Adults With Congenital Heart Disease Patients Research Articles

COVID-19 and Children with Congenital Heart Disease: Pandemic Implication

While the global coronavirus crisis worsens, a surprising feature of the disease appears that children might be immune from the worst form of it. Studies suggest that COVID- 19 is more likely to infect older adult men, particularly those with comorbidities. There is only limited data detailing the effects of COVID-19 on the pediatric population. Patients with underlying cardiovascular comorbidities are at increased risk of morbidity and mortality from SARS-CoV-2 infection. Certain adult patients with congenital heart disease (ACHD) and with complex congenital heart disease can be considered as high risk for complications related to COVID-19 infection on the basis of decreased functional reserve. While no study on COVID-19 has been included paediatric patients with congenital heart disease, it stands to reason that patients with congenital heart disease can be considered at higher risk for complications from COVID-19. Given the increased risk for severe COVID-19 in adults with underlying cardiac disease, there is concern that patients with congenital heart disease (CHD) may likewise be at increased risk for severe infection, as they are known to have higher risk for complications with viral illnesses including respiratory syncytial virus and influenza.
 DS (Child) H J 2020; 36(2): 138-145

Treatment of symptomatic atrial tachycardia in complex anatomic conditions: use and outcome of ablation in patients after damus-kaye-stansel surgery

Abstract Funding Acknowledgements Type of funding sources: None. Background Atrial tachycardias (AT) are amongst the most frequent complications in adult patients with congenital heart disease (ACHD). Their incidence mainly depends on age and complexity of underlying defect. AT has a marked impact on morbidity and mortality and is associated with sudden cardiac death particularly in complex ACHD patients. The arrhythmic substrate can be variable from re-entry to multifocal resulting in demanding requirements for electrophysiologic ablation procedures (EP) and thus, relatively high rates of recurrence. Methods and results Retrospective analysis of ACHD patients with prior Damus-Kaye-Stansel (DKS) surgery treated at one of the largest specialized tertiary centers for ACHD patients worldwide. Analysis focussed on occurrence of AT, respective treatment and outcome of EP. Over a median observation time of 15 years, 13 patients with prior DKS surgery and complex congenital heart defects of different types but uniformly Fontan circulation, were identified (median age 22.0 years (IQR 20.5 – 25.5), 76.9% male, 92.3% NYHA Class I, median systemic ventricular ejection fraction 58.5%, 69.2% extracardiac tunnel). Seven patients (53.8%) presented with symptomatic AT (median cyclic length 440 ms (IQR 360.0 – 517.5)). Of these, six patients underwent EP. Median procedure time was 180.5 min (IQR 160.8 – 274.5), median fluoroscopy time 1.56 min (IQR 0.51 – 4.73) and radiofrequency time 851 sec (265.0 -2005.0). Remote magnetic navigation was used to access ablation site through a retrograde access via the aortic as well as the neo-aortic valve. Three-dimensional image integration aided in mapping and ablation. Throughout the procedure as well as in follow-up visits, no reportable complications were observed. After a mean of 1.5 procedures, all analysed patients stayed complaint-free throughout a median time-interval of 13 months. Conclusion AT are frequent in complex univentricular situations after Damus-Kaye-Stansel surgery (53.8%). Although more than one procedure (mean 1.5) was necessary to achieve satisfactory results even in experienced hands, EP appears to be an appropriate treatment strategy in this young and otherwise healthy patient cohort. According to our analysis, EP is feasible and safe in centers with knowledge of specific anatomic characteristics of congenital heart disease and when the appropriate technical infrastructure is present. Access to ablation site was performed by remote magnetic navigation in the majority of procedures. The floppy catheter was navigated through the neo-aortic as well as through the aortic valve without observable peri- or postinterventional complications. Mapping with three-dimensional image integration facilitated EP and led to a relatively low radiation exposure with the known benefits for the patient as well as for the healthcare professionals involved in the procedure. Abstract Figure.

Impact of the New UNOS Heart Transplant Allocation System on Waitlist and Early Post-Transplant Mortality among Adults with Congenital Heart Disease

<h3>Purpose</h3> Adults with congenital heart disease (ACHD) are more likely to die or be delisted while awaiting heart transplant, less likely to receive a heart transplant and more likely to die in the early (30-day) post-transplant period compared to non-ACHD patients. On October 18, 2018; the UNOS heart allocation system was redesigned providing unique listing status for ACHD patients. The impact of this change on outcomes is unstudied. <h3>Methods</h3> Using the Scientific Registry of Transplant Recipients we compared ACHD patients listed for the first-time for heart transplant from 2 eras of equal duration (new era: October 18, 2018 to June 2, 2020 and prior era: March 3, 2017 to October 17, 2020). Patients with transplants occurring in the new era who were initially listed in the prior era were excluded. We compared baseline characteristics, Kaplan Meier curves and log-rank tests to assess differences in early post-transplant mortality and the composite of waitlist mortality or delisting due to clinical worsening among those with CHD between both eras. <h3>Results</h3> Out of 535 ACHD patients listed, 293 were in the new era (4.7% of total listings) and 242 were in the prior era (3.7%). Age, sex, racial distribution, renal function, hemodynamics and need for ECMO were identical between the two groups. 163 (56%) ACHD patients in the new era versus 150 (62%) in the prior era were transplanted; 11 (3.8%) versus 15 (6.2%) died on the waitlist; 32 (11%) versus 35 (14%) were delisted and 15 (9.2%) versus 19 (12.7%) died within 30 days of transplant respectively. In the new era, ACHD patients were significantly less likely to experience waitlist mortality or delisting due to clinical worsening but had similar early post-transplant mortality compared to the prior era. <h3>Conclusion</h3> The new UNOS heart transplant allocation system improved waitlist outcomes among ACHD patients listed for heart transplant.

COVID-19 in adults with congenital heart disease: early morbidity and mortality in two northern Italian specialist centers

There is no information on coronavirus disease 2019 (COVID-19)-related outcomes for adult patients with congenital heart disease (ACHD). We aim to assess the clinical burden of COVID-19 on ACHD patients during the initial stages of the pandemic in Europe. ACHD patients followed in 2 tertiary centers in the Lombardy region were screened for COVID-19-related symptoms and complications. 661 patients (52.3% male, mean age 34.9±11.7 years) with CHD of moderate (58.5%) or high (32.8%) complexity were contacted. Of these, 145 (21.9%) reported typical COVID-19 symptoms, but only 4.8% required hospitalization. We did not record confirmed COVID-19-related deaths. Patients hospitalized were elderly (P=0.03), more likely to be receiving diuretics (P=0.03) or beta-blockers (P=0.02), and have been admitted over the previous year (P=0.003). Of the 7 patients hospitalized, 2 had a systemic RV and 2 a Fontan circulation. COVID-19-related morbidity and mortality have been low in ACHD patients 6 weeks from the start of the outbreak, in one of the worse hit European regions.

Lessons learnt from COVID-19 in adult congenital heart patient in Tehran: a survey-based study of prevention, exposure, susceptibility, and outcomes.

COVD-19 pandemic has overwhelmed many healthcare systems worldwide. Underlying cardiovascular disease predisposes to greater disease susceptibility and more complications including mortality. Such data is unverified in adults with congenital heart disease (ACHD). The aim of the study is to report the Tehran experience with respect to preventative self-care measures, disease exposure, susceptibility, and outcomes after COVD-19 infection in ACHD patients. A telephone-based survey was conducted in ACHD patients, focusing on new-onset symptoms that might indicate COVID-19 infection, prevention measures, confirmed infection rates, and outcomes. Three-hundred and nine ACHD patients, with a mean age of 29.13 years (range from 14 to 72 years, SD = 10.64), and 170 (55%) women were assessed. The majority (86.7%) had moderate or complex ACHD. Two-thirds (67.3%) of the patients practiced high-level preventative self-care measures. After community exposure, 33.3% developed COVID-19, and after household exposure, 43.7% developed COVID-19. There was only one mortality in a post-operative patient. Thirty-seven patients (12%) reported new symptoms including cough (10%), fatigue (8%), fever (7%), and new dyspnoea (6.5%). Amongst 18 (6%) with confirmed COVID-19, there was only 1 mortality in a post-operative patient. Age (adjusted OR = 1.19, 95% CI: 1.07-1.31, p = 0.001), contact with confirmed COVID-19 cases (adjusted OR = 59.34, 95% CI: 3.68-955.10, p = 0.004) were independently associated with COVID-19 infection. Mortality risk associated with COVID-19 infection in ACHD patients with moderate or severe disease appears to be relatively low, similar to the general population. Such risk appears to act through conventional risk factors, and in this cohort, we demonstrated age as a significant risk factor in addition to exposure to the development of COVID-19 infection. Preventative self-care measures are a potentially significant and impactful intervention target for intervention and for improving outcomes.

Abstract 13468: Effect of Listing Criteria on Transplant Rate and Early Outcomes in Adults With Congenital Heart Disease

Introduction: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplant (HT) is one of the few options for the treatment of advanced HF in this growing population. In October 2018, the United Network for Organ Sharing (UNOS) implemented a change in organ allocation criteria. The effect of this change on outcomes in ACHD patients (pts) after listing and transplant has not been evaluated. Hypothesis: Change in organ allocation criteria negatively impacts outcomes in ACHD patients. Methods: Data from the Scientific Registry of Transplant Recipients in pts age &gt; 18 years old listed for HT between Oct. 2016 and 0ct. 2019 and followed through March 2020 were analyzed. Pts were grouped by diagnosis (ACHD and non-ACHD) and by the time of listing (pre- and post-change in allocation criteria). Differences in comorbidities, outcomes while listed, and 1-year Kaplan Meier survival post-HT were compared among groups. For comparison, post-change criteria (status 1-6) were equated to pre-change criteria (status 1A, 1B, 2). Results: Over 3 years, 11,931 patients were listed for HT; 459 had a primary diagnosis of ACHD. ACHD was present in 279/7942 pts listed in the 2 years pre-change and 180/3989 pts in the year post-change. ACHD pts listed post-change were less likely to have a history of cardiac surgery (88% vs. 79%, p=0.01) and more likely to have an abnormal BMI (p=0.015) than ACHD pts pre-change. Post-change, ACHD pts were listed at a higher priority status compared to pre-change ACHD. (Figure). The proportion of pts transplanted with ACHD increased slightly pre- and post-change (3.7% vs. 4.1%). There was no difference in 1-year survival in ACHD pts transplanted pre- and post-change (Figure). Conclusions: Recent changes to the UNOS organ allocation policy increased the proportion of ACHD patients transplanted with no change in early post-HT survival.

Abstract 16601: Impact of Insurance Status on Emergent Versus Non-Emergent Hospital Encounters Among Adults With Congenital Heart Disease

Background: Arguments against universal health insurance are ongoing, but insurance coverage may be beneficial in high-risk populations such as adults with congenital heart disease (ACHD). While the number of hospital visits have exponentially increased for ACHD over the last few decades, the impact of insurance on the type of hospital encounters remains unknown. Hypothesis: Uninsured ACHD patients are more likely to have emergent hospital encounters than those insured. Methods: Using California Office of Statewide Health Planning and Development Database from 01/2005 to 10/2015, we identified all hospital encounters that were emergent (which might or might not have resulted in an admission) or nonemergent among ACHD ≥18 years old. We determined the trends over time and odds of insurance status on emergent vs. nonemergent encounters. Results: Among 69,876,425 encounters, 72,142 were in patients with CHD diagnoses (mean age 49±12 years, 43% males, 52% Caucasian, 4% uninsured). 78% had severe CHD and 75% had a comorbidity. From 2005 to 2015, while all ACHD encounters increased by 108% (p for trend &lt;0.0001), there was significantly higher increase in emergent than nonemergent encounters (331% vs. 87%; p&lt;0.0001). The ratio of emergent:nonemergent encounters was significantly higher for uninsured than insured patients (3.18 vs. 1.02; p&lt;0.0001). Non-cardiac diagnoses were more common among emergent than non-emergent encounters (68% vs. 36%; p&lt;0.0001). Both before and after multivariable adjustment, uninsured status was associated with a significantly higher odds of emergent encounters for all ACHD (Figure), irrespective of CHD type, exhibiting the largest magnitude of effect compared to any other predictor. Conclusions: Efforts to enhance the ability to obtain and maintain insurance throughout the lifetime of ACHD patients might result in meaningful reductions in emergency encounters and a more efficient use of resources.

Abstract 15897: Patient Experience With Telemedicine in Adults With Congenital Heart Disease

Introduction: COVID19 pandemic has resulted in drastic changes in healthcare delivery with increase in use of telemedicine. There is limited data on patient experience with telemedicine in adults with congenital heart disease (ACHD). Hypothesis: We hypothesized that ACHD patients would prefer in-person clinic visits over telemedicine, and this would be especially true for patients &gt; 65 years who may be at a higher risk of experiencing technical limitations. Methods: We conducted a nurse administered telephone survey (Figure 1A) based on Agency for Healthcare Research and Quality recommendations to assess patient experience after ACHD telemedicine visits from March 2020 to June 2020. Results: Of 216 patients who had ACHD telemedicine visits, 136 (63%) agreed to participate in the survey. Mean age was 45 + 18 years, majority (65%) being video encounters. Most (98%) said that the telemedicine visit was successful in addressing their healthcare needs. Only 21 (15%) patients reported technical issues. Most patients (76%) were likely to choose telemedicine in the future instead of an in-person clinic visit provided testing was done separately. Given a choice between in-person clinic visit and telemedicine: 103 (76%) preferred telemedicine, 25 (18%) preferred in-person clinic visits, and 8 (6%) had no preference (Figure1B). Of the 25 patients over 65 years, 19 (76%) would choose telemedicine over the in-person clinic, and only one patient reported technical difficulties. Conclusion: In conclusion, patients reported a positive experience with ACHD telemedicine, which was true even among elderly patients. Technical limitations were infrequent even amongst the elderly. Majority of patients felt that their healthcare needs were addressed and would consider telemedicine over clinic visits if given a choice. This data is vital as payors move away from supporting telemedicine and hospitals restructure, with and without ongoing COVID 19 concerns.

Maternal and neonatal complications in women with congenital heart disease: results from a nationwide analysis including 7,231 pregnancies

Abstract Background The number of adult patients with congenital heart disease (ACHD) is increasing. Many ACHD women aim to start a family and require adequate pre-pregnancy counselling and pregnancy care. Limited population-based data on maternal and neonatal outcome and complications are available. Purpose We analysed the association between ACHD and maternal or neonatal outcomes based on data from one of the largest German Health Insurance Companies (approx. 9 million members). All pregnancies in women with ACHD and an age-matched non-congenital control group were included. The data covered all pregnancies and deliveries (in and out of hospital) between 2005 and 2018. Results The study population comprised 25,528 ACHD patients of childbearing age. Overall, 7,231 pregnancies occurred in 3,868 ACHD women (mean age at delivery 30.7±5.4 years). An age matched group of 6,513 women without ACHD (11,247 pregnancies, mean age 30.0±5.6 years) served as controls. Miscarriages/abortions occurred in 2,501 (34.6%) and 3,586 (31.9%) cases in the ACHD and non-ACHD cohort, respectively. Overall, there were 4,551 successful deliveries (99.5% in hospital) and 59 stillbirths in ACHD patients. Cesarean deliveries (CS) were significantly more common in ACHD patients (40.4% vs. 31.5% in the control group; p&amp;lt;0.0001) and the CS rate increased with disease complexity (Bethesda class I/II/III: 39.2/42.3/44.4%; p=0.035). There was one successful maternal resuscitation in each group, but no maternal deaths during pregnancy and up to 90 days postpartum. ACHD patients had a higher rate of maternal cerebral complications (1.0% vs. 0.17%; p&amp;gt;0.0001) compared to the control group. Neonatal mortality was low but significantly higher in the ACHD group (0.76% vs. 0.22% within the first 30 days of life; p&amp;lt;0.0001). Neonates to ACHD mothers were also significantly more often afflicted by low or extremely low birth weight or extreme immaturity (p&amp;lt;0.0001), and required resuscitation (1.04% vs. 0.34%; p&amp;lt;0.0001), mechanical ventilation (8.7% vs. 3.5%; p&amp;lt;0.0001) or blood transfusions (3.11% vs. 1.08%; p&amp;lt;0.0001) significantly more often compared to offspring from non-ACHD mothers. In addition, recurrence of congenital heart disease was 6.1 times higher in infants to ACHD mothers compared to controls (p&amp;lt;0.0001). Overall, 5.87% of all children born to ACHD mothers required cardiac surgical procedures in the first 6 years of life compared to only 0.39% of children to non-ACHD mothers (p&amp;lt;0.0001). Conclusion Our analysis confirms that maternal morbidity and neonatal morbidity and mortality are significantly increased in women with ACHD and their offspring compared to non-ACHD controls. Although, maternal mortality appears limited in high resource health care systems, the elevated maternal complication rate and particularly the high mortality and complication rate in neonates justifies specialized care to address specific risks and requirements of the condition. Funding Acknowledgement Type of funding source: None

Amiodarone-associated thyroid dysfunction in adult patients with congenital heart disease: a nationwide health insurance data analysis including &amp;gt;50.000 individuals

Abstract Background Arrhythmias are common in adult patients with congenital heart disease (ACHD). Amiodarone is widely used as an antiarrhythmic agent. Thyroid dysfunction represents a serious complication of amiodarone treatment. Systematic data on the prevalence of thyroid dysfunction, risk factors for complications and treatment options are lacking. Purpose Based on data from one of the largest German Health Insurance Companies (BARMER GEK, approx. 9 million members), we performed a retrospective analysis investigating the rate of thyroid complications under active amiodarone therapy in ACHD patients and a comparison group of cardiac patients without congenital heart disease on amiodarone between 2005 and 2018. Result Overall, 910 ACHD (34% female; median age 66 y; CHD complexity mild, moderate, severe in 64.6%, 23.6%,11.8%, respectively) and 49,782 non-ACHD patients (37% female, median age 73.4 y) received prescriptions for amiodarone without documented pre-existing thyroid disease or use of thyroid medication. Over a treatment period of 184,770 patient-years, 10,874 incidents of thyroid dysfunction occurred in the non-ACHD and 201 in the ACHD cohort, corresponding to an event-rate of 6% and 5.3% per patient year, respectively. Overall, 23.5% of the ACHD patients developed thyroid dysfunction (56.71% hypothyroidism, 43.3% hyperthyroidism). Risk factors for developing thyroid dysfunction on time dependent Cox-analysis were female gender (hazard ratio [HR] 1.44, 95% CI: 1.39–1.50; p&amp;lt;0.001), lower patient age (HR: 0.96 per 10 years, 95% CI: 0.94–0.98, p&amp;lt;0.001) renal dysfunction (HR 1.24, 95% CI: 1.19–1.29, p&amp;lt;0.001), history of alcohol abuse (HR 1.17, 95% CI: 1.07–1.27, p,0.001) and smoking (HR 1.12, 95% CI: 1.06–1.19, p&amp;lt;0.001). Congenital heart disease itself was not associated with a higher risk of thyroid dysfunction (HR 0.96, 95% CI: 0.83–1.10, p=0.53). Within the ACHD group, patients with complex disease had a significantly higher risk of thyroid dysfunction (HR 1.5, 95% CI: 1.00–2.25, p=0.049) compared to patients with simple diagnoses. Once thyroid disease occurred, 48.5% of patients were continued on amiodarone therapy, 12.8% of patients underwent an electrophysiologic procedure and only 2.1% of patients received class I antiarrhythmics. Specific thyroid therapy included thyroxine (62.6% of hypothyroid patients) or thiamazole (22.6% of hyperthyroid patients). Only 2.3% of patients required surgery or radiotherapy within 6 months after thyroid dysfunction. Conclusion Amiodarone-associated thyroid dysfunction is a frequent complication in ACHD patients. Overall, one in 4 ACHD patients on amiodarone developed thyroid dysfunction in our study. In itself, ACHD does not seem to increase the risk of thyroid dysfunction. However, female gender, complexity of disease, younger age and renal dysfunction emerged as independent risk factors. When amiodarone therapy can not be avoided, close follow-up and regular thyroid function tests are recommended. Funding Acknowledgement Type of funding source: None

Invasive electrophysiological studies in adult congenital heart disease- evolving use and impact of specialized centre therapy on recurrence of arrhythmia: a nationwird analysis based on 2.433 cases

Abstract Background Arrhythmias are a major contributor to morbidity and mortality in adult patients with congenital heart disease (ACHD). Advances in ablation technology have contributed to improved management and reduction of symptoms in this population. However, only limited data exist on the use and outcome of these technologies in large community-based cohorts of ACHD patients and on the impact of specialized centres on recurrence of arrhythmias. Purpose We performed a retrospective analysis based on data from one of the largest German Health Insurance Companies (approx. 9 million insured members), acquiring real-world data on frequency of invasive electrophysiological studies (EPS) in Germany between 2005 and 2018. ACHD patients were identified based on ICD codes and the spectrum of disease as well as the impact of EPS being performed at specialized ACHD centres was analysed. Results Out of 45,761 eligible ACHD patients in the database, we identified 2,433 EPS performed in 1,706 ACHD (51% female, median age 55.4 years, complexity of CHD mild, moderate and severe in 50.6%, 33.2%,16.2%, respectively). Over the study period the annual number of EPS increased by 207%. The majority of procedures were for supraventricular tachycardias (85.9%). Of these procedures atrial fibrillation accounted for 35.1%, atrial flutter for 29.4% and other supraventricular tachycardias/pre-excitation syndromes for 29.9% of cases. The majority of EPS (64.3%) was performed at non-specialized centres including 40.2% of cases in patients with complex disease. Overall, the re- intervention rate within 12 months of the primary EPS was 14.5%. Whereas in ACHD patients with simple and moderate complexity disease no obvious difference in 12-months re- intervention rate was observed between specialized and non-specialised centres (15.5% vs. 15.0%), in patients with complex disease the reintervention rate was 41.6% higher for non-specialized centres (13.6% vs. 9.6%). Conclusion Our large real- world analysis shows an increasing need for invasive electrophysiological studies in ACHD patients. The vast majority of procedures was performed for supraventricular arrhythmias including in atrial fibrillation and flutter ablation. Re-interventions were frequently required in this unique and anatomically challenging population. Alarmingly, many procedures were performed at non-specialized centres, although current guidelines recommend that interventions in complex patients should remain reserved for experienced high-volume centres. Funding Acknowledgement Type of funding source: None

Catheter ablation in adults with congenital heart disease: a 15-year perspective from a tertiary center

Abstract Background With the growing population of adults with congenital heart disease (ACHD), the number of catheter ablation procedures is expected to increase over time. Purpose We aimed to describe temporal trends in volume and outcomes of catheter ablation procedures in ACHD patients in a large tertiary center. Methods Retrospective observational study including all consecutive ACHD patients undergoing catheter ablation in a tertiary reference center over a 15-year period. Acute procedural success rate (including complete success in case of non-inducibility of any arrhythmia at the end of the procedure) as well as freedom from recurrence at 12 months were analyzed. Results From November 2004 to November 2019, 302 catheter ablations in 221 ACHD patients (43.6±15.0 years, 58.9% males) were performed. The annual number of catheter ablation increased progressively from 4 to 60 by year (p&amp;lt;0.001). Intra-atrial reentrant tachycardia/focal atrial tachycardia was the most common targeted arrhythmia (n=217, 71.9%). Over the study period, acute procedural success rate increased from 45.0% to 93.3% (p&amp;lt;0.001), including complete acute procedural success from 45.0% to 88.1% (p&amp;lt;0.001) (Figure 1). The use of irrigated catheters (30.0% to 94.8%, p&amp;lt;0.001), 3D-mapping systems (60.0% to 96.3%, p&amp;lt;0.001), contact force catheters (0.0% to 91.9%, &amp;lt;0.001), and high-density mapping (0.0% to 71.9%, p&amp;lt;0.01) increased significantly. Use of irrigated catheters (OR=3.96, 95% CI: 1.79–8.55), 3D-mapping system (OR=3.55, 95% CI: 1.62–7.55), contact force catheters (OR=3.46, 95% CI: 1.71–7.25), and high-density mapping (OR=3.85, 95% CI: 1.60–7.26) were associated with acute procedural success. The rate of freedom from any recurrence at 12 months increased from 29.4% to 66.2% (p=0.001). Seven (2.3%) non-fatal complications occurred. Conclusions The number of catheter ablation procedures in ACHD patients has considerably increased over the last 15 years. Advances in ablative technologies appear to be associated with a low rate of complications and a significant improvement in acute and midterm outcomes. Evolution of acute procedural success Funding Acknowledgement Type of funding source: None

Early extubation following cardiac surgery in adult congenital heart disease improves post-operative haemodynamics and reduces costs

Abstract Background In paediatric cardiac surgery, there has been a paradigm shift in perioperative management from a slow wean of mechanical ventilation in the intensive care unit (ICU), to “ultra fast-track” anaesthesia with early extubation (EE) in theatre to promote a faster recovery. Adults with congenital heart disease (ACHD) have multiple risk factors for prolonged intubation, including a greater proportion of re-do interventions, more co-morbidities and metabolic differences leading to slower emergence from anaesthesia. As a result, EE remains unproven and has not been widely adopted and in this patient group. Aim To assess the effects of EE on post-operative haemodynamics, hospital stay and associated healthcare costs. Methods Data were collected on ACHD patients, who underwent cardiac surgery in a high-volume tertiary centre between 2012 and 2018. Propensity score matching (1:1 or 2:1 where possible) was performed using the following variables: age, sex, body mass index, CHD complexity, functional class, length of surgery, systemic ventricular function, procedure-specific risk (adult congenital heart score; ACHS), urgent versus elective procedure, active endocarditis, pulmonary hypertension and renal dysfunction. Results 614 procedures were performed during the study period. After matching, 87 (14.2%) patients receiving EE were compared to 164 patients who received conventional care (CC). The overall complication rate was low, with no difference between the EE and CC groups (8.0% vs. 9.1%, p=0.77), and a very low reintubation rate (&amp;lt;1%). EE patients had a significantly shorter post-operative hospital length of stay in ICU and the high dependency unit (HDU; 48 [43–51] vs. 50 [47–69] hours, p&amp;lt;0.0001). EE patients required less inotropic &amp; vasopressor support, as demonstrated by a lower Vasoactive-Inotropic Score (VIS) compared to patients following NEE (median VIS 0.5 [0.0–1.8] vs. 2.0 [0.0–3.5], p&amp;lt;0.0001). The total fluid balance by the third post-operative day was more positive after CC than EE (1177±737mL vs. 927±780mL, p=0.004). Finally, lower combined ICU and HDU costs were incurred by EE compared with CC (£3.9K[2.8–4.2K] vs. £4.2K[3.9–6.3K], p&amp;lt;0.0001). Conclusion In ACHD patients undergoing cardiac surgery, including complex and redo procedures, EE was safe, associated with a shorter ITU and HDU stay and lower hospital costs. Funding Acknowledgement Type of funding source: None

Adults with congenital heart disease presenting to the emergency department : Potential pitfalls

Die Patientengruppe der Erwachsenen mit angeborenen Herzfehlern (EMAH) ist mittlerweile bereits größer als die der Kinder mit angeborenen Herzfehlern. EMAH-Patienten weisen auch nach Reparaturoperationen oft komplexe pathophysiologische und anatomische Verhältnisse auf. Bei Komplikationen kann es sehr rasch zu Notfallsituationen auch bei ansonsten asymptomatischen oder nur wenig symptomatischen Patienten kommen. Gemessen an der Gesamtzahl der Patienten, die von Notärzten und in den Notaufnahmen versorgt werden, sind EMAH-Notfallsituationen nach wie vor sehr selten. Diese Übersicht soll die Notfallbetreuung von EMAH-Patienten erleichtern. Für ca. zwei Drittel aller Notfälle sind Rhythmusstörungen und akute Herzinsuffizienz verantwortlich. Rhythmusstörungen müssen in der Regel zügig terminiert werden, weil sie unbehandelt rasch zur kardialen Dekompensation führen können. Bei Scheitern der medikamentösen Therapie oder hämodynamischer Instabilität müssen EMAH-Patienten mit tachykarden Rhythmusstörungen rasch elektrisch kardiovertiert werden. Symptomatische Bradykardien können eine rasche Schrittmacherversorgung erforderlich machen. Aufgrund der komplexen Anatomie kann das Einschwemmen eines transvenösen Interim-Schrittmachers bei einzelnen Vitien unmöglich sein. Die akute kardiale Dekompensation bei EMAH ist oft durch ein akutes Rechtsherzversagen verursacht. Weitere relativ häufige Aufnahmegründe sind Infektionen, Synkopen, Thromboembolien und Aortendissektion. Der Herzpass der Patienten informiert über das vorliegende Vitium. Die umgehende Kontaktaufnahme mit dem behandelnden EMAH-Zentrum wird dringend empfohlen.

Adult Congenital Heart Transplantation: Learning to Surf This Growing Wave?

Adult patients with congenital heart disease (ACHD) undergoing transplantation are a high-risk population. Based on current estimates, nearly 90% of children with repaired CHD survive into adulthood, approximately at 1 million.1 According to the most recent International Society of Heart and Lung Transplantation report, 3.3% of adult heart transplantations are done in CHD patients, representing a growth of over 40% in the past 2 decades.2,3 In this issue of The Annals of Thoracic Surgery, the current report by Kainuma and colleagues4 tries to study the differences between ACHD and non-ACHD patients with previous sternotomies undergoing heart transplantation.

Hospitalizations in adult patients with congenital heart disease: an emerging challenge.

The vast majority of patients with congenital heart disease (CHD) survive into adulthood, but many face lifelong complications, which often result in a hospital admission. The increasing number of hospitalizations in adults with CHD (ACHD) poses a significant challenge for healthcare systems globally, especially as heart failure (HF) is becoming increasingly common in this population and is the leading cause of morbidity and mortality. Besides HF, other major contributors to this increase in admission volume are hospitalizations related to mild lesions, comorbidities and pregnancies. Ιn-hospital mortality ranges between 0.8 and 6.1%, while hospitalizations related to HF predict medium-term mortality in ACHD population. Understanding the predictors of hospitalization and in-hospital mortality is, therefore, important for ACHD healthcare providers, who should identify patients at risk that require escalation of treatment and/or close monitoring. This article reviews the available literature on hospitalization patterns in ACHD patients, with a focus on HF-related hospital admissions and specific diagnostic subgroups.

Risk prediction models for heart failure admissions in adults with congenital heart disease

BackgroundHeart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH. MethodsData source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18–64 (1995–2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18–64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios. ResultsThe cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance. ConclusionsOne eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH.

Heart transplantation at a single tertiary adult congenital heart disease centre: Too little, too late?

BackgroundHeart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplantation can be an effective therapy for them, albeit unfavourable anatomy, end-organ damage, pulmonary vascular disease, HLA sensitization and lack of robust selection criteria currently limit its application. MethodsConsecutive CHD patients considered for heart or combined heart and lung transplantation at our tertiary ACHD centre between 2000 and 2018 constitute our study population. Baseline characteristics and outcome, including transplantation and death, were obtained for all patients from designated databases, medical records and the UK Office for National Statistics. ResultsFrom a total of more than 9000 active ACHD patients under follow-up, 166 (median age 40.4 years) fulfilled inclusion criteria, with a broad spectrum of underlying diagnosis: univentricular heart 22.3%, systemic right ventricle 22.3%, systemic-to-pulmonary shunts and Eisenmenger syndrome 16.3%, left sided valvular lesions 14.5%, tetralogy of Fallot 12.7%, CHD associated with cardiomyopathy 4.8% and other 7.2%. There was a high overall mortality with 39.2% of patients dying over a median follow-up of 2.7 years. A minority of patients (22.9%) were eventually listed and only 13.3% from the 166 patients were actually transplanted. Survival at 1 year after transplantation was 81.8% and remained high long-term (65.5% at 15 years). We describe herewith in detail characteristics and outcome of our cohort and present the transplantation pathway followed. ConclusionOf the small number of patients with ACHD considered for heart transplantation at a large tertiary centre, there was high overall mortality, with only a fraction of patients being actually transplanted. Patients who received transplantation, however, had a good outcome. Better patient selection and timing are clearly warranted so that more ACHD patients are considered and potentially benefit from this effective form of therapy.

Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance

BackgroundIn adult patients with congenital heart disease (ACHD), both underlying disease and lung restriction contribute to exercise intolerance. In ACHD the yet incompletely understood mechanism underlying restricted ventilation may be inspiratory muscle weakness. Therefore, this study comprehensively evaluated inspiratory muscle function in ACHD and associations with systemic inflammation and the clinical severity of exercise intolerance. Methods30 ACHD patients (21 men, 35 ± 12 years) and 30 healthy controls matched for age, gender and body mass index underwent spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Six-minute walking distance (6MWD) and New York Heart Association functional class were used to quantify exercise intolerance. Interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) levels were measured using enzyme-linked immunosorbent assays. ResultsACHD patients showed lower forced vital capacity (FVC), and maximum inspiratory (PImax) and expiratory (PEmax) pressures compared with controls (all p < 0.05). On ultrasound, ACHD patients showed a lower diaphragm thickening ratio (2.3 ± 0.5 vs. 2.8 ± 0.9, p < 0.01) and lower diaphragm excursion velocity during a voluntary sniff maneuver (5.7 ± 2.2 vs. 7.6 ± 2.0 cm/s, p < 0.01). Respiratory parameters, such as FVC (r = 0.53; p < 0.01) and PImax (r = 0.43; p = 0.02), correlated with 6MWD. Furthermore, amino terminal pro B-type natriuretic peptide levels were inversely correlated with FVC (r = −0.54; p < 0.01). Circulating pro-inflammatory cytokines were markedly increased, and IL-6 was correlated with 6MWD, dyspnea, and biomarkers of heart, lung and inspiratory muscle function (all p < 0.05). ConclusionsOur findings show that diaphragm dysfunction is present in ACHD and relates to restrictive ventilation disorder and exercise intolerance, possibly mediated by increased IL-6 levels.

Triage and management of the ACHD patient with COVID-19: A single center approach

With the recent emergence of SARS-CoV-2 and COVID-19, healthcare facilities and personnel are expected to rapidly triage and care for patients with even the most complex medical conditions. Adults with congenital heart disease (ACHD) represent an often-intimidating group of complex cardiovascular disorders. Given that general internists and general cardiologists will often be asked to evaluate this group during the pandemic, we propose here an abbreviated triage algorithm that will assist in identifying the patient's overarching ACHD phenotype and baseline cardiac status. The strategy outlined allows for rapid triage and groups various anatomic CHD variants into overarching phenotypes, permitting care teams to quickly review key points in the management of moderate to severely complex ACHD patients.