ABSTRACT
I. Introduction 499
II. Etiology 500
III. Prognosis and Natural History 501
IV. Patient Assessment and Selection 502
V. Signs 502
VI. Laboratory Studies 503
VII. Echocardiography 504
VIII. Operation: Pulmonary Thromboendarterectomy 508
A. Postoperative Care 509
B. Results 509
C. Medical Therapy 510
IX. Conclusions 510
References 510
I. Introduction
Pulmonary artery hypertension (PAH) is increasingly recognized as a source of
major morbidity and mortality in the population. A revised classification
system for the various causes of PAH has been recently proposed, which high-
lights the heterogeneous nature of this condition (1) The mainstay of therapy
for PAH is medical management, including the use of new potent pulmonary
vasodilator and antiproliferative agents (2). When medical therapy fails, lung
transplantation has been used to good effect, and pulmonary hypertension from
all causes accounts for approximately one-third of all lung transplants performed.
However, this surgical therapy carries a significant operative risk, and its effec-
tiveness is limited in the long-term by the side effects of immunosuppressive
therapy and allograft rejection (3).
