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Supplemental material, sj-pdf-1-pul-10.1177_20458940211032529 for Right ventricular function as assessed by cardiac magnetic resonance imaging-derived strain parameters compared to high-fidelity micromanometer catheter measurements by Takahiro Sato, Bharath Ambale-Venkatesh, Stefan L. Zimmerman, Ryan J. Tedford, Steven Hsu, Ela Chamera, Tomoki Fujii, Christopher J. Mullin, Valentina Mercurio, Rubina Khair, Celia P. Corona-Villalobos, Catherine E. Simpson, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Joao A.C. Lima, David A. Kass, Ichizo Tsujino and Paul M. Hassoun in Pulmonary Circulation

Supplemental material, sj-pdf-2-pul-10.1177_20458940211032529 for Right ventricular function as assessed by cardiac magnetic resonance imaging-derived strain parameters compared to high-fidelity micromanometer catheter measurements by Takahiro Sato, Bharath Ambale-Venkatesh, Stefan L. Zimmerman, Ryan J. Tedford, Steven Hsu, Ela Chamera, Tomoki Fujii, Christopher J. Mullin, Valentina Mercurio, Rubina Khair, Celia P. Corona-Villalobos, Catherine E. Simpson, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Joao A.C. Lima, David A. Kass, Ichizo Tsujino and Paul M. Hassoun in Pulmonary Circulation

Supplemental material, sj-pdf-2-pul-10.1177_20458940211032529 for Right ventricular function as assessed by cardiac magnetic resonance imaging-derived strain parameters compared to high-fidelity micromanometer catheter measurements by Takahiro Sato, Bharath Ambale-Venkatesh, Stefan L. Zimmerman, Ryan J. Tedford, Steven Hsu, Ela Chamera, Tomoki Fujii, Christopher J. Mullin, Valentina Mercurio, Rubina Khair, Celia P. Corona-Villalobos, Catherine E. Simpson, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Joao A.C. Lima, David A. Kass, Ichizo Tsujino and Paul M. Hassoun in Pulmonary Circulation

Supplemental Material for Serum uric acid as a marker of disease risk, severity, and survival in systemic sclerosis-related pulmonary arterial hypertension by Catherine E. Simpson, Rachel L. Damico, Laura Hummers, Rubina M. Khair, Todd M. Kolb, Paul M. Hassoun and Stephen C. Mathai in Pulmonary Circulation

Supplemental material, for The impact of ambrisentan and tadalafil upfront combination therapy on cardiac function in scleroderma associated pulmonary arterial hypertension patients: cardiac magnetic resonance feature tracking study by Takahiro Sato, Bharath Ambale-Venkatesh, Joao A.C. Lima, Stefan L. Zimmerman, Ryan J. Tedford, Tomoki Fujii, Olivia L. Hulme, Erica H. Pullins, Celia P. Corona-Villalobos, Roham T. Zamanian, Omar A. Minai, Reda E. Girgis, Kelly Chin, Rubina Khair, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Paul M. Hassoun in Pulmonary Circulation

Supplemental material, for The impact of ambrisentan and tadalafil upfront combination therapy on cardiac function in scleroderma associated pulmonary arterial hypertension patients: cardiac magnetic resonance feature tracking study by Takahiro Sato, Bharath Ambale-Venkatesh, Joao A.C. Lima, Stefan L. Zimmerman, Ryan J. Tedford, Tomoki Fujii, Olivia L. Hulme, Erica H. Pullins, Celia P. Corona-Villalobos, Roham T. Zamanian, Omar A. Minai, Reda E. Girgis, Kelly Chin, Rubina Khair, Rachel L. Damico, Todd M. Kolb, Stephen C. Mathai, Paul M. Hassoun in Pulmonary Circulation

Right ventricular function has prognostic significance in patients with pulmonary hypertension. We evaluated whether cardiac magnetic resonance-derived strain and strain rate parameters could reliably reflect right ventricular systolic and diastolic function in precapillary pulmonary hypertension. End-systolic elastance and the time constant of right ventricular relaxation tau, both derived from invasive high-fidelity micromanometer catheter measurements, were used as gold standards for assessing systolic and diastolic right ventricular function, respectively. Nineteen consecutive precapillary pulmonary hypertension patients underwent cardiac magnetic resonance and right heart catheterization prospectively. Cardiac magnetic resonance data were compared with those of 19 control subjects. In pulmonary hypertension patients, associations between strain- and strain rate-related parameters and invasive hemodynamic parameters were evaluated. Longitudinal peak systolic strain, strain rate, and early diastolic strain rate were lower in PAH patients than in controls; peak atrial-diastolic strain rate was higher in pulmonary hypertension patients. Similarly, circumferential peak systolic strain rate was lower and peak atrial-diastolic strain rate was higher in pulmonary hypertension. In pulmonary hypertension, no correlations existed between cardiac magnetic resonance-derived and hemodynamically derived measures of systolic right ventricular function. Regarding diastolic parameters, tau was significantly correlated with peak longitudinal atrial-diastolic strain rate ( r = −0.61), deceleration time ( r = 0.75), longitudinal systolic to diastolic time ratio ( r = 0.59), early diastolic strain rate ( r = −0.5), circumferential peak atrial-diastolic strain rate ( r = −0.52), and deceleration time ( r = 0.62). Strain analysis of the right ventricular diastolic phase is a reliable non-invasive method for detecting right ventricular diastolic dysfunction in PAH.

Pulmonary hypertension due to heart failure with preserved ejection fraction (PH-HFpEF) has been poorly studied in patients with systemic sclerosis (SSc). We sought to compare clinical characteristics and survival of SSc patients with PH-HFpEF (SSc-PH-HFpEF) versus pulmonary arterial hypertension (SSc-PAH). We hypothesized that patients with SSc-PH-HFpEF have a similar poor overall prognosis compared with patients with SSc-PAH when matched for total right ventricular load. The analysis included 117 patients with SSc-PH (93 with SSc-PAH versus 24 with SSc-PH-HFpEF) enrolled prospectively in the Johns Hopkins PH Registry. We examined baseline demographics and hemodynamics at diagnostic right heart catheterization (RHC), two-dimensional echocardiographic characteristics, six-minute walking distance (6MWD), treatment modalities, and laboratory values (serum NT-proBNP, creatinine, uric acid, and sodium), and assessed survival. Demographics and clinical features were similar between the two groups. Baseline RHC showed significantly higher pulmonary and right heart pressures in the SSc-PH-HFpEF compared with the SSc-PAH group. Trans-pulmonary gradient (TPG), however, was equally elevated without significant difference between the groups. SSc-PH-HFpEF patients had left atrial enlargement on echocardiography compared with SSc-PAH patients. No significant differences were found between groups for 6MWD, NT-proBNP, and other laboratory values. Although overall median survival time was 4.6 years with no difference in mortality rate between the two groups (SSc-PH-HFpEF versus SSc-PAH: 75% versus 59%; P = 0.26), patients with SSc-PH-HFpEF had a twofold increased risk of death compared with SSc-PAH patients after adjusting for hemodynamics. Concomitant intrinsic pulmonary vascular disease and HFpEF likely contribute to very poor survival in patients with SSc-PH-HFpEF.