Defining quality standards of care in CTD-PAH and management best practices: a Delphi panel consensus

Objective: This study aimed to develop a consensus of expert opinion on the quality standards of care and outline management best practices for pulmonary arterial hypertension (PAH) in SSc and other CTDs. Methods: Twenty physicians and four nurse practitioners (NPs) managing patients with CTD-PAH (including SSc-PAH) from six European countries participated in a modified Delphi panel. Consensus was defined as ≥80% agreement among the physicians. Results: The importance of a multimodal approach to screening and early detection of PAH through combining echocardiography, biomarker tests and symptom evaluation was emphasised. Consensus agreement was also reached on the usefulness and increased access of echocardiography for optimising screening and early detection processes, noting the importance of cardiology expertise for accurate assessment of right-heart variables. Panellists reached consensus agreement on the importance of a multidisciplinary approach to managing SSc- and other CTD-PAHs through collaboration between rheumatologists and pulmonary hypertension (PH) specialists, NPs and patients. Rheumatologists aligned on the usefulness of the DETECT screening algorithm, whereas cardiologists and pulmonologists remained divided, with consensus not being reached, thus highlighting the lack of agreement regarding the relevance of a stepwise approach. Similarly, no consensus was reached on the impact of the new haemodynamic definition of PH in CTD-PAH management, where greater evidence is required. Conclusions: Consensus was reached on key recommendations for optimising CTD-PAH management, including the standardisation of multimodal screening and promotion of closer collaboration between healthcare specialties and patients.