Faculty Bibliography

BACKGROUND:Guidelines recommend lifelong care with adult congenital heart disease (ACHD) specialists for adults with congenital heart disease (CHD). However, such gaps in visits at specialized ACHD centers have not been well-characterized from diverse US settings. METHODS:This retrospective study analyzed data from 12 centers in the national Patient-Centered Clinical Research Network. CHD conditions were classified using International Classification of Disease codes and a hierarchical algorithm. ACHD specialists were identified by investigators and encounter volumes. Data from the 'Pre-COVID' (2015-2019) and 'COVID' (2020-2022) periods were analyzed separately. Main outcome measures were: 1) Gaps in any ACHD specialist visit and recommended testing throughout the study period. 2) Gaps in recommended ACHD follow-up care. RESULTS:During pre-COVID (N=18,934) and COVID (N=22,453) periods, between 55.3%-55.8% were males, 27.2%-31.0% were 40+ years, 18.2%-19.6% had severe CHD, and 52.7-55.0% had CHD physiologic class B-D conditions. Between 47.0%-54.5% had gaps in specialist visit and 13.0%-24.6% had gaps in all the testing. Patients with gaps in specialist visits were 6.33-9.44 times more likely to have gaps in testing. Gaps were more common among patients with moderate (adjusted odds ratio [AOR]: 2.61) and simple (AOR: 2.84) CHD, those aged 40+ (AOR: 1.53) and non-physiologic class conditions (AOR 1.51). In both periods, 64.1%-71.5% of patients had gaps in follow-up care. CONCLUSIONS:Three-quarters of adults with CHD experienced gaps in specialized ACHD care while receiving services at high volume comprehensive tertiary health centers. To address these gaps, interventions such as fostering physician collaboration within tertiary centers might be needed, and targeted to patients with less severe CHD, non-physiologic class conditions, and those aged 40+ years.

BACKGROUND:Although routine outpatient visits with adult congenital heart defect (ACHD) specialists have been recommended, the evidence to support this remains sparse. OBJECTIVES/OBJECTIVE:The purpose of this study was to evaluate the association between outpatient ACHD visits and emergent admissions. METHODS:This observational study used standardized electronic health record data from 13 health centers from January 1, 2015 through December 31, 2019. Mixed logistic regression analyses examined the effects of number of outpatient ACHD specialist visits (1-2 considered routine) during a 6-month period on an emergent admission in the subsequent 6-month period, after adjusting for cohort characteristics, CHD severity, and center. Analysis was stratified by ACHD anatomic types and medical conditions associated with or without physiological CHD class. RESULTS:The 16,142 patients (median age 32 years, 44.9% women, 83.2% White) contributed to 118,079 person-periods, an average of 7.3 periods per person. Between 1.3% and 2.7% of patients had one emergent admission per 6-month period. Patients with zero, 3, 4, or more than 4 prior outpatient visits had 1.34, 1.67, 2.08-, and 2.48 times higher odds of emergent admission, respectively, than those with a prior routine visit (P < 0.05 using the Wald test). The stratified adjusted analysis demonstrated similar J-shaped (nonlinear) relationships by presence of medical conditions but not by anatomic type. CONCLUSIONS:Having routine outpatient ACHD specialist visits over a 6-month period reduces the likelihood of an emergent admission in the subsequent period, findings driven by presence of medical conditions and not anatomic severity. These findings provide supporting evidence for the ACHD guidelines and implementation science research.

BACKGROUND:There are multiple potential etiologies for right-to-left shunting across a large atrial septal defect (ASD). CASE SUMMARY/METHODS:A 34-year-old woman who presented with exertional dyspnea was found to be cyanotic due to right-to-left shunting across a secundum ASD and hypoplastic right ventricle (RV) due to an abnormal interventricular septum. Balloon occlusion of the ASD resulted in a significant drop in systemic cardiac output, so the patient underwent bidirectional Glenn shunt to both unload and partially bypass a hypoplastic RV, allowing for ASD closure. DISCUSSION/CONCLUSIONS:The bidirectional Glenn shunt is primarily used in the staged palliation of single-ventricle physiology in pediatric patients, and its use in adults is rare. In this case, its use allowed for hemodynamic tolerance of ASD closure with a hypoplastic RV. TAKE-HOME MESSAGE/CONCLUSIONS:This case exemplifies the complex hemodynamics that goes into consideration of ASD closure with an abnormal RV.

Medical and surgical advancements of the past 70 years have resulted in a remarkable shift in the natural history of congenital heart disease (CHD) such that survival to adulthood is expected for >90% of children born with congenital heart defects, including those with complex CHD. There are now more adults than children living with CHD, a majority of them are female. As significant strides have been made in the management of adult patients with CHD, there has been an evolving understanding of the important role inherent sex-specific differences play in impacting long-term outcomes for females with CHD including differences in sexual and reproductive health, risk and incidence of acquired cardiovascular disease, and health surveillance. Notably, care for the female CHD patient is a continuum that is not isolated to discrete stages but cumulative of health exposures and experiences over a lifetime. This review aims to provide a brief overview of the current understanding of the unique health needs and considerations for females with CHD over their lifetime for both the pediatric and adult provider to help identify opportunities for care optimization, continue to raise awareness of the necessity of lifelong care and advocate for the critical need of research that prospectively evaluates pregnancy and other health-related exposures on long-term quality of life and survival for females with CHD.

INTRODUCTION/BACKGROUND:Patients with congenital heart disease (CHD) are at increased risk of cancer. In patients with CHD and advanced heart failure, isolated heart transplantation (HT) can be considered. In the overall HT population, immunosuppression after HT increases the risk of post-transplant malignancy (PTM). However, cancer outcomes among adult HT patients with CHD have not been investigated. METHODS:Patients aged ≥ 18 years who received HT between January 1, 2010 and December 31, 2021 were identified using the United Network for Organ Sharing (UNOS) registry. Patients with CHD were compared to those without. T primary outcome was a composite outcome of PTM or death due to malignancy. Multivariable Fine-Gray competing-risk regression was used to estimate the subhazard ratio (SHR) of primary and secondary outcomes. RESULTS:Of the total of 29717 patients with HT were included, 1017 (3.4%) had CHD. Patients with CHD were younger, more likely to be female, and have had prior cardiac surgery. After multivariable competing-risk regression, CHD was associated with a higher risk of the primary outcome (SHR 1.43, 95% CI 1.15-1.80). Among patients who developed PTM, the median time to diagnosis of first PTM (median 36 vs. 46 months, p = 0.027) was shorter in patients with CHD. Among patients with CHD, survival after PTM was significantly lower compared with patients without malignancy (HR 3.32, 95% CI 2.03-5.43). CONCLUSIONS:Among adult patients with HT, CHD was associated with an increased risk of PTM. Further investigation is warranted to identify risk factors and screening strategies for malignancy in this patient population.

This paper presents the case of a 30-year-old man who was diagnosed with an apical-lateral wall left ventricular aneurysm with scarring, prominent left ventricular trabeculations, and mildly diminished systolic function. Working diagnosis was a congenital left ventricular aneurysm in the setting of left ventricular noncompaction, yet with a questionable defect of the pericardium.

Arrhythmias are highly prevalent in adults with congenital heart disease. For the clinician caring for this population, an understanding of pathophysiology, diagnosis, and management of arrhythmia is essential. Herein we review the latest updates in diagnostics and treatment of tachyarrhythmias and bradyarrhythmias, all in the context of congenital anatomy, hemodynamics, and standard invasive palliations for congenital heart disease.

BACKGROUND/UNASSIGNED:Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications. OBJECTIVES/UNASSIGNED:The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients. METHODS/UNASSIGNED:COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression. RESULTS/UNASSIGNED: < 0.0001) were independently associated with increased risk of TE/bleeding complications. CONCLUSIONS/UNASSIGNED:ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants.