Enormous gains have been made in the pathophysiologic understanding and therapy of pulmonary hypertension, particularly over the past decade. Pulmonary Hypertension aims to provide a current, comprehensive, and clinically relevant perspective on these gains, withcontributionsfromaccomplishedexperts.Asbackground, AlfredP.Fishman, MD, aleaderinthefieldformorethanfourdecades, offers his unique perspective on developments in the field over the pastcentury, includingthedevelopmentofrightheartcatheterization techniquesbyindividualswithwhomhesubsequentlyworked, thefirst descriptions of primary pulmonary hypertension, the formation of theNIHregistry, andearlierattemptstoclassifythedisease. The subsequent text provides an overview of the current state of the art. Descriptions of the present iteration of the classi- cationsystem(asrefinedbytheWorldHealthOrganization WHO] consensus conference in 2003) and diagnostic approach occupy the nexttwochapters, followedbyinsightsintopathophysiology, genetics, and the role of the right ventricle. These provide the foundation forChapters6 10, whichdiscussspecificconditionsassociatedwith pulmonaryhypertension, includingthosefromWHOGroups1,2,4, and5.Chapter10isauthoredbyexpertsfromthecenter(University of California, San Diego) with the greatest experience in surgical managementofthedisease.

Chapters 11 16 deal with currently available therapies, starting with a general discussion of the therapeutic approach and tra- tional therapies for pulmonary hypertension. The three classes of drugs currently approved in the United States for therapy of pulmonary hypertension prostacyclins, endothelin receptor anta- nists, and phosphodiesterase-5 inhibitors are examined in separate chapters.Resultsofclinicaltrialsthathaveevaluatedthesetherapies are analyzed and critiqued, and newer potential therapies, such as statins, arediscussed.Thelastchapterinthisgroupingaddressesthe issues of transitioning from one therapy to another and combining differenttherapies.Thesearepromisingtherapeuticavenuesthatare vii viii Preface currentlyofgreatinteresttothepulmonaryhypertensioncommunity and are undergoing intense investigation in a number of recently completedandongoingclinicaltrials. Surgical and interventional approaches to the management of pulmonary hypertension, including lung transplantation and atrial septostomy, are considered in the penultimate chapter. The final chaptercastsaforwardglance, asChristopherM.CarlinandAndrew J.

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