A clinically useful, treatment-based classification of pulmonary hypertension
divides the disease into 5 distinct categories: (1) pulmonary hypertension
associated with disorders of the respiratory system and/or hypoxemia; (2)
pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary
arterial hypertension; and (5) pulmonary hypertension due to disorders directly
affecting the pulmonary vasculature. Pulmonary arterial hypertension includes
individuals with primary pulmonary hypertension, congenital heart disease,
connective tissue disease, and liver disease. These heterogeneous diseases
have similar characteristic pathological changes, including in situ thrombosis,
smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization
is essential to confirm diagnosis, determine prognosis, and assign therapy.
A minority of patients have a favorable response to an acute vasodilator trial
and long-term benefit with calcium channel blocker therapy. Continuous intravenous
epoprostenol improves symptoms and survival in patients with advanced primary
pulmonary hypertension and has potential benefit in other forms of pulmonary
arterial hypertension. Lung transplantation remains an important option for
individuals in whom maximal medical therapy fails. The recent discovery of
the gene for familial primary pulmonary hypertension and the increase in new
drugs undergoing clinical trials are encouraging developments.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 80
Customize your page view by dragging & repositioning the boxes below.
More Listings atJAMACareerCenter.com >
The Rational Clinical Examination: Evidence-Based Clinical Diagnosis
All results at
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.