TY - JOUR
T1 - Secondary pulmonary arterial hypertension
T2 - to treat or not to treat?
AU - Tomic, Rade
AU - Hertz, Marshall I.
PY - 2018/6/1
Y1 - 2018/6/1
N2 - Purpose of review The purpose of this review is to review recent literature related to mechanisms and treatment options for 'secondary' (i.e., WHO Groups 3 and 5) pulmonary arterial hypertension (PAH). Recent findings Published randomized controlled trials, in general, do not support the use of approved therapies for 'primary' (i.e., WHO Group 1) PAH patients in patients with Group 3 PAH because of the small numbers of patients and inconsistent benefit. Therefore, we currently recommend against the use of these medications for Group 3 PAH. Similarly, there is limited evidence supporting the use of Group 1 PAH medications in Group 5 patients. In most patients with Group 5 PAH, treatment should be directed to the underlying disease. Summary The utility of PAH-specific therapy in WHO Group 3 PAH is unclear because of the small numbers of patients evaluated and inconsistent beneficial effects observed. There is limited evidence supporting the use of PAH medications in Group 5 patients, and they may be harmful in some cases.
AB - Purpose of review The purpose of this review is to review recent literature related to mechanisms and treatment options for 'secondary' (i.e., WHO Groups 3 and 5) pulmonary arterial hypertension (PAH). Recent findings Published randomized controlled trials, in general, do not support the use of approved therapies for 'primary' (i.e., WHO Group 1) PAH patients in patients with Group 3 PAH because of the small numbers of patients and inconsistent benefit. Therefore, we currently recommend against the use of these medications for Group 3 PAH. Similarly, there is limited evidence supporting the use of Group 1 PAH medications in Group 5 patients. In most patients with Group 5 PAH, treatment should be directed to the underlying disease. Summary The utility of PAH-specific therapy in WHO Group 3 PAH is unclear because of the small numbers of patients evaluated and inconsistent beneficial effects observed. There is limited evidence supporting the use of PAH medications in Group 5 patients, and they may be harmful in some cases.
KW - chronic obstructive pulmonary disease
KW - diffuse parenchymal lung disease
KW - idiopathic pulmonary fibrosis
KW - pulmonary arterial hypertension
KW - sarcoidosis
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U2 - 10.1097/MOT.0000000000000529
DO - 10.1097/MOT.0000000000000529
M3 - Review article
C2 - 29629993
AN - SCOPUS:85048110754
VL - 23
SP - 324
EP - 329
JO - Current Opinion in Organ Transplantation
JF - Current Opinion in Organ Transplantation
SN - 1087-2418
IS - 3
ER -