2. DEFINITION
• Pulmonary hypertension is defined by a mean pulmonary arterial pressure
(mPAP) >20 mmHg at rest measured by right heart catheterization.
• Non invasively by ECHO (estimate)
• Affects 1% of the general population
PATHOPHYSIOLOGY
• Vasoconstriction
• Proliferation
• Inflammation
• Medial hypertrophy and intimal fibrosis
10. SCREENING
Only for high risk individuals
• Systemic sclerosis
• BMPR2 mutation
• First degree relatives of patients with HPAH
• Patients undergoing assessment for liver transplantation
ANNUAL ECHO
11. DIAGNOSIS
• STEP 1(suspicion) – medical history,physical examination, blood
test(BNP/NT proBNP) and ECG
• STEP 2 (detection) – non invasive lung(PFT,CXR,ABG) and cardiac testing
(ECHO)
• STEP 3(confirmation) –referred to a PH centre
(1)when an intermediate/high probability of PH
(2)in the presence of risk factors for PAH or a history of PE
14. ECG
• P pulmonale (P>0.25 mV in lead II)
• Right axis deviation (QRS axis >90° or indeterminable)
• RV hypertrophy (R/S >1, with R >0.5 mV in V1; R in V1 + S in lead V5
>1 mV)
• Right bundle branch block—complete or incomplete (qR or rSR
patterns in V1)
• RV strain pattern (ST depression/T-wave inversion in the right
precordial V1–4 and inferior II, III, aVF leads)
• Prolonged QTc interval (unspecific)
17. ECHO
• Most important screening investigation
• Estimate PAP indirectly with TRV
sPAP = RVSP = 4× (TRV)2 + RAP
Normal < 37 mmhg
Abnormal >= 37 mmhg
• mPAP = (0.6×sPAP) + 2
Mild 20-40 mmhg
Moderate 40-55 mmhg based on mPAP
Severe >55 mmhg
18. • Current Recommendation --- Peak TRV >2.8m/s suggest PH
• Additional ECHO findings – 2/3 if present s/o PH
19.
20. V/Q SCAN
• Recommended for patients suspected or newly diagnosed PH to rule out or detect
signs of CTEPH
• In the absence of parenchymal lung disease , a normal perfusion scan excludes
CTEPH
CT SCAN
• PA-to-aorta ratio >0.9 and enlarged right heart chambers.
• A combination of three parameters
1. PA diameter ⩾30 mm
2. RVOT wall thickness ⩾6 mm highly predictive of PH
3. and septal deviation ⩾140° [or RV:LV ratio ⩾1])
• Non-contrast chest CT can help determine the cause of PH when there are features
of parenchymal lung disease.
21. CTPA
• Computed tomography pulmonary angiography (CTPA) is mainly used to
detect direct or indirect signs of CTEPH, such as filling defects (including
thrombus adhering to the vascular wall), webs or bands in the PAs, PA
retraction/dilatation, mosaic perfusion, and enlarged bronchial arteries
• Misses distal obstruction
PFT
• Pure PAH without lung disease – usually normal
22. RHC
• Right heart catheterization is the gold standard for diagnosing and classifying PH.
• Also used for the haemodynamic assessment of heart or Liver transplant candidates
and evaluating congenital cardiac shunts.
C/I
• A known thrombus or tumour in the RV or RA
• Recently implanted pacemaker(<1 month)
• Mechanical right heart valve
• Triclip
• Acute infection
Complication
The most feared complication of RHC is perforation of a PA.
23. VASOREACTIVITY
• The purpose of vasoreactivity testing in PAH is to identify acute
vasoresponders who may be candidates for treatment with high-dose
calcium channel blockers (CCBs)
• Pulmonary vasoreactivity testing is only recommended in patients
with IPAH, HPAH, or DPAH.
• Inhaled nitric oxide or inhaled iloprost are the recommended test
compounds for vasoreactivity testing.
• A positive acute response is defined as a reduction in mPAP by ⩾10
mmHg to reach an absolute value ⩽40 mmHg with increased or
unchanged CO.
26. FOLLOW-UP (4 STRATA) RISK ASSESSMENT
Mortality 0-2% 3-7% 9-19% >20%
Risk is calculated by dividing the sum of all grades by the number of variables and rounding to the next integer
28. MANAGEMENT
GENERAL MEASURES
• Exercise training and rehabilitation (under supervision)
• Psychosocial support
• Immunization against influenza , pneumonia, covid
• Diuretics (only for RHF with fluid overload)
• LTOT (only if Pao2<60 on ABG)
• Anemia (supplemental iron for iron deficiency anemia)
• Anticoagulants (individual basis)
• Pregnancy – discourage pregnancy and offer contraception
29.
30.
31.
32. RECOMMENDED COMBINATIONS
• Ambrisentan + Tadalafil
• Macitentan + Tadalafil
• PDE5- not useful in group 2 and group 3
• Riociguat + sildenafil should not be used – cause excessive
vasodilation and hypotension
• Bosentan + sildenafil should not be used due to drug interaction
