Right ventricular (RV) dysfunction and pulmonary hypertension (PH) occurs in approximately one-third of patients with interstitial lung disease (ILD) and is associated with reduced 6-minute walk distance (6MWD), and increased hospitalizations and mortality. Although the impact of RV dysfunction and PH has been well described in several types of ILD, data is scarce on antisynthetase syndrome. Therefore, we sought to examine the presence of RV dysfunction and PH in patients with antisynthetase syndrome and the impact on clinical outcomes. We conducted a retrospective study of patients with antisynthetase syndrome. Seventy-five subjects were identified. Fifty-one (68%) subjects had echocardiographic data. Patients were grouped into those with normal fractional area change (FAC) ≥ 35% and reduced FAC < 35%. Clinical, echocardiographic, and right heart catheterization data were compared between the two groups. Subjects with FAC < 35% had lower diffusion capacity of the lung for carbon monoxide (29% vs. 47%, p = 0.004), fibrotic features on computed tomography of the chest (79% vs. 33%, p = 0.005), larger RV diameter (5.4 vs. 3.9 cm, p < 0.001), higher right atrial pressures (8 vs. 5 mmHg, p = 0.02), and required supplemental oxygen more frequently (100% vs. 44%, p < 0.001) compared to those with FAC ≥ 35%. We found no difference in 6MWD and hospitalizations between the two groups.The presence of RV dysfunction in antisynthetase syndrome may identify patients at risk of poor outcomes.

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Children with congenital craniofacial conditions (CFCs) and their families may stand to benefit from day camps offering tailored psychosocial support and resources. Nonprofit ConnectMed International has held virtual and in-person day camps in San Diego for this community since 2018. We administered surveys before and after a series of day camps to understand the needs of this community and benefits perceived programming offered. Although studies have reported the psychosocial benefits of camps for children with rare diseases, few have examined the specific benefit for children with CFCs and their parents. Herein, we present the findings of our survey study as the first article to report a needs assessments and psychosocial benefits of day camps for children with CFCs and their parents.