Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows: While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

Prevalence and Trends of Slow Gait Speed in the United States.

Gait speed is a simple, effective indicator of age-related disease and disability. We sought to examine the prevalence and trends of slow gait speed in older Americans. Our unweighted analytic sample included 12,427 adults aged ≥ 65 years from the 2006-2016 waves of the Health and Retirement Study. Gait speed was measured in participant residences. Persons with gait speed < 0.8 or <0.6 m/s were slow. Sample weights were used to generate nationally representative estimates. The overall estimated prevalence of slow gait speed with the <0.8 m/s cut-point was 48.6% (95% confidence interval (CI): 47.4-49.8) in the 2006-2008 waves yet was 45.7% (CI: 44.3-47.1) in the 2014-2016 waves, but this downward trend was not statistically significant (p = 0.06). The estimated prevalence of slowness with the <0.6 m/s cut-point was 21.3% (CI: 20.4-22.3) for the 2006-2008 waves, 18.5% (CI: 17.5-19.4) for the 2010-2012 waves, and 19.2% (CI: 18.2-20.2) for the 2014-2016 waves, but there were again no significant trends (p = 0.61). Our findings showed that the estimated prevalence of slow gait speed in older Americans is pronounced, and different cut-points largely inform how slowness is categorized. Continued surveillance of slowness over time will help guide screening for disablement and identify sub-populations at greatest risk for targeted interventions.

Prevalence and Trends of Weakness Among Middle-Aged and Older Adults in the United States.

ABSTRACT: McGrath, R, FitzSimmons, S, Andrew, S, Black, K, Bradley, A, Christensen, BK, Collins, K, Klawitter, L, Kieser, J, Langford, M, Orr, M, and Hackney, KJ. Prevalence and trends of weakness among middle-aged and older adults in the United States. J Strength Cond Res 37(12): 2484-2490, 2023-Muscle weakness, which is often determined with low handgrip strength (HGS), is associated with several adverse health conditions; however, the prevalence and trends of weakness in the United States is not well-understood. We sought to estimate the prevalence and trends of weakness in Americans aged at least 50 years. The total unweighted analytic sample included 22,895 Americans from the 2006-2016 waves of the Health and Retirement Study. Handgrip strength was measured with a handgrip dynamometer. Men with weakness were below at least one of the absolute or normalized (body mass, body mass index) cut points: <35.5 kg, <0.45 kg/kg, <1.05 kg/kg/m 2 . The presence of any weakness in women was also identified as being below one of the absolute or normalized HGS cut points: <20.0 kg, <0.34 kg/kg, or <0.79 kg/kg/m 2 . There was an increasing trend in the prevalence of any weakness over time ( p < 0.001). The prevalence of weakness was 45.1% (95% confidence interval [CI]: 44.0-46.0) in the 2006-2008 waves and 52.6% (CI: 51.5-53.7) in the 2014-2016 waves. Weakness prevalence was higher for older (≥65 years) Americans (64.2%; CI: 62.8-65.5) compared with middle-aged (50-64 years) Americans (42.2%; CI: 40.6-43.8) in the 2014-2016 waves. Moreover, the prevalence of weakness in the 2014-2016 waves was generally higher in women (54.5%; CI: 53.1-55.9) than in men (50.4%; CI: 48.7-52.0). Differences existed in weakness prevalence across races and ethnicities. The findings from our investigation suggest that the prevalence of weakness is overall pronounced and increasing in Americans. Efforts for mitigating and better operationalizing weakness will elevate in importance as our older American population grows.

Cardiopulmonary resuscitation.

Performing cardiopulmonary resuscitation is a key competency for healthcare professionals. Training in immediate and advanced life support is a requirement for UK doctors; this is depicted in the Foundation training programme competencies and in the Internal Medicine Training curriculum. It requires being able to identify unwell patients, perform a structured assessment and treatment approach, master relevant procedural aspects and demonstrate non-technical skills including leading the resuscitation team. The Resuscitation Council UK has recently provided updated guidance on basic and advanced life support. These guidelines align with similar international guidelines, taking into account evidence from clinical trials of cardiac arrest management and national data on cardiac arrest outcomes in the community and in the hospital. The guidance includes considerations regarding individuals with suspected or confirmed coronavirus disease (COVID-19). The complex ethical aspects around escalation of care, advance care planning, 'Do Not Attempt Cardiopulmonary Resuscitation' decisions and communication with patients and their loved ones are also discussed. This chapter summarizes the current guidance on cardiopulmonary resuscitation.

Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom.

Background The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease with single ventricle physiology. "Fontan failure" is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult patients with Fontan physiology receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the United Kingdom. Methods and Results We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist congenital heart disease centers in England and Scotland between 2009 and 2019. Patients taking PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 patients with Fontan followed in specialist centers, only 76 (4.9%) received PAH therapies during follow-up. The vast majority (90.8%) were treated with a phosphodiesterase-5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, have a history of protein-losing enteropathy, or receive loop diuretics (P<0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median of 12 months (11-15 months), functional class was more likely to improve in the treated group (P=0.01), with no other changes in clinical parameters or safety issues. Conclusions PAH therapies are used in adult patients with Fontan circulation followed in specialist centers, targeting individuals with advanced disease or complications. Follow-up suggests stabilization of the clinical status after 12 months of therapy.

Acute-onset abdominal pain in a woman in her 30s.

CLINICAL INTRODUCTION: A woman in her 30s presented to the emergency department with sudden-onset abdominal pain with hypotension and tachycardia. She gave a history of congenital heart disease for which she had previously undergone multiple operations. On examination she demonstrated right upper quadrant tenderness. She underwent an urgent multiphase CT (figure 1A-C).heartjnl;105/4/275/F1F1F1Figure 1(A) Arterial phase coronal CT. (B) Arterial phase axial CT. (C) Portal venous phase axial CT. QUESTION: What is the underlying liver pathology?Hepatocellular adenomaCholangiocarcinomaHepatocellular carcinomaFocal nodular hyperplasiaHepatoblastoma.

Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week.

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.

Arrhythmia burden and related outcomes in Eisenmenger syndrome.

BACKGROUND: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death. OBJECTIVE: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger's syndrome and to identify risk factors for arrhythmias. METHODS: This retrospective study included patients aged ≥ 18 years of age with Eisenmenger's syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews. RESULTS: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The mean age was 38 ± 9 years (range: 18-63 years) with a majority in NYHA functional class II or III (57% and 32% respectively). Twenty-eight patients (17%) had significant tachyarrhythmia: paroxysmal supraventricular tachycardia (8 patients, 29%), atrial fibrillation (6 patients, 21%), atrial fibrillation and flutter (2 patients, 7%), nonsustained ventricular tachycardia (6 patients, 21%) and sustained ventricular tachycardia (6 patients, 21%). Among the entire study group, 26 patients (16%) were currently on antiarrhythmic therapy and 77 patients (49%) were on advanced therapies for pulmonary hypertension. Down syndrome was present in 78 patients (46%). There were 21 (13%) documented deaths, of which 8 (5%) were sudden death. Patients with arrhythmia were older [P = .01] and were more likely to have atrioventricular valvar regurgitation [Odds ratio: 4.33]. Advanced pulmonary hypertension therapy was associated with decreased all-cause mortality in logistic regression analysis [odds ratio: 0.31], while antiarrhythmic therapy was associated with sudden death [odds ratio: 6.24]. CONCLUSIONS: Arrhythmias are common among patients with Eisenmenger syndrome occurring in around 1 in 5 individuals and are associated with all-cause mortality and sudden death.

Exercise-Induced Systemic Venous Hypertension in the Fontan Circulation.

Increasingly end-organ injury is being demonstrated late after institution of the Fontan circulation, particularly liver fibrosis and cirrhosis. The exact mechanisms for these late phenomena remain largely elusive. Hypothesizing that exercise induces precipitous systemic venous hypertension and insufficient cardiac output for the exercise demand, that is, a possible mechanism for end-organ injury, we sought to demonstrate the dynamic exercise responses in systemic venous perfusion (SVP) and concurrent end-organ perfusion. Ten stable Fontan patients and 9 control subjects underwent incremental cycle ergometry-based cardiopulmonary exercise testing. SVP was monitored in the right upper limb, and regional tissue oxygen saturation was monitored in the brain and kidney using near-infrared spectroscopy. SVP rose profoundly in concert with workload in the Fontan group, described by the regression equation 15.97 + 0.073 watts per mm Hg. In contrast, SVP did not change in healthy controls. Regional renal (p <0.01) and cerebral tissue saturations (p <0.001) were significantly lower and decrease more rapidly in Fontan patients. We conclude that in a stable group of adult patients with Fontan circulation, high-intensity exercise was associated with systemic venous hypertension and reduced systemic oxygen delivery. This physiological substrate has the potential to contribute to end-organ injury.

Therapeutic pulmonary artery stenting for metastatic bronchial carcinoid.

We present a case of a middle-aged man with a 3-month history of progressive shortness of breath and peripheral oedema. Ten years prior to this, he had undergone a left pneumonectomy for metastatic bronchial carcinoid. Clinical examination revealed significant right heart failure, supported by transthoracic echocardiography. CT pulmonary angiogram revealed the cause to be marked progression of the bronchial carcinoid causing severe external compression of right pulmonary artery (RPA). In view of the distressing symptoms, a palliative endovascular intervention to the RPA was attempted to relieve obstruction, improve blood flow through the right lung and offload the right ventricle. This was performed under general anaesthesia involving interventional cardiology and radiology specialists together with a specialist anaesthetic team with extensive experience of managing carcinoid patients. The result was a marked improvement in symptoms and right heart function and the patient was discharged 2 days later.