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Halank, M; Zeder, KE; Sommer, N; Ulrich, S; Held, M; Köhler, T; Foris, V; Heberling, M; Neurohr, C; Ronczka, J; Holt, S; Skowasch, D; Kneidinger, N; Behr, J.
[Pulmonary hypertension associated with lung disease].
Pneumologie. 2023; 77(11): 916-925.
Doi: 10.1055/a-2145-4756
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PubMed
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- Leading authors Med Uni Graz
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Zeder Katarina Eleonora
- Co-authors Med Uni Graz
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Foris Vasile
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- Abstract:
- Lung diseases and hypoventilation syndromes are often associated with pulmonary hypertension (PH). In most cases, PH is not severe. This is defined hemodynamically by a mean pulmonary arterial pressure (PAPm) > 20 mmHg, a pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg and a pulmonary vascular resistance of ≤ 5 Wood units (WU). Both the non-severe (PVR ≤ 5 WU) and much more the severe PH (PVR > 5 WU) have an unfavorable prognosis.If PH is suspected, it is recommended to primarily check whether risk factors for pulmonary arterial hypertension (PAH, group 1 PH) or chronic thromboembolic pulmonary hypertension (CTEPH, group 4 PH) are present. If risk factors are present or there is a suspicion of severe PH in lung patients, it is recommended that the patient should be presented to a PH outpatient clinic promptly.For patients with severe PH associated with lung diseases, personalized, individual therapy is recommended - if possible within the framework of therapy studies. Currently, a therapy attempt with PH specific drugs should only be considered in COPD patients if the associated PH is severe and a "pulmonary vascular" phenotype (severe precapillary PH, but typically only mild to moderate airway obstruction, no or mild hypercapnia and DLCO < 45 % of predicted value) is present. In patients with severe PH associated with interstitial lung disease phosphodiesterase-5-inhibitors may be considered in individual cases. Inhaled treprostinil may be considered also in non-severe PH in this patient population.
- Find related publications in this database (Keywords)
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chronic obstructive pulmonary disease (COPD)
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emphysema
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interstitial lung disease (ILD)
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idiopathic pulmonary fibrosis
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combined pulmonary fibrosis and emphysema (CPFE)
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pulmonary hypertension