Background: Right ventricular (RV) maladaptation and failure determine outcome in pulmonary hypertension. The adaptation of RV function to loading (RV-pulmonary arterial coupling) is defined by a r...

Reserve of Right Ventricular-Arterial Coupling in the Setting of Chronic Overload.

Pulmonary hypertension (PH) is highly heterogeneous and despite treatment advances it remains a life-shortening condition. There have been significant advances in imaging technologies, but despite evidence of their potential clinical utility, practice remains variable, dependent in part on imaging availability and expertise. This statement summarizes current and emerging imaging modalities and their potential role in the diagnosis and assessment of suspected PH. It also includes a review of commonly encountered clinical and radiological scenarios, and imaging and modeling-based biomarkers. An expert panel was formed including clinicians, radiologists, imaging scientists, and computational modelers. Section editors generated a series of summary statements based on a review of the literature and professional experience and, following consensus review, a diagnostic algorithm and 55 statements were agreed. The diagnostic algorithm and summary statements emphasize the key role and added value of imaging in the diagnosis and assessment of PH and highlight areas requiring further research.

/pdf/statement-on-imaging-and-pulmonary-hypertension-from-the-41maw7o2yb.pdf

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

There is growing awareness of the importance of the right atrial (RA)-right ventricular (RV) axis in pulmonary hypertension (PH). Our results uncover alterations in RA phasic function that are rela...

/pdf/right-ventricular-function-correlates-of-right-atrial-strain-29by620jdy.pdf

Right ventricular function correlates of right atrial strain in pulmonary hypertension: a combined cardiac magnetic resonance and conductance catheter study

Background: Congenital diaphragmatic hernia (CDH) is an important cause of mortality and morbidity in the neonatal period. Pulmonary hypertension and pulmonary hypoplasia are key pathological findings. Cardiac function may also be an important determinant of disease severity, prognostic indicator, and therapeutic target in CDH. Objective: The aim of this study was to assess ventricular mechanics and synchrony in infants with CDH and controls using speckle tracking echocardiography (STE). Methods: Retrospective analysis was performed of echocardiograms obtained in the first 48 h of life in 27 infants with CDH and 20 controls. STE-derived longitudinal strain (LS) was measured in the right and left ventricles (RV, LV). Circumferential strain (CS) and radial strain (RS) were additionally measured in the LV. Mechanical dispersion (MD), a measure of synchrony, was assessed by calculation of the standard deviation of time to peak systolic strain in six ventricular segments. Results: RV LS and LV LS, LV CS, and LV RS were significantly reduced in CDH compared to controls. In the LV free wall, LS and RS were significantly reduced in CDH. LV LS correlated significantly with RV LS in CDH cases (r2 = 0.37, p = 0.002), but not controls (r2 = 0.19, p = 0.06). LV LS also correlated with LV MD in CDH (r2 = 0.25, p = 0.01) but not controls (r2 = 0.02, p = 0.54). Conclusions: Global impairment of RV and LV systolic function are present in newborn infants with CDH and are associated with primary left ventricular dysfunction, ventricular interdependence, and MD.

Ventricular Dysfunction, Interdependence, and Mechanical Dispersion in Newborn Infants with Congenital Diaphragmatic Hernia

Background Right ventricular function is impaired in chronic thromboembolic pulmonary hypertension (CTEPH). Tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) have been shown to help assess right ventricular function in pulmonary hypertension. Our goal was to (1) assess TAPSE and RVFAC before and after PTE, and (2) assess correlation of these variables with right heart catheterization data and PVR. Methods We evaluated 67 consecutive patients with CTEPH for pulmonary thromboendarterectomy (PTE). Of these 67 patients, 48 were deemed surgical candidates. Preoperative right heart catheterization was performed within 1.3±1.2 days of the preoperative echocardiogram. All postoperative right heart catheterizations were performed on the first postoperative day. Results TAPSE dropped from 18±6 to 10±3 mm after PTE (P Conclusion In patients with CTEPH, TAPSE paradoxically decreased by 50% early after PTE. TAPSE correlated inversely with PVR prior to PTE, but this correlation was lost completely after PTE. Thus, despite the immediate and marked decrease in afterload postoperatively, TAPSE did not improve; thus, TAPSE cannot be used as an early marker for surgical success.

Tricuspid annular plane systolic excursion in chronic thromboembolic pulmonary hypertension before and after pulmonary thromboendarterectomy.

Pulmonary hypertension (PH) is a progressive illness characterized by elevated pulmonary artery pressure; however, the main cause of mortality in PH patients is right ventricular (RV) failure. Historically, improving the hemodynamics of pulmonary circulation was the focus of treatment; however, it is now evident that cardiac response to a given level of pulmonary hemodynamic overload is variable but plays an important role in the subsequent prognosis. Non-invasive tests of RV function to determine prognosis and response to treatment in patients with PH is essential. Although the right ventricle is the focus of attention, it is clear that cardiac interaction can cause left ventricular dysfunction, thus biventricular assessment is paramount. There is also focus on the atrial chambers in their contribution to cardiac function in PH. Furthermore, there is evidence of regional dysfunction of the two ventricles in PH, so it would be useful to understand both global and regional components of dysfunction. In order to understand global and regional cardiac function in PH, the most obvious non-invasive imaging techniques are echocardiography and cardiac magnetic resonance imaging (CMRI). Both techniques have their advantages and disadvantages. Echocardiography is widely available, relatively inexpensive, provides information regarding RV function, and can be used to estimate RV pressures. CMRI, although expensive and less accessible, is the gold standard of biventricular functional measurements. The advent of 3D echocardiography and techniques including strain analysis and stress echocardiography have improved the usefulness of echocardiography while new CMRI technology allows the measurement of strain and measuring cardiac function during stress including exercise. In this review, we have analyzed the advantages and disadvantages of the two techniques and discuss pre-existing and novel forms of analysis where echocardiography and CMRI can be used to examine atrial, ventricular, and interventricular function in patients with PH at rest and under stress.

https://journals.sagepub.com/doi/pdf/10.1177/2045893217742000

Non-invasive imaging of global and regional cardiac function in pulmonary hypertension.

Several demographic and clinical factors have prognostic significance in idiopathic pulmonary arterial hypertension (IPAH). Studies in China and the USA have suggested an association between low socioeconomic status and reduced survival. The impact of social deprivation on IPAH survival in the UK is not known. 280 patients with IPAH and hereditary PAH (HPAH) attending the Scottish Pulmonary Vascular Unit (Glasgow, UK) were assigned to social deprivation quintiles using the Scottish Index of Multiple Deprivation database. The association between survival and social deprivation quintile was assessed using Cox proportional hazards regression analysis. The distribution of IPAH/HPAH patients was more socially deprived than would be expected based on Scottish citizenry as a whole (Chi-squared 16.16, p=0.003), suggesting referral and access to care is not impeded by socioeconomic status. Univariate analysis demonstrated no significant association between social deprivation and survival (p=0.81), and this association failed to reach significance with inclusion of time, sex and age as covariates in the model (p=0.23). There were no statistically significant correlations between social deprivation and baseline clinical variables of prognostic importance except for age, sex and quality of life. Social deprivation is not a significant referral barrier or prognostic factor for IPAH and HPAH in Scotland.

https://erj.ersjournals.com/content/erj/51/2/1700444.full.pdf

Timothy Crowe

Papers

Non-invasive imaging of global and regional cardiac function in pulmonary hypertension.

Social deprivation and prognosis in Scottish patients with pulmonary arterial hypertension.