International journal of clinical practice. Supplement
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Pulmonary hypertension (PH) may complicate the course of many forms of advanced interstitial lung disease (ILD) and has been shown to portend a worse outcome. The aetiology of PH is likely multifactorial with variable contribution of factors amongst the different diseases. The most common such conditions include idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-related ILD and pulmonary langerhans cell histiocytosis. Whether the course and impact of PH in these conditions can be modified by therapy requires further study.
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Int J Clin Pract Suppl · Jul 2008
ReviewRight heart function and haemodynamics in pulmonary hypertension.
The primary challenge in the care of the patient with advanced pulmonary arterial hypertension (PAH) is right ventricular dysfunction with concomitant right heart failure. Right heart function is closely tied to survival in this disease, and there is a growing interest in the study of this unique structure. ⋯ Several of the currently available treatments for PAH have been shown to have effects on the RV, not just the pulmonary vasculature, and, in future, therapies aimed at optimizing right ventricular function may allow better outcomes in this challenging disease. New directions in right ventricular assessment including measurement of pulmonary vascular impedance and more widespread availability of CMR may allow greater knowledge about this little studied, yet highly important, right side of the heart.