3. Overview
• Normal pulmonary circulation :
1. High-flow with low-resistance circuit capable of
accommodating the entire right ventricular
output at one fifth the pressure of the systemic
circulation level.
2. The right ventricle functions primarily as a flow-
generator pump and is particularly sensitive to
increases in its afterload.
3. Increased pulmonary artery pressure and
pulmonary vascular resistance
characterize pulmonary hypertension.
4. Pulmonary Hypertension
• Pulmonary hypertension. Chest radiograph in a patient with secondary pulmonary hypertension
reveals enlarged pulmonary arteries. This patient was found to have an atrial septal defect.
5. Natural History
• Resting mean pulmonary arterial pressure of
25 mmHg or more, or >30 mmHg with
exercise.
• Primary (Idiopathic) or secondary (many
known causes).
• Dyspnea (during exercise) , edema , and
abdominal distention (signs of elevated right
side pressure)
8. Plain X-ray
• By time of diagnosis , 90% of
patients have already
abnormalities.
• (low sensitivity and specificity)
• Findings:
1. Elevated apex due to right
ventricular hypertrophy (a
decrease in retrosternal area)
2. Enlarged right atrium (opacity
over right retrosternal space).
3. Enlarged pulmonary arteries.
9. Plain X-ray
4.Right hilar enlargement, can be
on both sides.
5.Pruning of peripheral
pulmonary vessels.
The X-ray shows gross enlargement of the
cardiac silhouette. The right border extends
far to the right indicating gross right atrial
enlargement .The right atrial enlargement may
be due to severe pulmonary hypertension and
right ventricular failure.
10. Plain X-ray
• Chest radiograph reveals enlargement of the pulmonary vasculature and
the central pulmonary arteries (arrows).
• Secondry hypertension By atrial septal defect.
11. Plain X-ray
• PA film of chest in a patient with
primary pulmonary HTN showing
right heart and main pulmonary with
its right and left branches.
• Lateral CXR of the same patient,
showing enlarged pulmonary artery.
12. Plain X-ray
• Cardiomegaly and prominent bilateral pulmonary arteries in the hilar areas can be
seen in the posteroanterior chest radiograph from a patient with idiopathic
pulmonary arterial hypertension. The lateral view also reveals enlarged pulmonary
arteries and cardiomegaly without any evidence of congestive heart failure.
14. Computed Tomography
• CT is good , noninvasive , used to
confirm presence of pulmonary
hypertension.
• CT pulmonary angiogram (CTPA)
is useful in delineating the
anatomic detail of the pulmonary
vasculature.
• CTPA is the best method for
demonstrating emboli.
• Contrast-enhanced images may
show intraluminal abnormalities
in the arteries and veins and can
detect emboli if it’s large.
CT pulmonary angiogram demonstrates
clots in both the right and left main
pulmonary arteries.
15. Computed Tomography
• Findings in CT :
• Extra cardiac vascular signs:
• Enlarged pulmonary trunk >29 mm diameter is often used as a general predictive cut-off
• Enlarged pulmonary arteries
• Mural calcification in central pulmonary arteries
• Evidence of previous pulmonary emboli
• Cardiac signs :
• Right ventricular hypertrophy: defined as wall thickness of more than 4 mm
• Straightening or bowing (towards the left ventricle) of the interventricular septum
• Right ventricular dilatation
• Decreased right ventricular ejection fraction
• Dilatation of the inferior vena cava and hepatic veins
• Pericardial effusion
• Parenchymal signs:
• Centrilobular ground-glass nodules (Cholesterol granuloma).
• Neovascularity: tiny serpiginous intrapulmonary vessels that often emerge from centrilobular arterioles.
16. CT of Idiopathic Pulmonary HTN
Axial contrast-enhanced CT scan obtained at initial
presentation shows central pulmonary artery
dilatation with aneurysmal enlargement of the left
lower lobe pulmonary artery (*) but no evidence of
intraluminal thrombi.
Axial contrast-enhanced CT scan obtained 2 years
later shows wall-adherent apposition thrombi(a
complication) (arrowheads) with recanalization
(arrows) in the pulmonary artery trunk and the right
main pulmonary artery. The left lower lobe
pulmonary artery (*) remains enlarged.
17. CT of Idiopathic Pulmonary HTN
• Spiral CT scan in a patient with pulmonary hypertension reveals
enlarged pulmonary arteries and an absence of thrombosis.
18. Computed Tomography
• High-resolution CT (HRCT)
scanning of the chest has a
role in the evaluation of
pulmonary HTN in patients
with suspected diffuse lung
disease, eg (COPD ,
interstitial lung disease).
• Axial contrast-enhanced CT image obtained
with lung window settings shows severe
emphysema with loss of lung parenchyma,
contributors to pulmonary hypertension.
19. CT of Pulmonary HTN with Thrombus
CTEPH1/3
• CTEPH in a 59-year-old man with a systolic pulmonary artery pressure of 100 mm Hg.
• Axial contrast-enhanced CT scan shows a thrombotic mass (straight arrows) in the right main
pulmonary artery, an intraluminal web (curved arrow) in the left lower lobe pulmonary
artery, and bronchial artery collateral vessels (arrowheads).
20. CT of Pulmonary HTN with Thrombus
CTEPH2/3
• Coronal reformatted image from contrast-enhanced CT more clearly
depicts collateral vessels (arrow).
21. CT of Pulmonary HTN with Thrombus
CTEPH3/3
• Axial contrast-enhanced CT scan shows a wall-adherent soft tissue mass (arrow) in the
right atrium, a finding that was confirmed to be a thrombus at pulmonary
thromboendarterectomy.
23. Echocardiography
• It’s performed to estimate the pulmonary artery systolic pressure and to
assess right ventricular size, thickness, and function.
• In addition, echocardiography can evaluate right atrial size, left ventricular
systolic and diastolic function, and valve function, while detecting
pericardial effusions and intracardiac shunts.
• Echocardiography uses Doppler ultrasound to estimate the pulmonary
artery systolic pressure. This technique takes advantage of the tricuspid
regurgitation that usually exists. The maximum tricuspid regurgitant jet
velocity is recorded and the pulmonary artery systolic pressure (PASP) is
then calculated:
PASP = (4 x [TRV]2) + RAP
24. Echocardiography
• Main findings are :
1. Right ventricular
enlargement (RVE).
2. Right ventricular
hypertrophy (RVH).
3. Right atrial enlargement
(RAE).
4. Functional tricuspid
regurgitation (TR) with a
high velocity regurgitant jet
by Doppler (TR jet), and a
mid-systolic notch on the
pulmonary artery Doppler
flow tracing (PA flow).
5. The interventricular septum
is shifted toward the left
ventricular cavity.
25. Echocardiography
• Panel A: Apical four-chamber
view from a patient with severe
idiopathic pulmonary arterial
hypertension associated with
tricuspid regurgitation. There is a
large apex-forming right ventricle
(RV), large right atrium (RA), and
small left ventricle (LV) and left
atrium (LA).
• Panel B: Agitated saline contrast
is injected intravenously and
results in RA and RV
opacification; four bubbles are
seen in the LV (arrow), possibly
due to right-to-left flow across a
patent foramen ovale.
26. Echocardiography
• Panel C shows an apical four
chamber view from a
patient with a large left-to-
right shunt due to an atrial
septal defect (ASD). The RV
is apex-forming but the RV
and RA are not as large as in
panel A.
• Panel D: Contrast is injected
intravenously and a few
bubbles are seen in the LV;
more importantly, there is a
prominent negative contrast
(nc) effect due to opacified
atrial blood.
27. Echocardiography
• Two-dimensional echocardiogram (parasternal short axis view at the level of the
aortic valve) with color flow Doppler shows significant left to right atrial flow
through two atrial septal defects.
28. Echocardiography
• The apical four chamber view from a 2-D echocardiogram with color flow
Doppler shows a small muscular ventricular septal defect (VSD) associated
with left to right shunting of blood.
29. Echocardiography
• The short axis view from a 2-D echocardiogram shows significant right
ventricular pressure and volume overload as a result of pulmonary
hypertension.
30. Echocardiography
• The short axis view from a 2-D echocardiogram shows significant
right ventricular pressure and volume overload as a result of
pulmonary hypertension.
31. Echocardiography
The short axis view at the level of the mitral chordae from a patient with advanced
pulmonary hypertension shows substantial morphologic changes, including severe
hypertrophy of the right ventricular (RV) wall, dilation of the RV chamber and
hypertrophy of the right side of the septum. The septum is flattened, strongly
suggesting pressure overload in the RV; this septal shape imparts a "D shape" to the left
ventricle (LV) which has relatively thin walls.
32. Echocardiography
• The four chamber view from a 2-D echocardiogram with color flow Doppler shows
significant tricuspid regurgitation with a dilated right atrium. There is a prosthetic
mitral valve suggesting that the etiology for tricuspid regurgitation is pulmonary
hypertension resulting from previous mitral valve disease.
33. Echocardiography
• The four chamber view from a 2-D echocardiogram with color flow
Doppler shows significant tricuspid regurgitation. There is enlargement of
the left atrium and limited mobility of the mitral valve which shows
doming in diastole, suggesting that tricuspid regurgitation is the result of
pulmonary hypertension due to mitral stenosis.
34. Summary
• By the time the diagnosis of pulmonary
arterial hypertension is made, 90% of patients
have an abnormal chest radiograph.
• Not specific nor sensitive.
• Main findings:
1. Enlarged right ventricle.
2. Enlarged right atrium.
3. Enlarged pulmonary vessels.
35. Summary
• CTPA is for the anatomic detail of the
pulmonary vasculature.
• CTPA is the best method for demonstrating
emboli.
• HRCT is for associated with lung diseases.
• Findings may be cardiac , vascular extra-
cardiac , and parenchymal.
36. Summary
• On echocardiography:
1. Right atrial and ventricular enlargement.
2. Paradoxical movement of the interventricular
Septum
3. Tricuspid regurgitation.
• Doppler echocardiography is the most reliable
noninvasive method for estimating pulmonary artery
pressure.
37. Sources
1-Pulmonary Hypertension Imaging.
Author: Davinder Jassal; Chief Editor: Eugene C Lin, MD.
2-Pulmonary hypertension by Dr Yuranga Weerakkody and Dr Frank
Gaillard et al.
3-CT Findings in Diseases Associated with Pulmonary Hypertension: A
Current Review
Claudia Grosse, MD, and , Alexandra Grosse, MD
4-Clinical features and diagnosis of pulmonary hypertension in adults
Author Lewis J Rubin, MD
William Hopkins, MD
5-Pulmonary hypertension in the elderly, part 1: Evaluation: Page 8 of 11
By Cynthia L. Bone-larson, MD, PhD and Kevin M. Chan, MD
6-CARDIOLOGY X-RAY QUIZ 9
By Prof. Dr. Johnson Francis, MD, DM, FACC, FRCP Edin, FRCP London
