Non‐invasive right ventricular load adaptability indices in patients with scleroderma‐associated pulmonary arterial hypertension

Abstract

Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is associated with worse outcome than idiopathic pulmonary arterial hypertension (IPAH), potentially due to worse right ventricular adaptation to load as suggested by pressure–volume loop analysis. The value of non-invasive load-adaptability metrics has not been fully explored in SSc-PAH. This study sought to assess whether patients with incident SSc-PAH have worse echocardiographic load-adaptability metrics than patients with IPAH. Twenty-two patients with incident SSc-PAH were matched 1:1 with IPAH based on pulmonary vascular resistance. Echocardiographic load-adaptability indices were divided into: surrogates of ventriculo-arterial coupling (e.g. right ventricular area change/end-systolic area), indices reflecting the proportionality of load adaptation (e.g. tricuspid regurgitation velocity-time integral normalized for average right ventricular radius), and simple ratios (e.g. tricuspid annular plane systolic excursion/right ventricular systolic pressure). The prognostic value of these indices for clinical worsening (i.e. death, transplant, or hospitalization for heart failure) at one year was explored. The two groups were comprised of patients of similar age, with similar cardiac index, pulmonary resistance, capacitance and NT-proBNP levels (p > 0.10). There was no difference in baseline right ventricular dimension, function or load-adaptability indices. At one year, eight (36.4%) SSc-PAH patients had experienced clinical worsening (eight hospitalizations and two deaths) versus one hospitalization in the IPAH group. Load adaptation at one year in survivors was not worse in SSc-PAH (p > 0.33). Patients with IPAH responded better to therapy than SSc-PAH in terms of reduction of right ventricular areas at one year (p < 0.05). Right ventricular load-adaptability echocardiographic indices do not appear to capture the increased risk of negative outcomes at one year associated with SSc-PAH.

Validation of the Tricuspid Annular Plane Systolic Excursion/Systolic Pulmonary Artery Pressure Ratio for the Assessment of Right Ventricular-Arterial Coupling in Severe Pulmonary Hypertension

“…13,15 RV area change/ESA was introduced as a surrogate for RV-arterial coupling in a small cohort of patients with scleroderma-associated PAH. 16 In that study, neither RV area change/ESA nor TAPSE/PASP had prognostic relevance. 16 TAPSE/PAAT was introduced as a substitute for TAPSE/PASP in children in whom a sufficient quality of Doppler velocity signal of tricuspid regurgitation could not be obtained, 33 and relied on the argument that PAAT would be a better measure of pulmonary vascular impedance (or afterload) than PASP.…”

“…RV area change/ESA was calculated as (end-diastolic area−ESA)/ESA. 16 Cardiac magnetic resonance imaging of RV volumes was performed with the Avanto 1.5 Tesla scanner system (Siemens Healthineers, Erlangen, Germany; gradient strength and slew rate: SQ-Engine (45mT/m @ 200 T/m/s). 27…”

“…We, therefore, assessed the relationship of echocardiographic TAPSE/PASP with Ees/Ea in severe PH. We also evaluated other suggested surrogates, including the ratios of RV fractional area change (FAC) to mean pulmonary artery pressure (mPAP, invasively measured), [13][14][15] RV area change to RV end-systolic area (ESA), 16 TAPSE to pulmonary artery acceleration time (PAAT), 17 and stroke volume (SV) to ESA (derived by dividing PASP/ESA as a surrogate of Ees 18,19 by PASP/ SV as a surrogate of Ea). 9 Because RV diastolic function may be altered independently of Ees or Ees/Ea and is associated with disease severity and outcome in PAH, 20,21 we also assessed the relationship of the surrogates with end-diastolic elastance (Eed).…”

Heart–lung interactions in COVID-19: prognostic impact and usefulness of bedside echocardiography for monitoring of the right ventricle involvement

“…The LAI RV allows distinguishing between pressure overloading-induced and impaired contractility-induced RV dysfunction, as well as between patients with similar FAC RV , but different RV myocardial inotropy [ 77 ]. In PAH, the LAI RV can reach values of 25 even in patients with relevant RV dysfunction, suggesting that in the vast majority of PAH patients, the cause of RV failure is the excessive pressure overload and not a relevantly impaired RV contractility [ 77 , 85 , 92 ]. This explains why RV failure is more likely and more faster reversible than LV failure of similar severity and also the reverse remodeling with normalization of RV function in the overwhelming majority of patients with pre-capillary PH-induced severe RV dysfunction, after they underwent lung transplantation [ 77 , 85 ].…”

Right heart failure in pulmonary hypertension: Diagnosis and new perspectives on vascular and direct right ventricular treatment

“…In recent years, several indices of RV function involving echocardiographic parameters have been proposed to reflect RV–arterial coupling in patients, including the ratio of RV FAC to mPAP (measured by right heart catheterisation; Prins et al, 2018), the ratio of RV area change to RV end‐systolic area (French et al, 2018), and the ratio of TAPSE to pulmonary artery acceleration time (Levy, El Khuffash, Woo, & Singh, 2018). However, none of these proposed echocardiographic surrogates have been directly compared with pressure–volume loop measures of RV–arterial coupling, contractility, or afterload.…”