Complex Adult Congenital Heart Disease Research Articles

(1074) - Outcomes of Complex Adult Congenital Heart Disease Patients Supported with a Fully Magnetically Levitated Ventricular Assist Device at a Single Center

(1074) - Outcomes of Complex Adult Congenital Heart Disease Patients Supported with a Fully Magnetically Levitated Ventricular Assist Device at a Single Center

Peak oxygen consumption by smartwatches compared with cardiopulmonary exercise test in complex congenital heart disease

ObjectiveTo evaluate for correlation between exercise capacity as assessed by peak oxygen consumption (pVO2) measurement during a cardiopulmonary exercise test (CPET) and smartwatches reporting this parameter in patients with adult...

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

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. 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. 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.

Heart rate: an accessible risk indicator in adult congenital heart disease

BackgroundHigher resting heart rate has been described as a risk factor for adverse outcome in healthy individuals and cardiovascular patients. The aim of this study was to evaluate resting heart...

Abstract 18044: Valvular Procedures in Pregnant Women With Congenital Heart Disease: The Magee Obstetric Maternal and Infant (MOMI) ACHD Substudy

Introduction: Pregnant women with adult congenital heart disease (ACHD) are at high risk of complications and may require interventions, including valvular surgery, after delivery. However, data on the long-term need for valvular surgery among pregnant women with ACHD are limited. Hypothesis: We hypothesized that increasing ACHD complexity and obstetric history are associated with the need for valvular surgery after delivery. Methods: Data from the University of Pittsburgh Medical Center clinical data warehouse were linked to all deliveries from 2004 to 2020 in pregnant women with ACHD (N=430) and without ACHD (N=876), who were included in the Magee Obstetric Maternal and Infant (MOMI) study. Women were matched at the delivery level for age, delivery year, and race. Valvular procedures were captured using Current Procedural Terminology codes until 8/31/2022. Results: Baseline characteristics and pregnancy outcomes are presented in Table 1 . Prior to index delivery, 8 women (6 with ACHD, 2 controls) had a valve replacement, Table 1. After a median of 5.1 (2.8, 8.8) years, 23 women with ACHD had a total of 25 valve procedures, corresponding to 1.0% per year (95%CI: 0.6 to 1.4). Most procedures were aortic valve (n=11, plus aortic root in 5) and pulmonary valve replacements (n=11), followed by tricuspid (n=5) and mitral (n=1) procedures; some women had >1 valve replaced in the same procedure. There were no valvular interventions among controls. After adjusting for maternal age and race, the strongest predictor of need for valvular interventions was ACHD complexity. Compared to simple lesions; moderate ACHD had a HR of 6.58; 95%CI 1.85 - 23.4; P=0.004 and complex ACHD a HR of 9.35; 95%CI 1.37 - 64.0; P=0.023). The number of previous deliveries (or total pregnancies) was not associated with the need for valvular procedures. Conclusions: Women with moderate or complex ACHD have increased rates of valvular procedures after delivery, but multiple pregnancies do not modify this risk.

Catheter-Based Techniques for Addressing Atrioventricular Valve Regurgitation in Adult Congenital Heart Disease Patients: A Descriptive Cohort.

Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

SPECIAL CONSIDERATIONS IN CRITICAL CARE OF THE CONGENITAL HEART DISEASE PATIENT.

Outcomes of congenital heart disease have improved markedly over the last 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management of complex adult congenital heart disease (ACHD) lesions not only from a cardiac perspective but also extra-cardiac organ consequences of years of abnormal circulation following surgical or palliative correction. Kidney and liver dysfunction, respiratory and hematological abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, these are classified in this review as medical non-cardiac, medical cardiac and surgical. Community/hospital acquired infections, cerebrovascular accidents, respiratory failure, alongside arrhythmias and heart failure are responsible for medical admissions. Surgical admissions include post-operative management after correction or palliation, but also medical optimization and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, palliative surgical shunts can be present and require additional consideration. This review focuses on the pathophysiology, long term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions is dependent on early involvement of specialized ACHD centers.

Factors associated with long-term outcomes in adult congenital heart disease patients with infective endocarditis: a 16-year tertiary single-centre experience.

Studies concerning factors associated with long-term outcomes in adult congenital heart disease (ACHD) patients after infective endocarditis (IE) are scarce, while IE-related mortality in these patients remains a burden. We evaluated the factors associated with long-term survival in ACHD patients admitted for IE. We performed a retrospective single-centre study of all ACHD patients admitted for IE to a tertiary cardiothoracic centre between 1999 and 2015. Underlying ACHD, detailed echocardiographic and clinical data, surgical treatment and long-term follow-up were analysed. We identified 151 ACHD patients admitted due to 176 episodes IE with 30-day, 6-month and 1-, 5- and 10-year survival of 95.4%, 92.7%, 92.7%, 84.7% and 75.6%, respectively. In a multivariable analysis, adjusted estimated probability of death was consistently higher after an IE episode among patients with complex as compared to simple/moderate ACHD: 10.6% vs 2.4% at 30 days, 15.0% vs 3.4% at 6 months and 1 year, 30.4% vs 7.8% at 5 years and 44.9% vs 13.1% at 10 years. Risk of death was higher among patients with prosthetic valve in comparison with those without (risk ratios 1.73-1.92). Surgical treatment was required in 76 (43.2%) episodes with 30-day mortality of 3.9%. Risk of death appeared to be lower than in the conservatively treated subgroup (risk ratios 0.71-0.78). We demonstrated satisfactory long-term survival in ACHD patients who were treated for IE in a tertiary cardiothoracic centre. Early mortality tended to be lower in the surgically treated subgroup. Factors negatively associated with long-term survival were complex ACHD and presence of prosthetic valve.

Heart Failure in Complex Congenital Heart Disease of the Adult.

Adult congenital heart disease (ACHD) patients have demonstrated improved survival, especially those with severely complex disease, mainly single-ventricle/Fontan physiology and those with a systemic right ventricle. We describe the heart failure phenotypes of complex CHD, reversible causes for heart failure, and considerations for advanced therapy. While initially marketed for application to patients with acquired causes for heart failure, newer devices and technologies have started to be used in the ACHD population. After reversible causes for heart failure in CHD are addressed, it is reasonable to consider use of new device-based technologies and orthotopic heart transplant (OHT) for end-stage disease. New heart failure technology and organ transplant should carefully be considered and applied in complex ACHD, where there may be significant improvement in morbidity and mortality.

O5-7 Feasibility Study- A physiotherapy led remote ACHD Cardiac Rehabiliation Program

ProblemPrevalence of adult congenital heart disease (ACHD) is growing. Exercise is safe and effective at enhancing exercise capacity and quality of life (QOL) for ACHD patients but they are predominately sedentary with reduced exercise capacity and obese compared to peers. ACHD patients are concerned about their inactivity, however are uncertain of exercise suitability and safety and are unable to access specialist advice or cardiac rehabilitation (CR). Many ACHD patients are managed in specialist centres covering vast areas meaning classes are inappropriate. Acquired heart disease CR is cost-effective, improving QOL, exercise capacity, mortality and morbidity. ACHD patients potentially gain the same benefits. Remote cardiac rehabilitation (RCR) using technology could be a feasible model providing accessible specialist knowledge and individualised exercise prescription.Description of the problemCardiologists referred sedentary, complex ACHD patients for a 12-week RCR providing individualised exercise prescription, education and motivation via telephone clinics and free apps (PT Momentum, Pacer and Active 10). Initially, habitual exercise was encouraged, participants were coached to achieve the recommended UK PA guidelines. Self-efficacy for Exercise (SEE) and Satisfaction with Life Score (SWLS) were assessed comparing patients' responses upon program completion to baseline. To determine the feasibility and acceptability of RCR to ACHD patients and quanitfy the impact of RCR on patient's physical activity (PA) and QOL.To discover if technology supported programme can increase the PA level of ACHD patients.ResultsFrom 23 referrals, 11 completed the program becoming more physically active, fulfilling the guidelines. Improvements were seen in both SEE (mean 30) and SWLS (mean 10). Patients deemed RCR an acceptable service delivery.Participants (9) responded well to apps. Reasons for not using apps included data capacity (1) or no device (2).LessonsACHD patients engaged with RCR making it; viable, acceptable and practical solution improving PA levels and QOL.RCR using app's is a feasible model however, alternatives are required.Main MessagesRCR enables ACHD patients to receive specialist knowledge, individualised, accessible and effective exercise prescription improving QOL and PA levels.RCR potentially leads to substantial cost savings and improvements in morbidity and mortality of ACHD patients.

The Critical Transfer From Paediatrics to Adult Care in Patients With Congenital Heart Disease: Predictors of Transfer and Retention of Care.

Congenital heart disease is the most common congenital birth defect and presents with differing degrees of complexity. Patients require lifelong specialized care. The transfer from paediatric to adult care is a time of risk that may result in lapses or loss of care. A successful transfer from paediatric to adult care is integral for improved patient outcomes. In this retrospective study, we used the paediatric cardiology database and the electronic records at the adult congenital heart disease (ACHD) clinic to identify referrals and successful transfer between 2008 and 2017. Successful transfer was defined as a patient referred to the ACHD clinic who was seen in the clinic and has ongoing follow-up. We also sought to identify predictors of a successful transfer. A total of 555 patients were referred to the ACHD clinic (2008-2017). Of all patients referred, 62% had a successful transfer and an ongoing specialist care. The remaining 38% either did not show for first appointments or missed 3 consecutive visits. Independent predictors of a successful transfer were the presence of moderate or complex ACHD, residing within the city limits, older age at the time of referral, and a more recent year of referral. Over one-third of patients did not achieve successful transfer, namely attendance at first clinic visit plus early retention in care. We were able to identify several variables that predict successful transfer. Further research is required to identify interventions that can be implemented to reduce lapses in patient care.

Patient monitoring and education over a tailored digital application platform for congenital heart disease: A feasibility pilot study

BackgroundPatients with adult congenital heart disease (ACHD) are a rapidly growing cardiovascular population with increasing health needs and co-morbidities. Furthermore, their management requires frequent and ongoing hospital visits which can be burdensome. Digital health and remote monitoring have been shown to have a vast potential to enhance delivery of healthcare for patients, reducing their need for travel to clinic appointments therefore reducing costs to the patient and the healthcare service. MethodsPatients over the age of 16 with a diagnosis of ACHD were invited to use the tailored digital application too. They were monitored for a period of 6 months. Information on patient demographics, time using the application, flagged events that prompted clinical reviews and their feedback through patient surveys were collected. ResultsA total of 103 patients were enrolled and registered to use the digital application tool. There were 57 (56%) males, median age at the time of enrolment was 39 (16–73) years. The majority (96%) had a moderate or complex ACHD according to the ACC/AHA classification. There was a total of 7 modules that were completed on a weekly basis. The median length of a participant session was 2.2 min and the mean time to complete a module was 21 s. In total, 35 (67%) felt that the application helped them better manage their cardiac condition. Almost all (94%) of patients expressed that they would like to continue using the application beyond the pilot. There were 18 flagged events during the 6 month observation period, and 50% of received early clinical intervention. ConclusionApplication based remote monitoring in this select group was well received and potentially holds large benefit to patients both clinically and economically. There were no safety concerns in our pilot feasibility study. Our data may inform much needed and timely investment in digital health.

Sacubitril/valsartan for heart failure in patients with complex adult congenital heart disease – Experience from a tertiary centre in Singapore

BackgroundPatients with adult congenital heart disease (ACHD) have longer life expectancies with the leading cause of death being heart failure (HF). Angiotensin receptor-neprilysin inhibitors (ARNI) have revolutionised contemporary HF management but is less well studied in the ACHD population. The aim of this paper is to describe the safety of ARNI use in complex ACHD and HF in our tertiary centre in Singapore. MethodsA retrospective analysis was conducted at our centre to identify ACHD patients of moderate to severe complexity prescribed with ARNI between December 2015 to November 2020. Demographic and clinical data were obtained from review of clinical documentation, laboratory results and imaging reports before and after ARNI initiation. Results16 patients were identified, 62% male, mean age 37 ​± ​9.9 years and mean follow up duration 708 ​± ​238 days. At baseline, majority of patients were NYHA II on appropriate guideline directed medical therapy, mean systolic blood pressure 124 ​± ​17.9 ​mmHg, BMI 23.5 ​± ​3.8 ​kg/m2 and creatinine 68.2 ​± ​12.6 ​μmol/L. ARNI uptitration was limited by symptomatic hypotension. The overall mean tolerated daily dose was 128 ​mg ​± ​68 ​mg. Renal function remained within normal limits and there were no episodes of hyperkalaemia. Although there were no HF hospitalisations for the duration of follow up, only 4 of the 16 patients reported improvement in their symptoms. ConclusionARNI use in our cohort of patients with HF secondary to ACHD of moderate to severe complexity was well tolerated at suboptimal doses with symptomatic hypotension limiting uptitration.

A feasibility pilot- a personalised physiotherapy led remote ACHD cardiac rehabilitation program

ObjectiveTo determine the feasibility of physiotherapy led remote cardiac rehabilitation (RCR) for Adult Congenital Heart Disease (ACHD) patients and quantify the impact on physical activity levels and well-being. MethodACHD cardiologists referred sedentary complex ACHD patients over 3 months, for 12-week physiotherapy-led RCR. RCR provided individualised exercise program and coaching via telephone clinics and apps. Outcomes in Self Efficacy for Exercise (SEE) and Satisfaction with Life Score (SWLC) were assessed by comparing patients' responses upon program completion to baseline. Results23 patients were referred, 11 completed the programme, 3 had learning difficulties. Participants were mainly female (9); age range 18–61 (median 24). Reasons for not completing RCR included; failure to attend initial appointments (7), lack of interest (1), too unwell (1), lost at follow up (1) and a death unrelated to RCR.Initially no patients were achieving the UK Physical Activity Guidelines; all 11 became more active fulfilling the guidelines. Improvements were seen in SEE with a mean increase of 23 (SD 12) and a mean improvement of 10 (SD 4) in SWLC. Participants (9) responded well to apps. Reasons for not using apps included limited phone storage capacity to download the app (1) or lack of device (2). Patients found RCR acceptable and a personalised approach was essential to improving efficacy. ConclusionRCR is feasible, allowing patients to access specialised, personalised exercise advice and prescription and become more active, improving quality of life (QOL). Future work is required to improve uptake and extend to larger patient numbers.

High-definition mapping of the atria using a novel multipolar mapping catheter in patients with complex adult congenital heart disease

BackgroundThe Advisor™ HD Grid Mapping Catheter (Abbott Technologies, Minneapolis, MN) has been recently introduced. Although the clinical use of HD Grid mapping catheter is well described in adults with no congenital heart disease, there is limited data on the feasibility of using the HD Grid multipolar catheter to create voltage and activation mapping in adults with congenital heart disease. The purpose of this study was to evaluate the safety and technical feasibility of using the Advisor™ HD Grid mapping catheter during the catheter ablation of atrial arrhythmias in adults with congenital heart disease. We included 6 consecutive adults with congenital heart disease suffering from atrial arrhythmias in our study. The HD Grid mapping catheter was used to perform voltage and activation mapping.ResultsSix patients with congenital heart diseases (d-TGA n = 1, Tricuspid atresia n = 1, atrioventricular defect repair n = 1, secundum atrial septal defect n = 1, double-inlet single-ventricle n = 1, Tetralogy of Fallot = 1); majority (84%) male, with the mean age was 35 ± 10 years included in our series. The mean ablation duration and the fluoroscopy time were 789 ± 433 and 502 ± 355 s, respectively. The mean radiation dose was 7.52 ± 9 milliGy/cm2. The HD Grid mapping catheter was used successfully for entire arrhythmia mapping in 5 out of 6 cases. During one procedure, HD Grid mapping catheter could not be used for the entire mapping due to suboptimal reach through baffle puncture. The acute success rate of ablation was 100% with no immediate complications.ConclusionsThe use of HD Grid mapping catheter is a safe and valuable adjunct to accurately create voltage and activation mapping in ACHD patients undergoing radiofrequency catheter ablation. However, a contact force-sensing ablation catheter should be considered in conjunction to supplement data acquisition in challenging anatomy and substrates.

Usefulness of cardiac fusion imaging with computed tomography and Doppler echocardiography in the assessment of conduit stenosis in complex adult congenital heart disease

BackgroundNoninvasive assessment of stenotic lesions in patients with complex adult congenital heart disease (ACHD) is challenging due to its complex morphology. The simultaneous two-screen display of multidetector-computed tomography (MDCT) and real-time echogram (STDME) technology can display a virtual multi-planar reconstruction from MDCT corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). We investigated the usefulness of the STDME technology for stenosis severity assessment in complex ACHD patients. MethodsTwenty-four complex ACHD patients with stenotic lesions were enrolled in this study. All patients underwent TTE and the STDME technology within a week after MDCT. Peak velocity and pressure gradient (PG) across the stenotic site were measured using continuous wave Doppler. Cardiac catheterization was performed in 17 patients. ResultsNine out of the twenty-four patients had undergone repair with a conduit. Peak velocity and PG from the STDME technology were higher than those from TTE (peak velocity: 3.1 ± 1.1 vs. 2.8 ± 1.0 m/s; peak PG: 43 ± 28 vs. 34 ± 21 mmHg; both p < 0.01). Peak PG from the STDME technology showed significant correlations with those from catheterization in patients with a conduit (n=7) and those without a conduit (n=10) (r = 0.795 and 0.880, respectively; both p < 0.05), while peak PG from TTE was correlated with catheterization measurements only in patients without a conduit (r = 0.850, p < 0.05). ConclusionsThe STDME technology enables more accurate assessment of conduit stenosis severity than does TTE in complex ACHD patients.

Evolution of echocardiography in adult congenital heart disease: from pulsed-wave Doppler to fusion imaging.

The number of patients with adult congenital heart disease (ACHD) has been dramatically increasing and adults with congenital heart disease now outnumber children with congenital heart disease. However, patients with ACHD are still at increased risk of morbidity and mortality due to residua and sequelae. Although echocardiography is an indispensable imaging modality in the comprehensive assessment of ACHD, accurate echocardiographic assessment of ACHD is challenging especially for physicians or sonographers who are not familiar with ACHD because of its complex morphology, physiology, and hemodynamics. A recently developed fusion imaging technology can provide synchronized display of real-time echocardiographic images and multiplanar reconstruction images of computed tomography or magnetic resonance imaging corresponding to the image plane of real-time echocardiography. We have reported the clinical utility of this fusion imaging technology for the precise evaluation of complex ACHD. On the other hand, conventional echocardiographic technology also plays an important role in assessing unique ACHD pathophysiology. For example, restrictive right ventricular physiology is a common finding after tetralogy of Fallot or pulmonary stenosis repair and can be evaluated by conventional pulsed-wave Doppler. In this review, we discuss the clinical usefulness of modern and conventional echocardiographic technologies for the evaluation of ACHD by presenting a case series.

Population and Resource Utilization Among Patients With Adult Congenital Heart Disease: A Snapshot View of a Moderate-Size Canadian Regional Centre

BackgroundHealth care resource utilization for patients with adult congenital heart disease (ACHD) has not been well characterized outside of large Canadian specialized regional centres. We sought to describe the ACHD population and resource utilization patterns seen in a medium regional Canadian centre providing specialized ACHD care. MethodsA cross-sectional retrospective study was done from a sample of patients seen in 2018 at the ACHD clinic in Manitoba, Canada. Demographic data were collected along with cardiac anatomy and repair type. Health care resource utilization, clinic visits, hospital admissions, unexpected hospital presentations, and cardiac interventions were measured over a 5-year period. ResultsA random sample of 262 patients was selected from our specialized ACHD clinic. Mean age was 33.5 (±13.7) years; 48% of the population was female, and >50% resided within the major city limits. A total of 21% of the population had simple anatomy, 44% had moderate anatomy, and 35% had complex anatomy. The most commonly used imaging modality was echocardiography, followed by cardiac magnetic resonance imaging, with more frequent imaging done in patients with complex anatomy. Unexpected hospital encounters occurred at a rate of 16 per 100 person-years. Total inpatient hospital days occurred at a rate of 33 per 100 person-years, and visits to the congenital clinic occurred at a rate of 90 per 100 person-years. ConclusionsHealth care resource utilization appears to be highest in older adults and those with more complex ACHD anatomy. As the overall cohort of adults with ACHD continues to age, resource needs are likely to increase.

Cardiac transplantation in adult congenital heart disease with prior sternotomy.

Adult congenital heart disease (ACHD) patients who require orthotopic heart transplantation are surgically complex due to anatomical abnormalities and multiple prior surgeries. In this study, we investigated these patients' outcomes using our institutional database. ACHD patients who had prior intracardiac repair and subsequent heart transplant were included (2008-2018). Adult patients without ACHD were extracted as a control. A comparison of patients with functional single ventricular (SV) and biventricular (BV) hearts was performed. There were 9 SV and 24 BV patients. The SV group had higher central venous pressure/pulmonary capillary wedge pressure (P=.028), hemoglobin concentration (P=.010), alkaline phosphatase (P=.022), and were more likely to have liver congestion (P=.006). Major complications included infection in 16 (48.5%), temporary dialysis in 12 (36.4%), and graft dysfunction requiring perioperative mechanical support in 7 (21.2%). Overall in-hospital mortality was 15.2%. Kaplan-Meier analysis showed a higher, but not statistically significant, survival after 10years between the ACHD and control groups (ACHD 84.9% vs. control 67.5%, P=.429). There was no significant difference in 10-year survival between SV and BV groups (78% vs. 88%, P=.467). Complex ACHD cardiac transplant recipients have a high incidence of early morbidities after transplantation. However, long-term outcomes were acceptable.

Subcutaneous implantable cardioverter defibrillator in complex adult congenital heart disease. Results from the S-ICD “Monaldi Care” registry

BackgroundImplantable cardioverter defibrillators (ICD) are widely accepted therapy in congenital heart disease (CHD) patients at risk of life-threatening ventricular arrhythmias or sudden cardiac death (SCD). Initial experience with subcutaneous ICD (S-ICD) systems has shown a high efficacy. However, the use of S-ICD in complex ACHD (adult congenital heart disease) implies some specific considerations, as the safety for these patients in unknown and recommendations among physicians may vary widely. MethodsWe review the data and studied the indications for S-ICD in complex ACHD and discuss its usefulness in clinical practice. ResultsFrom a large cohort of 297 patients enrolled in the S-ICD “Monaldi care” registry, that encompass all the patients implanted in the Monaldi Hospital of Naples, we considered 14 consecutive complex ACHD patients (aged 35.9 ± 16.7 years) who underwent S-ICD implant from February 2015 to June 2020. Mean follow-up was 23 ± 19.5 months. All the patients showed a good compliance to the device system with no complications (infections or skin erosions). Four patients (1 M/3F aged 38.2 ± 11.3) were listed for heart transplantation (HTX). Two of these underwent HTX, one died for refractory heart failure (HF) and one is still on the waiting list. ConclusionsThere might be a conceivable benefit from an extended use of the S-ICD in selected patients with CHD, especially in those with life-treating ventricular arrhythmias and complex anatomy or patients awaiting HTX. S-ICD appears to be a safe alternative to a transvenous system when a transvenous ICD cannot be implanted and pacing is not required.