Unique clinical challenges for the teenager with congenital heart disease.

PURPOSE OF REVIEW: On top of existing rites of passage, adolescents with congenital heart disease (CHD) face additional challenges as related to their underlying medical condition. These include compliance with medications and appointments, establishing heart-healthy behaviors, navigating potential at-risk situations, and transition to adult-oriented care. RECENT FINDINGS: Teenagers with CHD should focus on the importance of exercise as this seems to have important long-term benefits and may help improve weight management. Mental health concerns are a major issue for teens with CHD and awareness and appropriate screening are critical. Discussion regarding sexual health, contraception, pregnancy, and CHD risk in offspring occur rarely in clinical practice, and their absence sets up the CHD adolescent for potential complications. Establishing patterns of trust and communication between physicians and teenage CHD patients are critical to enable the appropriate transition of care to adult congenital care, yet gaps in transition remain common. SUMMARY: The outpatient cardiologist has a critical role to help the teenager with CHD navigate difficult terrain. Returning to the roots of careful history taking, motivational interviewing, and open-ended questions may be of great benefit in warding off complications and helping steer the adolescent with CHD towards a life as a successful adult living with CHD.

Considerations for Gender-Affirming Hormonal and Surgical Care Among Transgender and Gender Diverse Adolescents and Adults With Congenital Heart Disease.

BACKGROUND: Transgender and gender diverse (TGD) individuals and long-term survivors with adult congenital heart disease (ACHD) are both growing populations with specialized needs. No studies assess temporal trends or evaluate the care of TGD individuals with ACHD. METHODS AND RESULTS: Meetings between congenital cardiology and gender-affirming care specialists identified unique considerations in TGD individuals with ACHD. A retrospective chart review was then performed to describe patient factors and outpatient trends in those with an ACHD diagnosis undergoing gender-affirming hormonal or surgical care (GAHT/S) at 1 adult and 1 pediatric tertiary care center. Thirty-three TGD individuals with ACHD were identified, 21 with a history of GAHT/S. Fourteen (66%) had moderate or complex ACHD, 8 (38%) identified as transgender male, 9 (43%) transgender female, and 4 (19%) other gender identities. Three had undergone gender-affirming surgery. There were zero occurrences of the composite end point of unplanned hospitalization or thrombotic event over 71.1 person-years of gender-affirming care. Median age at first gender-affirming appointment was 16.8 years [interquartile range 14.8-21.5]. The most common treatment modification was changing estradiol administration from oral to transdermal to reduce thrombotic risk (n=3). An increasing trend was observed from zero TGD patients with ACHD attending a gender diversity appointment in 2012 to 14 patients in 2022. CONCLUSIONS: There is a growing population of TGD patients with ACHD and unique medical and psychosocial needs. Future studies must fully evaluate the reassuring safety profile observed in this small cohort. We share 10 actionable care considerations for providers with a goal of overseeing a safe and fulfilling gender transition across all TGD patients with ACHD.

Health Equity and Policy Considerations for Pediatric and Adult Congenital Heart Disease Care among Minoritized Populations in the United States.

Achieving health equity in populations with congenital heart disease (CHD) requires recognizing existing disparities throughout the lifespan that negatively and disproportionately impact specific groups of individuals. These disparities occur at individual, institutional, or system levels and often result in increased morbidity and mortality for marginalized or racially minoritized populations (population subgroups (e.g., ethnic, racial, social, religious) with differential power compared to those deemed to hold the majority power in the population). Creating actionable strategies and solutions to address these health disparities in patients with CHD requires critically examining multilevel factors and health policies that continue to drive health inequities, including varying social determinants of health (SDOH), systemic inequities, and structural racism. In this comprehensive review article, we focus on health equity solutions and health policy considerations for minoritized and marginalized populations with CHD throughout their lifespan in the United States. We review unique challenges that these populations may face and strategies for mitigating disparities in lifelong CHD care. We assess ways to deliver culturally competent CHD care and to help lower-health-literacy populations navigate CHD care. Finally, we review system-level health policies that impact reimbursement and research funding, as well as institutional policies that impact leadership diversity and representation in the workforce.

Disseminated histoplasmosis mimicking secondary syphilis.

A 34-year-old, HIV-positive man living in Texas presented with a 2-week history of fever, malaise, myalgias, oral ulcers, and papules on his chest, back, face, and extremities, including the palms. Initially secondary syphilis was suspected. However, RPR was negative. Histopathologic examination revealed a lymphocytic infiltrate with numerous intra-histiocytic fungal organisms. GMS and PAS stains were positive, consistent with the diagnosis of histoplasmosis. We report a case of disseminated histoplasmosis clinically mimicking secondary syphilis.

Atrial pacing in Fontan patients: The effect of transvenous lead on clot burden.

BACKGROUND: Transvenous permanent pacemaker (PPM) implantation is an available option for Fontan patients with sinus node dysfunction. However, the thrombogenic potential of leads within the Fontan baffle is unknown. OBJECTIVE: The purpose of this study was to compare the clot burden in Fontan patients with a transvenous atrial PPM to those without a PPM and those with an epicardial PPM. METHODS: This was a retrospective cohort study of all transvenous PPM implantations in Fontan patients followed at our institution (2000-2018). We performed frequency matching on Fontan type and age group. Primary outcome was identification of intracardiac clot, pulmonary embolus, or embolic stroke. RESULTS: Of 1920 Fontan patients, 58 patients (median age 23 years; interquartile range [25th-75th percentiles] 14-33) at the time of transvenous PPM implantation and 174 matched subjects formed our cohort. The type of Fontan performed in case subjects was right atrium-pulmonary artery or right atrium-right ventricle conduit (54%), lateral tunnel (43%), and extracardiac (3%). The cumulative incidence of clot was highest in patients with transvenous PPM, followed by patients with epicardial PPM and no PPM (1.2 vs 0.87 vs 0.67 per 100 person-years of follow-up, respectively). In multivariable analysis, anticoagulation and/or antiplatelet therapy were protective against clot and resulted in reduction of clot risk by 3-fold (incidence rate ratio 0.33; 95% confidence interval 0.21-0.53; P <.001). CONCLUSION: In a large cohort of Fontan patients matched for age and Fontan type, patients with transvenous PPM had a higher but not statistically significant incidence of clot compared to those with no PPM and epicardial PPM. Patients treated with warfarin/aspirin had lower clot risk.

Relation of Right Ventricular Dilation After Pulmonary Valve Replacement to Outcomes in Patients With Repaired Tetralogy of Fallot.

The rationale for timing of pulmonary valve replacement (PVR) in patients with repaired Tetralogy of Fallot (rTOF) has focused on pre-PVR threshold values of indexed right ventricular end-diastolic volume (RVEDVi) that lead to normalization of right ventricular (RV) size after valve implantation. The goal of this study was to determine whether persistent RV dilation after PVR is associated with adverse clinical outcomes. Subjects with rTOF who underwent PVR and had a cardiac magnetic resonance (CMR) exam after valve implantation at a single center from 2001 to 2017 were included. The composite clinical outcome after PVR included: death, aborted sudden cardiac death, sustained ventricular tachycardia (VT), or NYHA class ≥3. In 189 rTOF subjects, the mean age at PVR was 23.5 ± 11.7 years, median follow-up was 6.0 years (IQR 3.4 to 8.7), and the primary outcome occurred in 14 subjects (7%). The 5- and 10-year event-free rates were 97% and 91%, respectively. Post-PVR RVEDVi was not associated with the composite outcome (p = 0.59). Independent predictors of the outcome were older age at PVR (hazard ratios [HR] 1.06; 95% confidence interval [CI] 1.02 to 1.11; p = 0.004), post-PVR lower RV ejection fraction (HR 0.91; 95% CI 0.86 to 0.97; p = 0.002), and post-PVR atrial tachyarrhythmia (HR 7.60, 95% CI 1.65 to 35.05, p = 0.009). Our study shows that post-PVR RV dilation as measured by CMR-derived RVEDVi was not associated with the composite adverse clinical outcome in this cohort. These findings challenge the validity of current guidelines for PVR, which are based on pre-procedural threshold values of RVEDVi aimed at achieving normal post-procedural RV volumes.