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.