Wednesday, July 24, 2019

Chronic thromboembolic pulmonary hypertension (CTEPH)

Hello everyone,

We will be talking about CTEPH today.


Diagnostic criteria for CTEPH:
- Mean pulmonary artery pressure of 25 mm Hg or higher by right heart catheterization in the absence of left heart pressure overload
- Imaging evidence of chronic thromboembolism

Treatment: Lifelong anticoagulant therapy to help prevent further thromboembolism.

Definitive therapy: Pulmonary thromboendarterectomy.

Drug therapy: Riociguat may be used in those who are not candidates for surgery or have persistent PH following surgery.

Mechanism of action of Riociguat:
In healthy subjects, endothelial cell-derived nitric oxide (NO) induces vasodilation in vascular smooth muscle cells through the activation of sGC and the subsequent increase in production of cyclic guanosine monophosphate (cGMP). The production of endothelial cell-derived NO is reduced in patients with PAH, so targeting the NO/sGC/cGMP pathway is a logical therapeutic approach. Riociguat does so via a dual mechanism of action: It sensitizes sGC to endogenous NO, and it directly stimulates sGC receptors independent of NO availability, resulting in vasorelaxation and antiproliferative effects.''

-IkaN

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