Annals of Thoracic Medicine - Vol 9, Issue 3, July-September 2014 131

Saudi guidelines on the treatment

and management of pulmonary
hypertension 2014: Updates a fresh
understanding of pulmonary vascular
diseases in the developing world
Ghazwan Butrous
P
ul monary hypert ensi on i s def i ned
as a heterogeneous group of diseases,
characterized by progressive pathology in the
pulmonary circulation that creates an increase in
pulmonary vessels resistance, and consequently
leads to right ventricular failure. The pathology
is often secondary to other conditions, and
only a very small minority of the patients
is idiopathic or hereditary. Therefore, this
condition is not the exclusive domain of any
medical specialities, as it can involve respiratory,
cardiology, rheumatology, infectious diseases,
hematology, and pediatrics. For this reason,
the condition is very variable, and its clinical
presentation can vary from one region of the
world to another. Although there is no clinical
data to assess the global spectrum of pulmonary
hypertension worldwide, our initial observations
through the efforts of the Pulmonary Vascular
Research Institute has indicated that there is
an obvious difference between various regions,
and particularly between the developed and the
developing world. The main variety is likely due
to the pattern of the diseases. Infectious diseases,
genetic abnormalities, geographical, social and
environmental factors as well as the type and
standard of medical care in various countries
contribute to these differences. Initial unverifed
estimates suggest that pulmonary hypertension
in the developing world could be five to six
times more prevalent than in the developed
world. Thus, pathology, as well as the clinical
presentation and the clinical management of these
conditions, can vary from one country to another.
In the last two decades, there has been an
increase of interest in directing the medical
management towards evidence-based medicine,
and consequently various guidelines, consensus
reports and expert reports have been developed
and published by a number of institutions
and learned societies across the globe. These
guidelines, expert and consensus documents
aim to present all the relevant evidence on
a particular issue in order to help weigh the
risks and benefits of a specific diagnostic or
therapeutic feature. These documents must be
written in a concise and clear manner, with a very
careful selection of the degree of evidence, to help
physicians direct their clinical management. In
the area of pulmonary hypertension, the interest
in developing a clinical guideline has been
ongoing for the last two decades. One attempt
at developing these guidelines was made in the
early 1990s, after which the American College of
Chest physicians (ACCP) created the frst ACCP
consensus statement on pulmonary primary
hypertension, published in 1993.
[1]
This was likely
the result of increased activities in the developing
the understanding of pulmonary hypertension
after the frst international meeting in 1973 in
Geneva, Switzerland
[2]
and then at the subsequent
meetings of the (later named) World Symposium
of Pulmonary Hypertension.
[3]
During the
following thirty years, there were several seminal
events in the story of pulmonary hypertension.
One particular happening concerned the national
prospective study of primary pulmonary
hypertension by the National Institute of Health
in the United States,
[4]
which is considered as
one of the important early works to highlight
the poor prognosis of pulmonary hypertension.
By the middle of the 1990s, the establishment of
prostacyclin as the management of pulmonary
hypertension had kindled the interest of many
physicians in various disciplines.
[5]
But only
in the frst decade of the 21
st
century did the
breakthrough of establishing oral therapies,
by the introduction of endothelial antagonists,
phosphodiesterase inhibitors, and others classes
of drugs, make pulmonary hypertension a
prominent subject. The increased awareness of
this condition and possible treatment options
meant that many physicians started actively
treating these patients.
Consequently, this highlighted the necessity for
the establishment of a new guideline, and the
Address for
correspondence:
Prof. Ghazwan
Butrous, Department
of Cardiopulmonary
Sciences, Pulmonary
Vascular Research
Institute,
Kent Enterprise Hub,
University of Kent,
Giles Lane Canterbury,
Kent CT2 7NJ,
United Kingdom.
E-mail: g.butrous@kent.
ac.uk
Submission: 09-04-2014
Accepted: 12-04-2014
Professor of
Cardiopulmonary
Sciences, Department
of Cardiopulmonary
Sciences,
School of Pharmacy,
University of Kent,
Kent,United Kingdom
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DOI:
10.4103/1817-1737.134065
Editorial
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Butrous: Saudi Guidelines on pulmonary hypertension
132 Annals of Thoracic Medicine - Vol 9, Issue 3, July-September 2014
frst attempt was in the beginning of the 21
st
century, where
the European Society of Cardiology
[6]
and ACCP
[7]
started their
consensus evidence-based clinical practice guidelines, and were
subsequently updated.
[6,8]
Both of these guidelines have become
the standard for many centers worldwide. Furthermore, many
standard expert and consensus reports were published during
the World Symposium on Pulmonary Hypertension; the latest
was created during the Nice meeting in 2013 (the full report
can be found in a special supplement Journal of the American
College of Cardiology of December 2013).
[3]
These documents can
be considered as an accompaniment to the guidelines. Since
then, other guidelines tailored to more specifc manifestations
of the disease have also been developed. Examples include the
guidelines for the management of pulmonary hypertension in
connective tissue diseases
[9]
and guidelines for the management
of pulmonary hypertension in sickle cell diseases.
[10]
However,
it is important to note that most of these guidelines are based
only on the developed world, and generally result from big
clinical trials supported by the pharmaceutical industry. Some
efforts to establish local guidelines were initiated by a number
of organizations, of which the Saudi Association of Pulmonary
Hypertension is a prominent example. Their frst guidelines
were published in 2008 in this journal.
[11]
That document
was heavily based on the local issues in Saudi Arabia, but
was also strongly infuenced by the international guidelines.
Some other local guidelines were published in other countries,
and generally focused on certain issues prominent within
the region. A very good example are the Chinese guidelines
on the use of anticoagulants in acute pulmonary embolism,
and consequently in pulmonary vascular diseases, by Prof.
Xiansheng Cheng, from the Fuwai Hospital in Beijing.
[12]
It is crucial to note that the interest and increase in the spectrum
of pulmonary hypertension has to refect on particularly the
local issues. The Saudi Association of Pulmonary Hypertension
(SAPH) has therefore decided to update their guidelines, in line
with both the international standard and the organizations
experience of local issues that have been gained by their close
collaboration with many centers across the Middle East.
This has resulted in the publication of the guidelines published
in this issue of the Annals of Thoracic Medicine (reference needs
to be added by the Journal editor). It must be emphasized
that these guidelines heavily refect the practise in Saudi
Arabia specifcally, which has both very advanced diagnosis
and management and the availability of a most current
managements tools. However, what truly distinguishes
these guidelines is the inclusion of a special appendix, which
details other conditions: an unique feature compared to most
international current guidelines. This document was written
by experts from all over the world with a close association
with the SAPH, and therefore refects the understanding of
the local issues. Thus the updated SAPH guidelines are to be
appreciated for their comprehensiveness and their ease of use
as a real reference.
Considering the above criteria, this document could be a
good starting point towards establishing a global guideline
for pulmonary vascular diseases. This global guideline
could reflect the local issues in various countries of the
world, specifcally conditions whereby pulmonary vascular
disease is due to local conditions. Such issues would include
schistosomiasis, which is considered one of the most common
causes of pulmonary hypertension worldwide; hemolytic
conditions, particularly sickle cell anemia; and issues like
congenital heart diseases in the developing world, where often
the management is not as appropriate and leads to many of
these patients developing pulmonary vascular diseases, even
though this phenomenon is now slowly disappearing in the
developed world due to early intervention. Additionally,
we may have to consider issues such as high altitude and
the genetic polymorphisms that are represented all over the
world.
All of these issues have been addressed well in the current
guidelines, but unfortunately this guideline cannot make
a sound recommendation for these conditions due to
the paucity of clinical evidence in the developing world.
However, at present, the guideline helps to establish
diagnosis and increase awareness, [] which will
hopefully act as a catalyst for the steady development of
global pulmonary vascular disease guidelines. This may
further stimulate research towards the diagnosis []
and management of these specifc causes of pulmonary
hypertension in the developing world.
Finally, the SAPH, Dr. Majdy Idrees, and his team, are to
be appreciated for this enormous effort, which is a step in
the right direction to help patients in the Kingdom of Saudi
Arabia, and towards the global management of pulmonary
hypertension.
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Butrous: Saudi Guidelines on pulmonary hypertension
Annals of Thoracic Medicine - Vol 9, Issue 3, July-September 2014 133
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How to cite this article: Butrous G. Saudi guidelines on the treatment
and management of pulmonary hypertension 2014: Updates a fresh
understanding of pulmonary vascular diseases in the developing
world. Ann Thorac Med 2014;9:131-3.
Source of Support: Nil, Confict of Interest: None declared.
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