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Research ArticleSymposium Papers

Diagnosis and Management of Pulmonary Arterial Hypertension: Implications for Respiratory Care

Deborah Jo Levine
Respiratory Care April 2006, 51 (4) 368-381;
Deborah Jo Levine
Division of Cardiothoracic Surgery, University of Texas Health Science Center, San Antonio, Texas.
MD
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Abstract

Pulmonary arterial hypertension (PAH) is a pathological condition of the small pulmonary arteries. PAH is characterized histopathologically by vasoconstriction, vascular proliferation, in situ thrombosis, and remodeling of all 3 levels of the vascular walls. These pathologic changes result in progressive increases in the mean pulmonary-artery pressure and pulmonary vascular resistance, which, if untreated, leads to right-ventricular failure and death. PAH can be associated with multiple conditions or risk factors (eg, collagen vascular diseases, liver disease, human immuno-deficiency virus, congenital heart disease, or ingestion of certain medications or toxins) or it can be idiopathic. Up to 10% of the idiopathic cases are familial. Regardless of the etiology, the clinical presentation, histopathologic lesions, and response to therapy are all similar. Early in the disease process, the signs and symptoms of PAH are often subtle and nonspecific, making diagnosis challenging. Patients most often present with progressively worsening dyspnea and fatigue. An extensive evaluation is indicated to diagnose PAH, decipher its etiology, and determine long-term treatment goals. Transthoracic echocardiogram is an excellent screening tool to evaluate PAH, but every patient requires a right-side heart catheterization to help stage the disease and guide therapy. Prior to a decade ago, clinicians were only able to offer symptomatic therapy to this challenging group of patients. Earlier diagnosis, rapidly advancing understanding of the pathogenesis, and an increasing number of treatment options have changed the course of PAH, which was once thought to be invariably fatal.

  • pulmonary arterial hypertension
  • pulmonary hypertension
  • pulmonary vascular disease
  • right heart failure
  • cor pulmonale

Footnotes

  • Correspondence: Deborah Jo Levine MD, Division of Cardiothoracic Surgery, University of Texas Health Science Center, 7703 Floyd Curl Drive, Mail Code 7841, San Antonio TX 78229-3900. E-mail: levinedj{at}uthscsa.edu.
  • Copyright © 2006 by Daedalus Enterprises Inc.
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Respiratory Care: 51 (4)
Respiratory Care
Vol. 51, Issue 4
1 Apr 2006
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Diagnosis and Management of Pulmonary Arterial Hypertension: Implications for Respiratory Care
Deborah Jo Levine
Respiratory Care Apr 2006, 51 (4) 368-381;

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Diagnosis and Management of Pulmonary Arterial Hypertension: Implications for Respiratory Care
Deborah Jo Levine
Respiratory Care Apr 2006, 51 (4) 368-381;
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Keywords

  • pulmonary arterial hypertension
  • pulmonary hypertension
  • pulmonary vascular disease
  • right heart failure
  • cor pulmonale

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