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Esophageal dysmotility is identified as a contraindication to lung transplantation at some centers due to increased risks of acute rejection, pulmonary infection, and chronic lung allograft dysfunction. Phosphodiesterase-type 5 inhibitors (PDE5i) are a cornerstone pharmacotherapy for pulmonary arterial hypertension (PAH) and are known to exert off-target effects that may impact lung transplant candidacy, including impaired esophageal contractility and decreased lower esophageal sphincter tone. We report 2 patients with PAH who were initially declined listing for lung transplantation due to iatrogenic esophageal dysmotility induced by PDE5is. Upon discontinuation of PDE5i therapy, these patients experienced significant improvement in esophageal motility within 14 days and met the criteria for transplant listing at their centers. Recognizing and mitigating the off-target effects of PDE5i medications is critical for maximizing access to transplant for patients with PAH.

Background: Patients undergoing evaluation for advanced heart failure therapies (AHT) are at a critical juncture of their illness. Guidelines recommend shared decision-making with patients and caregivers; however, the approach and content of these discussions are unknown. Methods: Patients referred to a specialty palliative care service during AHT evaluation between October 2018 and September 2022 were identified from electronic health care records. Goals of care conversations documented in an advance care planning module were analyzed for participants, content, and timing before the transplant selection committee meeting. Primary outcomes included the prevalence of documented hopes, worries, shared prognostic communication, illness understanding, treatment expectations, and information preferences. Patients were followed for a minimum of 6 months to evaluate secondary processes and clinical outcomes. Results: Ninety-four patients (mean age 52.8 years, 76.6% male) met the criteria. Most patients (97.9%, n = 92) had illness understanding documented. In initial discussions, 76.3% (n = 71) hoped to “live longer” and 67.7% (n = 63) to “be independent”; thematic analysis supported these hopes with 91.0% of patients expecting “better functionality.” Most patients preferred multimodal information and communication. During follow-up, 27.7% (n = 26) had treatment decisions deferred at the initial committee presentation, and 43.6% (n = 41) were reviewed at multiple selection committee meetings. By the end of the study period, 53.2% (n = 50) underwent one or more AHT and 24.5% (n = 23) had died. Conclusions: Patients undergoing the AHT evaluation process face considerable uncertainty. A standardized approach by a specialty palliative care service elicits patients’ illness understanding, hopes, worries, treatment expectations, and information preferences to promote shared decision-making at this critical time.

Background Frailty is common among patients with advanced heart failure (HF), and screening for frailty to guide care is recommended. Although multiple tools are available to screen for frailty, the feasibility of routinely incorporating frailty screening into daily clinical practice among hospitalised advanced HF patients has not been rigorously tested.Methods This was a prospective, single-centre, quality improvement study. Two brief frailty screening tools were incorporated into palliative care consultations for all patients ≥50 years from August 2021 to October 2022. In the first phase, the Clinical Frailty Scale (CFS) was implemented, followed by the Study of Osteoporotic Fracture (SOF) tool or a modified SOF (mSOF) version in the second phase. The primary outcome was feasibility (%) of performing frailty screenings for this high-risk population.Results A total of 212 patients (mean age 69±10 years, 69% male, 79% white, 30% with ischaemic HF) were referred for palliative care consultation during the study period. Overall, frailty screens were completed in 86% (n=183) of patients. CFS and mSOF reached >80% of adoption, while SOF adoption was 54%. Altogether, 52% of the population screened frail by use of CFS and 52% also by mSOF. All clinicians (n=6) participating in the study reported that frailty screening tools were useful and acceptable, and 83% reported plans for continued utilisation in future clinical practice.Conclusions Frailty screening with CFS or mSOF tools was feasible in hospitalised patients with advanced HF. Tools that require physical assessment were more challenging to implement. These data support the feasibility of incorporating questionnaire-based frailty screening in a busy hospital setting.

Background: Biomarkers are increasingly part of assessing and managing heart failure (HF) in adults with congenital heart disease (CHD). Objectives: To understand the response of cardiac biomarkers with therapy for acute decompensated heart failure (ADHF) and the relationship to prognosis after discharge in adults with CHD. Design: A prospective, observational cohort study with serial blood biomarker measurements. Settings: Single-center study in the inpatient setting with outpatient follow-up. Participants: Adults (≥18 years old) with CHD admitted with ADHF between August 1, 2019, and March 1, 2020. Exposure: We measured body mass, Kansas City Cardiomyopathy Questionnaire (KCCQ-12) score, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and high-sensitivity C-reactive protein (hsCRP) at enrollment, discharge, and 1st clinic follow-up visit; soluble suppression of tumorigenicity 2 (sST2) was measured at the first two time points. Measures: Univariate regression assessed the association between changes in weight, biomarkers, and changes in KCCQ-12 scores, between enrollment and discharge (ΔHospitalization) and between discharge and 1st clinical follow-up visit (ΔPost−discharge). Wilcoxon rank-sum tests assessed the association between change in biomarkers, KCCQ-12 scores, and the composite outcome of cardiovascular death or rehospitalization for ADHF. Results: A total of 26 patients were enrolled. The median age was 51.9 years [IQR: 38.8, 61.2], 13 (54.2%) were women, and median hospital stay was 6.5 days [IQR: 4.0, 15.0] with an associated weight loss of 2.8 ​kg [IQR -5.1, −1.7]. All three cardiac biomarkers decreased during hospitalization with diuresis while KCCQ-12 scores improved; a greater decrease in sST2 was associated with an improved KCCQ-12 symptom frequency (SF) subdomain score (p ​= ​0.012), but otherwise, there was no significant relationship between biomarkers and KCCQ-12 change. Change in hsCRP and NT-proBNP after discharge was not associated with the composite outcome (n ​= ​8, vs. n ​= ​16 who did not experience the outcome; Δ Post-discharge hsCRP +5.1 vs. −1.0 ​mg/l, p ​= ​0.061; NT-proBNP +785.0 vs. +130.0 ​pg/ml, p ​= ​0.220). Conclusions: Serial biomarker measurements respond to acute diuresis in adults with CHD hospitalized for ADHF. These results should motivate further research into the use of biomarkers to inform HF therapy in adults with CHD.