Inflammation in chronic thromboembolic pulmonary hypertension: accomplice or bystander in altered angiogenesis?

Abstract

@ERSpublicationsEvidence for the role of inflammation in the pathogenesis of PAH: a new study demonstrates inflammation in CTEPH http://ow.ly/OxixF Chronic thromboembolic pulmonary hypertension (CTEPH) is not an uncommon cause of pre-capillary pulmonary hypertension (PH). It is thought to be a long-term complication of symptomatic or asymptomatic pulmonary embolism (PE) or venous thromboembolism (VTE) [1][2][3]. It now appears clear from a retrospective analysis of large European registries [4] and an international prospective registry [5] that CTEPH, as its name indicates, is somehow associated with one or multiple thromboembolic events. However, the reason why a minority of patients (∼0.1-9%) [6,7] who suffer an initial VTE or PE eventually develop CTEPH while the vast majority manage to resolve their clot and resume normal life remains an enigma. Some clues into the aetiology of the disease are provided by the identification of predisposing factors found in epidemiological studies [4], such as infection (osteomyelitis) and malignancy, indwelling catheters or ventriculo-atrial shunts, splenectomy, chronic inflammatory conditions (e.g. inflammatory bowel disease), and genetic predisposition involving the coagulation system [4,6].

Chronic Thromboembolic Pulmonary Hypertension: An Update

“…However, the process of resolution of the thrombus after acute pulmonary embolism is ineffective in patients with CTEPH and leads to a process of reorganization of the thromboembolic material in the vessel wall [ 31 ]. This process is characterized by a thickening of the inner layer by the formation of fibrotic tissue, with deposition of collagen and elastin fibers by alpha-SMA positive cells, potentially deriving from the differentiation of smooth muscle fiber cells that have migrated from the tunica media [ 47 , 48 , 49 , 50 , 51 ].…”

Oligonucleotide, Therapy, and Applications

“…In this use case, the drugs would increase the expression of proteins repressed by the miRNA binding partners. The lncRNAs and proteins targeted might be proteins with anti-inflammatory effects [91], antiproliferative effects, or antimigratory effects. Another interesting strategy that remains untested is to disrupt lncRNA function with small molecules that bind to RNA secondary structure or disrupt the tertiary structure of ribonucleoprotein complexes [92].…”

Evaluation of the hemodynamics and right ventricular function in pulmonary hypertension by echocardiography compared with right-sided heart catheterization

“…PH is diagnosed by a mean pulmonary artery pressure (mPAP) ≥25 mmHg measured during right-sided heart catheterization (RHC) at rest whilst at sea level ( 4 ). PH is a hemodynamic condition caused by a variety of diseases of the lungs and heart, or other systemic diseases ( 5 – 8 ). RHC remains the primary standard for the evaluation of PH, with the ability to exclude left-to-right cardiac shunt and other severe left-sided heart conditions ( 9 , 10 ).…”