Pulmonary hypertension and right heart dysfunction in chronic lung disease

Biomed Res Int. 2014:2014:739674. doi: 10.1155/2014/739674. Epub 2014 Jul 24.

Abstract

Group 3 pulmonary hypertension (PH) is a common complication of chronic lung disease (CLD), including chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor prognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and only a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right ventricle (RV) range between early RV remodeling, hypertrophy, dilatation, and eventual failure with associated increased mortality. The golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical examination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management of the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as phosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients with CLD and moderate-to-severe PH.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Echocardiography, Doppler
  • Endothelin Receptor Antagonists / therapeutic use
  • Heart Failure / drug therapy*
  • Heart Failure / etiology
  • Heart Failure / physiopathology
  • Heart Ventricles / pathology
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology
  • Lung Diseases, Interstitial / complications
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / pathology*
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Prostaglandins / therapeutic use
  • Pulmonary Disease, Chronic Obstructive / complications
  • Pulmonary Disease, Chronic Obstructive / drug therapy
  • Pulmonary Disease, Chronic Obstructive / physiopathology*
  • Sleep Apnea Syndromes / complications
  • Sleep Apnea Syndromes / drug therapy
  • Sleep Apnea Syndromes / pathology

Substances

  • Endothelin Receptor Antagonists
  • Phosphodiesterase 5 Inhibitors
  • Prostaglandins