Pulmonary Hypertension Associated with Left Heart and Lung Diseases

Pulmonary hypertension (PH) associated with left heart diseases (PH-LHD) and PH associated with lung diseases (PH-lung) are common types of PH which typically represent more advanced disease stages of left heart and lung disease, characterised by more severe symptoms and worse prognosis. The tailored work-up should include an assessment of the severity and haemodynamic constellation of PH and the mechanism of the underlying left heart or lung disease and the respective therapeutic targets. Transthoracic echocardiography is the primary noninvasive imaging tool to estimate the likelihood of PH. However, the accuracy of echocardiography is limited in patients with only moderately elevated pulmonary pressures, and acoustic windows in patients with lung disease are often poor. Therefore, right heart catheterisation is always required if exact knowledge of the haemodynamic constellation is required. Echocardiography, cardiac catheterisation, blood gas analysis, lung function testing and ergospirometry as well as additional modalities such as high-resolution computed tomography, overnight sleep studies and cardiac magnetic resonance imaging may be required to characterise the underlying left heart or lung disease and the targets for therapy. The general treatment principle for the management of PH-LHD and PH-lung is to treat the underlying left heart or lung disease in an optimal manner. Specific vasodilator therapies currently do not play a prominent role in PH-LHD and PH-lung but additional trials are underway and may define subgroups deriving benefit.