Pulmonary arterial hypertension: Classification, diagnosis, and prognosis

Terence K. Trow, Naureen Khan

Research output: Contribution to journalReview articlepeer-review

Abstract

Our understanding of the pathobiology of pulmonary arterial hypertension (PAH) has evolved considerably over the past 2 decades, with increasing recognition of the important role that aberrant vasoproliferative responses play in conjunction with disordered vasoconstriction. Classification of the many forms of PAH into categories sharing a similar pathophysiology and clinical presentations help the practicing clinician approach a complex differential diagnosis. Noninvasive tests can be used to narrow this differential but must be applied with an appreciation for their limitations. Transthoracic echocardiography is the screening tool of choice; the workup should also include chest radiography and electrocardiography. However, right heart catheterization is ultimately required to establish the diagnosis. While PAH remains a progressive and generally fatal disease, existing therapies have a significant impact on survival and new therapeutic targets offer great hope for improving the prognosis.

Original languageEnglish (US)
Pages (from-to)487-493
Number of pages7
JournalJournal of Respiratory Diseases
Volume27
Issue number11
StatePublished - Nov 1 2006

Keywords

  • Idiopathic pulmonary arterial hypertension
  • Primary pulmonary hypertension
  • Pulmonary arterial hypertension
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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