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Chronic Lung Diseases (CLDs) constitute one of the most frequent causes of pulmonary hypertension (PH) and rank second after PH due to left
heart disease. In PH-CLD, destruction of lung parenchyma is associated with decreased functional capacity, diminished quality of life, increased
oxygen requirements, and a heightened risk of death.
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As both pulmonary arterial hypertension (PAH) and PH due to lung disease are categorized as precapillary PH, it is essential to distinguish
between the two conditions, particularly when the lung disease is present with the severe form of PH. If the diagnosis confirms a severe form of
PH-CLD, then individualised care is essential in patients with severe lung disease and evidence of right-sided heart failure, particularly those with
lower-limb oedema. Moreover, echocardiography (ECHO) and N-terminal-pro hormone B-type natriuretic peptide (NT-proBNP) evaluation are of
high importance and must be ordered by the pulmonologist. Right Heart Catheterisation (RHC) is also essential for the determination of the extent
and severity of pulmonary vascular involvement. The use of inhaled vasodilators, along with a referral for right heart catheterisation, should be
considered at an earlier phase of interstitial lung disease. In addition to therapy, cardiopulmonary rehabilitation programmes have been found to
improve six-minute walking tests (6MWT), oxygenation, and functional capacity.
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This article discusses the screening of PH-CLD along with the treatment algorithm and suggests the promising role of vasodilators in PH-CLD.