Life and Death: A Multicenter Study Evaluating Cardiologists' Approach to Difficult Conversations with Fontan Patients and Families.

Outpatient cardiologists provide longitudinal care for Fontan patients. As these patients age, they face mounting morbidities, necessitating challenging conversations about prognosis and goals of care. We created a novel survey to evaluate cardiologists' attitudes surrounding risk counseling for patients/caregivers. Cardiologists were recruited during concomitant outpatient enrollment of individuals with Fontan operation > age 10. Physician demographic data, expectations of timing in discussing adverse event risk, and perceived barriers were collected. Barriers were analyzed using a thematic approach. 40 cardiologists (9 institutions) responded regarding 155 patients (mean age 21.2 years, SD 7.7). Physicians were mostly male (58%) with mean practice of 21 years post-fellowship (SD 12). Most felt the time was right to have a conversation with patient (55%) and family (62%), and majority thought patient (53%) and family (75%) were ready for such a conversation. Most had previously discussed prognosis with patient (72%) and family (75%). Providers were inclined to discuss risk with caregivers earlier (mean patient age 9 years, SD 11) than patients (mean patient age 17 years, SD 6.4). Nevertheless, 42% of physicians perceived significant barriers and provided 58 narrative comments categorized into 4 major themes: (1) Patient-related (53.4%), including cognitive limitations and mental health; (2) Provider-related (16.4%), including lack of familiarity, preservation of happiness, and discomfort; (3) Family related (12.3%), including protection/denial and psychosocial stressors; (4) Other (26%), including social barriers. Experienced cardiologists are willing to have difficult conversations; nearly half reported largely patient-related barriers. Facilitating these conversations is critical for the adolescent/young adult with Fontan physiology.

Number of CAG repeats and mortality in middle aged and older men.

DESIGN: The androgen receptor (AR) mediates peripheral effects of testosterone. Previous data suggests an association between the number of CAG repeats in exon-1 of the AR gene and AR transcriptional activity. The aim of this analysis was to determine the association between the number of AR CAG repeats and all-cause mortality in men and the influence of testosterone level on the association. PATIENTS AND MEASUREMENTS: Follow-up data to 27 January 2018 were available for men aged 40-79 years recruited across six countries of the European Male Aging Study between 2003 and 2005. Cox proportional hazards modelling was used to determine the association between CAG repeat number/mortality. Results were expressed as hazard ratios (HR)/95% confidence intervals (CI). RESULTS: One thousand nine hundred and seventy-seven men were followed up. Mean baseline age was 60 ± 11.1 years. Mean duration of follow-up was 12.2 years. At follow up 25.1% of men had died. CAG repeat length ranged from 6 to 39, with the highest proportion of CAG repeat number at 21 repeats (16.4%). In a multivariable model, compared to men with 22-23 AR CAG repeats: for men with <22 and >23 AR CAG HR, 95% CI for mortality were, <22 CAG repeats 1.17 (0.93-1.49) and >23 CAG repeats 1.14 (0.88-1.47). In a post-hoc analysis, the association was significant for men in the lowest tertile of baseline testosterone (<14.2 nmol/L) with >23 CAG repeats: in the adjusted model for <22 and >23 CAG repeats, respectively, 1.49 (0.97-2.27) and 1.68 (1.06-2.67) versus 22-23 repeats. CONCLUSIONS: Our European-wide cohort data overall found no association of androgen receptor CAG repeat number and mortality in men. However, post hoc analysis suggested that an association might be present in men with lower baseline testosterone concentrations, which merits further investigation.

The number of androgen receptor CAG repeats and mortality in men.

INTRODUCTION: The androgen receptor (AR) mediates peripheral effects of testosterone. Evidence suggests that the number of CAG repeats in exon-1 of the AR gene negatively correlates with AR transcriptional activity. The aim of this analysis was to determine the association between CAG repeat number and mortality in men. METHODS: Men aged 40-79 years were recruited from primary care for participation in the UK arm of the European Male Aging Study between 2003 and 2005. Cox proportional hazards modelling was used to determine the association between CAG repeat number/mortality. Results were expressed as hazard ratios(HR)/95% confidence intervals (CI). RESULTS: 312 men were followed up. The mean baseline age was 59.5 years. At follow up, 85/312(27%) men had died. CAG repeat length ranged from 14 to 39, with the highest proportion of CAG repeat number at 21 repeats(16.4%). In a multivariable model, using men with CAG repeat numbers of 22-23 as the reference, men with a lower number of CAG repeats(<22) showed a trend for a higher mortality in the follow-up period (HR 1.46 (0.75, 2.81)) as did men with higher number of repeats (>23) (1.37 (0.65, 2.91)). CONCLUSION: Our data suggest that CAG repeat number may partially influence the risk of mortality in men. Further larger studies are required to quantify the effect.

Timing of Delivery in Women with Cardiac Disease.

OBJECTIVE: Guidelines do not exist to determine timing of delivery for women with cardiovascular disease (CVD) in pregnancy. The neonatal benefit of a term delivery as compared with an early term delivery is well described. We sought to examine maternal outcomes in women with CVD who delivered in the early term period (370/7 through 386/7 weeks) compared with those who delivered later. STUDY DESIGN: This is a prospective cohort study examining cardiac and obstetric outcomes in women with CVD delivering between September 2011 and December 2016. The associations between gestational age at delivery and maternal, fetal, and obstetric characteristics were evaluated. RESULTS: Two-hundred twenty-five women with CVD were included, 83 (37%) delivered in the early term period and 142 (63%) delivered at term. While the early term group had significantly higher rates of any hypertension during pregnancy (18.1 vs. 7%, p = 0.01) and intrauterine growth restriction (22.9 vs. 2.8%, p < 0.001), there was no difference in high-risk cardiac or obstetric characteristics. No difference in composite cardiac morbidity was found (4.8 vs. 3.5%, p = 0.24). Women in the early term group were more likely to undergo cesarean delivery than women in the term group (43.4 vs. 24.7%, p = 0.004). CONCLUSION: There is no maternal benefit of an early term delivery in otherwise healthy women with CVD. Given the known fetal consequences of early term delivery, this study offers support to existing literature suggesting term delivery in these women. KEY POINTS: · Question of delivery timing in women with cardiac disease.. · No difference in cardiac morbidity, term versus early term.. · Term delivery in women with asymptomatic cardiac disease..

Physician assistant utilization in U.S. emergency departments; 2010 to 2017.

BACKGROUND: Physician Assistants (PAs) are widely used in United States (US) Emergency Departments (EDs). We sought to characterize ED PA utilization and practice characteristics in US EDs 2010-2017. METHODS: A retrospective, secondary analysis of the 2010 to 2017 Center for Disease Control's National Hospital Ambulatory Medical Care Survey (NHAMCS) was performed. National estimates of ED visits involving PAs alone (PA), PAs with physician involvement (PA+), or physician only (PHYS) were analyzed for patient demographics and hospital characteristics. RESULTS: Between 2010 and 2017, an estimated 1 billion US ED visits occurred. 5.0% (95% confidence interval [CI] ±2.7%) of visits were seen by a PA, and 8.2% (±2.7%) by a PA+; 76.3% (±7.2%) by PHYS. No linear trends by year were identified in PA or PA+ visits. PA acuity was highest for semi-urgent/nonurgent (56.4%, ±10.7%). PA see the minority of ambulance arrival [5.8% (±1.3)] and admit less patients [1.7% (±0.7%)]. Less laboratory [53.3% (±10.2%) vs. 67.0% (±6.2%)] and radiographic [38.8% (±6.6%) vs. 51.6% (±4.6%) studies were performed during PA vs. PHYS visits. PA visits were most common for patients 25-44 years old (yo) (32.9%, ±6.2%) and 15-24 yo (19.2%, ±3.7%). Most PA visits result in a length of stay (LOS) between 1 and 1.9 h (32.9%, ±6.7%) compared to most PHYS visits resulting in a LOS >3 h (40.3%, ±3%). CONCLUSIONS: From 2010 to 2017, no linear trends in US ED PA and PA+ utilization were identified. PHYS continue to see the majority of ED patients.

Intraperitoneal corticosteroids for recurrent ascites in patients with Fontan circulation: Initial clinical experience.

Individuals with single-ventricle congenital heart disease who are palliated to a Fontan circulation are at risk for heart failure and liver disease, with recurrent ascites being a potentially debilitating cause of late morbidity. Although ascites associated with heart failure or liver failure is usually characterized by a high serum-ascites albumin gradient (SAAG), we have observed multiple instances of ascites in Fontan patients with low SAAG, suggesting an inflammatory process. We present three cases in which recalcitrant ascites severely and adversely impacted the quality of life and describe our initial experience with intraperitoneal corticosteroids in this setting.

Mechanisms for heart failure in systemic right ventricle.

There are two predominant scenarios where the right ventricle acts as the systemic ventricle, namely congenitally corrected transposition of the great arteries (CC-TGA) and patients with D-transposition of the great arteries (D-TGA) who have undergone an atrial switch operation. There is evidence that having a right ventricle in the systemic position predisposes to the development of heart failure. In this review, we aim to explore the proposed mechanisms and pathophysiology for heart failure in patients with a systemic right ventricle in these two subsets of patients.

Assessment and management of heart failure in the systemic right ventricle.

Transposition of the great arteries (TGA) is a common congenital cardiac malformation, in which many patients survive into adulthood. From the 1960s through much of the 1980s, the majority of those with D-TGA were repaired with an atrial switch procedure, in which the right ventricle (RV) remains the systemic ventricle. Congenitally corrected TGA patients often remain with a systemic RV. In these patients, heart failure risks result from residual sequelae of childhood repair, which often include tricuspid regurgitation, RV failure, and atrial and ventricular arrhythmias. Imaging techniques, including echocardiography, cardiac magnetic resonance imaging, multi-detector computed tomography, and radionuclide ventriculography focus on evaluation of anatomy and function as both diagnostic and prognostic tools. Biomarkers are used for risk stratification. Structural and surgical interventions are targeted for palliation and prevention of further decompensation in conjunction with pharmacologic, ablative, and device-based therapies for acute and chronic management of heart failure.

Anticoagulant Reversal Strategies in the Emergency Department Setting: Recommendations of a Multidisciplinary Expert Panel.

Bleeding is the most common complication of anticoagulant use. The evaluation and management of the bleeding patient is a core competency of emergency medicine. As the prevalence of patients receiving anticoagulant agents and variety of anticoagulants with different mechanisms of action, pharmacokinetics, indications, and corresponding reversal agents increase, physicians and other clinicians working in the emergency department require a current and nuanced understanding of how best to assess, treat, and reverse anticoagulated patients. In this project, we convened an expert panel to create a consensus decision tree and framework for assessment of the bleeding patient receiving an anticoagulant, as well as use of anticoagulant reversal or coagulation factor replacement, and to address controversies and gaps relevant to this topic. To support decision tree interpretation, the panel also reached agreement on key definitions of life-threatening bleeding, bleeding at a critical site, and emergency surgery or urgent invasive procedure. To reach consensus recommendations, we used a structured literature review and a modified Delphi technique by an expert panel of academic and community physicians with training in emergency medicine, cardiology, hematology, internal medicine/thrombology, pharmacology, toxicology, transfusion medicine and hemostasis, neurology, and surgery, and by other key stakeholder groups.

Physician assistant and nurse practitioner utilization in U.S. emergency departments, 2010 to 2017.

BACKGROUND: Physician Assistants (PAs) and Nurse Practitioners (NPs) are widely utilized in United States (US) Emergency Departments(EDs). We sought to characterize ED PA and NP utilization and practice characteristics in US EDs 2010-2017. METHODS: A retrospective, secondary analysis of the Center for Disease Control's National Hospital Ambulatory Medical Care Survey was performed. National estimates of ED visits involving PAs/NPs alone (PA/NP), PAs/NPs with physician involvement (PA/NP+), or physician only (PHYS) were analyzed for patient demographics and hospital characteristics. RESULTS: Between 2010 and 2017, 1 billion US ED visits occurred. 8.4% (±4.2%) of visits were seen by a PA/NP, and 11.8% (±4.5%) by a PA/NP+; 76.3% (±7.2%) by PHYS. There was an increase in utilization by PA/NP observed in 2016. PA/NP acuity was highest for semi-urgent/nonurgent (53.2%, ±8.6%). PA/NP see the minority of ambulance arrivals [5.4% (±1.2%)] and admit less patients overall [1.6% (±0.7%)]. Less laboratory [53.6% (±10.0%) vs. 67.0% (±6.2%)] and radiographic [38.0% (±7.0%) vs. 51.6% (±4.6%)] studies were performed during PA/NP only vs. PHYS visits. PA/NP visits were most common for patients 25-44 years old (yo) (31.1%, ±5.5%) and 0-15 yo (23.9%, ±4.7%). Most PA/NP visits result in a length of stay (LOS) between 1 and 1.9 h (33.4%, ±5.7%) compared to most PHYS visits resulting in a LOS greater than 3 h (40.3%, ±3%). CONCLUSIONS: From 2010 to 2015, PA/NP utilization remained stable until an increase in 2016. There was a decrease in 2017. Various PA/NP patient characteristics are significant compared to PHYS. PHYS continue to see most ED patients.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

Clinical outcome and prognostic factors for central neurocytoma: twenty year institutional experience.

Central neurocytomas are uncommon intraventricular neoplasms whose optimal management remains controversial due to their rarity. We assessed outcomes for a historical cohort of neurocytoma patients and evaluated effects of tumor atypia, size, resection extent, and adjuvant radiotherapy. Progression-free survival (PFS) was measured by Kaplan-Meier and Cox proportional hazards methods. A total of 28 patients (15 males, 13 females) were treated between 1995 and 2014, with a median age at diagnosis of 26 years (range 5-61). Median follow-up was 62.2 months and 3 patients were lost to follow-up postoperatively. Thirteen patients experienced recurrent/progressive disease and 2-year PFS was 75% (95% CI 53-88%). Two-year PFS was 48% for MIB-1 labeling >4% versus 90% for ≤4% (HR 5.4, CI 2.2-27.8, p = 0.0026). Nine patients (32%) had gross total resections (GTR) and 19 (68%) had subtotal resections (STR). PFS for >80% resection was 83 versus 67% for ≤80% resection (HR 0.67, CI 0.23-2.0, p = 0.47). Three STR patients (16%) received adjuvant radiation which significantly improved overall PFS (p = 0.049). Estimated 5-year PFS was 67% for STR with radiotherapy versus 53% for STR without radiotherapy. Salvage therapy regimens were diverse and resulted in stable disease for 54% of patients and additional progression for 38 %. Two patients with neuropathology-confirmed atypical neurocytomas died at 4.3 and 113.4 months after initial surgery. For central neurocytomas, MIB-1 labeling index >4% is predictive of poorer outcome and our data suggest that adjuvant radiotherapy after STR may improve PFS. Most patients requiring salvage therapy will be stabilized and multiple modalities can be effectively utilized.

Clinical Approaches to the Patient with a Failing Fontan Procedure.

The Fontan operation has been nothing short of revolutionary in its influence on the modern management of patients born with functionally univentricular hearts. The vast majority of these individuals are now surviving well into adulthood. In the 45 years since its introduction, however, there has been increasing recognition of Fontan survivors as a vulnerable population with an altered physiology that has remarkably broad, adverse impact on their long-term health. In this review, the authors discuss the varied manifestations, both cardiac and extracardiac, of the failing Fontan circulation and potential therapeutic options. In addition, a general clinical approach to the patient presenting with Fontan failure is proposed. Ultimately, the key to improving our care and understanding of the Fontan population lies in multi-institutional collaboration and partnership between subspecialty cardiologists and specialists in other organ systems.

Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity.

Impaired exercise capacity is common after the Fontan procedure and is attributed to cardiovascular limits. The Fontan circulation, however, is also distinctively vulnerable to unfavorable lung mechanics. This study aimed to define the prevalence and physiological relevance of pulmonary dysfunction in patients with Fontan physiology. We analyzed data from the Pediatric Heart Network Fontan Cross-Sectional Study to assess the prevalence and pattern of abnormal spirometry in Fontan patients (6-18 yr old) and investigated the relationship between low forced vital capacity (FVC) and maximum exercise variables, including peak O2 consumption (Vo2peak), among those who demonstrated adequate effort (n = 260). Average ages at the time of exercise testing and Fontan completion were 13.2 ± 3.0 and 3.5 ± 2.2 yr old, respectively. Aerobic capacity was reduced (Vo2peak: 67.3 ± 15.6% predicted). FVC averaged 79.0 ± 14.8% predicted, with 45.8% having a FVC less then the lower limit of normal. Only 7.8% demonstrated obstructive spirometry. Patients with low FVC had lower Vo2peak (64.4 ± 15.9% vs. 69.7 ± 14.9% predicted, P < 0.01); low FVC independently predicted lower Vo2peak after adjusting for relevant covariates. Among those with Vo2peak < 80% predicted (n = 204/260), 22.5% demonstrated a pulmonary mechanical contribution to exercise limitation (breathing reserve < 20%). Those with both low FVC and ventilatory inefficiency (minute ventilation/CO2 production > 40) had markedly reduced Vo2peak (61.5 ± 15.3% vs. 72.0 ± 14.9% predicted, P < 0.01) and a higher prevalence of pulmonary mechanical limit compared with patients with normal FVC and efficient ventilation (36.1% vs. 4.8%). In conclusion, abnormal FVC is common in young patients after the Fontan procedure and is independently associated with reduced exercise capacity. A large subset has a pathologically low breathing reserve, consistent with a pulmonary mechanical contribution to exercise limitation.

How Good Are Cardiologists at Predicting Major Adverse Events in Fontan Patients?

Background: It is unknown how well cardiologists predict which Fontan patients are at risk for major adverse events (MAEs). Objectives: The purpose of this study was to examine the accuracy of cardiologists' ability to identify the "good Fontan" patient, free from MAE within the following year, and compare that predicted risk cohort to patients who experienced MAE. Methods: This prospective, multicenter study included patients ≥10 years with lateral tunnel or extracardiac Fontan. The cardiologist was asked the yes/no "surprise" question: would you be surprised if your patient has a MAE in the next year? After 12 months, the cardiologist was surveyed to assess MAE. Agreement between cardiologist predictions of MAE and observed MAE was determined using the simple kappa coefficient. Multivariable generalized linear mixed effects models were performed to identify factors associated with MAE. Results: Overall, 146 patients were enrolled, and 99/146 (68%) patients w`ere predicted to be a "good Fontan." After 12 months, 17 (12%) experienced a MAE. The simple kappa coefficient of cardiologists' prediction was 0.17 (95% CI: 0.02-0.32), suggesting prediction of MAE was 17% better than random chance. In the multivariable cardiologist-predicted MAE (N = 47) model, diuretic/beta-blocker use (P ≤ 0.001) and systolic dysfunction (P = 0.005) were associated with MAE. In the observed multivariable MAE (N = 17) model, prior unplanned cardiac admission (P = 0.006), diuretic/beta-blocker use (P = 0.028), and ≥moderate atrioventricular valve regurgitation (P = 0.049) were associated with MAE. Conclusions: Cardiologists are marginally able to predict which Fontan patients are at risk for MAE over a year. There was overlap between factors associated with a cardiologist's prediction of risk and observed MAE, namely the use of diuretic/beta-blocker.