IMAGING IN

PULMONARY
HYPERTENSION
Dr. dr. Rusli Muljadi, Sp. Rad (K)
OUTLINE
1. Introduction
2. Radiology Modalities
3. Classification
4. Imaging workup
5. Approach CT
1. INTRODUCTION
Pulmonary hypertension
Pulmonary hypertension is hemodynamically defined as
a mean pulmonary artery pressure >25 mm Hg at rest
or >30 mm Hg during exercise

with an increased pulmonary vascular resistance

Pulmonary hypertension
• PH may primarily affect either

Arterial (precapillary) pulmonary circulation.

Venous (postcapillary) pulmonary circulation.

Peña Elena, et al. RadioGraphics 2012; 32:9–32
Pulmonary Arterial Hypertension (PAH)
A. Idiopathic
B. Arise in association with:
• Pulmonary thromboembolism • Human immunodeficiency virus

• Parasitic or foreign material infection

• Parenchymal lung disease; • Longstanding cardiac left-to-right

shunt due to congenital anomaly.
• Liver disease
• Vasculitis
Pulmonary Venous Hypertension (PVH)
• Pulmonary veno-occlusive disease

• Pulmonary venous compression by extrinsic lesions

• Left-sided cardiac disease

• Pulmonary vein stenosis.

• Evidence of coexistent pulmonary arterial hypertension due to retrograde

transmission of elevated venous pressures across the capillary bed also is
commonly seen at imaging and histopathologic analysis.
Pulmonary Hypertension
• A spectrum of idiopathic and associated diseases
• that result in vascular remodeling: intimal thickening, medial
hypertrophy and extension of media into acinar arterioles
• all of which may be secondary reaction to localized fibrosis or
inflammation of any etiology.
• A large component of the morbidity of PH of any cause is the
secondary right heart failure
Peña Elena, et al. RadioGraphics 2012; 32:9–32
Peña Elena, et al. RadioGraphics 2012; 32:9–32
Diagnosis based on
• Clinical assessment of hemodynamic parameters

• Medical history

• Pulmonary function this is only for testing

• Radiologic and histologic findings

• Patients with PH experience nonspecific cardiovascular and
respiratory symptoms including dyspnea, fatigue, chest pain or
angina, and syncope
Role of Imaging
in patients with pulmonary hypertension.
• Imaging plays a central role in establishing the diagnosis

• Defining the cause

• Quantifying hemodynamic compromise

• Therapeutic planning and monitoring

• Complications
2. RADIOLOGY MODALITIES
(Diagnosis)
2.1. Chest radiography,
• The central pulmonary arteries are classically enlarged with
rapid tapering of peripheral pulmonary vasculature (pruning)

• Chest radiography may also depict right-sided cardiac

enlargement in idiopathic PAH.
Moderate cardiomegaly, marked enlargement of the
pulmonary trunk and right pulmonary artery
Primary PH Progression
2.1. Chest radiographs
• Abnormal in 90% of patients with idiopathic pulmonary arterial
hypertension (PAH) at the time of diagnosis

• Chest radiography may help assess the presence of PH, but the
degree of PH does not correlate with the extent of imaging
abnormalities
Radiographic
Classification

CATEGORY PRE CAPILLARY LUNG RELATED POST CAPILLARY

PULMONARY
VESSLES ARTERIAL ARTERIAL VENOUS
PRIMARILY
INVOVLED
EVIDENCE OF
ADDITIONAL PRUNED PERIPHERAL PULMONARY VENOUS
IMAGING PULMONARY VESSELS, MOSAIC LUNG PARENCHYMAL DISEASE HTN –
FINDINGS ATTENUATION septal lines, ground glass
opacities, pleural effusions

• Idiopathic PAH • COPD • Left ventricular failure

EXAMPLES • FamilialPAH • ILD • LV inflow or outflow
• Drug‐related • Sleep Apnea obstruction
• L to R cardiacshunt • Developmental lung • Pulmonary venoocclusive
disease
disease
• Mediastinal fibrosis

Frazier et al. Radiographics 20.2 (2000):491‐524.

PH (ASD) PH (COPD) PH (LV Failure)
2.2. Transthoracic two-dimensional
Doppler echocardiography
• Transthoracic two-dimensional Doppler echocardiography is the
first-line modality for diagnosis of PH

• It is widely available and is the most common imaging modality

used to assess ejection fraction, left-sided heart disease, or
intracardiac shunts
• For detection of moderate PH, echocardiography has sensitivity
of 79%–100% and specificity of 68%–98%

• However, echocardiography has limited capability for evaluation

of the pulmonary arteries beyond the main pulmonary artery
and is quite limited in evaluation of right ventricular function
Severe right ventricular enlargement and dislocation of the
interventricular septum (IVS) toward the left ventricle (LV)

Apical four-chamber
parasternal short-axis views
Impaired Right Ventricular Function
Tricuspid Regurgitation Dilated Inferior Vena Cava
2.3. Ventilation-perfusion (V/Q)
scintigraphy
• To identify or exclude Chronic Thromboembolic PH (CTEPH) in
patients with suspected CTEPH

• Accurate assessment of parenchymal perfusion

• It does not provide the anatomic information

2.4. CT Scan
• CT is a routinely used imaging modality for evaluation of patients with
suspected PH

• Studies may be performed without intravenous contrast material using a

high-resolution protocol when the lung parenchyma is the sole question.

• Intravenous contrast material may be used (often with a pulmonary

angiography protocol) when CTEPH or other nonpulmonary causes of
PH are suspected, such as fibrosing mediastinitis
The CT approach to diagnosis of PH
• Identifying an enlarged pulmonary artery diameter >29 mm,
which is usually larger than that of the ascending aorta at
the same level
• This diameter must be measured in the axial plane at the bifurcation,
orthogonal to the long axis of the pulmonary artery
• Main Pulmonary Artery Diameter > 29 mm is associated with P.H.,
measured 2 cm from the pulmonary valve, has a sensitivity of 84%, a
specificity of 75% and a PPV of 97%
• Other findings such as increased segmental artery–to-bronchus ratio
greater than 1:1 in three or more lobes increase the specificity for
diagnosis of PH
Main Pulmonary artery dilatation > 29 mm
CT features of pulmonary Arterial
hypertension
• Dilatation of the pulmonary artery trunk (29 mm or more)

• Diameter of which frequently exceeds that of the ascending aorta (greater than or
equal to 1)

• Dilatation of the right and left main pulmonary arteries (left PA: >28 mm Right PA:
>24.3 mm)

• Abrupt narrowing and tapering of the peripheral pulmonary vessels

• Right ventricular hypertrophy

• Right ventricular and atrial enlargement with inversion of the interventricular

septum and dilatation of the tricuspid valve annulus.
 RV Dilatation

Pulmonary artery > bronchus Reflux

CT features of pulmonary Venous
hypertension
• Interlobular septal thickening

• Pleural effusion

• Airspace opacities.
• CT is more available than other modalities

• CT findings in the lung parenchyma, mediastinum (including the

presence of bronchial arteries), pulmonary arteries, and heart
can guide the radiologist to classify PH (Will discuss in next
section)
2.5. Cardiac MR imaging
• Cardiac MR imaging is one of the most accurate methods for
assessing right ventricular size, morphology, and function

• It can also be used to assess the anatomy of the pulmonary arteries

and pulmonary blood flow

• Cardiac MR imaging is used to make sure that the right ventricle is

not showing signs of failure (eg, dilatation or reduced ejection
fraction) that would warrant additional intervention or medication
2.6. Right heart catheterization
• Right heart catheterization allows direct measurement of pulmonary
pressures, pulmonary resistance, and cardiac output
• It remains the Standard of Reference for diagnosis of PH.
• Right heart catheterization may also be used to help predict the response
to vasodilators
• As it is invasive and provides little information about the lungs or
mediastinum, right heart catheterization is usually performed in
conjunction with other modalities
PH (CTEPH)
Why a diagnosis of P.H. is not enough?

• Because very different pathophysiological pathways may

lead to the same pressure endpoint

• Imaging pattern depends on mechanism, as do prognosis

and treatment strategies

• Therefore, the role of imaging is to establish the mechanism

and the most likely cause of P.H., beyond simply establishing
the diagnosis of P.H. Etiologies
3. CLASSIFICATION
(Etiologies)
5th World Symposium Classification, Nice, France2013
Group
I Pulmonary Arterial Hypertension
(Includes all causes that leadto
structural narrowing of the
pulmonary vessels)
II Pulmonary Hypertension due to
left sided heart disease
III Pulmonary Hypertension related
to lung disease orhypoxia
IV Chronic
Thromboembolic
Pulmonary Hypertension
V Pulmonary Hypertension related
to multifactorial mechanisms
Subcategory
• 1.1 Idiopathic PAH
• 1.2 Heritable PAH
• 1.3 Drug andtoxin induced PAH
• 1.4 PAH associated with: Connective tissue diseases, HIV, Portal hypertension, Congenital
heart disease, Schistosomiasis
• 1’ Pulmonary veno‐occlusive disease and/or Pulmonarycapillary hemangiomatosis
• 1” Persistent pulmonary hypertension of the newborn
• 2.1 LV Systolic dysfunction
• 2.2 LV Diastolic dysfunction
• 2.3 ValvularDisease
• 2.4 Congenital/acquired left heart inflow/outflow tract obstruction and Congenital
cardiomyopathies
• 3.1 Chronic obstructive pulmonarydisease
• 3.2 Interstitial lung disease
• 3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
• 3.4 Sleep breathingdisorders
• 3.5 Alveolar hypoventilationdisorders
• 3.6 Chronic high altitudeexposure
• 3.7 Developmental lungdisease
• 5.1 Hematologic disorders
• 5.2 Systemic Disorders: Sarcoidosis, Pulmonary histiocytosis, Lymphangioleiomyomatosis
• 5.3 Metabolicdisorders: Glycogen storage disorders, Gaucher disease
• 5.4 Other: Fibrosing Mediastinits, Tumoral calcinosis, Renalfailure
Simonneau et al. JACC 62.25 (2013):D34‐D41.
Group 1: PAH
• Group 1 disease involves conditions with a significant
arteriolar component of disease and the potential to respond
to vasodilator therapy.

• One of the main diagnoses in this group is idiopathic PAH

 Group I ‐ Idiopathic/Heritable PAH (IPAH)

• Pulmonary arterial hypertension

– no clinically discernible cause
– normal pulmonary arterial wedge pressure
– no evidence of left‐to‐right shunt
• 3:1 female to male ratio
• Mean survival of 2.8 years w/o treatment
• Heritable PAH is similar to the idiopathic form
– Familial predilection 35 year old with IPAH. Chest radiograph
reveals dilated central pulmonary
– Not well understood arteries (white arrows) with rapid
– Bone morphogenetic protein receptor type II (BMPR2) tapering to oligemic peripherallungs.
mutation in over half of patients
Farber et al. NEJM 351.16 (2004): 1655‐1665.
Group I –PVOD/PCH

Pulmonary Veno‐occlusiveDisease: Pulmonary Capillary Hemangiomatosis:

Evidence of sepal lines and subtle Groundglass nodules with peripheral
groundglass nodules. vascular pruning. No significantinterlobular
septal thickening demonstrated.

Frazier et al. Radiographics27.3 (2007): 867‐882.

 Group I – Congenital Heart Disease

• Systemic to Pulmonary Shunt

– Atrial Septal Defect
– Ventricular Septal Defect
– Patent Ductus Arteriosus
– Eisenmenger syndrome
– Total or PartialAnomalous
Pulmonary Venous Return
• Caused by a large increase in
pulmonary arterial blood
flow
44 year old with a secundum type ASD (black arrow).
Chest CT reveals enlargement of main pulmonaryartery
with right sided heartenlargement.

Simonneau et al. JACC 54.1s1 (2009):S43‐S54.

Group 2: PH Due to Left Heart Disease
• PH induced by left heart disease is the most prevalent form
wordwide

• It begins as increased back-pressure with congestion of the

pulmonary capillary bed resulting in elevated left heart pressures
(passive PH)

• Group 2 PH is seen in patients with heart failure with preserved or

reduced ejection fraction and in left-sided valvular diseases.
Group II – PH due to Left Heart Dysfunction

• Backup of flow into the

pulmonary venous system
raising venous pressure
and eventually arterial
pressure
• May be causedby:
– restriction of flow into
(pulmonary vein
stenosis) or through
the left atrium (mitral
valve disease)
– poor flow into
(diastolic dysfunction)
or through the left
ventricle (systolic
dysfunction or aortic Patient with prolonged mitral stenosis (black arrow)
demonstrates evidence of venous pulmonary HTN
valve disease) with septal lines.

Simonneau et al. JACC 54.1s1 (2009):S43‐S54.

Group 3: PH Due to Lung Diseases
and/or Hypoxia
• Almost 66% of patients with chronic obstructive pulmonary disease
(COPD) have some degree of PH, usually mild
• The prevalence of PH in interstitial lung disease varies from 32% to
84%
• PH is seen in 20%–30% of patients with obstructive sleep apnea.
• Ten percent to 15% of patients with sleep apnea have concomitant
COPD, which increases the likelihood of PH
Group III – Related to underlying Lung Disease
• Caused by multiple
factors
– Hypoxic induced
vasoconstriction
– Mechanical stress due
to the hyper‐inflated
lungs
– Destruction of the
pulmonary capillaries
by emphysema/fibrosis

54 year old female with sarcoidosis. CT chest demonstrates

upper lobe predominant fibrosis related to end stage
sarcoidosis. The MPA was enlarged and the patient was
found to have PH, due to the underlying lung disease.
Group 4: CTEPH
• The pathophysiology of CTEPH remains unclear and may follow
even just one episode of pulmonary embolism
Group IV ‐ Chronic ThromboembolicDisease

• Chronic emboli tightlyadhere

to the medial layer of the
artery, replacing the normal
intima and causing
stenosis/obstruction
• Mechanical obstruction leads
to decrease in the cross‐
sectional area and increased
resistance within the
pulmonary arteries
CT PE study demonstrates enlarged MPA
• Can be considered in the Pre due to large chronic pulmonary emboli.
Capillary Classification Organization of the thrombi with its
Radiographic Scheme eccentric location and internal
calcification (arrows) prove itschronicity.

Castañer, Eva, et al. Radiographics 29.1 (2009): 31‐50.

Group IV ‐ Chronic Thromboembolic Disease(CTEPH)
Additional Image Findings:

Mosaic Attenuation: Enlarged BronchialArteries:

Reflecting geographic variation in bloodflow Due to the development ofsystemic–pulmonary
anastomoses, to maintain pulmonary bloodflow

Castañer, Eva, et al. Radiographics 29.1 (2009): 31‐50.

Group 5: PH with
Unclear Multifactorial Mechanisms
• Hematologic disorders: chronic hemolytic anemia, myeloproliferative
disorders, splenectomy
• Systemic disorders: sarcoidosis, pulmonary histiocytosis,
lymphangioleiomyomatosis
• Metabolic disorders: glycogen-storage disease, Gaucher disease, thyroid
disorders
• Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure,
segmental PH
Group V – Related to Multifactorial Mechanisms

• Related to different
underlying
systemic disease
• Causal relationship
either
multifactorial
and/or not well
understood
Mediastinal Fibrosis: Coronal oblique CT reconstructions
reveal constriction of the pulmonary venous drainage into
the left atrium, causing post capillary pulmonary
hypertension with septal lines (black arrows). Precapillary
hypertension is also evident with MPA dilatation.

Botticelli et al. Circulation 1966; 33:862–871.

Complications
Right Ventricular Changes

Increased Myocardial RV dilatation,

RV failureand
pulmonary RV pressure remodeling increased wall
Cor
arterial overload with RV stress and
Pulmonale
pressure hypertrophy dysfunction

Peña, Elena, et al. Radiographics 32.1 (2011): 9‐32.

Cor Pulmonale : RV Strain andHypertrophy

COR PULMONALE MRI demonstrates a dilated RV with free wall

•Anterior Free Wall RV > 6 mm thickening and delayed hyperenhancment at
•Dilatation of RV chamber (RV/LV >1) the RV insertion points (arrows) related to RV
•Septal flattening or curvereversal strain.
•Regurgitant flow: Pulmonary & tricuspidvalves

Peña, Elena, et al. Radiographics 32.1 (2011): 9‐32.

Compression to Coronary Artery

Compression to Bronchus
4. IMAGING WORK UP
Peña Elena, et al. RadioGraphics 2012; 32:9–32
• Every patient with suspected PH should undergo Chest
Radiograph and Echocardiography, with the goals of
excluding the most common causes of PH => LV failure and
diffuse lung diseases (COPD and ILD).

• Echo can also assess valve disease and RV function

• If there is a clear cause of PH

• If PH is deemed proportional to the severity of LV dysfunction

or pulmonary parenchymal disease

 Appropriate treatment for the underlying disease is initiated,

and usually no further work up of PH is necessary.
• If there is no clear cause

• If the degree of PH is disproportionate

• If there is RV failure

 Chest CT for pulmonary parenchymal diseases and PTE

disease (CE). V/Q scan for PTE disease.
• Cardiac MRI for RV dysfunction, suspected shunts, valve
disease. Emerging role for direct evaluation of pulmonary
artery flow dynamics.

• Right heart catheterization may be required, particularly if

idiopathic arterial hypertension is suspected.
Peña Elena, et al. RadioGraphics 2012; 32:9–32
5. Approach to CT
CT Findings in PH
• A structured approach to the CT findings in a patient with PH allows the
radiologist to play an important role in appropriate categorization according to the
Nice classification.

• These specific findings can be grouped into

- Pulmonary artery

- Pulmonary parenchymal
- Cardiac

- Mediastinal findings.
 WHO Classification ‐‐ RadiologicalFlowsheet

Enlarged MPA

Variable
No other Pulmonary Involvement/
abnormalities Heart Lungs Vasculature Systemic
Disease

Congenital Left Sided Underlying CTEPH

IPAH
Heart Disease Dysfunction Lung Disease GROUP V
GROUP I GROUP II GROUP IV
GROUP I GROUP III

Simonneau et al. JACC 62.25 (2013):D34‐D41.

5.1.Pulmonary artery findings

• Peripheral calcification : Group 4 (CTEPH), Group 2, Group 1

• Peripheral dilatation : Group 1, Group 5

• Eccentric filling defects : Group 4, Group 1

• Intra-arterial tumor Almost always Group 5

 Peripheral calcification

Acute pulmonary embolism

Pulmonary Artery filling defect

Chronic pulmonary embolism

Peripheral dilatation in portal
hypertension
5.2. Pulmonary parenchymal findings
• Centrilobular nodules : Group 1: PCH, Group 1: PVOD

• Mosaic attenuation : Group 4: CTEPH, Group 1, Group 2, and less frequently group 3

• Interlobular septal thickening : Group 3: irregular thickening; Group 2: smooth thickening;

Group 1: PVOD, connective tissue disorders;

• Bronchiectasis : Group 3; Group 4: CTEPH; Less frequently group 5

• Subpleural peripheral opacities : Group 4: CTEPH; Less frequently group 3

• Ground-glass opacities : Group 1; Group 3; Group 2

• Diffuse solid nodules Almost always group 5

IPF
Bronkiektasis
Kavitas dan infiltrat paru kanan, pneumotoraks
loculated kiri, bula, bronkopleura fistula kiri
5.3. Cardiac findings
• Congenital lesions Group 2: Eisenmenger syndrome and
congenital heart disease Group 1

• Left ventricular disease and signs of left ventricular failure :

Group 2; Group 1

• Valve anomalies : Group 2; Group 1

Left Atrial Tumor (Myxoma?)
ASD

Large ostium secundum ASD Superior sinus venosus ASD

VSD
Left Heart Failure
 Dilated
Cardiomyopathy

Hyperthropic
Cardiomyopathy
Valve Calcification

Mitral valve Aortic valve

5.4. Mediastinal findings
Hypertrophied bronchial arteries

• Group 4: CTEPH

• Group 2: Eisenmenger syndrome and congenital heart disease

5.5.No associated pulmonary or
mediastinal findings
• Group 1: Idiopathic PH
Summary − Take Home Messages

• Imaging plays a central role in establishing the diagnosis, defining

the cause, quantifying hemodynamic compromise, and
therapeutic planning and monitoring in patients with pulmonary
hypertension
• CT assessment of the lung parenchyma, pulmonary arteries,
bronchial arteries, and heart may provide valuable information as
to the subtype of PH according to the 2013 Nice classification and
help facilitate appropriate categorization