Native myocardial T1 and right ventricular size by CMR predict outcome in systemic sclerosis-associated pulmonary hypertension

Daniel S Knight; Ruta Virsinskaite; Nina Karia; Alice R Cole; Rory H Maclean; James T Brown; Rishi K Patel; Yousuf Razvi; Lucia Venneri; Tushar Kotecha; Ana Martinez-Naharro; Peter Kellman; Ann M Scott-Russell; Benjamin E Schreiber; Voon H Ong; Christopher P Denton; Marianna Fontana; J Gerry Coghlan; Vivek Muthurangu

doi:10.1093/rheumatology/keae141

Native myocardial T1 and right ventricular size by CMR predict outcome in systemic sclerosis-associated pulmonary hypertension

Rheumatology (Oxford). 2024 May 17:keae141. doi: 10.1093/rheumatology/keae141. Online ahead of print.

Authors

Daniel S Knight^{1

2

3}, Ruta Virsinskaite^{1

2

3}, Nina Karia^{1

3}, Alice R Cole⁴, Rory H Maclean⁴, James T Brown^{1

2

3}, Rishi K Patel^{2

5}, Yousuf Razvi^{2

5}, Lucia Venneri², Tushar Kotecha^{1

2

3}, Ana Martinez-Naharro², Peter Kellman⁶, Ann M Scott-Russell⁷, Benjamin E Schreiber¹, Voon H Ong⁴, Christopher P Denton⁴, Marianna Fontana^{2

5}, J Gerry Coghlan¹, Vivek Muthurangu³

Affiliations

¹ National Pulmonary Hypertension Service, Royal Free London NHS Foundation Trust, London, UK.
² Department of Cardiac MRI, Royal Free London NHS Foundation Trust, London, UK.
³ Institute of Cardiovascular Science, University College London, London, UK.
⁴ Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School (Royal Free Campus), London, UK.
⁵ Division of Medicine, University College London, London, UK.
⁶ National Heart, Lung, and Blood Institute, National Institute of Health, Bethesda, MD, USA.
⁷ Department of Rheumatology, Queen Alexandra Hospital, Portsmouth, UK.

PMID: 38759116
DOI: 10.1093/rheumatology/keae141

Abstract

Objectives: Measures of right heart size and function are prognostic in systemic sclerosis-associated pulmonary hypertension (SSc-PH), but the importance of myocardial tissue characterisation remains unclear. We aimed to investigate the predictive potential and interaction of cardiovascular magnetic resonance (CMR) myocardial tissue characterisation and right heart size and function in SSc-PH.

Methods: A retrospective, single-centre, observational study of 148 SSc-PH patients confirmed by right heart catheterization who underwent clinically-indicated CMR including native myocardial T1 and T2 mapping from 2016 to 2023 was performed.

Results: Sixty-six (45%) patients died during follow-up (median 3.5 years, range 0.1-7.3). Patients who died were older (65 vs 60 years, p= 0.035) with more dilated (RVEDVi and RVESVi, p< 0.001), hypertrophied (RVMi, p= 0.013) and impaired (RVEF, p< 0.001) right ventricles, more dilated right atria (RAi, p= 0.043) and higher native myocardial T1 (p< 0.001).After adjustment for age, RVESVi (p = 0.0023) and native T1 (p = 0.0024) were independent predictors of all-cause mortality. Both RVESVi and native T1 remained independently predictive after adjusting for age and PH subtype (RVESVi p < 0.001, T1 p = 0.0056). Optimal prognostic thresholds for RVESVi and native T1 were ≤38 mL/m2 and ≤1119 ms, respectively (p < 0.001). Patients with RVESVi ≤ 38 mL/m2 and native T1 ≤ 1119 ms had significantly better outcomes than all other combinations (p < 0.001). Furthermore, patients with RVESVi > 38mL/m2 and native T1 ≤ 1119 ms had significantly better survival than patients with RVESVi > 38mL/m2 and native T1 > 1119ms (p = 0.017).

Conclusion: We identified prognostically relevant CMR metrics and thresholds for patients with SSc-PH. Assessing myocardial tissue characterisation alongside RV function confers added value in SSc-PH and may represent an additional treatment target.

Keywords: CMR; T1 mapping; prognosis; pulmonary hypertension; right ventricle; systemic sclerosis.