Characteristics and Outcomes of Adults With Congenital Heart Disease in the Cardiac Intensive Care Unit.

Background: Little is known regarding the characteristics, treatment patterns, and outcomes in patients with adult congenital heart disease (ACHD) admitted to cardiac intensive care units (CICUs). Objectives: The authors sought to better define the contemporary epidemiology, treatment patterns, and outcomes of ACHD admissions in the CICU. Methods: The Critical Care Cardiology Trials Network is a multicenter network of CICUs in North America. Participating centers contributed prospective data from consecutive admissions during 2-month annual snapshots from 2017 to 2022. We analyzed characteristics and outcomes of admissions with ACHD compared with those without ACHD. Multivariable logistic regression was used to assess mortality in ACHD vs non-ACHD admissions. Results: Of 23,299 CICU admissions across 42 sites, there were 441 (1.9%) ACHD admissions. Shunt lesions were most common (46.1%), followed by right-sided lesions (29.5%) and complex lesions (28.7%). ACHD admissions were younger (median age 46 vs 67 years) than non-ACHD admissions. ACHD admissions were more commonly for heart failure (21.3% vs 15.7%, P < 0.001), general medical problems (15.6% vs 6.0%, P < 0.001), and atrial arrhythmias (8.6% vs 4.9%, P < 0.001). ACHD admissions had a higher median presenting Sequential Organ Failure Assessment score (5.0 vs 3.0, P < 0.001). Total hospital stay was longer for ACHD admissions (8.2 vs 5.9 days, P < 0.01), though in-hospital mortality was not different (12.7% vs 13.6%; age- and sex-adjusted OR: 1.19 [95% CI: 0.89-1.59], P = 0.239). Conclusions: This study illustrates the unique aspects of the ACHD CICU admission. Further investigation into the best approach to manage specific ACHD-related CICU admissions, such as cardiogenic shock and acute respiratory failure, is warranted.

Medical Management of Isolated Partial Anomalous Pulmonary Venous Connection With Symptomatic Right Ventricular Failure and Pulmonary Hypertension.

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease in which one or more pulmonary veins drain into the systemic venous circulation. The abnormal connection between the pulmonary vein and the right atrium can result in a right-sided volume overload due to a left-to-right shunt, followed by eventual right-sided pressure overload and right ventricular failure. PAPVC is usually associated with an atrial septal defect but can present as an isolated finding. We present a case of isolated PAPVC resulting in right heart failure and predominantly pre-capillary pulmonary hypertension. We discuss the challenges in the diagnosis and medical management of isolated PAPVC and highlight the clinical and hemodynamic indications for pulmonary vasodilators and diuretics.

Right Coronary Artery to Left Ventricular Fistula Complicated by Symptomatic Arrhythmia.

Coronary cameral fistulas (CCFs) are rare and are characterized by an abnormal connection between a coronary artery and any of the four chambers of the heart. Most cases of CCFs are asymptomatic. The most common presentation in symptomatic patients includes chest pain or heart failure; however, arrhythmias are rarely associated. We report the case of a 32-year-old male previously unknown to have any medical illnesses. He presented to the clinic with complaints of frequent palpitations, necessitating recurrent admissions. His electrocardiograms revealed regular wide complex tachycardia with a right bundle branch block pattern, suggestive of fascicular ventricular tachycardia. During hospitalization, an elective coronary angiography showed a large CCF originating from the right posterior descending coronary artery and draining into the left ventricle. Moreover, cardiac magnetic resonance imaging did not show any scar or evidence of cardiomyopathies. The patient underwent a successful catheter-based right coronary artery to left ventricular fistula occlusion with coils. In addition, the patient underwent a complex electrophysiological study with three-dimensional mapping and ablation. The presented case underscores the rarity and complexity of such clinical presentations. It also highlights the importance of a multidisciplinary approach in addressing this unique cardiac anomaly.

Challenges and opportunities in patients with adult congenital heart disease, a narrative review.

Adult congenital heart disease Pregnancy Transition of care Challenges heart failure.

Successful thromboaspiration of bilateral pulmonary thromboembolism in a Fontan circulation patient: a case report.

Background: Fontan surgery aims to palliate univentricular congenital heart diseases in which biventricular repair is not feasible. A large spectrum of early and late complications has been described in literature. However, pulmonary thromboembolism represents a rare complication in these patients, leading to a scarcity of evidence regarding diagnosis and treatment strategies. Case summary: We present a case of a 27-year-old woman born with a complex cyanotic congenital heart disease, namely pulmonary and tricuspid stenosis with subaortic interventricular communication and atrial septal defect, who underwent palliation surgery with Blalock-Taussig shunt, bidirectional Glenn, and extracardiac Fontan. She developed acute respiratory failure and was admitted to the hospital, being diagnosed with bilateral thromboembolism. Since she was haemodynamically stable, initially, a conservative approach was chosen. However, due to no clinical improvement, she subsequently underwent bilateral thromboaspiration with restoration of pulmonary circulation. Discussion: Due to the unique Fontan pathophysiology, the possible physiological and clinical implications of pulmonary thromboembolism in this condition are profound. Thus, care and imaging tests in specialized centres are important as the management of these patients is different from those with biventricular physiology.

Cor Triatriatum Sinister in a Young Adult: An Unusual Cause of Syncope.

A 25-year-old male with no prior medical history presented with a one-month history of nausea, weight loss, and dyspnea that progressed to syncope. The initial echocardiogram showed a dilated right ventricle with signs of systolic failure. The patient was admitted for suspected pulmonary embolism, but chest computed tomography (CT) revealed interstitial pneumonia. A transthoracic echocardiogram on day 6 of admission diagnosed cor triatriatum sinister (CTS), severe pulmonary hypertension, chronic cor pulmonale, and reduced right ventricular function. The patient was managed conservatively in the intensive care unit (ICU) without the need for mechanical ventilation and discharged after clinical improvement. This case highlights the importance of the early diagnosis of rare congenital heart defects such as cor triatriatum sinister, which can present with nonspecific symptoms and rapidly progress to right heart failure.

Current approach to genetic testing and genetic evaluation referrals for adults with congenital heart disease.

Background: Congenital heart disease (CHD) is the most common congenital anomaly. Up to 33% have an identifiable genetic etiology. Improved medical and surgical management of CHD has translated into longer life expectancy and a rapidly growing population of adults living with CHD. The adult CHD (ACHD) population did not have access during childhood to the genetic technologies available today and therefore have not had a robust genetic evaluation that is currently recommended for infants with CHD. Given this potential benefit; the aims of this study were to determine how ACHD cardiologists offer genetics services to patients and identify the indications that influence decision-making for genetics care. Methods: We performed a descriptive cross-sectional study of ACHD cardiologists. A study-developed questionnaire was distributed via emailed REDCap link. The recruitment email was sent to 104 potential respondents. The survey was open from 06/2022 to 01/2023. Results: Thirty-five cardiologists participated in the study (response rate of 34%). Most cardiologists identified as white (77%) and male (66%). Cardiologists were more likely to refer patients to genetics (91%) than to order testing themselves (57%). Of the testing ordered, chromosomal testing (55%) was ordered more than gene sequencing (14%). Most cardiologists would refer a patient with a conotruncal lesion (interrupted aortic arch) over other indications for a genetics evaluation. There were more reported barriers to ordering genetic testing (66%) compared to referring to genetics for a genetics evaluation (23%). Cardiologists were more confident recognizing features suggestive of a genetic syndrome than ordering the correct test (p = 0.001). Regarding associations between clinical factors and current practices, more years in practice trended towards less referrals and testing. Evaluating a greater number of patients (p = 0.11) and greater confidence recognizing syndromic features (p = 0.12) and ordering the correct test (p = 0.09) were all associated with ordering more testing. Conclusion: Testing for microdeletion syndromes is being offered and completed in the ACHD population, however testing for single-gene disorders associated with CHD is being under-utilized. Developing guidelines for genetic testing in adults with CHD could increase access to genetic services, impact medical management, reduce uncertainty regarding prognosis, and inform recurrence risk estimates.

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

The Value of the Electrocardiogram in Adult Congenital Heart Disease.

The electrocardiogram is the first test that is undertaken when evaluating a patient's heart. Diagnosing congenital heart disease in an adult (ACHD) can be facilitated by knowing the classical electrocardiographic (EKG) findings. These EKG findings often result from the congenital defect that prevents a part of the cardiac conduction system from occupying its normal anatomic position. When these classical EKG findings are not present, the clinician should consider alternate diagnoses. As the patient with congenital heart disease ages, with native anatomy or after surgical or device repair, the EKG can be used to assess the patient's status and to decide if and when treatment requires adjustment. This is because the electrocardiogram (EKG) can diagnose the hypertrophy or enlargement in a cardiac chamber that results from the congenital defect or anomaly and can diagnose an arrhythmia that might compromise an otherwise stable anatomy. While ACHD often involves intracardiac shunting, in many cases the abnormality only involves cardiac electrical conduction block or ventricular repolarization. These life-threatening diseases can be diagnosed with an EKG. This review will demonstrate and explain how the EKG can be used to diagnose and follow adults with congenital heart disease. When coupled with history and physical examination, the value of the EKG in ACHD will be apparent. A diagnosis can then be made or a differential diagnosis proposed, before an imaging study is ordered.

Chest Radiography Pearls in Select Adult Congenital Heart Disease.

Congenital heart disease in adult patients (ACHD) includes individuals with native anatomic deformities and those who have benefited from corrective, ameliorative, or interventional heart and vascular interventions. Congenital heart disease is the most common birth defect, although with interventions most survive into adulthood. Newborns and children with complex congenital heart diseases that feature cyanosis fail to thrive, and once this is identified, heart failure can promptly undergo diagnostic evaluations and treatment. However, patients with simple congenital heart disease and subtle clinical signs and symptoms may escape diagnosis until adulthood or experience changes in their cardiac hemodynamics and physiology in settings such as pregnancy or newly diagnosed arrhythmias. The chest X-ray (CXR) is the most common X-ray among all radiological procedures. Individual features or a constellation of features on a CXR are often present in patients who have congenital heart disease. The ability to recognize these CXR features is a valuable skill for making the diagnosis of ACHD and for following these patients as they age, and can complement echocardiographic findings. When used well to diagnose ACHD, the CXR will be the sharpest arrow in the quiver.

PATHFINDER-CHD: prospective registry on adults with congenital heart disease, abnormal ventricular function, and/or heart failure as a foundation for establishing rehabilitative, prehabilitative, preventive, and health-promoting measures: rationale, aims, design and methods.

BACKGROUND: Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. AIMS: The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). METHODS: This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. DESIGN: The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. PROCESSES: Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. ASSESSMENTS: Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. DISCUSSION OF THE DESIGN: The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. CONCLUSION: The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Breastfeeding in primiparous women with congenital heart disease - a register study.

BACKGROUND: The number of pregnant women with congenital heart disease (CHD) is rising, and the disease poses increased risks of cardiovascular and obstetric complications during pregnancy, potentially impacting breastfeeding success. This study aimed to investigate breastfeeding in primiparous women with CHD compared to primiparous women without CHD, and to examine potential hindering factors for breastfeeding in women with CHD. METHODS: The data were gathered between 2014 and 2019 and obtained by merging the Swedish Congenital Heart Disease Register (SWEDCON) with the Swedish Pregnancy Register. Primiparous women ≥ 18 years of age with CHD (n = 578) were matched by age and municipality to 3049 women without CHD, giving birth after 22 gestational weeks. Multivariable logistic regression analysis was used to identify factors associated with non-breastfeeding in women with CHD. RESULTS: Fewer women with CHD breastfed than women without CHD two days (94% vs. 97%, p = 0.001) and four weeks after birth (84% vs. 89%, p = 0.006). When all women were analysed, having CHD was associated with non-breastfeeding at both two days and four weeks after birth. For women with CHD, body mass index (BMI) ≥ 30 (OR 3.1; 95% CI 1.4, 7.3), preterm birth (OR 6.4; 95% CI 2.1, 19.0), self-reported history of psychiatric illness (OR 2.4; 95% CI 1.2, 5.1), small for gestational age (OR 4.2; 95% CI 1.4, 12.2), and New York Heart Association Stages of Heart Failure class II - III (OR 6.0; 95% CI 1.4, 26.7) were associated with non-breastfeeding two days after birth. Four weeks after birth, factors associated with non-breastfeeding were BMI ≥ 30 (OR 4.3; 95% CI 2.1, 9.0), self-reported history of psychiatric illness (OR 2.2; 95% CI 1.2, 4.2), and preterm birth (OR 8.9; 95% CI 2.8, 27.9). CONCLUSIONS: The study shows that most women with CHD breastfeed, however, at a slightly lower proportion compared to women without CHD. In addition, factors related to the heart disease were not associated with non-breastfeeding four weeks after birth. Since preterm birth, BMI ≥ 30, and psychiatric illness are associated with non-breastfeeding, healthcare professionals should provide greater support to women with CHD having these conditions.

Adults with Congenital Heart Disease and Transplant: Challenges, Opportunities, and Policy.

The rate of heart transplantation in adults with congenital heart disease (ACHD) is rising, but the 1-year mortality posttransplantation remains higher than non-ACHD patients. A robust pretransplant assessment and operative and postoperative planning can mitigate much of the perioperative risk. Importantly, ACHD patients who survive the first year have significantly better 10-year survival compared with non-ACHD patients. The current allocation system gives ACHD patients a relatively high priority, but providers must use the prespecified exception requests for higher status, especially for patients with the Fontan circulation. It is vital that ACHD patients with end-stage heart failure are cared for at centers with ACHD subspecialty care.

Unveiling a Unique Case of Scimitar Syndrome: Clinical Significance and Multidisciplinary Management Challenges in Pakistan.

Scimitar syndrome, a rare congenital cardiac anomaly, involves abnormal pulmonary vein drainage into systemic veins, leading to distinct imaging features resembling a curved-blade sword. This case report presents a unique instance of scimitar syndrome in Pakistan, emphasizing its clinical importance and the challenges of management. A 26-year-old female with a history of recurrent pulmonary infections and respiratory symptoms since childhood was diagnosed with scimitar syndrome. Radiological assessments, including chest X-rays, computed tomography pulmonary angiograms (CTPA), and transthoracic echocardiography, confirmed the presence of a curved vessel originating from the right hemidiaphragm and connecting with the inferior vena cava (IVC). The patient and her medical team opted for conservative management, involving multidisciplinary care, tailored treatment for infections, and regular monitoring. The rarity of Scimitar syndrome necessitates careful diagnosis and management decisions. While surgical intervention is often recommended, this case demonstrates the complexities of choosing conservative management based on patient preferences and the evolving clinical course. A literature review reveals varied outcomes of surgical and conservative approaches, emphasizing the need for personalized strategies. Radiological techniques, such as CTPA and MRI, play pivotal roles in diagnosis and monitoring. This case report underscores the clinical significance of scimitar syndrome, particularly in regions with limited reported cases, like Pakistan. The multidisciplinary management approach, the decision-making process regarding conservative treatment, and the unique radiological findings contribute to the medical community's understanding of this rare condition.

The burden of psychological trauma and post-traumatic stress disorder among adults with congenital heart disease: PTSD in ACHD.

Psychological trauma, symptoms of post-traumatic stress disorder (PTSD), and mental health conditions are common in adult congenital heart disease (ACHD). There is a gap in research examining PTSD in ACHD using the current Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) criteria in assessing patient characteristics and experiences with trauma-focused treatment. Surveys were offered to outpatients over a 6-month enrollment period to be completed by way of a QR code on their personal smart phone. Patient-reported items include a detailed medical and psychosocial history, the Oslo social support scale, adverse childhood experiences survey, and the PTSD checklist for DSM-5. Of 158 patients (77% moderate or complex heart disease) who provided complete data, a provisional diagnosis of PTSD was found in 48 patients (30%) using a PTSD checklist for DSM-5 cut-off score of ≥31. A positive PTSD screen was associated with younger age, nonwhite race, presence of heart failure, lower New York Heart Association functional class, lower linear quality of life score, lower Oslo social support scale score, an insecure caregiver relation, period of unemployment, emergency department visits, medication nonadherence, and coexisting mental health disorders. Complexity of heart disease and number of surgical and/or catheter-based interventions were not associated with PTSD, although having undergone no cardiac surgeries until adulthood (aged ≥18 years) was associated with a lower prevalence of PTSD. Those who screened positive for PTSD were more likely to report multiple traumatic events, including noncardiac traumatic events. Only 14 of 48 patients (29%) reported a known diagnosis of PTSD, although 44 patients (92%) reported having ever seen a mental health provider. A total of 18 patients (38%) reported currently having a mental health provider. A total of 30 patients (62%) had heard of at least 1 evidence-based trauma-informed therapy, and 14 (29%) had tried at least 1. In conclusion, using the DSM-5 criteria, we observed a high prevalence of potential PTSD in ACHD associated with several novel cardiac and psychosocial patient factors. Future longitudinal studies will be necessary to establish causality. Few patients with ACHD have been formally diagnosed with PTSD or have experience with evidence-based trauma-informed therapies.

Management of Unrepaired Tetralogy of Fallot in an 86-Year-Old Patient Undergoing Transcatheter Aortic Valve Replacement.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect, typically requiring early treatment in infancy. Untreated TOF is associated with poor survival, with most uncorrected patients not surviving beyond the third decade. Here, we present a unique case of an 86-year-old female with uncorrected TOF who underwent a transcatheter aortic valve replacement (TAVR) procedure due to severe aortic stenosis (AS). The patient's TOF was identified during infancy, and she was categorized as an acyanotic "pink baby." Notably, the first palliative surgery for TOF was performed in 1944, when the patient was seven years old. The patient never underwent corrective surgery for TOF and continued to lead a symptom-free life until she developed severe AS later in life. The TAVR procedure significantly improved her symptoms, illustrating the importance of considering alternative etiologies for symptoms in elderly patients with uncorrected TOF and AS. In addition, we underscore the anesthetic management during TAVR, specifically highlighting the challenges addressed, such as the utilization of general anesthesia with transesophageal echocardiography (TEE) and the placement of a pulmonary artery (PA) catheter.

The Unrepaired Tetralogy of Fallot: A Tale of Delayed Presentation and Limited Access to Care.

Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease characterized by four distinct anatomical features. While surgical repair has significantly improved long-term outcomes, some individuals, particularly those from low socioeconomic backgrounds who lack access to medical care, may suffer from complications such as pulmonary hypertension (pHTN) and heart failure. We present a case report of a young female with unrepaired TOF who presented with acute-on-chronic hypoxic respiratory failure and heart failure, highlighting the complex nature and challenges associated with this condition.

Role of ECG in the Accidental Finding of an Atrioventricular Septal Defect in an Asymptomatic Patient Undergoing Cosmetic Surgery.

Electrocardiogram (ECG) is an important diagnostic tool in identifying congenital heart disease (CHD), as demonstrated by this case of a 48-year-old female who presented for a preoperative evaluation for cosmetic surgery. ECG showed a right bundle branch block (RBBB) and first-degree atrioventricular (AV) block, and further testing revealed a primum atrial septal defect (ASD) with mitral valve anterior leaflet cleft and a membranous ventricular septal defect (VSD). She underwent successful surgical repair and was discharged home without complications. This case highlights the importance of performing additional tests like echocardiography or other imaging modalities in cases of abnormal ECG findings to accurately diagnose the underlying heart condition and ensure proper treatment.

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.