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Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed. Of 17 referrals for cardiac transplantation, seven Fontan patients underwent CHLT. All patients who underwent CHLT had either advanced fibrosis or cirrhosis on liver biopsy. There were no perioperative deaths. The most common postoperative morbidity was acute kidney injury. Short-term complications include one episode of acute liver rejection but no cardiac rejection greater than 1R. CHLT is an acceptable therapeutic option for patients with failing Fontan physiology who exhibit concomitant advanced liver fibrosis. However, optimal patient selection is currently undefined, and long-term outcomes are not known.

BackgroundAdults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life.MethodsAdults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review.ResultsOf 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001).ConclusionsAmong adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.

Objective To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death. Methods Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as “no‐show” or “same‐day cancellation.” Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017. Results Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic characteristics such as race, income, and insurance type were associated with non‐attendance (all P values < .05), whereas age, gender, and disease complexity were not. On multivariable analysis, predictors of nonattendance were black race (adjusted odds ratio [AOR] 4.95; P < .001), other race (AOR 3.54; P = .003), and history of no‐show in the past (AOR 4.95; P < .001). Compared to patients who attended clinic, patients with a nonattended visit had a threefold increased odds of multiple ED visits and a significantly lower rate of ED‐free survival over time. There were no significant differences in hospitalizations or death by attendance. Conclusions ACHD clinic nonattendance is associated with race and prior history of no‐show, and may serve as a marker of higher ED utilization for patients with ACHD.

Objective To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insurance, and family planning. Design Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning. Results Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work‐related problems due to CHD and a quarter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on a 0‐10 scale) were 3.5 (employment), 6.0 (insurance), and 8.0 (family planning). Few patients recalled discussions about employment (19%) or health insurance (20%). Over half recalled discussions about family planning, although males were less likely to have had these discussions than females (24% vs 86%, P < .001). Across the three domains, patients identified 16‐18 years as the most appropriate age to initiate discussion, although for patients who recalled discussions, they typically occurred between 20 and 25 years. Conclusions Adults with CHD commonly face employment, health insurance, and family planning challenges. However, discussions about these matters occur with less frequency than recommended and at older ages than patients would prefer. Communication about such issues should be incorporated into a comprehensive educational curriculum for adolescents during the process of transition to adult care.

Quality indicators for adult congenital heart disease (ACHD) were recently published due to a lack of consensus regarding delivery of care to adults with congenital heart disease (CHD). The objective of this study was to examine adherence to quality indicators for the care of patients with tetralogy of Fallot. Adults with tetralogy of Fallot seen in outpatient cardiology clinics at a tertiary care facility between July 2014 and June 2015 were included, and electronic medical records for each visit were reviewed. Completion rates for eight proposed quality indicator metrics were recorded and results for ACHD and non-ACHD cardiologists were compared. A total of 96 eligible patients completed 179 cardiology visits (134 ACHD and 45 non-ACHD). The quality indicator completion rates were over 80% for 7 of the 8 indicators. Metric 5 (cardiac magnetic resonance imaging every five years) had the lowest completion rate at 38.7%. Compared to non-ACHD cardiologists, ACHD cardiologists had higher completion rates for QRS assessment (88.1% vs. 75.6%, p = 0.04), echocardiogram by CHD expert (97.8% vs. 80.0%, p < 0.001), and infective endocarditis counseling (95.9% vs. 77.4%, p = 0.001). In this single center study, there was a wide range of quality indicator completion rates for tetralogy of Fallot. Routine cardiac MRI by an expert in CHD was identified as an area for improvement. There were significant differences in quality indicator completion between ACHD and non-ACHD cardiologists.

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