Pathophysiology of exercise intolerance in pulmonary arterial hypertension.
Respirology
; 23(2): 148-159, 2018 02.
Article
in En
| MEDLINE
| ID: mdl-28845582
ABSTRACT
Pulmonary arterial hypertension (PAH) is a chronic disease with poor prognosis and important exercise limitation despite the proliferation of treatment options in the last decade. Chronically increased right ventricular (RV) afterload results in right heart failure and without treatment, rapid clinical deterioration is common. Exercise intolerance is the cardinal feature of the disease impacting upon quality of life and clinical outcome. The pathophysiological mechanisms that lead to reduced exercise capacity in this population are complex with ventriculoarterial uncoupling likely to be the predominant feature. The relative contributions of additional factors that contribute to exercise limitation beyond ventriculoarterial uncoupling have not been characterized. This review addresses these factors with a focus on recent developments and uncertainties. RV maladaptation and the intricate interplay between the heart, abnormal pulmonary vascular bed and peripheral factors such as dysfunction of the respiratory and peripheral muscles are discussed in detail.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Exercise Tolerance
/
Familial Primary Pulmonary Hypertension
Type of study:
Etiology_studies
/
Prognostic_studies
Aspects:
Patient_preference
Limits:
Humans
Language:
En
Journal:
Respirology
Year:
2018
Document type:
Article