Phillips, Sabrina D.

Background There is a global mismatch between the number of adults with congenital heart disease and adult congenital heart disease (ACHD) cardiologists, potentially impacting patient outcomes. This survey explores factors influencing trainees and cardiologists' career choices in ACHD. Methods An online, anonymous global survey on demographics, ACHD as a career choice, encouraging and deterring factors assessed via a 5‐point Likert scale and open‐ended questions on recommending ACHD was distributed through targeted emails and social media. Responses meeting a 75% completion criterion were analyzed using descriptive statistics and thematic analysis. Results Among 811 included responses, most were from women (53.5%), aged 31 to 40 years (48.3%), White individuals (43.3%), non‐Hispanic individuals (85%), from outside the ACHD field (79.0%), and from North America (42.7%). Only 30.9% of non‐ACHD physicians considered specializing in ACHD despite 78.9% recommending it. Encouraging factors included awareness of ACHD significance, mentor influence, and clinical exposure. Key deterrents were inadequate financial incentives, limited job opportunities, and lengthy training. Regional variations showed that longer training duration and lack of compensation incentives were the main deterrents in North America. Suggested solutions included structured training programs, salary improvements, and early ACHD exposure during medical education. Conclusions Less than a third of respondents considered ACHD as a career largely due to inadequate financial incentives, limited job opportunities, and long training duration. Addressing these barriers could enhance recruitment and help meet the growing demands of the ACHD population.

INTRODUCTION: There is a significant shortage of specialized Adult Congenital Heart Disease (ACHD) cardiologists despite a rising prevalence of adults with this condition. AIM: Our study explores the specific motivations and deterrents that influence adult and pediatric cardiologists’ ACHD career decisions, which could serve as the basis for future solutions to overcome this shortage in each specific region. METHODS: An anonymous global survey was distributed to adult and pediatric cardiologists and trainees via email, social media, and medical society newsletters. The survey included demographics, ACHD exposure, career considerations, Likert scale ratings, and open-ended inquiries on ACHD career recommendations. Respondents included non-ACHD and ACHD cardiologists (defined as those caring for ACHD with or without formal training). The analysis covered regional variations across continents. Responses with less than 75% completion were excluded. RESULTS: A total of 811 valid responses were collected (53.5% female, 48.3% aged 31-40, and 43.3% White). ACHD cardiologists were 21%, mostly practicing in North America (44.1%) and Europe (32.9%). Almost all ACHD cardiologists (94.7%) recommended it as a career. Among the 641 non-ACHD cardiologists, 85.7% had been exposed to ACHD, however, only 35.1% considered pursuing it themselves. For ACHD cardiologists the top global motivators for pursuing an ACHD career were awareness of the field and its significance, mentor's influence, and sufficient clinical exposure during residency (Figure 1A). Factors that deterred non-ACHD cardiologists included a lack of compensation compared to other cardiology subspecialties, limited job opportunities, and the longer duration of training (Figure 1B). Absence of specialized training and educational programs emerged as a major deterrent in all continents except North America. The lack of mentorship or role models in ACHD was particularly pronounced in Africa and Central and South America. Limited exposure to ACHD during training was a significant issue in Europe and Africa. CONCLUSION: Most ACHD cardiologists recommend a career in ACHD. However, only a few non-ACHD cardiologists recommend it. Influential mentors, clinical exposure motivate; low compensation, lengthy training deter. Regional disparities require targeted interventions for better ACHD cardiologist recruitment.

Introduction: The subcutaneous implantable cardioverter defibrillator (S-ICD) is an attractive option for patients with congenital heart disease (CHD) in whom a transvenous system is contraindicated. However, more than 10% of adult patients with CHD fail S-ICD screening due to a large T- to R-wave ratio. We hypothesized right-sternal electrodes may permit S-ICD placement in patients who fail left-sternal screen. Methods: We prospectively screened 40 patients with CHD for S-ICD using the standard proprietary approach. We also screened with right sternal electrodes (with left can electrode position). Results: The mean age of the cohort was 46+/-15 years; 24 males were enrolled. CHD anomalies consisted of tetralogy of Fallot (n=11), Ebstein’s anomaly (n=7), atrial septal defect (n=6), dextro-looped transposition of the great artery (n=5) and other (n=11). Left side screening ECG failed in 9 patients (23%), five of whom passed with right sternal electrodes, increasing the pass rate from 77% to 90%. Figure shows the screening ECG and chest x-ray of a patient who failed in all three vectors on the left side but passed in the alternative vector on the right side and underwent right sided implantation. Conclusions: The addition of right sternal electrodes during screening increases S-ICD eligibility and may avoid thoracotomy for high risk patients with CHD.

Introduction: Fontan pathway obstruction is a serious complication characterized by an anatomical or functional narrowing anywhere in the cavo-pulmonary pathways. Here we report the first case where an innovative Fontan conduit rehabilitation procedure with intravascular lithotripsy was used achieving a significant increase in the pathway size. Case: A 28-year-old male with tricuspid atresia status-post Fontan palliation (age 23 months) with bare metal stent implant to treat obstruction at age 15, presented with syncope, ascites and varicose veins. Imaging studies revealed a cirrhotic liver morphology and Fontan pathway obstruction with minimal diameter of 12 mm. Cardiac catheterization confirmed severe Fontan conduit obstruction in addition to LPA stenosis. Serial covered Cheatham-Platinum stents were implanted for conduit coverage followed by Palmaz stents. Non-compliant Atlas balloon dilation to 20 atm was performed without expansion of the Fontan pathway beyond 12 mm due to severe calcification. A 7.0 x 60 mm shock-wave lithotripsy balloon was added alongside the Atlas balloon to modulate the heavily calcified wall with subsequent expansion of the conduit to 21 mm after inflating the Atlas balloon to 14 atm. Discussion: Several studies demonstrated that transcatheter stenting of stenotic Fontan conduits can be safely performed. Intravascular lithotripsy has emerged as a novel therapy for the treatment of coronary vascular calcification. We report the first case of an innovative percutaneous Fontan conduit rehabilitation procedure using a lithotripsy shock wave balloon in an adult with severely calcified Fontan pathway obstruction and prior stents, achieving a 75% increase in the pathway size without significant complications. Further studies are needed to evaluate the long-term safety and efficacy of this novel approach, but this case presents an exciting option for future directions in the percutaneous treatment of Fontan pathway obstruction.

Background: Patients with ccTGA have a high incidence of atrial arrhythmias and premature failure of the systemic right ventricle. We sought to better define this risk. Methods: A retrospective review of all clinical data for patients with ccTGA followed at a large tertiary care institution was performed. The primary outcome was a composite of death and cardiac transplantation. Results: A cohort of 165 patients (age 41±16 years, 53% male) were followed for 11±8 years; 41 patients died, 7 underwent cardiac transplantation, and 4 underwent VAD placement. Male sex (HR 2.7, p=0.002), older age (HR 1.04, p<0.001), clinical heart failure symptoms (HR 7, p<0.001), heart failure hospitalization (HR 4.9, p<0.001), syncope (HR 1.8, p=0.04) and pulmonary hypertension (HR 2.77, p<0.001) were significant predictors of the primary outcome. Atrial fibrillation (HR 3.5, p=0.002), atrial flutter (HR 3.05, p=0.04), and prolonged QRS duration (HR 1.01, p=0.004) on the first ECG, as well as right and left atrial enlargement (HR 4, p<0.001 and 3.7, p<0.003) and severe systemic AV valve regurgitation (HR 3.8, p=0.03) on the first echocardiogram were associated with poor outcomes. Atrial arrhythmias occurred in 110(67%) patients: atrial fibrillation in 75(45%), atrial flutter in 50(30%), and supraventricular tachycardia in 16(9.7%) patients. Arrhythmias were largely asymptomatic in 109(66%) patients, while 25(14.5%) experienced palpitations, dizziness or presyncope, and 10(6%) presented with decompensated heart failure. Cardioversion and antiarrhythmic therapy were utilized in 53(32%) patients. Surgical pulmonary vein isolation or atrial MAZE were performed in 15(9%) patients, cryoablation of the CTI in 3 patients, and surgical ablation of an accessory pathway in 2 patients. One or more catheter ablation procedures were also performed in 18(11%) patients. Conclusions: Atrial fibrillation and flutter, heart failure, prolonged QRS duration, systemic AV valve regurgitation and pulmonary hypertension predict worse outcomes in ccTGA. Although asymptomatic in a majority of patients, atrial arrhythmias are associated with significant morbidity and mortality. Whether a rhythm control strategy even in asymptomatic patients may improve outcomes remains unknown.

Abstract Background Pregnancy related cardiomyopathy is a significant cause of maternal morbidity and mortality globally. A presumed overlap between normal pregnancy-associated symptoms and clinical symptoms of cardiomyopathy contributes to delays in diagnosis and increased risk of maternal mortality. Purpose We sought to evaluate the association between patient-reported cardiovascular symptoms and the presence of cardiomyopathy among pregnant and postpartum patients. We hypothesize that individual cardiovascular symptoms are unrelated to the presence of cardiomyopathy. We also evaluated the use of a novel adaptation of a validated health status questionnaire in relation to cardiomyopathy. Methods We enrolled 48 pregnant (&gt;13 weeks) and postpartum (up to 12 months) participants in a prospective study between October 2021 and February 2022. All study participants completed a baseline questionnaire, which included current cardiovascular symptoms, an assessment of health status using an adapted version of the Kansas City Cardiomyopathy Questionnaire (KCCQ-12), followed by a resting transthoracic echocardiogram on the same day. We defined cardiomyopathy as a left ventricular ejection fraction (LVEF) &lt;50% based on 2-D echocardiography. Fisher's exact and Wilcoxon rank-sum tests were employed to evaluate the association between reported cardiovascular symptoms, the adapted KCCQ-12 (KCC-A) score, and cardiomyopathy. Results At the time of enrollment, 67% were pregnant and 33% postpartum. Forty-eight percent identified as White, 31% as Black, 10% as Asian, and 10% as other race. The median age was 31 years (Q1: 28, Q3: 35) and 6% had an LVEF &lt;50%. We found no statistically significant association between four reported cardiovascular symptoms (shortness of breath, orthopnea, fast breathing, and episodes of “asthma” that did not improve with inhalers or other treatment) and cardiomyopathy or medial E/e' ratio. KCC-A scores were low in the study population overall (median 52; Q1:40, Q3: 61). We demonstrated a significantly lower KCC-A score among women with LVEF &lt;50% (median 24; Q1: 15, Q3: 44) compared to women with LVEF ≥50% (median 54; Q1: 44, Q3: 61) p=0.02. Conclusions We showed no significant association between individual cardiovascular symptoms and cardiomyopathy in an obstetric population. However, we demonstrate for the first time that an adapted KCCQ-12 questionnaire for health status assessment could potentially identify women with a high-likelihood of cardiomyopathy during the peripartum period who may benefit from additional evaluation including echocardiography. Larger studies are needed to validate this finding. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): This study was funded by a research grant from the Miami Heart Research Institute, Florida Heart Foundation. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health

Background: Patients with D-transposition of the great arteries and atrial switch have a high incidence of atrial arrhythmias. We sought to analyze the arrhythmia substrate, ablation strategies, and outcomes for catheter ablation in this population. Methods: An in-depth analysis of all clinical and procedural data in patients with D-transposition of the great arteries, atrial baffles, and atrial arrhythmia ablation was performed. Results: A cohort of 32 patients (72% male, mean age 38±7 years) underwent ablation for non-AV nodal reentrant tachycardia atrial arrhythmias, and 4 patients underwent AV nodal reentrant tachycardia ablation. Cavotricuspid isthmus flutter (CTI-flutter) was the most common arrhythmia, encountered in 75% of patients, followed by scar-related intraatrial reentrant tachycardia (non-CTI intraatrial reentrant tachycardia, 53%) and focal atrial tachycardia (focal atrial tachycardia, 6%). Among the 32 patients, 26 underwent 31 procedures at our institution. For patients with prior outside intervention, the index ablation at our institution revealed CTI-dependent flutter in 3/5 cases. However, redo ablation after an index ablation with demonstrated bidirectional CTI block revealed different/new arrhythmia substrates (80% non-CTI intraatrial reentrant tachycardia, 40% focal atrial tachycardia). Achieving bidirectional block across the CTI often required ablating on both sides of the baffle (retroaortic access, 81%; using a baffle leak, 11.5%; or transbaffle puncture, 7.7%). Combined approaches were necessary in 19% to reach the critical tissue. Acute procedural success was 81%, and recurrence was documented in 58% of patients. Despite recurrence, clinical arrhythmia burden was significantly reduced post-ablation ( P <0.001), with rare episodes, amenable to antiarrhythmic therapy. Redo ablation was required in 5 (19%) patients and uncovered new arrhythmia substrates. AV nodal reentrant tachycardia ablation also required transbaffle approaches in 3/4 patients. Conclusions: CTI-dependent flutter was the most common arrhythmia in patients with Dextro-Transposition of the Great Arteries and atrial switch. Transbaffle approaches were often necessary, and, provided that bidirectional CTI block was achieved at the index ablation, late recurrence was due to different arrhythmia mechanisms. Despite recurrence, ablation was associated with significant clinical improvement.

Introduction: Atrial arrhythmias are common in patients with D-TGA and atrial switch. We sought to analyze the arrhythmia substrate and catheter ablation approaches and outcomes. Methods: We performed a retrospective review of all clinical and procedural data in patients with D-TGA followed at a large tertiary care center. Results: In a cohort of 152 patients (mean age 30±11 years), atrial tachycardia was present in 69(45%) patients. Ablations were performed in 39(26%) patients: macro-reentrant atrial flutter (N=37), atrial fibrillation (N=4), and focal automatic atrial tachycardia (N=3). Detailed electrophysiology study data was available for 34 patients. At first ablation (N=28), cavo-tricuspid isthmus dependent flutter (CTI, 23(82%)) was most common, followed by right atriotomy-related flutter (IART, 16(57%)) and focal atrial tachycardia (FAT, 1(3.5%)). Bidirectional CTI block often required ablation on both sides of the baffle to complete the isthmus line. Access to the pulmonary venous atrium was obtained in 82% of first-time ablations (via retrograde aortic access, 74%, baffle puncture, 6%, or baffle fenestration, 12%) and in 100% of redo procedures. The first ablation was acutely successful in 25 (89%) patients; the other 3 patients had either partial procedural success (1), failed ablation (1), or underwent an empirical ablation (1). Long-term arrhythmia recurrence occurred in 13(46%) after the first ablation and class III antiarrhythmic medications were utilized in 12 patients. At least one long-term recurrence occurred in 11(44%) patients. Importantly, clinical arrhythmia burden was significantly reduced post-ablation, with rare and short-limited episodes amenable to antiarrhythmic drugs or cardioversion. Repeat ablation was required in 3 cases. Long-term arrhythmia recurrence after a previously completed CTI line involved different arrhythmia mechanisms: scar-related reentry (80%) and automatic atrial tachycardia (40%). Conclusions: Atrial arrhythmia in patients with D-TGA often involves the CTI and atriotomy scars. Ablation of the CTI typically requires access to the pulmonary venous atrium to achieve bidirectional block. Despite late recurrence, the clinical arrhythmic burden is substantially improved.

Abstract Background Cardiovascular disease (CVD) has been identified as a major threat to maternal health in the US and UK with cardiomyopathy being one of the most common acquired CVD in the pregnant and postpartum period. Diagnosing cardiomyopathy in pregnancy is challenging due to an overlap of cardiovascular symptoms with normal pregnancy symptoms. Purpose The purpose of this study was to evaluate the effectiveness of an ECG based deep learning model in identifying cardiomyopathy among pregnant and postpartum women. Methods We utilized an ECG based deep learning model to detect cardiomyopathy in a cohort of pregnant or postpartum women seen at multiple hospital sites. Model performance was evaluated using area under the curve (AUC), accuracy, sensitivity, and specificity. We compared the diagnostic probabilities of the deep learning model with natriuretic peptides and a multivariable model consisting of demographic and clinical parameters. Results 1,807 women were included. 7%, 10% and 13% had LVEF ≤35%, &lt;45% and &lt;50% respectively. The ECG based deep learning model identified cardiomyopathy with an AUC of 0.92 for left ventricular ejection fraction (LVEF) ≤35%, 0.89 for LVEF &lt;45% and 0.87 for LVEF &lt;50%. For LVEF ≤35%, AUC was higher in Black (0.95) and Hispanic (0.98) women compared to white (0.91). Natriuretic peptides and the multivariable model had AUCs of 0.85 and 0.72 respectively. Conclusions A deep learning model effectively identifies cardiomyopathy in pregnant or postpartum women, outperforms natriuretic peptides and traditional clinical parameters with the potential to become a powerful initial screening tool for cardiomyopathy in the obstetric care setting. Funding Acknowledgement Type of funding sources: Other. Main funding source(s): This study was made possible using resources supported by the Mayo Clinic Women's Health Research Center and the Mayo Clinic Building Interdisciplinary Research Careers in Women's Health (BIRCWH) Program funded by the National Institutes of Health (NIH), grant number K12 HD065987. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

Abstract Background Cardiovascular disease (CVD) has been identified as a major threat to maternal health in the US and UK with cardiomyopathy being one of the most common acquired CVD in the pregnant and postpartum period. Diagnosing cardiomyopathy in pregnancy is challenging due to an overlap of cardiovascular symptoms with normal pregnancy symptoms. Purpose The purpose of this study was to evaluate the effectiveness of an ECG based deep learning model in identifying cardiomyopathy among pregnant and postpartum women. Methods We utilized an ECG based deep learning model to detect cardiomyopathy in a cohort of pregnant or postpartum women seen at multiple hospital sites. Model performance was evaluated using area under the curve (AUC), accuracy, sensitivity, and specificity. We compared the diagnostic probabilities of the deep learning model with natriuretic peptides and a multivariable model consisting of demographic and clinical parameters. Results 1,807 women were included. 7%, 10% and 13% had LVEF ≤35%, &lt;45% and &lt;50% respectively. The ECG based deep learning model identified cardiomyopathy with an AUC of 0.92 for left ventricular ejection fraction (LVEF) ≤35%, 0.89 for LVEF &lt;45% and 0.87 for LVEF &lt;50%. For LVEF ≤35%, AUC was higher in Black (0.95) and Hispanic (0.98) women compared to white (0.91). Natriuretic peptides and the multivariable model had AUCs of 0.85 and 0.72 respectively. Conclusions A deep learning model effectively identifies cardiomyopathy in pregnant or postpartum women, outperforms natriuretic peptides and traditional clinical parameters with the potential to become a powerful initial screening tool for cardiomyopathy in the obstetric care setting. Funding Acknowledgement Type of funding sources: Other. Main funding source(s): This study was made possible using resources supported by the Mayo Clinic Women's Health Research Center and the Mayo Clinic Building Interdisciplinary Research Careers in Women's Health (BIRCWH) Program funded by the National Institutes of Health (NIH), grant number K12 HD065987. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.