Anton Vonk Noordegraaf

Auger WR

Benza R

Champion HC

Galiè N

Hovnanian A

Montani D

Simonneau G

Vonk Noordegraaf, A.

NARCIS 

The image of pulmonary hypertension

DSpace at VU

J Bras Pneumol. 2011;37(3):283-284
hypertension, they can cause considerable 
harm if the underlying cause of the pulmonary 
hypertension is left heart disease or COPD. The 
choice of diagnostic tools is based not only 
on current recommendations and guidelines 
but also on the availability of techniques and 
expertise.(3) For instance, although the current 
guidelines strongly recommend the use of 
ventilation/perfusion imaging to exclude chronic 
thromboembolic pulmonary hypertension, this 
technique is not always available. At facilities 
staffed with well-trained radiologists who 
are familiar with the diagnosis of chronic 
thromboembolism, CT and magnetic resonance 
(MR) angiography present potential alternatives 
to ventilation/perfusion imaging. The ideal 
situation is that in which the various techniques 
are available and are used in a complementary 
manner, experienced hands making optimal 
use of the strengths of each imaging modality. 
For instance, when pulmonary hypertension 
is suspected, ventilation/perfusion imaging 
is considered the most sensitive tool for the 
diagnosis of chronic thromboembolic pulmonary 
hypertension. However, this technique provides 
no information on the pulmonary vasculature. 
Therefore, if ventilation/perfusion imaging 
indicates chronic thromboembolic pulmonary 
hypertension, additional investigation is 
required. Although CT angiography provides 
excellent data on the central pulmonary arteries, 
it is limited in the detection of small peripheral 
artery lesions.(4) In addition, although pulmonary 
angiography provides excellent information on 
peripheral chronic pulmonary embolism, it can 
miss centrally located lesions adhered to the 
pulmonary vascular wall. By combining CT 
angiography and pulmonary angiography, we 
can obtain excellent information on the central 
and peripheral pulmonary vasculature, which is 
needed in order to determine whether surgery is 
a therapeutic option or not.
How do the new imaging modalities, such 
as MR imaging (MRI), fit into this picture? 
Because MRI can be used for perfusion imaging 
and MR angiography, it can be considered a 
Pulmonary hypertension is a condition 
defined by a mean pulmonary artery pressure 
above 25 mmHg. The first step in the assessment 
of pulmonary hypertension is to consider it 
within the context of the symptoms or the 
underlying medical condition. When pulmonary 
hypertension is suspected, echocardiography 
is the next logical step in the diagnostic 
process. Pulmonary hypertension is sometimes 
diagnosed on the basis of accidental findings 
on chest X-rays or CT scans made for other 
purposes. In a review published in the current 
issue of the Brazilian Journal of Pulmonology, 
Hovnanian et al.(1) summarize the major 
diagnostic information that can be used in order 
to detect pulmonary hypertension. Although 
the results of noninvasive tests can raise the 
diagnostic suspicion of pulmonary hypertension, 
there is as yet no simple, specific, inexpensive 
instrument that is also highly sensitive for the 
diagnosis of pulmonary hypertension. It is 
therefore of interest to explore the potential of 
electrical impedance tomography in this respect, 
as proposed by Hovnanian et al.(1)
If noninvasive tests produce evidence of 
pulmonary hypertension, further diagnostic 
tests are required in order to diagnose the 
underlying condition. According to The World 
Health Organization, the causes of pulmonary 
hypertension are divided into five major groups: 
pulmonary arterial hypertension; pulmonary 
hypertension secondary to left heart disease; 
pulmonary hypertension secondary to lung 
diseases (obstructive or interstitial) or sleep-
disordered breathing; pulmonary hypertension 
caused by blood clots in the lungs or blood 
clotting disorders; and pulmonary hypertension 
secondary to various other diseases, including 
blood disorders, systemic disorders, and 
metabolic disorders, as well as other conditions, 
such as tumors.(2) An accurate diagnosis of 
pulmonary hypertension is important for the 
patient, because the various diagnostic classes 
require different medical treatments. For 
instance, although the use of endothelin receptor 
antagonists is indicated in pulmonary arterial 
The image of pulmonary hypertension
A imagem da hipertensão pulmonar
Anton Vonk Noordegraaf
Editorial
284 
J Bras Pneumol. 2011;37(3):283-284
References
1. Hovnanian A, Menezes E, Hoette S, Jardim C, 
Jasinowodolinski D, Souza R. The role of imaging 
techniques in the assessment of pulmonary circulation. 
J Bras Pneumol. 2011;37(3):389-403.
2. Simonneau G, Robbins IM, Beghetti M, Channick 
RN, Delcroix M, Denton CP, et al. Updated clinical 
classification of pulmonary hypertension. J Am Coll 
Cardiol. 2009;54(1 Suppl):S43-54.
3. Task Force for Diagnosis and Treatment of Pulmonary 
Hypertension of European Society of Cardiology (ESC); 
European Respiratory Society (ERS); International 
Society of Heart and Lung Transplantation (ISHLT), 
Galiè N, Hoeper MM, Humbert M, et al. Guidelines for 
the diagnosis and treatment of pulmonary hypertension. 
Eur Respir J. 2009;34(6):1219-63.
4. Auger WR, Kim NH, Trow TK. Chronic thromboembolic 
pulmonary hypertension. Clin Chest Med. 
2010;31(4):741-58.
5. Benza R, Biederman R, Murali S, Gupta H. Role of 
cardiac magnetic resonance imaging in the management 
of patients with pulmonary arterial hypertension. J Am 
Coll Cardiol. 2008;52(21):1683-92.
6. Champion HC, Michelakis ED, Hassoun PM 
Comprehensive invasive and noninvasive approach to the 
right ventricle-pulmonary circulation unit: state of the 
art and clinical and research implications. Circulation. 
2009;120(11):992-1007.
7. Montani D, Kemp K, Dorfmuller P, Sitbon O, Simonneau 
G, Humbert M. Idiopathic pulmonary arterial 
hypertension and pulmonary veno-occlusive disease: 
similarities and differences. Semin Respir Crit Care Med. 
2009;30(4):411-20.
one-stop shop for the diagnosis of pulmonary 
hypertension, given that it can also provide 
detailed information on the right ventricle.(5,6) 
However, due to issues related to availability and 
costs, the application of this technique is limited 
in most countries around the world.
An accurate diagnosis of pulmonary 
hypertension depends not only on the correct 
use of imaging techniques, as is nicely outlined 
in the review by Hovnanian et al.,(1) but also on 
expertise in the diagnosis of this condition. In 
particular, rare conditions, such as pulmonary 
veno-occlusive disease(7) and pulmonary 
hypertension in patients with amyloidosis, can 
be easily overlooked by non-experts. Therefore, 
patients with pulmonary hypertension should be 
referred to a large referral center where not only 
are the required imaging techniques available 
but, more importantly, the staff knows how to 
use them for the benefit of the patient.
Anton Vonk Noordegraaf  
Department of Pulmonary Diseases,  
Vrij Universiteit –  
VU, Free University –  
University Medical Center,  
Amsterdam, The Netherlands


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