[PDF] Vascular and right ventricular remodelling in chronic thromboembolic pulmonary hypertension | Semantic Scholar (2024)

An overview of RV involvement in PAH and CTEPH is provided, with a focus on methods utilized to assess and characterize RV function, as well as the mechanisms underlying maladaptive changes that have been elucidated to date.

This review systematically summarizes the current knowledge on adaptive and maladaptive remodeling of the right heart in CTEPH from molecular mechanisms to clinical practice.

Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease.

The severity of PH is related to PAC in precapillary PH and the different degree of RV adaptation in the subgroups is shown by RVSWI as an invasive parameter.

The issues surrounding CTEPH are reviewed, including its etiology and pathogenesis, epidemiology, diagnosis, treatment options, and associated prognosis, as well as challenges in assessing surgical candidacy and predicting postoperative outcome.

In this review, the current knowledge on the processes underlying development and progression of CTEPH are discussed, and on how animal models can help enlarge understanding of these processes are discussed.

The surgery is curative in a large proportion of patients with resolution of the pulmonary hypertension and leads to major clinical improvement due to improved haemodynamic parameters and oxygenation, and a reduction in dead space ventilation.

Microvascular disease is partly due to post-capillary remodelling in human and experimental CTEPH and appears to be related to bronchial-to-pulmonary venous shunting, and further studies are needed to clinically assess the functional importance of this finding.

A multiscale, image-to-hemodynamics pipeline for BPA therapy planning for inoperable CTEPH patients is provided, providing an integrative, holistic tool for identifying optimal target lesions for treatment.

CTEPH patients showed more evident RV dyssynchrony than PAH patients, and age-adjusted multiple logistic analysis showed that RV-6SD and PAC were predictors of CTEPH rather thanPAH.

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A simple measurement that is easily integrated into routine echocardiography, for great clinical relevance and a lot of physiological sense is introduced, which would allow for the identification of peripheral small vessel disease that is not amenable to surgery.

The results show that differences in RV afterload among groups with different forms of PH can be quantified with a windkessel model and suggest that the RC time and the elastic properties of the large pulmonary arteries remain unchanged in PH.

This overview identifies the gaps in the understanding of RV failure and attempts to fill them, when possible, and aims to encourage the pulmonary hypertension research community to direct some of their attention to the RV, in parallel to their focus on the pulmonary vasculature.

Rup variability in patients with CTEPH and PAH is suggestive of pathophysiological heterogeneity and Rup is significantly increased in operable proximalCTEPH compared with non-operable distal CT EPH and IPAH/CTD-PAH.

It is concluded that postobstructive vasculopathy substantially raises pulmonary vascular resistance, mainly upstream from the site of entry of the bronchial circulation.

During therapy for PH, R and C remain inversely related and changes in both R andC better explain changes in cardiac index than either of them alone.

The magnitude of the VA/Q abnormality correlated poorly with the PVR, the mean Ppa, or the magnitude of vascular obstruction, and the low cardiac output and resulting low P-VO2 were responsible for approximately 33% of the increased AaPO2.

There exists no animal model for CTEPH; therefore, experimentation in the future must include human tissues and clinical data in parallel, and based on available data one may speculate that PE may be followed by a pulmonary vascular remodelling process modified by infection, immune phenomena, inflammation and thyroid hormone replacement or malignancy.

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