Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?

BACKGROUND: With congenital heart disease patients increasingly living into adulthood, there is a growing population of adult congenital heart disease (ACHD) patients suffering from heart failure. Limited data exist evaluating heart transplant in this population. METHODS: Retrospective review was performed of ACHD patients undergoing heart transplantation 11/1990-1/2023. Kaplan-Meier, cumulative incidence accounting for competing risk of death, and subgroup analyses comparing those with biventricular (BiV) and univentricular (UniV) physiology were performed. Data are presented as median (interquartile range) or counts (%). RESULTS: 77 patients with a median age of 36 years (27, 45) were identified, including 57 (74%) BiV and 20 (26%) UniV. Preoperatively, UniV patients were more likely to have cirrhosis (9/20 [45.0%] vs 4/57 [7.0%], p<0.001) and protein losing enteropathy (4/20 [20.0%] vs 1/57 [1.8%], p=0.015). Multiorgan transplantation was performed in 23 patients (30%) and more frequently in UniV patients (10 [50%] vs. 13[23%], p=0.04). Operative mortality was 6.5%, 2/20 (10%) among UniV and 2/57 (4%) among BiV patients, p=0.276. Median clinical follow-up was 6.0 (1.4, 13.1) years. Survival tended to be lower among UniV patients compared to BiV patients, particularly within the first year (p=0.09), but was similar for survivors beyond one year. At 5 years, incidence of rejection was 28% (17%, 38%) and coronary allograft vasculopathy was 16% (7%, 24%). CONCLUSIONS: Underlying liver disease and need for heart/liver transplantation were significantly higher among UniV patients. Survival tended to be lower among UniV, particularly within the first year, but was similar for survivors beyond one year.

Sex Differences in Outcomes of Adults With Isolated Coarctation of the Aorta.

Background: Data are limited about the effect (or lack thereof) of sex on clinical outcomes in adults with coarctation of the aorta (COA). The purpose of this study was to compare atherosclerotic cardiovascular disease (ASCVD) risk profile, blood pressure (BP) data, echocardiographic indices, and mortality between men and women with COA. Methods: Retrospective study of adults with COA, and no associated left-sided obstructive lesions, who received care at Mayo Clinic (2003-2022). ASCVD risk profile was assessed as the prevalence of hypertension, hyperlipidemia, type 2 diabetes, obesity, smoking history, and coronary artery disease. A 24-hour BP monitor was used to assess daytime and nighttime BP and calculate nocturnal dipping. Results: Of 621 patients with isolated COA, 375 (60%) were men, and 246 (40%) were women. Women had similar ASCVD risk profile and daytime BP as men. However, women had less nocturnal dipping (7 ± 5 mm Hg vs 16 ± 7 mm Hg, P < 0.001), higher pulmonary artery mean pressure (23 mm Hg [interquartile range: 16-31] vs 20 mm Hg [interquartile range: 15-28], P = 0.04), and higher pulmonary vascular resistance index (3.41 ± 1.14 WU · m2 vs 3.02 ± 0.76 WU · m2, P = 0.006). Female sex was associated with all-cause mortality (adjusted hazard ratio 1.26, 95% confidence interval 1.04-1.94) and cardiovascular mortality (adjusted hazard ratio 1.38, 95% confidence interval 1.09-2.18). Conclusions: Women had a higher risk of both cardiovascular mortality and all-cause mortality compared to the risks in men. This difference may be related to the higher-than-expected ASCVD risk factors, abnormal nocturnal blood pressure, and pulmonary hypertension observed in women in this cohort. Further studies are required to identify optimal measures to address these risk factors.

Contexte: Il existe peu de données sur l'issue clinique en fonction du sexe chez les adultes présentant une coarctation de l'aorte (CoA). Le but de cette étude consistait donc à comparer le profil de risque de maladie cardiovasculaire athéroscléreuse (MCVAS), les données relatives à la pression artérielle (PA), les indices échocardiographiques et le taux de mortalité chez des hommes et des femmes présentant une CoA. Méthodologie: Il s'agissait d'une étude rétrospective réalisée chez des adultes présentant une CoA en l'absence de lésions obstructives gauches, soignés à la clinique Mayo entre 2003 et 2022. Le profil de risque de MCVAS a été évalué en fonction de la prévalence de l'hypertension, de l'hyperlipidémie, du diabète de type 2, de l'obésité, des antécédents tabagiques et de la coronaropathie. Une surveillance sur 24 heures a été utilisée pour évaluer la PA diurne et nocturne, en plus de calculer la chute nocturne de la PA. Résultats: Parmi les 621 patients présentant une CoA isolée, 375 (60 %) étaient des hommes et 246 (40 %) étaient des femmes. Les femmes présentaient une PA diurne et un profil de risque de MCVAS semblables aux hommes. Elles présentaient néanmoins une chute nocturne de la PA moins prononcée (7 ± 5 mmHg vs 16 ± 7 mmHg, p < 0,001), une pression artérielle pulmonaire moyenne plus haute (23 mmHg [max.-min. : 16-31] vs 20 mmHg [max.-min. : 15-28], p = 0,04) et un indice de résistance vasculaire pulmonaire plus élevé (3,41 ± 1,14 UW · m2 vs 3,02 ± 0,76 UW · m2, p = 0,006). Le sexe féminin a été associé à un plus fort taux de mortalité toutes causes confondues (rapport de risques ajusté : 1,26; intervalle de confiance à 95 % : 1,04-1,94) et de mortalité cardiovasculaire (rapport de risques ajusté : 1,38; intervalle de confiance à 95 % : 1,09-2,18). Conclusions: Les femmes sont exposées à un risque de mortalité cardiovasculaire et de mortalité toutes causes confondues plus élevé que les hommes. Cette différence pourrait être attribuable au rôle plus important que prévu joué par les facteurs de risque de MCVAS ainsi qu'à la pression artérielle nocturne anormale et à l'hypertension pulmonaire chez les femmes de cette cohorte. D'autres études sont nécessaires pour savoir quels seraient les paramètres optimaux qui permettraient d'évaluer ces facteurs de risque.

Mitral Annulus Calcium Score in Patients With Calcific Mitral Stenosis Undergoing Invasive Hemodynamic Assessment.

BACKGROUND: Aortic valve calcium score is associated with hemodynamic severity of aortic stenosis. Whether this association is present in calcific mitral stenosis remains unknown. METHODS AND RESULTS: This study was a retrospective analysis of consecutive patients with mitral stenosis secondary to mitral annular calcification (MAC) undergoing transseptal catheterization. All patients underwent invasive mitral valve assessment via direct left atrial and left ventricular pressure measurement. Computed tomography within 1 year of cardiac catheterization and with adequate visualization of the mitral annulus was included. MAC calcium score quantification by Agatston method was obtained offline using dedicated software (Aquarius, TeraRecon, V.4). Median patient age was 66.9±11.2 years, 47% of patients were women, 50% had coronary artery disease, 40% had atrial fibrillation, 47% had prior cardiac surgery, and 33% had prior chest radiation. Median diastolic mitral valve gradient was 9.4±3.4 mm Hg on echocardiography and 8.5±4 mm Hg invasively. Invasive median mitral valve area using the Gorlin formula was 1.87±0.9 cm2. Median MAC calcium score for the cohort was 7280±7937 Hounsfield units. MAC calcium score correlated with the presence of atrial fibrillation (P=0.02) but was not associated with other comorbidities. There was no correlation between MAC calcium score and mitral valve area (r=0.07; P=0.6) or mitral valve gradient (r=-0.03; P=0.8). CONCLUSIONS: MAC calcium score did not correlate with invasively measured mitral valve gradient and mitral valve area in patients with MAC-related mitral stenosis, suggesting that calcium score should not be used as a surrogate for invasive hemodynamic parameters.

Outcomes of Surgical Repair of Aberrant Subclavian Arteries in Adults.

BACKGROUND: Symptoms, imaging characteristics, and early and midterm surgical outcomes for aberrant subclavian arteries (ASCA) are not well defined in the adult population. METHODS: A single-institution retrospective review was conducted of adults undergoing surgical repair of ASCA and descending aorta origin/Kommerell diverticulum (KD) from January 1, 2002, to December 31, 2021. Symptom improvement and differences in imaging characteristics between anatomic groups and the number of symptoms were assessed. RESULTS: Mean age was 46 ± 17 years. There were 23 of 37 left aortic arches with right ASCA (62%) and 14 of 37 right aortic arches with left ASCA (38%). Of these, 31 of 37 (84%) were symptomatic, and 19 of 37 (51%) had KD size/growth meeting criteria for surgical repair. KD aortic origin diameter was larger in more symptomatic patients: 20.60 mm (interquartile range [IQR], 16.42-30.68 mm) in patients with ≥3 symptoms vs 22.05 mm (IQR, 17.52-24.21 mm) for 2 symptoms vs 13.72 mm (IQR, 12.70-15.95 mm) for 1 symptom (P = .018). Aortic replacement was required in 22 of 37 (59%). There were no early deaths. Complications occurred in 11 of 37 (30%): vocal cord dysfunction (4 of 37 [11%]), chylothorax (3 of 37 [8%]), Horner syndrome (2 of 37 [5%]), spinal deficit (2 of 37 [5%]), stroke (1 of 37 [3%]), and temporary dialysis requirement (1 of 37 [3%]). Over a median follow-up of 2.3 years (IQR, 0.8-3.9 years), there was 1 endovascular reintervention and no reoperations. Dysphagia and shortness of breath resolved in 92% and 89%, respectively, whereas gastroesophageal reflux persisted in 47%. CONCLUSIONS: The KD aortic origin diameter correlates with the number of symptoms, and surgical repair of ASCA and descending aorta origin/KD effectively relieves symptoms, with low rates of reintervention. Given the operative complexity, surgical repair should be performed in patients meeting size criteria or with significant dysphagia or shortness of breath symptoms.

Surgical Considerations for the Mitral Valve in Congenitally Corrected Transposition.

BACKGROUND: Systemic atrioventricular valve (morphologic tricuspid valve [TV]) regurgitation has been implicated in the development of systemic ventricular failure in congenitally corrected transposition of the great arteries (ccTGA), leading to timely referral for valve replacement. However, the surgical management of subpulmonary atrioventricular valve (morphologic mitral valve [MV]) regurgitation and outcomes has not been well studied. METHODS: Of 108 ccTGA patients undergoing atrioventricular valve surgery from 1979 to 2022, 22 patients (20%) underwent MV surgery. Demographics, etiology of valve regurgitation, operative details, and outcomes of these 22 patients were retrospectively reviewed. Follow-up at 1, 5, and 10 years was available for 18 (82%), 13 (59%), and 11 patients (50%), respectively. RESULTS: Median age was 37 years (interquartile range, 29-57 years). Intrinsic MV pathology was present in 18 individuals (82%). Operations included repair in 16 patients (73%), replacement in 6 (27%), and concomitant replacement of TV in 16 (73%). There was 1 perioperative death (5%) in a patient undergoing an emergent operation for severe acute-on-chronic heart failure due to worsening TV regurgitation. During a median follow-up of 12 years (interquartile range, 2-19 years), 7 patients (32%) died. Among the 16 patients who underwent repair, recurrent moderate or greater regurgitation was seen in 15%, 29%, and 43% of repairs in patients with annular, leaflet, and lead-induced regurgitation, respectively. CONCLUSIONS: Concomitant TV and MV disease occur much less frequently than isolated TV disease in ccTGA. Intrinsic MV disease is most commonly observed but appears less amenable to successful repair compared with mitral repair in the systemic position and suggests MV replacement may be preferred in ccTGA patients.

Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels.

BACKGROUND: Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA). OBJECTIVES: The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA. METHODS: We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data. RESULTS: The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P < 0.0001). Predictors of postoperative mortality were age at operation (P = 0.01) and preoperative SVEF (P = 0.04). Preoperative SVEF (P < 0.001), complex ccTGA (P = 0.02), severe SAVV regurgitation (P = 0.04), and preoperative creatinine (P = 0.003) were predictors of late postoperative SVEF. CONCLUSIONS: SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF ≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.

Risk factors and early outcomes of repeat sternotomy in 1960 adults with congenital heart disease: A 30-year, single-center study.

OBJECTIVE: Patients with congenital heart disease (CHD) increasingly live into adulthood, often requiring cardiac reoperation. We aimed to assess the outcomes of adults with CHD (ACHD) undergoing repeat sternotomy at our institution. METHODS: Review of our institution's cardiac surgery database identified 1960 ACHD patients undergoing repeat median sternotomy from 1993 to 2023. The primary outcome was early mortality, and the secondary outcome was a composite end point of mortality and significant morbidity. Univariable and multivariable logistic regression models were used to determine factors independently associated with outcomes. RESULTS: Of the 1960 ACHDs patient undergoing repeat sternotomy, 1183 (60.3%) underwent a second, third (n = 506, 25.8%), fourth (n = 168, 8.5%), fifth (n = 70, 3.5%), and sixth sternotomy or greater (n = 33, 1.6%). CHD diagnoses were minor complexity (n = 145, 7.4%), moderate complexity (n = 1380, 70.4%), and major complexity (n = 435, 22.1%). Distribution of procedures included valve (n = 549, 28%), congenital (n = 625, 32%), aortic (n = 104, 5.3%), and major procedural combinations (n = 682, 34.7%). Overall early mortality was 3.1%. Factors independently associated with early mortality were older age at surgery, CHD of major complexity, preoperative renal failure, preoperative ejection fraction, urgent operation, and postoperative blood transfusion. In addition, sternotomy number and bypass time were independently associated with the composite outcome. CONCLUSIONS: Despite the increase in early mortality with sternotomy number, sternotomy number was not independently associated with early mortality but with increased morbidity. Improvement strategies should target factors leading to urgent operations, early referral, along with operative efficiency including bypass time and blood conservation.

Late Outcomes of Konno-Rastan and Modified Konno for Left Ventricular Outflow Tract Obstruction.

BACKGROUND: While early outcomes of the Konno-Rastan and modified Konno procedures are known, long-term outcomes remain undetermined. Our objective was to examine long-term clinical and echocardiographic outcomes. METHODS: Retrospective review was performed of patients undergoing Konno-Rastan and modified Konno for complex left ventricular outflow tract (LVOT) obstruction from January 1980 to January 2021. The Ross-Konno procedure was excluded due to its often limited ventriculotomy. RESULTS: A total of 92 patients were identified: 73 Konno-Rastan and 19 modified Konno. The modified Konno group was significantly younger (median [interquartile range, IQR]: 6, [2-12] years vs 16 [9-32] years, P = .001). LVOT obstruction was multilevel (39 of 92 [42.4%]), tunnel subaortic (35 of 92 [38.0%]), or aortic valve stenosis/annular hypoplasia (18 of 92 [19.6%]). Shone syndrome was present in 20 of 92 (21.7%) patients. Operative mortality was 7 of 92 (7.6%); 4 of 86 (4.7%) in elective vs 3 of 6 (50.0%) in urgent cases (P = .005). Median follow-up was 12 (IQR, 5-22) years. Aortic valve/LVOT mean gradient was 13 (IQR, 10-18) mm Hg at 1 year but gradually increased to 25 (IQR, 13-46) mm Hg at 10 years. Ejection fraction remained normal. Freedom from reoperation at 5 and 10 years was 77.2% and 58.4% among Konno-Rastan and 57.7% and 41.7% among modified Konno patients (P = .28), respectively. Overall survival at 5, 10, and 15 years was 82.9%, 76.3%, and 65.5%. Risk factors for mortality included older age, New York Heart Association class III/IV, longer cardiopulmonary bypass time, and multilevel LVOT obstruction. CONCLUSIONS: While LVOT obstruction is alleviated early, recurrent LVOT obstruction occurs over time after Konno-Rastan and modified Konno procedures. Additionally, despite preserved left ventricular systolic function, late overall survival is poor in this young population.

Clinical benefits of angiotensin receptor-Neprilysin inhibitor in adults with congenital heart disease.

BACKGROUND: There are limited data about the clinical benefits of angiotensin receptor-neprilysin inhibitor (ARNI) in adults with congenital heart disease (CHD). The purpose of the study was to assess the clinical benefits (chamber function and heart failure indices) of ARNI in adults with CHD. METHOD: In this retrospective cohort study, we compared the temporal change in chamber function and heart failure indices between 35 patients that received ARNI for >6 months, and a propensity matched control group (n = 70) of patients that received angiotensin converting enzyme inhibitor or angiotensin-II receptor blocker (ACEI/ARB) within the same period. RESULTS: Of the 35 patients in the ARNI group, 21 (60%) had systemic left ventricle (LV) while 14 (40%) had systemic right ventricle (RV). Compared to the ACEI/ARB group, the ARNI group had greater relative improvement in LV global longitudinal strain (GLS) (28% versus 11% increase from baseline, p < 0.001) and RV-GLS (11% versus 4% increase from baseline, p < 0.001), and greater relative improvement in New York Heart Association functional class (-14 versus -2% change from baseline, p = 0.006) and N-terminal pro-brain natriuretic peptide levels (-29% versus -13% change from baseline, p < 0.001). These results were consistent across different systemic ventricular morphologies. CONCLUSIONS: ARNI was associated with improvement in biventricular systolic function, functional status, and neurohormonal activation, suggesting prognostic benefit. These results provide a foundation for a randomized clinical trial to empirically test the prognostic benefits of ARNI in adults with CHD, as the next step towards evidence-based recommendations for heart failure management in this population.

Atrial Appendage Aneurysms: Natural History and Outcomes.

BACKGROUND: Left (LAAA) and right atrial appendage aneurysms (RAAA) are rare entities, and their natural history, management, and long-term outcomes are not well studied. METHODS: This retrospective review includes all patients from 2000 to 2021 with atrial appendage aneurysms at our institution identified using an electronic search tool. LAAA and RAAA were confirmed using multimodality imaging and intraoperative findings. RESULTS: We identified 13 (87%) patients with LAAA and 2 (13%) with RAAA. At diagnosis, 11 (73%) were female, mean age was 51.4 ± 18.8 years, and ejection fraction 56.5 ± 13.1%. Three (20%) patients had congenital heart disease including 2 (13%) with atrioventricular septal defects and 1 (7%) with congenitally corrected transposition. LAAA/RAAA was diagnosed due to new-onset atrial fibrillation (AF) in 6 (40%) patients and embolic stroke in 2 (13%). Ten patients had preexisting AF diagnosed 2.9 ± 1.4 years earlier at a mean age of 50.2 ± 15.5 years. Thrombus within the aneurysm was identified in 2 (15%) LAAA patients. All patients were on anticoagulation and follow-up of the cohort from diagnosis was 7.1 ± 6.2 years. Eleven (73%) patients were surgically managed with 7 (64%) lesions excised, 1 (9%) stapled, and 3 (27%) ligated. Postoperative complications occurred in 2 (18%) patients with 1 (7%) developing tricuspid regurgitation and another with pericardial effusion and tamponade. CONCLUSIONS: Atrial appendage aneurysm is a rare disease and nearly half of patients present with AF. Surgical treatment with concomitant AF ablation is a reasonable and safe management option.

Outcomes of a protocolized approach for surgical unroofing of intramural anomalous aortic origin of coronary artery in children and adults.

OBJECTIVE: Management of anomalous aortic origin of coronary arteries has been variable, and limited data are available on early and late outcomes. METHODS: We report a single institution's experience with a protocolized approach to 148 consecutive patients who underwent surgical unroofing of intramural anomalous aortic origin of coronary arteries (June 2003 to December 2020). The management algorithm included preoperative and postoperative cross-sectional and echocardiographic imaging, exercise testing, and a standardized operative technique. RESULTS: The median age of the cohort was 44.4 years (range, 4 months to 83 years); 130 patients had an anomalous right coronary artery, and 19 patients had an anomalous left coronary artery. Surgical unroofing was an isolated procedure in 118 patients (80%) and a concomitant procedure in 30 patients (20.3%). There were 2 (1%) early deaths; both were reoperations for aortic root or valve replacement. Over a median follow-up of 9.5 (interquartile range, 5-12.3) years, there were 5 late deaths, 3 due to noncardiac causes and 2 due to unknown cause. Late survival after anomalous aortic origin of coronary arteries repair at 10 and 15 years was 94.5% and 94.5%, respectively. There were no early or late deaths in the pediatric cohort with a median follow-up of 10.9 years (interquartile range, 6.9-12.1). At a median clinical follow-up of 3.9 years (interquartile range, 1.1-9.5), 36 patients had chest pain but none with evidence of ischemia related to the unroofing. CONCLUSIONS: Surgical unroofing of anomalous aortic origin of coronary arteries can be performed safely with low early mortality, even in the setting of concomitant procedures. Late survival is excellent, with the vast majority being symptom free.

Partial Anomalous Pulmonary Venous Connection With Intact Atrial Septum: Early and Midterm Outcomes.

BACKGROUND: Partial anomalous pulmonary venous return with intact atrial septum warrants greater understanding and evaluation in the literature. METHODS: From January 1993 to December 2018, 293 patients with partial anomalous pulmonary venous return underwent surgical repair. Of these, 45 patients (15.3%) had an intact atrial septum. The median age was 36 years (interquartile range, 24-48). Direct reimplantation was used in 17 patients (38%), intracardiac baffling in 15 (33%), and caval division (Warden) technique in 13 (29%). Descriptive statistics were used to assess the data, and Kaplan-Meier analysis was used to assess survival. RESULTS: Anomalous veins were right-sided in 27 patients (60%), left-sided in 16 patients (36%), and bilateral in 2 patients (4%). The insertion sites were the superior vena cava in 23 patients (51%), innominate vein in 12 (27%), inferior vena cava in 6 (13%), coronary sinus in 2 (4%), right atrium in 1 patient (2%), and unknown in 1 patient (2%). Scimitar syndrome was noted in 8 patients (18%). There was no postoperative mortality or residual defects. Postoperative echocardiography excluded any obstruction of pulmonary or systemic veins. Postoperative complications included atrial fibrillation in 9 patients (20%) and pneumothorax requiring chest tube in 5 patients (11%). Survival at 1, 5, and 10 years was 100%, 95%, and 95%, respectively. Two patients underwent pulmonary vein dilation, one at 3 years and the other at 7 years. CONCLUSIONS: Surgical repair of partial anomalous pulmonary venous connection with intact atrial septum can be performed with excellent early and midterm outcomes. The overall incidence of midterm systemic or pulmonary vein stenosis is low.

Haemodynamic and prognostic associations of liver fibrosis scores in Fontan-associated liver disease.

OBJECTIVES: Fontan-associated liver disease (FALD) is universal post-Fontan palliation; however, its impact on survival remains controversial and current diagnostic tools have limitations. We aimed to assess the prognostic role of liver fibrosis scores (aminotransferase to platelet ratio [APRI] and fibrosis-4 [FIB-4]) and their association with haemodynamics and other markers of liver disease. METHODS: 159 adults (age ≥18 years) post-Fontan undergoing catheterisation at Mayo Clinic, Minnesota, between 1999 and 2017 were included. Invasive haemodynamics and FALD-related laboratory, imaging and pathology data were documented. RESULTS: Mean age was 31.5±9.3 years, while median age at Fontan procedure was 7.5 years (4-14). Median APRI score (n=159) was 0.49 (0.33-0.61) and median FIB-4 score (n=94) was 1.12 (0.71-1.65). Correlations between APRI and FIB-4 scores and Fontan pressures (r=0.30, p=0.0002; r=0.34, p=0.0008, respectively) and pulmonary arterial wedge pressure (r=0.25, p=0.002; r=0.30, p=0.005, respectively) were weak. Median average hepatic stiffness by magnetic resonance elastography was 4.9 kPa (4.3-6.0; n=26) and 24 (77.4%) showed stage 3 or 4 liver fibrosis on biopsy; these variables were not associated with APRI/FIB-4 scores. On multivariable analyses, APRI and FIB-4 scores were independently associated with overall mortality (HR 1.31 [1.07-1.55] per unit increase, p=0.003; HR 2.15 [1.31-3.54] per unit increase, p=0.003, respectively). CONCLUSIONS: APRI and FIB-4 scores were associated with long-term all-cause mortality in Fontan patients independent of other prognostic markers. Correlations between haemodynamic status and liver scores were weak; furthermore, most markers of liver fibrosis failed to correlate with non-invasive indices, underscoring the complexity of FALD.

Echo-Doppler and strain assessment of filling pressures in adults with congenitally corrected transposition and systemic right ventricles.

AIMS: Systolic dysfunction of the systemic right ventricle (sRV) is common in adults with transposition of the great arteries and sRV. In acquired disease, diastology analysis for assessment of filling pressures (FP) is paramount in patient care. METHODS AND RESULTS: Retrospective analysis of 47 adults with sRV without prior systemic tricuspid valve surgery undergoing catheterization and echocardiography within 7 days (median -2 [-1, -3]) from January 2000 to February 2021 at our institution. Median age was 48 (31, 55) years, and 16 (34.0%) patients were female. FPs were normal in 21 patients (44.7%). Left atrial size was enlarged in most patients (83.0%) with mean indexed value 58.3 ± 23.4 mL/m2. Tissue Doppler e' was not significantly different between those with high FPs vs. normal (medial 0.07 ± 0.03 vs. 0.08 ± 0.03 m/s, P = 0.63; lateral 0.08 ± 0.04 vs. 0.08 ± 0.04 m/s, P = 0.88). E velocity and subpulmonic mitral regurgitant velocity were higher in those with high FPs (0.9 ± 0.3 vs. 0.6 ± 0.2 m/s, P = 0.005; 3.8 ± 1.1 vs. 2.8 ± 0.9 m/s, P = 0.004). Left atrial reservoir strain, sRV global longitudinal strain, and subpulmonic left ventricular strain were worse in those with high FP (18.0 ± 7.6 vs. 27.9 ± 10.2%, P = 0.0009; -13.0 ± 4.4 vs. -17.9 ± 5.0%, P = 0.002; -16.8 ± 5.7 vs. -23.0 ± 3.8%, P = 0.001). CONCLUSION: Despite the complex anatomy, FPs can be assessed non-invasively in adults with sRV without prior systemic tricuspid valve surgery. The current guideline algorithm for diastolic dysfunction in acquired heart disease has limited applicability in this population. Given the limitations of Doppler in this heterogeneous population, strain analysis can be a helpful adjunct for estimation of FPs.

Subaortic Stenosis in Adult Patients With Atrioventricular Septal Defect.

BACKGROUND: Patients with atrioventricular septal defects (AVSD) are at risk for development of subaortic stenosis throughout their lifetime. The early and midterm outcomes of adults with AVSD undergoing primary operation or reoperation for subaortic stenosis remain unknown. METHODS: All patients aged 18 years or more with partial or complete AVSD who underwent operation for subaortic stenosis at our institution from 1992 to 2020 were retrospectively reviewed. RESULTS: Nineteen patients were identified: 15 patients with partial AVSD (79%); 3 (16%) with complete AVSD; and 1 (5%) with transitional AVSD. Fifteen patients (79%) had previously corrected AVSD (median 8 years; interquartile range, 3.6-23.1) and 7 (37%) had previous repair of subaortic stenosis. The mechanism for obstruction included subaortic membrane (n = 19, 100%); septal hypertrophy (n = 11, 58%); anomalous papillary muscle, chordae, or left atrioventricular valve tissue (n = 9, 47%); and tunnel obstruction (n = 5, 26%). All patients underwent transaortic membrane resection, and septal myectomy was done in 18 patients (95%). There was no early mortality. During follow-up (median 8.3 years, maximum 28), survival was 100% at 5 years and 95% at 10 years. One patient required reintervention for subaortic stenosis 15 years after the operation at our institution. CONCLUSIONS: Surgical correction of subaortic obstruction in adult patients with AVSD can be accomplished with low morbidity and mortality. Subaortic stenosis can appear late after the initial repair of AVSD, and these patients remain at risk for recurrence after resection.

Relationship between left atrial myopathy and atrial fibrillation in adults with coarctation of aorta.

Background: Although patients with coarctation of aorta (COA) have clinical risk factors for atrial fibrillation (AF), there are limited data about AF prevalence, and role of left atrial (LA) indices for risk stratification in this population. We hypothesized that LA indices (LA reservoir strain and LA volume index) were associated with AF, and would identify patients at risk for AF progression. Methods: We analyzed electrocardiograms/Holters, and echocardiograms of adult COA patients at Mayo Clinic (2000-2018). Results: Of 776 patients, 726(94 %), 46(5.9 %) and 4(0.5 %) had no history of AF, paroxysmal AF, and persistent AF respectively; yielding AF prevalence of 6.4 %. LA reservoir strain (AUC 0.782 [0.751-0.808]) had more robust association with AF as compared to LA volume index (AUC difference -0.115, p < 0.001).Among 726 patients without prior AF, 25(3.4 %) had new-onset AF during follow-up. LA reservoir strain <25 % and LA volume index >34 ml/m2 were independent predictors of new-onset AF (HR 1.81 [1.15-3.85], and HR 1.41 [1.03-4.78], respectively). Of 46 patients with paroxysmal AF, 22(48 %) had recurrent AF, and LA reservoir strain <25 % was an independent predictor of recurrent AF (HR 1.94 [1.41-4.17]). LV pressure overload and stiffness indices were associated with progressive LA dysfunction and new-onset AF. Conclusions: Collectively, these data suggest that LA strain can potentially be used for AF risk stratification. Further studies are required to determine whether LA strain can proactively identify patients that will respond favorably to different antiarrhythmic therapies, and whether interventions to reduce LV pressure overload will improve LA function and reduce AF progression.

Early and Mid-Term Outcomes of Coronary Artery Bypass Grafting in Adults With Congenital Heart Disease.

Atherosclerotic coronary artery disease (CAD) is well-studied in acquired cardiac diseases; however, little data exist regarding outcomes of adult congenital heart disease (ACHD) with CAD. This study examined patients who underwent coronary artery bypass grafting (CABG) during repair of ACHD. This was a retrospective study of patients who underwent CABG for CAD concomitant with ACHD repair 1972-2021. Demographic information, ACHD diagnosis, surgical history, operative details, and outcomes were analyzed. Data are presented as median (interquartile range [IQR]). 157 patients were identified with a median age of 63 (IQR 17) years. Left anterior descending (LAD) was the predominant diseased artery (109 patients [69%]); of those 83 (76%) were treated with mammary artery. 90 (57.3%) patients had 1 bypass, 42 (26.7%) 2, 19 (12%) 3, and 6 (3.8%) had 4. There has been no early mortality since 1988. There was no long-term survival difference between the patients with LAD disease treated with mammary compared to vein (P = 0.68), but early mortality was higher in those treated with vein (10.3% vs 0%, P = 0.018). Late recurrent angina was found in 18 patients (12%) and recurrent CAD found in 17 patients (11%), with 16 patients (10%) requiring CAD reintervention. At most recent follow-up (7.2 [IQR 11.4] years), 101 (64.3%) patients were deceased at 10 (IQR 13.1) years after surgery. Surgical revascularization for CAD may be necessary during the treatment of ACHD, most commonly for LAD disease. Early mortality was low in recent decades. Continued surveillance for recurrent CAD is required.

Outcome of New-Onset Postoperative Atrial Fibrillation After Cardiac Surgery in Adults With Congenital Heart Disease.

BACKGROUND: Postoperative atrial fibrillation (POAF) is one of the most common complications after cardiac surgery. POAF is associated with a longer hospital stay, higher healthcare resource utilization, and higher risk of morbidity and mortality. As a result, the American and European guidelines recommend the use of beta-blockers and amiodarone for the prevention of POAF, and in turn, avoid the complications associated with POAF. OBJECTIVES: The purpose of this study was to determine the incidence, risk factors, and prognostic implications of new-onset POAF after cardiac surgery in adults with congenital heart disease (CHD). METHODS: A retrospective study was conducted among adults with CHD who underwent cardiac surgery (2003-2019). POAF and late-onset atrial fibrillation (AF) were defined as AF occurring within and after 30 days postoperatively, respectively. RESULTS: Of 1,598 patients (mean age 39 ± 13 years, 51% men), 335 (21%) developed POAF. Risk factors associated with POAF were older age, hypertension, left atrial (LA) reservoir strain and right atrial (RA) dysfunction, and nonsystemic atrioventricular valve regurgitation. Of 1,291 patients (81%) with follow-up ≥12 months, the annual incidence of late-onset AF was 1.5% and was higher in patients with POAF compared with those without POAF (5.9% vs 0.4%; P < 0.001). Risk factors associated with late-onset AF were POAF, older age, severe CHD, and LA and RA dysfunction. Of the 1,291 patients, 63 (5%) died during follow-up, and the risk factors associated with all-cause mortality were older age, severe CHD, hypertension, left ventricular systolic dysfunction, and LA and RA dysfunction. POAF was not associated with all-cause mortality. CONCLUSIONS: POAF was common in adults with CHD and was associated with late-onset AF but not all-cause mortality. Atrial dysfunction was independently associated with POAF, late-onset AF, and all-cause mortality. These risk factors can be used to identify patients at risk for POAF and provide a foundation for prospective studies assessing the efficacy of prophylactic therapies in this population.