Correspondence Address:
Ghazwan Butrous
Department of Cardiopulmonary Sciences, Pulmonary Vascular Research Institute, Kent Enterprise Hub, University of Kent, Giles Lane Canterbury, Kent CT2 7NJ
United Kingdom

Full Text

Pulmonary hypertension is defined 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 unverified 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 first 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 first 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 first 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 first 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 specific 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 first 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 influenced 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 reflect 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 organization's 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 reflect the practise in Saudi Arabia specifically, 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 reflects 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, specifically 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 specific 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.

References