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Background: The impact of donor-recipient age difference in adult congenital heart disease (ACHD) patients undergoing heart transplant (HT) is unknown. Methods: ACHD patients (≥18 years old) who underwent HT (2000-2020) were identified using the United Network for Organ Sharing database. Graft survival through 10 years based on donor-recipient age difference was evaluated by comparing outcomes of donors >5 years older than recipients (Older), donors within 5 years of recipient age (Equal Age), and donors >5 years younger than recipients (Younger, reference group). Cox multivariable analysis was performed to evaluate the effect of donor-recipient age difference on early and late graft survival. Results: A total of 1,275 ACHD patients underwent HT (60% male, median 35 years old (interquartile range 24-46) with median graft survival of 13.7 years (95% confidence interval [CI] 11.7-16.0). Compared to Younger donors (n = 306 [24%]), graft survival was similar with Equal Age donors (n = 698 [55%]; log-rank p = 0.61), though significantly reduced with Older donors (n = 271 [21%]; log-rank p = 0.03). In multivariable analysis, late graft survival was similar with Equal Age donors but lower with Older donors (adjusted hazard ratio 1.63, 95% CI 1.16-2.28, p = 0.005), with a trend of reduced survival with Older donors in recipients

Background Data on the use of implanted hemodynamic monitoring (IHM) in patients with Fontan circulation are limited. This study reports our experience using the CardioMEMS HF system in adults with Fontan circulation. Methods and Results This single‐center, retrospective study evaluated heart failure hospitalizations, procedural complications, and device‐related complications in patients with Fontan circulation referred for IHM placement (2015–2022). The association of pulmonary artery pressure (by most recent catheterization and median IHM pressure within 30 days of placement) with both death and follow‐up Model for End‐Stage Liver Disease Excluding International Normalized Ratio score were evaluated. Of 18 patients referred for IHM placement, 17 were successful (median age, 30 [range 21–48] years, 6 women). Procedural complications (access site hematomas, pulmonary artery staining) occurred in 3 patients, without device‐related procedural complications. In follow‐up (median, 35 [range, 6–83] months), 1 patient developed a pulmonary embolism (possibly device‐related). Heart failure hospitalizations/year were similar before and after IHM (median, 1 [interquartile range, 0–1.0] versus 0.6 [0–2.3]; P=0.268), though only 46% of heart failure hospitalizations had associated IHM transmissions. IHM pressures were associated with Model for End‐Stage Liver Disease Excluding International Normalized Ratio scores (R2=0.588, P

Background: Pulmonary hypertension (PH) due to left heart disease (World Health Organization (WHO) Group 2 PH) is the largest PH subgroup, however most reports of PH in pregnancy focus on patients with pulmonary arterial hypertension (WHO Group 1 PH). We evaluated pregnancy outcomes across WHO PH subgroups. Methods: We performed a retrospective single center cohort study of maternal and fetal outcomes in pregnant women with PH (2004–2018). Results: We analyzed outcomes of 70 pregnancies in 70 women with PH (30 ± 6 years-old), classified as WHO Group 1 PH (12 (17%)), Group 2 PH (45 (64%)), Group 3 PH (4 (6%)) and Group 5 PH (9 (13%)). Although no peripartum death occurred, 3 (4.3%) women with WHO Group 2 PH had late mortality (7 ± 4 months post-partum). Additionally, 33 major adverse cardiac events occurred in 26 (37%) women, preterm birth occurred in 32 (49%), and post-partum hemorrhage in 10 (14%), such that only 24 (37%) women completed a viable pregnancy free of an adverse cardiac, obstetric or fetal/neonatal event. Major adverse cardiac events were predominantly due to heart failure (24 (73%)), occurring only in WHO Groups 1 and 2 PH (3 (25%) women vs. 17 (38%), p = 0.07), and significantly associated with pre-eclampsia, left ventricular ejection fraction ≤45%, maternal diabetes, and systemic hypertension. Conclusions: WHO Group 2 PH carries similar risk for maternal cardiovascular events when compared to women with WHO Group 1 PH. Further studies evaluating maternal risk in this cohort are needed.

Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.

As more adults survive with congenital heart disease, the need to better understand the long‐term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality‐based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration. In this document we examine these foundational principles and offer suggestions for developing an ethically responsible and inclusive framework for national ACHD data collection.

Background: Pulmonary arterial hypertension (PAH) is a disease characterized by increased pulmonary vascular resistance (PVR). Pregnancy in the setting of PAH is associated with significant maternal morbidity and mortality, typically present during labor and the early postpartum. Historically, observed maternal mortality has been as high as 56%. However, with improved recognition, understanding, and treatment of PAH, reassessment of maternal outcomes is of increasing interest. Study design: This is a retrospective observational case series of all women with a diagnosis of moderate to severe World Health Organization Group 1 PAH who had pregnancy managed in our tertiary care center between 2004 and 2018. Results: Ten women (Age 28 [SD 6] years, mean pulmonary artery pressure 48 [SD 16.5] mmHg, PVR 7.5 [SD 4.3] Wood units) were identified and included. Seven women (70%) had an antepartum diagnosis of PAH, 2 women (20%) were diagnosed during pregnancy and 1 (10%) postpartum. Four women (40%) delivered preterm, and 3 women (30%) had a successful vaginal delivery. Cardiovascular complications occurred in 4 women (40%) and non-cardiovascular complications occurred in 5 women (50%). There were no significant changes in right ventricular function or non-invasive assessment of PA pressures at long-term follow-up. All patients are alive to date at a mean follow-up of 8 [SD 4] years. Conclusion: In our single center, 10 women with moderate to severe PAH receiving directed medical therapy survived pregnancy and delivery. Further evaluation and review are required to optimize medical management for women with PAH who pursue pregnancy.

Pulmonary arterial hypertension (PAH) and novel coronavirus (SARS-CoV-2) disease COVID-19 are characterized by extensive endothelial dysfunction and inflammation leading to vascular remodeling and severe microthrombi and microvascular obliterative disease. It is hypothesized that those patients with underlying lung disease, like PAH, represent a high-risk cohort in this pandemic. However, reports of COVID-19 in this cohort of patient have been scaring and an observational survey showed that the disease was relatively well tolerated. We postulate that specific PAH vasodilator may offer some protection and/or advantage in the case of concomitant COVID-19. Here we review the literature describing mechanisms of action for each of the broad categories of PAH therapy, and offer potential hypothesis about why this therapy may impact outcomes in COVID-19.

Abstract The corona virus disease ‐2019 (COVID‐19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID‐19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID‐19 include myocarditis, arrhythmia and myocardial infarction. Importantly, the pandemic has stretched health care systems and many care team members are at risk for contracting and possibly transmitting the disease which may further impact the care of patients with cardiovascular disease. In this review, we describe the effects of COVID‐19 in the pediatric and young adult population and review the cardiovascular involvement in COVID‐19 focusing on implications for patients with congenital heart disease in particular.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly that results in a left-to-right shunt. Based on the shunt fraction, PAPVR has a wide spectrum of presentations. If a significant left-to-right shunt is left unrepaired, pulmonary vascular remodeling can occur resulting in the development of pulmonary arterial hypertension (PAH). Furthermore, if the condition is associated with an atrial septal defect (ASD), the patient can develop shunt reversal and Eisenmenger's syndrome in setting of severe PAH. Management plans include close observation, surgical repair, and treatment with pulmonary artery vasodilator therapies. Here, we present multiple cases of PAPVR to highlight the wide spectrum of presentations and the individualized treatment for each case. Keywords: PAPVR, PAH, CHD, ASD

Background: Disease-related stressors for survivors of congenital heart disease (CHD) have been qualitatively described but not quantified nor examined in relationship to important patient-reported outcomes (PROs)., Objective: The aims of this study are to (1) identify the types and degree of disease-related stress experienced by CHD survivors based on age, functional status, and sex, (2) examine differences in stress and PROs by age, functional status, and sex, and (3) determine the unique contribution of perceived stress to variability in PROs., Methods: A cross-sectional study of 173 adolescents and emerging and young adults who were recruited from both pediatric and adult CHD clinics was conducted. Participants rated the degree to which they found various aspects of CHD stressful and completed PROs of health-related quality of life and emotional distress. Differences in perceptions of stress across predictors were determined using analyses of variance and χ analyses. The relative contribution of perceived stress predicting PROs was examined using stepwise linear regression., Results: Two items emerged as being stressful for almost half of the sample, including concerns about future health and having scars or other signs of medical procedures. Adolescents reported less perceived stress than emerging or young adults, and survivors with even mild functional limitations reported higher perceived stress than did those without any symptoms. Perceptions of stress significantly contributed to variability in PROs above and beyond other predictors and was the only variable to explain unique variance in emotional distress., Conclusions: Having even mild functional impairment may have significant deleterious consequences on PROs via increased perceptions of stress. Stress may be modifiable using cognitive behavioral therapy.