Book Review

Pulmonary Hypertension

N Engl J Med 2008; 359:2180-2181November 13, 2008

Article

Pulmonary Hypertension
(Contemporary Cardiology.) Edited by Nicholas S. Hill and Harrison W. Farber. 444 pp., illustrated. Totowa, NJ, Humana Press, 2008. $149. ISBN: 978-1-58829-661-0

Pulmonary hypertension and its effects on right heart function have long been an enigma to most clinicians. However, remarkable progress has been made in recent years. We are now able to accurately classify the condition and have a better understanding of the mechanisms that contribute to the pulmonary vascular remodeling that leads to elevated pulmonary-artery pressure and right heart failure. These achievements have led to the development of innovative medical therapies that target the dysfunctional pathways that are characteristic of the disease, as well as the development of surgical approaches that include lung transplantation and pulmonary endarterectomy. It is thus timely to release an updated textbook to the growing number of physicians who have an interest in pulmonary vascular medicine. Pulmonary Hypertension will undoubtedly provide useful information on the condition to readers.

The introductory chapter, written by Alfred P. Fishman, a pioneer in the field of pulmonary vascular medicine who has witnessed and led many advances in the past five decades, is of major interest. In a few pages, Fishman describes what has been a series of amazing achievements, including the development of techniques for right heart catheterization, the first description of “primary” pulmonary hypertension, and the progress that has been made since then. Fishman also reminds us of the epidemics of pulmonary hypertension that the use of anorectic agents caused in Europe and in the United States.

Authors of subsequent chapters address the current classification of pulmonary hypertension as well as invasive and noninvasive diagnostic approaches. Physicians need both approaches to identify the causes and assess the severity of patients' illnesses. These chapters will be very helpful to the busy clinician who is treating patients with pulmonary hypertension. The descriptions of the pathophysiology and genetics of pulmonary hypertension nicely summarize the many imbalanced pathways that have led to the discovery of targeted therapies for pulmonary hypertension. The emphasis on the so-called dark side of the heart, the right ventricle, allows for a better understanding of the specifics of heart failure in patients with pulmonary hypertension. Many conditions that promote pulmonary arterial hypertension — including congenital heart diseases, connective tissue diseases, human immunodeficiency virus infection, portal hypertension, sarcoidosis, and sickle cell disease — are discussed, with clear take-home messages and appropriate references to the literature.

Chronic thromboembolic pulmonary hypertension is one of the important clinical variants of pulmonary hypertension, and it is the only curable form of the disease. The chapter on this subject is of great interest because the authors extensively discuss how the condition should be identified and describe the referral of patients to specialized centers where pulmonary endarterectomy, a lifesaving procedure, is performed.

All the chapters that deal with medical management are well balanced and clear and include discussions of the management of fluid overload, hypoxemia, thrombosis, and the use of vasodilators in a small subset of patients with documented vasoreactivity. Targeted therapies (such as prostacyclin derivatives, endothelin-receptor antagonists, and phosphodiesterase type 5 inhibitors) have been developed on the basis of our understanding of unbalanced pathways in the pulmonary arteries of patients with pulmonary hypertension — but we are far from a cure.

We have progressed a long way since 1973, when the World Health Organization sponsored the first international meeting on a mysterious condition called “primary pulmonary hypertension,” spurred on by the sudden increase in pulmonary hypertension in patients who had used the anorexigen aminorex fumarate. It remains widely believed that pulmonary hypertension is a rare disease. Although this is true in the case of idiopathic pulmonary arterial hypertension (formerly known as primary pulmonary hypertension), a condition that affects approximately 6 persons per 1 million in Europe, the true extent of the burden of pulmonary hypertension is currently unknown and largely underestimated worldwide. There is no doubt that this book will be of interest to nurses, medical trainees, and physicians from various fields who witness the burden of pulmonary hypertension in their practice.

Marc Humbert, M.D.
Hôpital Antoine-Béclère, 92140 Clamart, France
marc.humbert@abc.aphp.fr

Citing Articles (1)

Citing Articles

1. 1

   Dan Dan Sun, Hong Mao Chen, Yun You Duan, Li Jun Yuan, Fu Jun Shang, Ning Nan Liang, Wei Cao. (2011) Analysis of Doppler Flow Spectra of the Superior Vena Cava in a Canine Model of Acute Thromboembolic Pulmonary Hypertension : Analysis of SVC Spectra in Canine ATEPH Model. Echocardiographyno
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