatation through the NO/cGMP pathway.

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× Sildenafil demonstrated similar results in
improving exercise tolerance, symptoms and
haemodynamics, as was previously
demonstrated by the use of ERAs
× Phosphodiesterase5 inhibitors are
recommended in patients In NYHA class II and
III

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× The majority of PAH patient(theres no effect
with single type of drug)  combination
therapy with two or more of PH specific drug
× The underlying disease should be treated

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 Surgical treatment

× Endarterectomy  not all CTEPH recurs or

persist
× The study indicated improvement in
haemodynamics and clinical condition
× NYHA functionalclass IV with
× Right heart failure  do not respond to PH spe-
cific drug therapy should be considered for
balloon
× Atrialseptostomy or heart/lung transplantation
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× Shunt  patien with SpO2 > 80%
× Poor prognosis  transplantation

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 Conclusion

- Pulmonary hypertension is a complex

condition diagnosis and in the treatment of
the disease.
- PH centre by a multidisciplinary team  to
evaluate and treat PH patients.
- It is essential to diagnose the underlying
cause  for the therapeutic strategies.
- Important to recognise PH and start
treatment at a nearly stage of the disease as
this will improve prognosis.
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