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Year : 2013  |  Volume : 137  |  Issue : 2  |  Page : 401-402

Pulmonary vascular disorders, Progress in respiratory research

Bhopal Memorial Hospital & Research Centre & National Institute for Research in Environmental Health (ICMR) Bhopal 462 038, India

Date of Web Publication26-Mar-2013

Correspondence Address:
V K Vijayan
Bhopal Memorial Hospital & Research Centre & National Institute for Research in Environmental Health (ICMR) Bhopal 462 038
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Source of Support: None, Conflict of Interest: None

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How to cite this article:
Vijayan V K. Pulmonary vascular disorders, Progress in respiratory research. Indian J Med Res 2013;137:401-2

How to cite this URL:
Vijayan V K. Pulmonary vascular disorders, Progress in respiratory research. Indian J Med Res [serial online] 2013 [cited 2021 Jan 18];137:401-2. Available from:

Pulmonary vascular disorders, Progress in respiratory research , vol. 41, Humbert M., Souza R., Simonneau G., editors (S. Karger, Basel, Switzerland) 2012. 290 pages. Price: US$ 249.00, EUR 177.00, CHF 212.00

ISBN 978-3-8055-9914-6

This book describes the major advances that have occurred in the area of pulmonary arterial hypertension (PAH), especially in understanding the patho-physiological mechanisms and in the management during the last one decade. Each chapter is contributed by global experts in the field of PAH. The first chapter describes the updated "Dana Point" classification of PAH as adopted at the 4 th World Symposium on Pulmonary Hypertension held in Dana Point, California, USA in 2008. Schistosomiasis and chronic haemolytic anaemia are added as separate entities that cause PAH. The second chapter deals with pathology of PAH describing in detail the vascular lesions seen in PAH. The chapter on "Invasive rest and exercise haemodynamics in the management of pulmonary vascular disease" emphasises that it is essential to have invasive haemodynamic studies for diagnosis, assessing prognosis and monitoring the effect of therapy in pulmonary vascular disease. This chapter clearly states that additional or complementary measurements such as echocardiography or MRI cannot replace the invasive haemodynamic measurement which is the "gold standard" investigation, an important message to those dealing with PAH patients. The important role of exercise testing for the assessment of disease severity and prognostication as well as for monitoring response to treatment in idiopathic PAH is highlighted in the next chapter. The 6-minute walking test has been advocated as a simple, reproducible and non-invasive test for the routine assessment of PAH patients.

The next chapter on "Non-invasive exploration of the pulmonary circulation and the right heart" describes the advances in echocardiography and MRI that enable us to measure right heart ventricular volume and mass and pulmonary artery flow. The role biomarkers such as uric acid, troponin, asymmetric dimethyl arginine, endothelin, D-dimer and von Willebrand factor and natriuretic peptides to describe disease severity and prognosis are critically discussed in a separate chapter. It has been stressed that a combination of different biomarkers may be the future approach for the evaluation of patients with PAH. The importance of genetic screening to identify and analyse clinical characteristics of PAH patients with a heritable condition is described in a chapter, as germ line mutations of BMPR2 gene (bone morphogenetic protein receptor type 2), ACVRL1 (activin A receptor type II-like 1), ENG (endoglin) and Smad8 genes have been identified in patients with PAH. It has been suggested that screening of asymptomatic relative carriers of BMPR2 mutation can benefit because they have a high risk of developing PAH.

A separate chapter on idiopathic pulmonary arterial hypertension (IPAH) compares the clinical characteristics of IPAH in patients from developed and developing countries. This chapter emphasises the need to have pulmonary hypertension registries in developing countries so that differences in the clinical characteristics can be scientifically documented. Many subsequent chapters are devoted to description of PAH due to various aetiologies and diseases. Pulmonary hypertension due to drugs and toxins, connective tissue disorders, HIV and other viral infections, hepatopulmonary syndrome, congenital heart diseases, sickle cell disease, schistosomiasis, pulmonary veno-occlusive disease, left heart disease, chronic obstructive pulmonary disease, interstitial and granulomatous lung diseases and high altitude are described in each chapter. The pathophysiology, clinical features, diagnostic methods, treatment strategies and prognosis of such patients with PAH are elaborated in each chapter.

Acute pulmonary venous thromboembolism and the role of anti-coagulants in venous thromboembolism are described in two chapters. There is a critical assessment of recent improvements and unresolved issues in anti-coagulant treatment of venous thromboembolism. A separate chapter deals with the epidemiology, pathophysiology, clinical features, diagnosis, surgical and medical treatment of chronic thromboembolic pulmonary hypertension. A chapter on treatment of PAH discusses the currently licensed treatments, viz. prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase type-5 (PDE 5) inhibitors. As disease progression is frequently observed in patients treated with single drug, it has been suggested that combination with two or more drugs may improve. It has also been suggested that general measures and supportive therapy with oxygen administration and diuretics may provide symptomatic benefit.

As there are patients who may not respond to optimised medical therapy, lung transplantation is a therapeutic option. The role of lung transplantation and the novel extracorporeal support in pulmonary hypertension is discussed in a chapter. Bilateral lung transplantation is the preferred procedure in most patients. The recent introduction of venoarterial extracorporeal membrane oxygenation (ECMO) in awake and non intubated patients to stabilise them for several weeks allowing bridging to transplantation has also been discussed. The International Society of Heart and Lung Transplantation (ISHLT) registry has reported that the cumulative median survival for all procedures performed was 6.6 years after bilateral lung transplantation and 4.6 years after single lung transplantation, and the long-term outcome with 5-year-survival was reported to be approximately 50%. Atrial septostomy, another option available for treatment of PAH is discussed in a separate chapter. Atrial septostomy is offered to a selected group of patients and this acts as a therapeutic option as a bridge to transplantation and as a destination therapy for patients failing on medical treatment. A whole chapter deals with pulmonary vascular disorders in hereditary haemorrhagic telangiectasia. The last chapter is an overview of future perspectives in PAH.

The pulmonary circulation and the diseases associated with it are a challenge to physicians to make a diagnosis and to provide optimal treatment. This book has dealt with most of the recent developments in pulmonary hypertension especially in relation to pathophysiology, clinical, diagnostic and treatment aspects. This book focusses on the clinical aspects of pulmonary hypertension and thus will be an essential resource book for clinicians who deal with PAH patients, viz. chest physicians, cardiologists, internists, thoracic surgeons and postgraduate medical students.


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