Background: Scleroderma (SCL) is an important cause of pulmonary arterial hypertension (PAH), but there is high prevalence of left ventricular diastolic dysfunction (LVDD) and post-capillary pulmonary hypertension (PH). We previously demonstrated a high incidence of occult left heart disease (LHD) in patients with SCL and PH using catheterization-derived Left Ventricular End Diastolic Pressure measurements and optional saline challenge. Indexed left atrial volume (LAVi) is a validated measurement supporting a diagnosis of LHD and LVDD. It has previously been studied in PH from LHD, but not other forms of PH or SCL. We hypothesized that LAVi can predict pre- vs. post-capillary PH etiology.
Methods: We retrospectively studied 131 patients from 2010 to 2017, reviewing the index right/left catheterization (RHC/LHC) with saline challenge, and complete transthoracic echocardiogram (TTE) performed within 3 months. TTEs were re-analyzed for LAVi and LHD indices, RV function and Pulmonary Pressures by a blinded echocardiographer. LHD was determined by RHC/LHC measure of PAWP or LVEDP > 15 mm interpreted by a blinded physician.
Results: There were 30 subjects with no PH, 57 with PAH, and 44 with LHD by RHC/LHC. ROC analysis yielded an AUC of 0.578 for LAVi to discriminate between pre-capillary and post-capillary PH. A LAVi >= 33 mL/m2 yielded a sensitivity of 0.29 and specificity of 0.89 to rule-in LHD.
Conclusion: Measurement of LAVi by TTE can assist in detecting LHD in SCL and/or PH. Many centers rely on RHC alone, without saline challenge. In a diuresed patient with PH and a low wedge pressure, LHD may thus be missed. LAVi > 33 mL/m2 should suggest LHD even when hemodynamics suggest otherwise. LAVi may be used as an exclusionary adjunct to RHC/LHC to confirm a PAH diagnosis. LAVi may prove useful in screening patients with PH to avoid catheterization.