Clinical Worsening in Pulmonary Arterial Hypertension Is Predicted by Right Ventricular End-Systolic Area (RESA) and RV/LV Area Ratio During Recumbent Exercise Stress Echocardiography

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.

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Dobutamine stress for evaluation of right ventricular reserve in pulmonary arterial hypertension

European Respiratory Journal ◽

10.1183/09031936.00089914 ◽

2014 ◽

Vol 45 (3) ◽

pp. 700-708 ◽

Cited By ~ 45

Author(s):

Tripura Sharma ◽

Edmund M.T. Lau ◽

Preeti Choudhary ◽

Paul J. Torzillo ◽

Phillip A. Munoz ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Ventricular ◽

Stress Echocardiography ◽

Dobutamine Stress Echocardiography ◽

Systolic Function ◽

Cardiopulmonary Exercise Testing ◽

Dobutamine Stress ◽

Contractile Reserve ◽

Pulmonary Arterial

Right ventricular contractile response to pharmacological stress in pulmonary arterial hypertension (PAH) has not been characterised. We evaluated right ventricular contractile reserve in adults with PAH using dobutamine stress echocardiography.16 PAH patients and 18 age-matched controls underwent low-dose dobutamine stress echocardiography. Contractile reserve was assessed by the change (Δ; peak stress minus rest value) in tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (S′). A subgroup of 13 PAH patients underwent treadmill cardiopulmonary exercise testing for peak oxygen uptake (V′O2peak).At rest, TAPSE and S′ were reduced in the PAH group compared with controls (1.7±0.4 versus 2.4±0.2 cm and 9.7±2.6 versus 12.5±1.2 cm·s−1, respectively; p<0.05). Contractile reserve was markedly attenuated in PAH compared to controls (ΔTAPSE 0.1±0.2 versus 0.6±0.3 cm and ΔS′ 4.6±2.8 versus 11.2±3.6 cm·s−1; p<0.0001). In the sub-group of PAH patients with preserved right ventricular systolic function at rest, contractile reserve remained depressed compared to controls. V′O2peak was significantly correlated with ΔS′ (r=0.87, p=0.0003) and change in stroke volume (r=0.59, p=0.03).Dobutamine stress can reveal sub-clinical reduction in right ventricular contractile reserve in patients with PAH. A correlation with exercise capacity suggests potential clinical value beyond resting measurements.

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Multi‐Beat Right Ventricular‐Arterial Coupling Predicts Clinical Worsening in Pulmonary Arterial Hypertension

Journal of the American Heart Association ◽

10.1161/jaha.119.016031 ◽

2020 ◽

Vol 9 (10) ◽

Author(s):

Steven Hsu ◽

Catherine E. Simpson ◽

Brian A. Houston ◽

Alison Wand ◽

Takahiro Sato ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Ventricular ◽

Pulmonary Arterial ◽

Clinical Worsening

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Right ventricular stroke work correlates with outcomes in pediatric pulmonary arterial hypertension

Pulmonary Circulation ◽

10.1177/2045894018780534 ◽

2018 ◽

Vol 8 (3) ◽

pp. 204589401878053 ◽

Cited By ~ 8

Author(s):

Weiguang Yang ◽

Alison L. Marsden ◽

Michelle T. Ogawa ◽

Charlotte Sakarovitch ◽

Keeley K. Hall ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Ejection Fraction ◽

Arterial Hypertension ◽

Right Ventricular ◽

Optimization Techniques ◽

Stroke Work ◽

Characteristic Analysis ◽

Potential Biomarker ◽

Pulmonary Arterial ◽

Clinical Worsening

Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR). Optimizing treatment strategies and timing for transplant remains challenging. Thus, a quantitative measure to predict disease progression would be greatly beneficial in treatment planning. We devised a novel method to assess right ventricular (RV) stroke work (RVSW) as a potential biomarker of the failing heart that correlates with clinical worsening. Pediatric patients with idiopathic PAH or PAH secondary to congenital heart disease who had serial, temporally matched cardiac catheterization and magnetic resonance imaging (MRI) data were included. RV and PA hemodynamics were numerically determined by using a lumped parameter (circuit analogy) model to create pressure-volume (P-V) loops. The model was tuned using optimization techniques to match MRI and catheterization derived RV volumes and pressures for each time point. RVSW was calculated from the corresponding P-V loop and indexed by ejection fraction and body surface area (RVSWEF) to compare across patients. Seventeen patients (8 boys; median age = 9.4 years; age range = 4.4–16.3 years) were enrolled. Nine were clinically stable; the others had clinical worsening between the time of their initial matched studies and their most recent follow-up (mean time = 3.9 years; range = 1.1–8.0 years). RVSWEF and the ratio of pulmonary to systemic resistance (Rp:Rs) values were found to have more significant associations with clinical worsening within one, two, and five years following the measurements, when compared with PVR index (PVRI). A receiver operating characteristic analysis showed RVSWEF outperforms PVRI, Rp:Rs and ejection fraction for predicting clinical worsening. RVSWEF correlates with clinical worsening in pediatric PAH, shows promising results towards predicting adverse outcomes, and may serve as an indicator of future clinical worsening.

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