High Prevalence of Abnormal Hemoglobin A1c in the Adolescent and Young Adult Fontan Population.

Little is known about diabetes risk in adolescents and young adults with Fontan palliation. We sought to understand the prevalence of abnormal hemoglobin A1c (HbA1c) in the adolescent and young adult population with Fontan palliation. Between 2015 and 2021, 78 Fontan patients > 10 years of age were seen in our single ventricle clinic; 66 underwent screening with HbA1c. 50% of the study cohort (n = 33) had HbA1c ≥ 5.7%; 2% (n = 1) had HbA1c ≥ 6.5%. There was no correlation between BMI and HbA1c, with no difference in the prevalence of overweight or obesity (BMI ≥ 85th percentile) between those with and without abnormal HbA1c (31% versus 27%, p = 0.69). While 20% of the cohort had a family history of diabetes, there was no difference in family history between those with and without abnormal HbA1c (21% versus 19%, p = 0.85). There were no differences in other risk factors and characteristics (race, glomerular filtration rate, liver function, liver elastography, hematocrit, and years from Fontan surgery) between those with and without abnormal HbA1c. Our results highlight the importance of recognizing that abnormal HbA1c is highly prevalent in the Fontan population. Whether abnormal HbA1c in this population correlates with atherosclerotic cardiovascular disease in adulthood is not known. The mechanism for an abnormal HbA1c in the adolescent and young adult Fontan population remains unclear and further studies are needed.

Tetralogy of Fallot and Aortic Dissection: Implications in Management.

We present the case of a 61-year-old man with tetralogy of Fallot postrepair and mechanical aortic valve replacement with an aortic root/ascending/arch aneurysm with chronic type A aortic dissection. He underwent uncomplicated aortic root and total arch replacement. Continued surveillance for aortic aneurysm is necessary in the tetralogy of Fallot population. (Level of Difficulty: Intermediate.).

The challenges of an aging tetralogy of Fallot population.

Introduction: Advancements in surgery and management have resulted in a growing population of aging adults with tetralogy of Fallot (TOF). As a result, there has been a parallel growth in late complications associated with the sequelae from the underlying cardiac anomalies as well as the surgical and other interventional treatments.Areas covered: Here, we review challenges related to an aging population of patients with TOF, particularly late complications, and highlight advances in management and key areas for future research. Pulmonary regurgitation, heart failure, arrhythmias, and aortic complications are some of these late complications. There is also a growing incidence of acquired cardiovascular disease, obesity, and diabetes associated with aging. Management of these late complications and acquired comorbidities continues to evolve as research provides insights into long-term outcomes from medical therapies and surgical interventions.Expert opinion: The management of an aging TOF population will continue to transform with advances in imaging technologies to identify subclinical disease and valve replacement technologies that will prevent and mitigate disease progression. In the coming years, we speculate that there will be more data to support the use of novel heart failure therapies in TOF and consensus guidelines on the management of refractory arrhythmias and aortic complications.

Effect of donor, component, and recipient characteristics on hemoglobin increments following red blood cell transfusion.

Significant research has focused individually on blood donors, product preparation and storage, and optimal transfusion practice. To better understand the interplay between these factors on measures of red blood cell (RBC) transfusion efficacy, we conducted a linked analysis of blood donor and component data with patients who received single-unit RBC transfusions between 2008 and 2016. Hemoglobin levels before and after RBC transfusions and at 24- and 48-hour intervals after transfusion were analyzed. Generalized estimating equation linear regression models were fit to examine hemoglobin increments after RBC transfusion adjusting for donor and recipient demographic characteristics, collection method, additive solution, gamma irradiation, and storage duration. We linked data on 23 194 transfusion recipients who received one or more single-unit RBC transfusions (n = 38 019 units) to donor demographic and component characteristics. Donor and recipient sex, Rh-D status, collection method, gamma irradiation, recipient age and body mass index, and pretransfusion hemoglobin levels were significant predictors of hemoglobin increments in univariate and multivariable analyses (P < .01). For hemoglobin increments 24 hours after transfusion, the coefficient of determination for the generalized estimating equation models was 0.25, with an estimated correlation between actual and predicted values of 0.5. Collectively, blood donor demographic characteristics, collection and processing methods, and recipient characteristics accounted for significant variation in hemoglobin increments related to RBC transfusion. Multivariable modeling allows the prediction of changes in hemoglobin using donor-, component-, and patient-level characteristics. Accounting for these factors will be critical for future analyses of donor and component factors, including genetic polymorphisms, on posttransfusion increments and other patient outcomes.

The Rogue P-wave: Atrial Dissociation Following Bicaval Heart Transplantation.

We report the first case, to our knowledge, of atrial dissociation in a patient who underwent bicaval orthotopic heart transplantation. It was believed that atrioventricular dissociation prompting pacemaker implantation likely represented donor sinus bradycardia/arrest with intact atrioventricular conduction with surgically isolated recipient atrial activity.