IMAGING SPECTRUM OF

PULMONARY ARTERIAL
HYPERTENSION
BM. Traoré ; AA. Traoré ; Y. Alami Lamrani ; M. Boubbou ; M. Maaroufi ; B. Alami

Service de radiologie CHU HASSAN II Fès-Maroc

Faculté de médecine et de pharmacie

Université Sidi Mohammed Ben Abdellah

JFR 2017
 PLAN
• INTRODUCTION

• METHODS AND MATERIELS

• RESULTS

• DISCUSSIONS

• TAKE HOME MESSAGES

 INTRODUCTION
• Pulmonary hypertension is hemodynamically defined as a mean pulmonary artery pressure
greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise with an increased
pulmonary vascular resistance.

• The diagnosis is based on a clinical assessment of hemodynamic parameters, medical

history, results of pulmonary function testing, and radiologic and histologic findings.

• Pulmonary multidetector CT angiography is considered the gold standard of imaging

techniques used to assess PH-related pulmonary vessel changes in the differential diagnosis
 PURPOSE
• To learn about pathophysiologic characteristics of PAH.

• To become familiar with imaging techniques and findings of PAH.

• To be aware of the main etiologies of PAH.

 METHODS AND MATERIALS

Materials :

We report a retrospective study that evaluates 90 cases of pulmonary arterial hypertension seen

during two year- period from January 2014 to December 2015. Patients were admitted with

dyspnea, chest pain and fatigue.

 METHODS AND MATERIALS

METHODS :

Our patients underwent plain chest x-ray (n=90), CT pulmonary angiography (n=90), chest

MRI (n=26).

We used the Dana point 2008 classification for etiological diagnosis.

 RESULTS

Epidemiology

• Male: 50% (45/90) and Female : 50% (45/90).

• Sex ratio: 1

• Average age : 50 years .

 RESULTS
The results that were obtained:

• Group 1: long-standing Left-to-Right Shunt (n=7),

• Group 1′: Pulmonary Capillary Hemangiomatosis and Veno-occlusive Disease (n=10),

• Group 2: Left-sided Heart Disease (n=20),

• Group 3: Lung Disease (n=34),

• Group 4: Chronic Thromboembolic Disease (n=12),

• Group 5: Other Conditions: Sarcoidosis (n=2), histiocytosis X (n=2), lymphangiomatosis

(n=2), fibrosing mediastinitis (n=1).
 RESULTS

Groupe 5
Groupe 1
8%
8%
Groupe 1'
Groupe 4 11%
13%

Groupe 2
22%
Groupe 3
38%

Groupe 1 Groupe 1' Groupe 2 Groupe 3 Groupe 4 Groupe 5

 DISCUSSION
Pathology :

PAH can result from either increased pulmonary venous resistance (most common) or
increased pulmonary venous flow, such as with a left-to- right shunt .

Even in cases of increased flow, the main factor in generating severe PAH is an arteriopathy,
which has four main components :
• muscular hypertrophy ; intimal thickening ; adventitial thickening

• plexiform lesions : focal proliferation of endothelial channels

 DISCUSSION
Clinical presentation :

Symptoms are non-specific and begin with fatigue and breathlessness on exertion, and can include
angina, palpitations and pre-syncope. Later, as right ventricular failure develops, signs in advanced
cases include peripheral oedema and ascites, and syncope.

PAH is caused by a number of diseases affecting the heart and lungs. The clinical classification is
helpful in understanding the different aetiology and determining treatment.
 Classification
The clinical classification is helpful in understanding the different aetiology and determining treatment.
Dana Point Classification of Pulmonary Hypertension
Group Description
1 Pulmonary arterial hypertension
1.1 Idiopathic pulmonary arterial hypertension
1.2 Heritable
1.3 Drug and toxin induced
1.4 Associated with connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, schistosomiasis, and chronic
hemolytic anemia
1.5 Persistent pulmonary hypertension in newborns.
1’ Pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis
2 Pulmonary hypertension due to left-sided heart disease
3 Pulmonary hypertension due to lung diseases or hypoxia
4 Chronic thromboembolic pulmonary hypertension
5 Pulmonary hypertension with unclear multifactorial mechanisms :
• Haematological disorders: myeloproliferative disorders, splenectomy
• Systemic disorders: sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis , vasculitis
• Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders
• Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis
 RADIOGRAPHIC FEATURES
Chest radiography :

Not enough for a definitive diagnosis. The following chest radiographic findings may indicate
the presence of pulmonary hypertension :
• enlargement of the right and left main pulmonary arteries;

• hilar enlargement; tapering or pruning of peripheral pulmonary arteries;

• enlargement of the right interlobar artery (greater than 15 mm diameter on posteroanterior frontal
radiograph);

• right atrial and right ventricular enlargement;

• and areas of oligemia, which appear as increased lucency and decreased vascularity
 Fig. Hilum convergence sign

Fig. Plain film findings : central pulmonary

Pulmonary vessels converge arterial dilatation and pruning of the
Andjoin a dilated pulmonary artery. peripheral arteries
 Fig. Plain film findings : right interlobar artery
dilatation
Fig. Plain findings : Right atrium and right
ventricule enlargement
 RADIOGRAPHIC FEATURES

Transthoracic Echocardiography :

Complementing chest radiography. It yields semi quantitative functional and anatomic

information and is an invaluable tool for the diagnosis :

• Systolic and diastolic dysfunction;

• The left and right ventricles,

• Intracardiac shunt,

• Valvular stenosis and regurgitation.

 CT PROTOCOL IN PAH

• Scanning Range • From the Base of Lungs to the Apicies

• Detetector Collimation • 64 x 0.6 mm
• kVp • 120
• mA • 240
• Rotation Time (ms) • 330
• Pitch • 0.6
• Reconstruction Thickness • 1.25 x 0.625 mm ; 2.5 x 2.5
• Site of Cannulation • Right Cubital Fossa
• Contrast Volume • 80 ml
• Saline Volume • 60 ml
• Contrast Flow rate • 4 mls/sec
• Level of Dynamic Scan • Pulmonary Arch
• Type of Triggering • Bolous Triggering
• ROI • 120 HU
 CT

• The classic MDCT findings of pulmonary hypertension may be divided into three categories:
vascular, cardiac, and parenchymal.

Vascular signs :

• => Enlarged pulmonary trunk:

 29 mm diameter is often used as a general predictive cut-off, Larger than the adjacent ascending
aorta Pulmonary trunk enlargement is a poor predictor of PAH in patients with interstitial lung
disease (specificity ~ 40%)

 The ratio of the main pulmonary arterial diameter to that of the ascending aorta is also ≥ 1.
 CT
Vascular signs :

Axial MDCT angiogram images show dilatation of the

main pulmonary artery.
 CT
Vascular signs :
=> Enlarged pulmonary arteries:
• A segmental artery-to-bronchus diameter ratio of 1:1 or more in three or
four lobes has a specificity of 100% for the presence of pulmonary
hypertension
 CT
Cardiac signs Right ventricular dilatation ( as a right ventricle
to left ventricle diameter ratio of more than 1:1
Straightening or bowing (towards the left at the midventricular level on axial MDCT
ventricle) of the interventricular septum on angiogram).
axial MDCT.

Fig. Cardiac signs of pulmonary hypertension

 CT
Cardiac signs

• Dilatation of the inferior

vena cava
• and Hepatic veins.

Axial MDCT angiogram shows reflux of

contarast into the inferior vena cava. which is
dilated, and hepatic veins
Suggesting tricuspid regurgitztion and dilated
right ventricule

Fig. Cardiac signs of pulmonary hypertension

 CT

Parenchymal Signs :

• Centrilobular ground-glass nodules are a feature of pulmonary hypertension: - Especially

common in patients with idiopathic PAH

- Also seen in patients with pulmonary capillary hemangiomatosis

 MRI
Cardiac MR imaging is used to assess ventricular volume, morphologic characteristics, mass,
function, and changes in pulmonary circulation.

Cardiac MR protocol in patients with PAH

• FIESTA cine short axis, 2-3-4 chambers (morphology, function, and wall motion)

• Phase contrast on pulmonary artery (flow measurements)

• T1 black-blood (morphology)

• Contrast enhancement MR angiography (pulmonary arteries)

• Delayed enhancement (viability)

 MRI

Morphological abnormalities in patients with PAH:

• Hypertrophy, increased trabeculation, dilatation of RV, increased RV mass

• Dilatation of Right atrium (RA)

• Concentric shape of Right ventricle (RV)

• ''D-shaped'' morphology of Left ventricle ( LV)

• Dilatation of pulmonary arteries

59-year old patient with secondary to chronic thromboembolic disease.
Properative short-axis oblique steady-state free precession cine MR image,
obtained at the midventricular level in early diastole, shows right ventricular
dilatation (✽), hypertrophy (black arrow) and a D-shaped left ventricular
(white arrow) secondary to leftward bowing of the interventricular septum
(arrow head).

Fig. Morphologic cardiac findings of pulmonary hypertension

 MRI
Functional abnormalities in patients with PAH :
• Reduced contractility (hypokinesis of RV)

• Dysfunction : Right ventricle end-systolic volume (RVEDV); Right ventricle

• end-systolic volume (RVESV) ; Ejection fraction (EF); Cardiac index (CI) ;

• Stroke volume (SV)

• Paradoxical movement of Interventricular septum( IVS)

• Valve insufficiency (tricuspid or pulmonary)

• Reduced flow velocity in pulmonary artery

• Decreased systolic in blood flow right coronary artery

52-year olf female with severe PAH.
a. The right ventricule is grossly dilated and hypertrophied. Note a
pericardial effusion due to the difficult drainage of the systemic
circulation

b. The distorted interventricular septum is bowed (white arrow) towards the left
ventricule because of right ventricular overload.

Fig. Functional cardiac findings in patient with PAH

 THE MAIN ETIOLOGIES OF PAH
Group 1: long-standing Left-to-Right Shunt

• Eisenmenger syndrome :

Complication of an uncorrected high-flow, high-pressure congenital heart anomaly leading to

chronic pulmonary arterial hypertension and shunt reversal.

- The three lesions that account for most cases are:

• ventricular septal defect (VSD)

• atrioventricular septal defect (AVSD)

• patent ductus arteriosus (PDA)

Axial MDCT images in patient with Eisenmenger syndrome show:

Large main PA
Ventricular septal defect
Right ventricular hypertrophy wall thickness > 4mm.
 THE MAIN ETIOLOGIES OF PAH

Group 1#: Pulmonary Capillary Hemangiomatosis and Veno-occlusive Disease

- Rare causes of pulmonary arterial hypertension

- Characterized by specific pathologic changes that lead to obliteration of the postcapillary veins

- Commonly affects children and young adults

Axial MDCT images in patient with veno-occlusive Disease show:
Thickened interlobular septa
Poorly defined centrilobular ground-glass nodules
lymphadenopathy
 THE MAIN ETIOLOGIES OF PAH
Group 2 :Left-sided Heart Disease:

- One of the most common causes of pulmonary hypertension.

- It results from backward transmission of elevated left atrial pressure into the pulmonary

venous circulation.

- The most common causative entities are left ventricular systolic or diastolic dysfunction,

valvular disorders, and left atrial tumors.

Dilatation of the pulmonary artery
Interlobular septal thickening Alveolar edema : bat wing
Pleural effusion pulmonary opacities

Fig. Signs of pulmonary edema resulting from left heart failure

 Pulmonary vein dilatation
Upper lobe redistribution Interlobular septal thickening
of pulmonary vessels Pleural effusion

Fig. Signs of pulmonary edema resulting from left heart failure

 THE MAIN ETIOLOGIES OF PAH
Group 3: Lung Disease

• The most common lung diseases that are associated with PAH include chronic obstructive
pulmonary disease, interstitial lung disease

• CT findings:
• The combination of emphysema in the upper lobes and pulmonary fibrosis in the lower lobes appears
to be associated with a higher prevalence of pulmonary hypertension

• In patients with advanced pulmonary fibrosis, the ratio of the pulmonary artery diameter to that of the
ascending aorta is a more reliable indicator of pulmonary hypertension than the absolute main
pulmonary artery diameter
Patient with interstitial lung disease and
PAH at axial MDCT image shows:
pulmonary fibrosis (Honeycombing).

Fig. CT findings in Patient with interstitial lung disease and PAH

 THE MAIN ETIOLOGIES OF PAH

Group 4: Chronic Thromboembolic Disease

CT findings

Parenchymal Findings:

• Scars from prior pulmonary infarction

• Mosaic pattern of perfusion

• Cylindrical bronchial airway dilatation

• Systemic perfusion of the peripheral pulmonary arterial bed

 Peripheral scarring due to old pulmonary infraction

Fig. Parenchymal findings of chronic thromboembolic pulmonary hypertension.

 THE MAIN ETIOLOGIES OF PAH

Vascular Findings:

• Complete obstruction, indicated by a tapered occluded vessel

• Partial filling defects, indicated by obtuse mural margins with the vessel wall

• Collateral systemic supply

• Partial filling defect, indicated by the presence of webs or bands

Eccentric mural thickening
espesiallyin the right and
interlobar arteries

Fig. Vascular findings of chronic thromboembolic pulmonary hypertension.

Fig. Axial images MDCT in patients with Chronic Thromboembolic Disease show:
Large main PA
Dilatation of the pulmonary artery
Pleural effusion
 THE MAIN ETIOLOGIES OF PAH
Group 5: Other Conditions

=> Histiocytosis X

• Pulmonary histiocytosis X is an uncommon but important cause of pulmonary fibrosis and honeycombing
in young adults.

CT findings:
• Predominantly upper lobe nodules and cysts are virtually pathognomonic of this disorder. As pulmonary
histiocytosis X progresses, the nodules decrease in number, leaving multiple thin-walled cysts.

• ground-glass opacities,

• emphysema

• mosaic attenuation
Axial MDCT image of a patient with pulmonary
histiocytosis X and PAH shows a combination of
cysts and nodules . The cysts are round
and well defined

Fig. CT findings of histiocytosis X

 THE MAIN ETIOLOGIES OF PAH
=> Lymphangiomyomatosis (LAM) :

- Uncommon interstitial lung disease that exclusively affects women, usually during their
reproductive years.

- LAM is characterized pathologically by abnormal proliferation of LAM cells in the lungs and in
thoracic and retroperitoneal lymphatics.

CT findings:
• thin walled cysts of variable sizes surrounded by normal lung parenchyma
• interlobular septal thickening
• may show a dilated thoracic duct
• haemorrhages may be seen as areas of increased attenuation
Axial MDCT image, obtained in patient with
progressive dyspnea and pulmonary hypertension
shows the round, and thin-walled cysts typical of
lymphangiomyomatosis.

Fig. CT findings of Lymphangiomyomatosis

 THE MAIN ETIOLOGIES OF PAH
=> Fibrosing mediastinitis

- Rare condition that results from a fibrotic reaction caused by granulomatous infection that
causes narrowing of the pulmonary arteries or veins. It also may affect the central airways.

CT findings:

• mediastinal or hilar mass : especially in localised disease

• infiltrative region of soft-tissue attenuation which obliterates normal

• mediastinal fat planes and encases or invades adjacent structures : diffuse form
Fibrosing mediastinitis in a 77-year-old man with a history of
right pulmonary artery stenosis and histoplasmosis.
Axial MDCT angiogram shows marked narrowing of the right
and interlobular arteries resulting from a partially soft-tissue
mass (arrow)

Fig. CT findings of fibrosing mediastinitis

 TAKE HOME MESSAGES
• Imaging plays a fundamental role in the management of patients suffering from PAH.

• Radiologist plays an increasingly important role in the diagnosis and management of

pulmonary arterial hypertension

 REFERENCES
1. B. Alami, et al. What every radiologist needs to know about pulmonary arterial hypertension .
European society of cardiac radiology (ESCR) october 2013.
2. Marie M. Budev et al. Diagnosis and evaluation of pulmonary hypertension. Cleveland clinic
journal of medicine volume 70 • Supplement 1 April 2003
3. Nazzareno Galie et al. Guidelines for the diagnosis and treatment of pulmonary hypertension.
European Heart Journal (2009) 30, 2493-2537
4. Elena Peña et al. Pulmonary Hypertension: How the Radiologist Can Help. Radiographics ; Volume
32 ; Number 1 ; January-February 2012
5. Gianluca Marrone et al.The role of 1.5T cardiac MRI in the diagnosis, prognosis and management
of pulmonary arterial hypertension. Int J Cardiovasc Imaging
6. Yuranga Weerakkody et al. Pulmonary arterial hypertension. www.Radiopaedia.org
7. Lombard. V et al. Hypertension artérielle pulmonaire: quel rôle à jouer pour le radiologue? CHU
NANCY-BRABOIS; JFR 2010.
8. O.Sanchez et al. Imaging of pulmonary arterial hypertension. Revue des maladies respiratoires.
Volume 24, Issue 2, february 2007, Pages 155-169.
9. J.P.Alunni et al. Hypertension arterielle pulmonaire (HTAP) : correlations entre IRM cardiaque et
catheterisme cardiaque droit. Journal de Radiologie volume 89, Issue 10, October 2008, Page 1367