Adult Congenital Heart Disease Research Articles

A survey on the implementation of cardiovascular surgery for congenital heart disease in China between 2017 and 2021

Objective: To investigate the inplementation of cardiovascular surgery for congenital heart disease (CHD) in China. Methods: A cross-sectional study was carried out. The CHD cardiovascular surgery data collected by the Chinese Society of Extracorporeal Circulation from 2017 to 2021 in 31 provinces (autonomous regions/municipalities) of China were retrospectively reviewed, the implementation of CHD cardiovascular surgery in different provinces, regions, general/specialized hospitals, and different age groups (whether≤18 years old) were summarized, and the correlation analysis between the number of surgeries carried out in each province/region and the gross regional product and the number of the regional population was performed. Results: Between 2017 and 2021, the annual volume of CHD cardiovascular surgery was 77 120, 77 634, 81 161, 62 663 and 71 492, respectively, showing a decreasing trend. Meanwhile, the proportion of CHD patients aged≤18 years who underwent cardiovascular surgery also showed a downward trend, from 79.8% (61 557/77 120) in 2017 to 58.6% (41 871/71 492) in 2021 (P=0.027). The number of surgical cases varied greatly among different provinces, including 4 provinces with≥5 000 cases and 9 provinces with 2 000-5 000 cases. In the five years, the number of CHD cardiovascular surgeries in Central and East China was the largest, accounting for 41.1%-45.5% of the total surgical cases. The proportion of CHD surgery cases≤18 years old was the highest in Southwest China (69.7%-87.4%) and the lowest in Northeast China (28.2%-68.9%). Except for 2021, the number of cases carried out by each region between 2017 and 2020 was correlated with the gross regional product (r=0.929, 0.929, 0.893 and 0.964, respectively, all P<0.05) and the population (r=0.821, 0.893, 0.821 and 0.857, respectively, all P<0.05). Hospitals that performed more than 100 operations (20.5%±1.2% of the total number of hospitals) completed 86.2%±1.2% of the total number of operations in China during the 5-year period. In 2017 and 2021, the number of CHD cardiovascular surgeries preformed in children's/women's and children's specialized hospitals accounted for 24.3% (18 772/77 120) and 23.8% (17 012/71 492) of the total number of cases in China, respectively. Conclusions: From 2017 to 2021, the number of cardiovascular surgery for CHD decreases slightly, but the proportion of surgery for adult CHD patients increases significantly.There is a strong correlation between the number of CHD operations in each region and their economic development status. The scale of CHD cardiovascular surgery performed in children's hospitals/women's and children's hospitals accounts for about a quarter of the total volume in China.

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

BackgroundCongenital 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.MethodsWe 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.ResultsThirty-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.ConclusionTesting 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.

Concomitant large atrial septal defect closure, biatrial cryoablation and tricuspid valve replacement with mitral homograft.

The concomitant large atrial septal defect closure, endocardial biatrial cryoablation and tricuspid valve replacement with mitral homograft in a patient with adult congenital heart disease is presented. Because of the severely dilated right ventricle and leaflet tenting, tricuspid valve replacement was considered. The patient refused both mechanical and stented biological prosthesis due to personal beliefs, therefore, the alternative valve substitute was proposed. Relevant decision-making process, preoperative diagnostic work-up and surgical technique are highlighted with satisfactory outcomes.

Empirical Findings on Associations between Anxiety, Depression, and Congenital Heart Disease in Adults – A Systematic Review and Meta-analysis

Empirical Findings on Associations between Anxiety, Depression, and Congenital Heart Disease in Adults – A Systematic Review and Meta-analysis

Key Psychosocial Health Outcomes and Association With Resilience Among Patients With Adult Congenital Heart Disease

BackgroundAdult congenital heart disease (ACHD) can negatively impact quality of life (QOL). Strengthening resilience may improve this and other psychosocial outcomes important for living a meaningful life. ObjectivesThe purpose of this study was to describe resilience and key psychosocial health outcomes in ACHD and evaluate the associations between resilience and these outcomes. MethodsWe conducted a prospective cohort study of outpatients with moderate or complex ACHD between May 2021 and June 2022. Participants completed surveys at baseline and 3 months, evaluating resilience (Connor-Davidson Resilience Scale-10), health-related QOL (EQ5D-3L, linear analog scale), health status (Euroqol visual analog scale), self-competence (Perceived Competence Scale), and psychological symptom burden (Hospital Anxiety and Depression Scale) and distress (Kessler-6). ResultsThe mean participant age (N = 138) was 41 ± 14 years, 51% were female, and 83% self-identified as non-Hispanic White. ACHD was moderate for 75%; 57% were physiologic class B. Mean (SD) baseline resilience score (Connor-Davidson Resilience Scale-10) was 29.20 ± 7.54. Participants had relatively good health-related QOL, health status, and self-competence, and low psychological symptom burden and distress. Higher baseline resilience was associated with better values of all outcomes at 3 months (eg, 1 point higher resilience was associated with 0.92 higher linear analog scale; 95% CI: 0.52-1.32) with or without adjustment for demographics. After further adjusting for the baseline psychosocial measure, only the association between resilience and QOL measures at 3 months remained statistically significant. ConclusionsResilience is positively associated with health-related QOL for outpatients with moderate or complex ACHD, though relationships are small in magnitude. Study findings can guide the application of resilience-building interventions to the ACHD population.

How to Recognize and Overcome Pulmonary Hypertension Crisis During Patent Ductus Arteriosus Closure by Device in Adult

Background: Managing a patent ductus arteriosus (PDA) with severe pulmonary hypertension (PH) is challenging since closing the PDA can result in reduced cardiac output and right ventricular (RV) failure. The latest guideline for Adult Congenital Heart Disease (ACHD) stated it is harmful to close the defect in patients with pulmonary vascular resistance (PVR) ≤5 WU and flow ratio (FR) &lt;1.5. Case presentation: A 37-year-old female was referred with cough, fever, low saturation, and murmur findings. After serial examination, she was diagnosed with large PDA and severe PH her peripheral saturation (SaO2) was 88%-89% and non-responder to acute vasoreactivity test. After a year of PH therapy, vasoreactivity showed a response to the vasoreactivity test and improved on clinical presentation with SaO2 91%-92%. Patient fell in to PH crisis condition during the procedure of device closure, prostacyclin analogue intravenous (IV) and phosphodiesterase inhibitors inhalation was administered, and the procedure was carried on. The device was successfully implanted, and the patient had SaO2 97% in all four extremities before discharge. Conclusion: With established PH therapy, PDA with severe PH can underwent PDA closure by device with satisfying outcome. Keyword: Case Report, Congenital Heart Disease Intervention, Patent Ductus Arteriosus, Pulmonary Hypertension Crisis.

In-Hospital Death and End-of-Life Status Among Patients With Adult Congenital Heart Disease　- A Retrospective Study Using the JROAD-DPC Database in Japan.

The end-of-life (EOL) status, including age at death and treatment details, of patients with adult congenital heart disease (ACHD) remains unclear. This study investigated the EOL status of patients with ACHD using a nationwide Japanese database.Methods and Results: Data on the last hospitalization of 26,438 patients with ACHD aged ≥15 years, admitted between 2013 and 2017, were included. Disease complexity (simple, moderate, or great) was classified using International Classification of Diseases, 10th Revision codes. Of the 853 deaths, 831 patients with classifiable disease complexity were evaluated for EOL status. The median age at death of patients in the simple, moderate, and great disease complexity groups was 77.0, 66.5, and 39.0 years , respectively. The treatments administered before death to patients in the simple, moderate, and great complexity groups included cardiopulmonary resuscitation (30.1%, 35.7%, and 41.9%, respectively), percutaneous cardiopulmonary support (7.2%, 16.5%, and 16.3%, respectively), and mechanical ventilation (58.7%, 72.2%, and 75.6%, respectively). Overall, 70% of patients died outside of specialized facilities, with >25% dying after ≥31 days of hospitalization. Nationwide data showed that patients with ACHD with greater disease complexity died at a younger age and underwent more invasive treatments before death, with many dying after ≥1 month of hospitalization. Discussing EOL options with patients at the appropriate time is important, particularly for patients with greater disease complexity.

Arrhythmia Burden Among Adult PatientsWith Congenital Heart Disease: APopulation-Based Study.

A significant percentage of patients with congenital heart disease surviving into adulthood will develop arrhythmias. These arrhythmias are associated with an increased risk of adverse events and death. We aimed to assess arrhythmia prevalence, risk factors, and associated health care usage in a large national cohort of patients with adult congenital heart disease. Adults with a documented diagnosis of congenital heart disease, insured by Clalit and Maccabi health services between January 2007 and December 2011, were included. We assessed the associations between arrhythmia and subsequent hospitalization rates and death with mixed negative binomial and Cox proportional hazard models, respectively. Among 11 653 patients with adult congenital heart disease (median age, 47 years [interquartile range, 31-62]), 8.7% had a tachyarrhythmia at baseline, 1.5% had a conduction disturbance, and 0.5% had both. Among those without a baseline arrhythmia, 9.2% developed tachyarrhythmias, 0.9% developed a conduction disturbance, and 0.3% developed both during the study period. Compared with no arrhythmia (reference group), arrhythmia in the previous 6 months was associated with a higher multivariable adjusted hospitalization rate, 1.33-fold higher than the rate of the reference group (95% CI, 1.00-1.76) for ventricular arrhythmia, 1.27-fold higher (95% CI, 1.17-1.38) for atrial arrhythmias, and 1.33-fold higher (95% CI, 1.04-1.71) for atrioventricular block. Atrial tachyarrhythmias were associated with an adjusted mortality hazard ratio (HR) of 1.65 (95% CI, 1.44-2.94), and ventricular tachyarrhythmias with a >2-fold increase in mortality risk (HR, 2.06 [95% CI, 1.44-2.94]). Arrhythmias are significant comorbidities in the adult congenital heart disease population and have a significant impact on health care usage and survival.

The state of adult congenital heart disease training from the trainee perspective: A call for action

The state of adult congenital heart disease training from the trainee perspective: A call for action

Impact of Social Determinants of Health on Outcomes for Patients with Adult Congenital Heart Disease.

The study of adults with congenital heart disease (ACHD) is a rapidly growing field; however, more research is needed on the disparities affecting outcomes. With advances in medicine, a high percentage of patients with congenital heart disease (CHD) are advancing to adulthood, leading to an increase in the number of ACHD. This creates a pressing need to evaluate the factors, specifically the social determinants of health (SDOH) contributing to the outcomes for ACHD. A myriad of factors, including, but not limited to, race, education, and socioeconomic status, have been shown to affect ACHD outcomes. Existing data from hospitalizations, mortality and morbidity, advanced care planning, patient and physician awareness, financial factors, and education alongside race and socioeconomic status present differences in ACHD outcomes. With SDOH having a significant impact on ACHD subspecialty care outcomes, ACHD centers need to be constantly adapting and innovating, incorporating SDOH into patient management, and providing additional healthcare resources to manage the care of ACHD.

Anesthesia management of laparoscopic right colectomy in an older patient with postoperative tetralogy of Fallot with residual anomaly

BackgroundDiversity in hemodynamics of adult congenital heart disease necessitates a case-by-case selection of appropriate surgical and anesthetic options. However, previous case reports regarding the management of laparoscopic surgery in adult patients with congenital heart disease are limited.Case presentationA 72-year-old man who underwent a laparoscopic right colectomy for colon cancer had a residual ventricular septal defect and right ventricular outflow tract obstruction despite post-repair of tetralogy of Fallot. Pulmonary hypertension or right ventricular dysfunction was not observed. The preoperative pulmonary to systemic blood flow ratio (Qp/Qs) was 2.3. After positive pressure ventilation and insufflation, the amount of left-to-right ventricular shunting decreased, and the Qp/Qs approached 1.0, as calculated from pulmonary arterial and systemic arterial blood gas analysis.ConclusionsLaparoscopic surgery might be tolerable in patients with tetralogy of Fallot who have preserved the right ventricular function, left-to-right ventricular shunting, and no high pulmonary vascular resistance.

C-Reactive Protein and Long-Term Prognosis in Adult Patients with Congenital Heart Disease.

Background/Objectives: Prognostic biomarkers may provide information about the patient's cardiovascular outcomes. However, there are doubts regarding how high-sensitivity C-reactive protein (hs-CRP) impacts patients with congenital heart disease (CHD). The main objective is to evaluate whether high hs-CRP levels predict a worse prognosis in patients with CHD. Methods: Observational and prospective cohort study. Adult CHD patients and controls were matched for age and sex. Results: In total, 434 CHD patients (cases) and 820 controls were studied. The median age in the CHD patients was 30 (18-62) years and 256 (59%) were male. A total of 51%, 30%, and 19% of patients with CHD had mild, moderate, and great complexity defects, respectively. The body mass index [1.07 (1.01-1.13), p = 0.022)], diabetes mellitus [3.57 (1.07-11.97), p = 0.039], high NT-pro-BNP levels [1.00 (1.00-1.01), p = 0.021], and low serum iron concentrations [0.98 (0.97-0.99), p = 0.001] predicted high hs-CRP levels (≥0.3 mg/dL) in patients with CHD. During a follow-up time of 6.81 (1.17-10.46) years, major cardiovascular events (MACE) occurred in 40 CHD patients, showing the Kaplan-Meier test demonstrated a worse outcome among patients with hs-CRP levels above 0.3 mg/dL (p = 0.012). Also, hs-CRP showed statistical significance in the univariate Cox regression survival analysis. However, after adjusting for other variables, this significance was lost and the remaining predictors of MACE were age [HR 1.03 (1.01-1.06), p = 0.001], great complexity defects [HR 2.46 (1.07-5.69), p = 0.035], and an NT pro-BNP cutoff value for heart failure > 125 pg/mL [HR 7.73 (2.54-23.5), p < 0.001]. Conclusions: Hs-CRP obtained statistical significance in the univariate survival analysis. However, this significance was lost in the multivariate analysis in favor of age, CHD complexity, and heart failure.

Caring for the Critically Ill Adult Congenital Heart Disease Patient.

This review aims to discuss the unique challenges that adult congenital heart disease (ACHD) patients present in the intensive care unit. Recent studies suggest that ACHD patients make up an increasing number of ICU admissions, and that their care greatly improves in centers with specialized ACHD care. Common reasons for admission include arrhythmia, hemorrhage, heart failure, and pulmonary disease. It is critical that the modern intensivist understand not only the congenital anatomy and subsequent repairs an ACHD patient has undergone, but also how that anatomy can predispose the patient to critical illness. Additionally, intensivists should rely on a multidisciplinary team, which includes an ACHD specialist, in the care of these patients.

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.

Value of Extended Arrhythmia Screening in Adult Congenital Heart Disease Patients

The European Society of Cardiology guidelines for the management of adult congenital heart disease patients recommend screening for arrhythmias and bradycardias in symptomatic patients, often being done by means of an ambulatory 24–48-hour Holter or implantable loop recorder (ILR). However, nowadays non-invasive instruments, such as patches, smartwatches and smartphones based on single-lead ECGs that perform extended monitoring, are also available. The aim of this narrative review was to assess whether these instruments, when they detect arrhythmias and bradycardias in patients with adult congenital heart disease, will lead to meaningful changes in clinical care. Clinically meaningful changes include adjustment of medication, cardioversion, electrophysiology study, ablation or implantation of a cardiovascular implantable electronic device. The following monitoring instruments are discussed: cumulative Holter, 2-week continuous monitor, smartwatch- and smartphone-based single-lead ECG, and ILR. The diagnostic yield of extended rhythm monitoring is high, and varies between 18% (smartphone-based single-lead ECG) and 41% with ILR. In conclusion, contemporary arrhythmia screening includes various new non-invasive technologies that are promising new tools as an alternative to Holter monitoring or ILR. However, the optimal mode of detection is still unclear due to the lack of head-to-head comparisons.

Safety and Efficacy of Percutaneous Pulmonary Valve Implantation: The Singapore Experience

Background: Right ventricular outflow tract (RVOT) dysfunction in adult congenital heart disease patients frequently requires repeated interventions. Percutaneous pulmonary valve implantation (PPVI) is being used increasingly to treat pulmonary stenosis (PS) and regurgitation (PR), improving symptoms, right ventricular haemodynamics and function and, indirectly, left ventricular filling and function. This article explores the authors’ early local experience with PPVI. Methods: Between 2017 and 2022, PPVI was attempted in 10 patients at a single tertiary centre, including six with PS and four with PR. Patients underwent multimodality imaging and dental clearance. PPVI was then performed. Patients were continued on lifelong single antiplatelet therapy and dental hygiene was reinforced. Results: Overall, there was an 80% success rate of Melody PPVI in the 10 adult congenital heart disease (ACHD) patients (mean age 35.8 ± 13.7 years; 60% male), with significant improvements in RVOT pathology, right ventricle function and pulmonary pressures. Melody PPVI was cancelled in one patient due to risk of coronary compression, and an Edwards S3 PPVI was implanted in another patient instead due to a large RVOT size causing stent embolisation. Two patients experienced complications of bleeding and stent fracture, both of which were conservatively managed and had good long-term outcomes. Conclusion: The authors present their early local experience with PPVI in ACHD patients at a single tertiary centre. PPVI has proven to be safe and efficacious, improving PS and PR, right and left ventricle function and pulmonary pressures. Nonetheless, precautions must be taken to minimise complications such as coronary compression, access site bleeding, device embolisation and stent fracture.

The Impact of Local Programmatic Decisions on Outcomes in Transplant-Listed Adults With Congenital Heart Disease

We investigated variables impacting waitlist time and negative waitlist outcomes in adult congenital heart disease (ACHD) orthotopic heart transplant (OHT) candidates following the 2018 allocation change. Adult OHT candidates listed between 10/18/2018-12/31/2022 in the United Network for Organ Sharing database were categorized as ACHD vs. non-ACHD. Waitlist time and time to upgrade for those upgraded into status 1-3 were compared using rank-sum tests. Death/delisting for deterioration was assessed using Fine-Gray sub-distribution hazard ratios (SHRs). Of 15,424 OHT candidates, 589 (3.8%) were ACHD. ACHD vs. non-ACHD candidates had less urgent status at initial listing (4.2% vs. 4.7% listed at status 1; 17.2% vs. 23.7% listed at status 2, p<0.001), but not final listing (5.9% vs. 7.6% final status 1; 35.6% vs. 36.8% final status 2, p<0.001). ACHD vs. non-ACHD candidates upgraded into status 1 (65.0 vs. 30.0 days, p=0.09) and status 2 (113.0 vs. 64.0 days, p=0.003) spent longer on the waitlist. ACHD vs. non-ACHD candidates spent longer waiting for an upgrade into status 1 (51.4 vs. 17.6 days, p=0.027) and status 2 (76.7 vs. 34.7 days, p=0.003). Once upgraded, there was no difference between groups in waitlist time as status 1 (9.7 vs. 5.5 days, p=0.66). ACHD vs. non-ACHD candidates with a final status of 1 (20.0% vs. 8.6%; SHR 2.47 [95%CI=1.19-5.16], p=0.02) and 2 (8.9% vs. 2.3%; SHR 3.59 [95%CI=2.18-5.91], p<0.001) experienced higher death/deterioration. ACHD candidates have longer waitlist times, lower priority status at initial listing, wait longer for upgrade, and have higher mortality at the same final status as non-ACHD candidates, suggesting that they are being upgraded too late.

Pregnancy in Women with Congenital Heart Disease: New Insights into Neonatal Risk Prediction

BackgroundAdvances in managing adult congenital heart disease (ACHD) have led to an increased number of women with CHD reaching childbearing age. This demographic shift underscores the need for improved understanding and prediction of complications during pregnancy in this specific ACHD population. Despite progress in maternal cardiac risk assessment, the prediction of neonatal outcomes for ACHD pregnancies remains underdeveloped. Therefore, the aims of this study are (1) to assess neonatal outcomes in a CHD women population, (2) to identify their predictive factors and (3) to propose a new risk score for predicting neonatal complications. MethodsThis registry study included all women born between 1975 and 1996 diagnosed with ACHD who underwent at least one cardiology consultation for ACHD in Cliniques Universitaires Saint-Luc. A multivariate analysis was performed to identify predictors of neonatal complications and these were incorporated into a new risk index. Its validity was assessed using bootstrap method. This score was then compared with scores adapted from the ZAHARA and CARPREG studies for offspring events prediction. ResultsAnalysis of 491 pregnancies revealed 31.4% of neonatal complications. Four significant predictors of adverse neonatal outcomes were identified: cardiac treatment during pregnancy (OR 14.8, 95%CI [3.4-66]), hypertensive disorders of pregnancy (OR 11.4, 95%CI [3.4-39.0]), smoking during pregnancy (OR 10.6, 95% CI [2.8-40.6]), and pre-pregnancy BMI < 18.5 kg/m² (OR 6.5, 95% CI [2.5-16.5]). The risk model demonstrated an AUC of 0.70 (95% CI [0.65-0.75), which remained stable after bootstrap validation. This model significantly outperformed the scores adapted from ZAHARA and CARPREG data. Based on the regression coefficients, a risk score was subsequently developed comprising 5 risk categories. ConclusionsOne third of ACHD pregnancies are complicated by poor neonatal outcome. These complications are determined by 4 independent factors relating to the cardiac and non-cardiac status of the patients, which have been incorporated into a risk score. Our study is one of the first to propose a predictive risk score of neonatal outcomes in ACHD pregancies, and paves the way for other validation and confirmation studies.

Characteristics and Outcomes of a Single-Centre Cohort of Adult Congenital Heart Disease Patients Referred for Heart Transplant

Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021). Database analysis of patient demographics, characteristics, wait-listing, and transplantation outcomes was performed. A total of 45 patients (mean age 37±9.9 years old; 69% male) were referred for HTx with a mean follow-up of 5.9±6.3 years. Of these, 22 of 45 (49%) were listed and transplanted, including one heart-lung transplant. The commonest diagnosis was dextro-transposition of the great arteries (13/45, 29%). Most patients, 33 of 45 (73.3%) had undergone at least one cardiac surgery in childhood. Indications for HTx referral included HF in 34 of 45 (75%), followed by pulmonary hypertension in 7 of 45 (11%). Median transplant wait-list time was 145 days (interquartile range, 112-256). Of the 23 patients not wait-listed, the reasons included clinical stability in 13 of 45 (29%), psychosocial factors in 2 of 45 (4.4%) and prohibitive surgical risk, including multiorgan dysfunction, in 8 of 45 (17.7%). Transplant was of a single organ in most, 21 of 22 (95.5%). Overall mortality was 5 of 22 (22.7%) in those after HTx, and 14 of 23 (60.9%) in those not listed (p=0.0156). Increasingly, ACHD patients demonstrate the need for advanced HF treatments. HTx decision-making is complex, and increased mortality is seen in those not wait-listed. Ultimately, the referral of ACHD patients for HTx is underpinned by local decision-making and experience, wait-list times and outcomes.

Prognostic significance of echocardiographic deformation imaging in adult congenital heart disease.

Due to medical and surgical advancements, the population of adult patients with congenital heart disease (ACHD) is growing. Despite successful therapy, ACHD patients face structural sequalae, placing them at increased risk for heart failure and arrhythmias. Left and right ventricular function are important predictors for adverse clinical outcomes. In acquired heart disease it has been shown that echocardiographic deformation imaging is of superior prognostic value as compared to conventional parameters as ejection fraction. However, in adult congenital heart disease, the clinical significance of deformation imaging has not been systematically assessed and remains unclear. According to the Preferred Reporting Items for Systematic Reviews checklist, this systematic review included studies that reported on the prognostic value of echocardiographic left and/or right ventricular strain by 2-dimensional speckle tracking for hard clinical end-points (death, heart failure hospitalization, arrhythmias) in the most frequent forms of adult congenital heart disease. In total, 19 contemporary studies were included. Current data shows that left ventricular and right ventricular global longitudinal strain (GLS) predict heart failure, transplantation, ventricular arrhythmias and mortality in patients with Ebstein's disease and tetralogy of Fallot, and that GLS of the systemic right ventricle predicts heart failure and mortality in patients post atrial switch operation or with a congenitally corrected transposition of the great arteries. Deformation imaging can potentially impact the clinical decision making in ACHD patients. Further studies are needed to establish disease-specific reference strain values and ranges of impaired strain that would indicate the need for medical or structural intervention.