Book Review

Pulmonary Circulation: Diseases and their Treatment

N Engl J Med 2005; 352:2359-2360June 2, 2005

Article

Pulmonary Circulation: Diseases and their Treatment
Second edition. Edited by Andrew J. Peacock and Lewis J. Rubin. 614 pp., illustrated. London, Arnold, 2004. $198.50. ISBN: 0-340-80782-2

This second edition of Pulmonary Circulation is timely. Nine years have elapsed since its first edition in 1996, years in which we have witnessed such major scientific advances as the description of the human genome (2001) and, directly relevant to the pulmonary circulation, alteration of the gene for bone morphogenetic protein receptor type 2 (BMPR2) on chromosome 2q33. Two groups of investigators identified BMPR2 mutations in about 50 percent of familial cases and 26 percent of sporadic cases of idiopathic pulmonary arterial hypertension at the midpoint (2000) between publication of the first and second editions of Pulmonary Circulation. Other notable events that occurred during the period between editions were the development of a new classification of pulmonary hypertension under the sponsorship of the World Health Organization, a revised classification, and new treatments that emerged from large multicenter controlled trials involving patients with pulmonary arterial hypertension — a disease that, just two decades ago, had a mean survival period of less than three years.

The book is a combined effort of investigators heavily invested in the study of the pulmonary circulation from the United States, many parts of Europe, Mexico, Canada, and Australia (the list of contributors is a who's who of pulmonary-hypertension experts in the world). These authors collectively have contributed to a better understanding of the pathogenesis of pulmonary hypertension and to the development of new drug treatments, which lend a “front stage” feeling to the book for the casual reader as well as the expert in the field.

The book's nine sections, with a total of 52 chapters, cover most known aspects of the pathology and physiology of the pulmonary circulation. The orientation is heavily tilted in favor of the diagnosis and treatment of pulmonary arterial hypertension (covered in parts 3, 4, and 6, which represent more than 350 pages), a prototypical disease of the pulmonary circulation and the most common syndrome affecting the pulmonary circulation. The various causes of pulmonary hypertension receive generous and comprehensive coverage. One excellent and concise chapter deals with the genetics of pulmonary hypertension, and the topic is further covered in other chapters. The section on pulmonary arterial hypertension is capped by a masterly overview of the currently available treatments that have emerged from recent large clinical trials, promising emerging drugs, and a rationale for future therapeutic research. This section is a comprehensive and important one for the expert in pulmonary hypertension as well as the general practitioner.

Part 5 elucidates the diagnosis and treatment of chronic (in addition to acute) thromboembolic disease and pulmonary vascular tumors. Both of these conditions, despite their rarity, are deservedly emphasized because there is a surgical treatment that favorably alters the prognosis. The chapters are written by investigators with some of the broadest expertise in these challenging disorders.

Several practical points make the reading of this book enjoyable: the extensive index and reference material, the stand-alone chapter structure, the explicit pathological slides and photographs, and the inventory of key points summarized at the end of each chapter. Up-to-date and practical guidelines are provided for each topic.

This book is at the vanguard of its type in the field of the pulmonary circulation, and it achieves the editors' ambitious goal of providing a balance between scientific review and clinically relevant, comprehensive guidelines for the busy practicing physician. One deficiency is the absence of a historical perspective on pulmonary circulation, the study of which dates back to Ibn Nafis (1210–1288), William Harvey (1578–1657), and more recently, David Dresdale, Peter Harris, and Paul Hamilton Wood, among many others. The late John (“Jack”) Reeves (who died unexpectedly this year after a bicycle accident, just after participating in a conference on pulmonary circulation) would have been aptly suited to this task. Short of this, in the foreword to this second edition of Pulmonary Circulation, Reeves gives us an insightful perspective on the breadth of the book and its raison d'être.

Paul M. Hassoun, M.D.
Johns Hopkins Hospital, Baltimore, MD 21224
phassoun@jhmi.edu