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12.5 Pulmonary hypertension associated with left heart disease
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Published:November 2023
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Abstract
Although pulmonary hypertension (PH) is most commonly due to left heart disease (LHD), we have the least information on this condition regarding pathology, pathophysiology, genetics, and treatments. New guidelines are labelling the condition as PH associated with LHD, although PH is in a majority of cases due to LHD (PH-LHD). Apart fromthe difficulty of finding adequate models and identifying molecular mechanisms of disease, the precise nomenclature of PH-LHD remains controversial, with two proposed sub-categories of PH-LHD, isolated post-capillary (iPC-PH) and combined pre- and post-capillary pulmonary hypertension (CPC-PH) that carry different prognoses. With regards to treatments, current recommendations do not differentiate between PH-LHD subtypes, and suggest a primary focus on the underlying left heart disease condition. Additional difficulties in this group of PH arise from: (1) heterogeneity of clinical presentation, (2) dependence of haemodynamics and on hydration status, (3) uncertainties as to whether PH in PH-LHD is to be classified as a comorbidity of LHD or whether LHD is to be classified as the comorbidity of PH, thus resulting in uncertainty whether the heart or the lung are the primary treatment target, and (4) irreversibility of the condition. The field has been helped by a rigorous scientific approach to patients’ characteristics by a ‘molecular’ dissection of associated conditions with phenomapping, modern imaging, randomized controlled clinical treatment trials, in-depth understanding, and arising treatments of a few specific conditions that lead to PH-LHD, such as cardiac amyloidosis or hypertrophic cardiomyopathy.
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