2. When to suspect Pulmonary Hypertension
•Any case of breathlessness without overt
signs of specific heart and lung disease
•Increased breathlessness unexplained
by the underlying disease itself
•Symptoms and/or physical signs of
pulmonary hypertension in presence of
co-morbid conditions like
CTD / ILD, CHD with systemic to
pulmonary shunt, portal hypertension,
HIV infection, sleep apnea syndromes,
deep vein thrombosis
•Incidental suspicion via abnormal ECG , CXR
echocardiographic finding .
3. The evaluation process of a patient with
suspected PH requires a series of investigations
intended to
1. confirm the diagnosis
2. clarify the clinical group of PH and the
specific aetiology within the PAH group
3. evaluate the functional and haemodynamic
impairment.
4. Clinical presentation
• Dyspnea 60%
• Fatigue 19%
• Near syncope/syncope 13%
• Chest pain 7%
• Palpitations 5%
• LE edema 3%
• Hoarseness of voice 2%
(Ortners syndrome)
Symptoms of PAH
7. Electrocardiogram
• RV hypertrophy and
strain
• Right atrial dilatation.
• Right axis deviation
• An R wave/S wave ratio
greater than one in lead
V1
• Incomplete or complete
right bundle branch
block
• Increased P wave
amplitude in lead II
8. Chest Radiograph
In 90% of patients with IPAH the chest radiograph
is abnormal at
the time of diagnosis.
Findings include
•central pulmonary arterial dilatation
•‘pruning’ (loss) of the peripheral blood vessels
•Right atrium and RV enlargement
9. CXR in PH
Large central
Pulmonary
arteries
Right
Ventricular
Hypertrophy
Rapid attenuation
of pulmonary
vessels
Clear Lung Fields
10. Echocardiogram
Transthoracic echocardiography provides several
variables which correlate with right heart
haemodynamics including PAP
•Order for screening when clinical suspicion
exists
•Order for standard interval screening in selected
groups:
•Family of those with IPAH or with known BMPR2
mutation
•Scleroderma spectrum
•Pre-liver transplant
11. Echocardiogram Findings
TR
Right atrial and ventricular
hypertrophy
Flattening of interventricular septum
Small LV dimension
Dilated PA
Pericardial effusion
• Poor prognostic sign
• RA pressure so high it impedes
normal drainage from pericardium
• Do not drain, usually does not
induce tamponade since RV under
high-pressure and non-collapsible
12. •Pulmonary function tests and arterial
blood gases
Pulmonary function tests and arterial blood gases will
identify the contribution of underlying airway or
parenchymal lung disease.
•Ventilation/perfusion lung scan
The ventilation/perfusion lung scan should be performed in
patients with PH to look for potentially treatable CTEPH
13. •High-resolution CT
High-resolution CT provides detailed views of the lung
parenchyma and facilitates the diagnosis of interstitial lung
disease and emphysema. Highresolution CT may be very
helpful where there is a clinical suspicion of PVOD
•Blood tests and immunology
Routine biochemistry, haematology, and thyroid function tests are
required in all patients. Serological testing is important to detect
underlying CTD, HIV, and hepatitis.
•Abdominal ultrasound scan
Liver cirrhosis and/or portal hypertension can be reliably excluded
by the use of abdominal ultrasound.
14. Right heart catheterization and
vasoreactivity
RHC is required
•to confirm the diagnosis of PAH,
•to assess the severity of the haemodynamic
impairment, and
•to test the vasoreactivity of the pulmonary circulation
