Faculty Bibliography

BACKGROUND/UNASSIGNED:Although infective endocarditis during pregnancy is rare, it carries significant morbidity and mortality for both mother and foetus. While professional societies provide recommendations for the treatment of infective endocarditis, there are no specific guidelines for the management of pregnancy-associated infective endocarditis. In this report, we present two cases of infective endocarditis presenting during the second trimester of pregnancy that required surgical intervention, focusing on the unique considerations when caring for pregnant individuals. CASE SUMMARIES/UNASSIGNED:Two patients in the second trimester of pregnancy presented with fevers and malaise. Both were found to have positive blood cultures and mitral valve vegetations, leading to diagnoses of mitral valve endocarditis. Their hospital courses were complicated by embolic strokes, and one patient required transcatheter embolization of a mycotic aneurysm. Both patients underwent surgical valve replacements with bioprosthetic valves. Ultimately, both patients delivered at term without complication. DISCUSSION/UNASSIGNED:In addition to the standard management of infective endocarditis, pregnancy-associated infective endocarditis requires multidisciplinary collaboration regarding the relative timing of cardiac surgery and delivery, the use of intraoperative foetal monitoring, and the choice of valve replacement and anticoagulation. Each of these decisions requires balancing the risk of morbidity and mortality to the patient, the risk of neonatal prematurity and associated complications and disability, and the risk of foetal death during cardiopulmonary bypass. We discuss our teams' decision-making processes with a focus on the relevant considerations for each of these challenging decisions.

OBJECTIVE/UNASSIGNED:The arterial switch operation (ASO) is the standard repair for d-transposition of the greater arteries, although coronary reimplantation remains technically complex and carries the risk of both early and late complications. Coronary ostial plasty for late stenosis has been described using various patch materials, each with its own advantages and limitations. However, the optimal patch material remains unclear. METHODS/UNASSIGNED:We report 2 cases of symptomatic late left main coronary artery (LMCA) stenosis after ASO in young adults with complex coronary artery. Both patients underwent coronary ostial plasty in which a femoral artery homograft patch was used. The patients' preoperative presentation, imaging findings, operative details, and postoperative course are reviewed. RESULTS/UNASSIGNED:Case 1 was a 22-year-old man with a single coronary artery arising from the right anterior sinus and >75% LMCA ostial stenosis. Case 2 was a 25-year-old young man with interarterial LMCA atresia and collateralization from a dominant right coronary artery. In both cases, surgical repair involved extending the aortotomy into the LMCA and performing ostial plasty using a femoral artery homograft patch. Both patients had uneventful recoveries and remain asymptomatic with excellent functional status. CONCLUSIONS/UNASSIGNED:Late coronary artery stenosis is a recognized complication after ASO, particularly in patients with complex coronary anatomy. Although multiple patch materials have been used for ostial plasty, each has drawbacks. Femoral artery homograft may represent a promising alternative in these challenging anatomical settings.

Venous thrombosis in the Fontan circulation can be a devastating complication, and its management has traditionally been surgical. Here, we present two cases of extensive Fontan thrombosis that have both been successfully medically managed, with survival beyond two years.

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.