Moderate CHD Research Articles

Atrial flutter/fibrillation in adult congenital heart disease: Insights from a large Asian cohort

BackgroundThe incidence of atrial fibrillation/atrial flutter (AF/AFL) in general population is lower in Asia compared to Western countries. It is unclear whether a similar trend exists among adults with congenital heart disease (ACHD). We determine the profile, risk factors, and impact of AF/AFL in an Asian ACHD cohort. MethodsWe included all ACHD patients diagnosed in an Asia tertiary care center between 2007 and 2018, analyzing AF (sustained and paroxysmal AF) and AFL, collectively./Purpose. ResultsThe study encompassed 4391 patients (55.9% women), with 81% having simple, 16.3% moderate and 2.8% severe CHD. AF/AFL was observed in 6.7% of the patients, with 54.6% having paroxysmal AF, 27.3% sustained AF, and 18.1% AFL. Incidence of AF/AFL increased with age and was higher in patients with pulmonary hypertension (PH), severe CHD, and metabolic syndrome. We found a progressive trend in the onset age of arrhythmia: AFL at a younger age, followed by paroxysmal and sustained AF. Risk factors for AF/AFL included severe and moderate CHD, PH, previous interventions, and male sex (odds ratio 11.2 and 3.15, 2.03, 1.75, and 1.71, respectively). When stratifying by CHD severity, PH and male sex were significant risk factors in simple CHD, while only PH in severe CHD. Patients with AF/AFL had a significantly lower major adverse cardiovascular events-free survival rate. ConclusionsThis large ACHD cohort from Asia exhibited a high incidence of AF/AFL, similar to Western reports. The risk of AF/AFL was primarily associated with hemodynamic factors such as PH and CHD severity.

Comparison in the adult congenital heart disease severity classification of ACC/AHA and ESC guidelines in a 3,459 Mexican population

BackgroundLatin American registries of clinical and demographic profiles of ACHD are scarce. International guidelines classify disease complexity with different approaches. With these two regards, a registry was carried out to examine factors associated with mortality and to compare severity classifications in our population. Methods and resultsCross-sectional study conducted on ACHD between 2018 and 2022 to evaluate clinical and demographic characteristics and to assess the agreement between the 2020 ESC Guidelines and 2018 AHA/ACC Guidelines for the Management of Adults with Congenital Heart Disease using the kappa method. Binomial logistic regression models were used to examine correlates of mortality. 3459 patients were included [56 % women, median age 34 years (IQR 24–50)]; 83.41 % were alive and 4.11 % died. The subjects had the following characteristics: 74.18 % were in NYHA I FC, 87.30 % had SVEF ≥50 %, 18.42 % developed arrhythmias, 58.92 % were surgically repaired, 7.05 % received palliative management, and 0.03 % were in heart transplant protocol. The agreement between ESC and AHA/ACC complexity classifications was low (43.29 %) in moderate ACHD, and high (83.10 %) in severe disease. Mortality was higher in patients with NYHA III-IV FC, arrhythmias and under palliative care. ConclusionThis study found that ESC and AHA/ACC complexity classifications have limited concordance in categorizing moderate complexity CHD. Reparative procedures had lower mortality odds than palliative care.

Perfusion index in newborns with CHD without clinical signs of hypoperfusion and heart failure: comparison with healthy newborns.

Peripheral perfusion index has been proposed as a possible method for detecting circulatory impairment. We aimed to determine the normal range of peripheral perfusion index in healthy newborns and compare it with that of newborns with CHD. Right-hand saturation and right-hand peripheral perfusion index levels were recorded, and physical examination and echocardiography were performed in newborns who were 0-28 days old and whom were evaluated in our paediatric cardiology outpatient clinic. The saturation and peripheral perfusion index levels of newborns with normal heart anatomy and function were compared with those of newborns with CHD. Out of 358 newborns (238 mature and 75 premature) enrolled in the study, 39 had CHD (20 mild CHD, 13 moderate CHD, and 6 severe CHD), of which 29 had CHD with left-to-right shunting, 5 had obstructive CHD, and 5 had cyanotic CHD. No newborn had clinical signs of hypoperfusion or heart failure, such as prolonged capillary refill, weakened pulses, or coldness of extremities. Peripheral perfusion index level was median (interquartile range) 1.7 (0.6) in healthy newborns, 1.8 (0.7) in newborns with mild CHD, and 1.8 (0.4) in newborns with moderate and severe CHD, and there was no significant difference between the groups regarding peripheral perfusion index level. Peripheral perfusion index remains unchanged in newborns with CHD without the clinical signs of hypoperfusion or heart failure. Larger studies with repeated peripheral perfusion index measurements can determine how valuable this method will be in the follow-up of newborns with CHD.

Temporal Changes in Clinical Characteristics and Outcomes of Adults With Congenital Heart Disease

The purpose of this study was to assess differences in the clinical characteristics (defined by congenital heart disease [CHD] anatomic and physiologic classification scheme) of adults with CHD across different eras, and how these differences influence outcomes (heart failure hospitalization and all-cause mortality). Patients were divided into depending on year of baseline encounter: cohort #1 (1991-2000, n=1,984 [27%]), cohort #2 (2001-2010, n=2,448 [34%]), and cohort #3 (2011-2020, n=2,847 [39%]). Patients were classified into 3 anatomic groups (simple, moderate, and complex CHD) and 4 physiologic stages (stage A-D). There was a temporal increase in the proportion of patients in physiologic stage C (17% vs 21% vs 24%, P < .001), and stage D (7% vs 8% vs 10%, P=.09), with a corresponding decrease in physiologic stage A (39% vs 35% vs 28%, P < .001). No temporal change in anatomic groups. There was a temporal decrease in the incidence of all-cause mortality (12.7 vs 10.6 vs 9.5 per 1,000 patient-years, P < .001). However, there was a temporal increase in the incidence of heart failure hospitalization (6.8 vs 8.4 vs 11.2 per 1,000 patient-years, P < .001). CHD physiologic stage (but not anatomic groups) was associated with heart failure hospitalization and all-cause mortality. There is a need for better strategies to identify and treat heart failure, and to modify the risk factors associated with heart failure and all-cause mortality.

Very high power- short duration radiofrequency ablation in patients with congenital heart disease and atrial fibrillation: safety and outcome

Abstract Funding Acknowledgements Type of funding sources: None. Background It has been shown recently that ablation of paroxysmal and persistent atrial fibrillation (AF) using very high-power-short duration (vHPSD) ablation protocol is safe and effective. Data for vHPSD ablation protocol in adult patients with congenital heart disease (ACHD) are lacking. We searched our database for ACHD patients undergoing AF ablation using vHPSD ablation regarding periprocedural safety and outcome. Methods The data of 62 consecutive ACHD patients with mild (n=43; 69.4%), moderate (n=15; 24.2%) or complex (n= 4; 6.4%) CHD were analysed (Table 1 ). Mean age was 60,43 ± 12,7 years ( 46% male) with a Cha2ds2-VASc2 Score median of 2. Patients were diagnosed with paroxysmal AF (n= 24; 38%) or persistent AF (n= 38; 61%). In 6 patients (9,6 %), a previous conventional pulmonary vein isolation (PVI) had been performed. Settings used for vHPSD ablation were 70 W/5-7 s or 60 W/7-10 s using irrigated-tip catheters. Circumferential PVI was performed in all 62 patients; in 30 patients (48%) additional substrate ablation was performed. The 30-day incidence of adverse events was analyzed and patients followed-up in regular visits at our outpatient clinic using repetitive 7 days Holter ECGs every 3 months. Results No adverse events such as cardiac tamponade, pericardial effusion &amp;gt;10mm, transient ischemic attack/stroke, atrio-esophageal fistula, cardiac arrest or death occurred. Vascular access complications were detected in 7/62 patients (11%), including 4 patients with a need of intervention (surgical n=2, thrombin injection n=2). Complete PVI was performed in all patients (mean procedure time 129 ± 42min, mean fluoroscopy time 9,09 ± 5.44 min. Radiofrequency (RF) time was 127.27 ± 7,25 min (111,43 ± 27,83 min PVI only, 148,76 ± 46,07 min PVI + substrate ablation). After a mean follow up of 249 days, freedom of any atrial arrhythmia off antiarrhythmic drugs (AAD) after a single ablation procedure was present in 26/47 patients (55.3%). Success rate was significantly higher in patients with paroxysmal AF (n=16/18; 88%) compared to patients with persistent AF (n=10/29; 34.5%; p&amp;lt; 0.001; Figure 1) Conclusion: In this first study using vHPSD in ACHD patients of variable complexity with paroxysmal or persistent AF, the technique was safe and effective. Major periprocedural complications were rare and limited to vascular access problems. No vHPSD ablation modality-related complications occurred. vHPSD was fast in all patintes regardless of CHD complexity. Results in the paroxysmal CHD group were excellent and encouraging for persistent AF.

Adults with CHD balancing motivations and concerns in pregnancy decision-making.

While the incidence of pregnancy has increased among individuals with adult CHD, little has been described about considerations and experiences of patients with adult CHD regarding pregnancy. We aimed to explore patients' motivations, concerns, and decision-making processes regarding pregnancy. In April 2019-January 2020, we conducted in-depth telephone interviews with patients (n = 25) with simple, moderate, or complex adult CHD, who received prenatal care at the University of Washington during 2010-2019 and experienced a live birth. Transcripts were analysed using thematic analysis. Participants described motivations for pregnancy as both internal desires (motherhood, marriage fulfillment, biological connection, fetal personhood, self-efficacy) and external drivers (family or community), as well as concerns for the health and survival of themselves and the fetus. Factors that enabled their decision to maintain a pregnancy included having a desire that outweighed their perceived risk, using available data to guide their decision, planning for contingencies and knowing their beliefs about termination, plus having a trusted healthcare team, social support, and resources. Factors that led to insurmountable risk in subsequent pregnancies included desire having been fulfilled by the first pregnancy, compounding risk with age and additional pregnancies, new responsibility to an existing child, and reduced healthcare team and social support. Understanding individuals' motivations and concerns, and how they weigh their decisions to become or remain pregnant, can help clinicians better support patients with adult CHD considering pregnancy. Clinician education on patient experiences is warranted.

Algorithmic complexity stratification for congenital heart disease patients

BackgroundCongenital Heart Disease (CHD) encompasses a huge variety of rare diagnoses that range in complexity and comorbidity. To help build clinical guidelines, plan health services and conduct statistically powerful research on such a disparate set of diseases there have been various attempts to group pathologies into mild, moderate, or severe disease. So far, however, these complexity scores have required manual specialist input for every case, and are therefore missing in large databases where this is impractical, or quickly outdated when guidelines are revised. MethodsWe used the up-to-date European Society of Cardiology guidelines to create an algorithm to assign complexity scores to CHD patients using only their diagnosis list. Two CHD specialists then independently assigned complexity scores to a random sample of patients. ResultsOur algorithm was 96% accurate where both specialists agreed on a complexity score; this occurred 68% of the time overall, and 79% of the time in moderate or complex CHD. The algorithm “failed” mainly when diagnoses were insufficiently specific, usually for septal defects (where size was unspecified), or where complexity depends on the procedure performed (e.g. atrial/arterial switch for transposition of the great arteries). ConclusionsWe were able to algorithmically determine the complexity scores of a majority of patients with CHD based on their diagnosis list alone. This could allow for automatic complexity scoring of most patients in large CHD databases, for example our own Registry of the Congenital Heart Alliance of Australia and New Zealand. This will facilitate targeted research into the management, outcomes and burden of CHD.

Abstract 13115: Impact of Socioeconomic Status and Remoteness of Residence on Access to Cardiac Care for Pediatric Heart Disease in Alberta, a Jurisdiction of Universal and Centralized Cardiac Healthcare

Introduction: Socioeconomic status (SES) and remoteness of residence (RoR) impact access to care and outcomes in congenital (CHD) and acquired (AHD) pediatric heart disease. Whether the universal, centralized Canadian healthcare system mitigates such inequities is unclear. We examined associations between SES and RoR on age at cardiac diagnosis (ACD), time to first intervention (TFI), and annual primary care (PCV) and cardiology (CV) visits as evidence of healthcare access in Alberta. Methods: All children born and diagnosed in Alberta with CHD or AHD from 2005-2017 were included. CHD was classified as mild, moderate, and severe base on the Bethesda Task Force definitions. Geospatial modelling was used to determine drive times (&lt;60, 60-180, &gt;180 minutes) to 1 of 2 provincial cardiac programs. Cox proportional hazards regression models were used to examine relationships between RoR and SES with ACD and TFI, and Poisson models using generalized estimating equations for annual PCV and CV. Results: Of 12,542 children, 9347 had mild, 1733 moderate and 717 severe CHD and 745 AHD. Most (8,833, 70.4%) lived &lt;60 minutes to a cardiac center, and the largest proportion were in SES quintile 1 (least vulnerable, 3,074, 24.5%) and smallest in quintile 5 (most vulnerable, 2003, 16.0%). Median ACD was 1(IQR 0-108) days for mild, 0(0-54) days for moderate, and 0(0-0) days for severe CHD, and 340(41-1312) days for AHD. Intervention occurred in 1086(62.7%) with moderate CHD (TFI 63(7-150) days), 588(82.0%) with severe CHD (11(5-58) days), and 42 with AHD (1828(793-2866) days). ACD was impacted slightly by SES and only in AHD (Table). TFI and PCVs were not impacted by RoR or SES. Finally, CVs were inversely related to RoR for CHD and AHD. Conclusions: In Alberta, SES and RoR do not importantly impact ACD, TFI and PCVs among CHD and AHD patients. Greater RoR but not SES, however, is associated with fewer annual CVs, suggesting a need to optimize cardiac outreach services.

Non-VKA Oral Anticoagulants in Adult Congenital Heart Disease: a Single-Center Study.

Non-VKA oral anticoagulants (NOACs) prescription is increasing in adults with congenital heart disease (ACHD). However, data on efficacy and safety in ACHD is unclear, particularly in severe CHD. The study aimed to review the safety and efficacy of NOACs in ACHD. Retrospective evaluation of ACHD patients started on NOACs from 2014 to 2020, with the primary endpoints of bleeding or thromboembolic events (TE). CHA2DS2-VASc and HAS-BLED scores were calculated, mortality was assessed, and risk factors for bleeding were identified. A total of 93 patients were included, the mean age was 52 ± 15years, 58% were female, 55.9% had moderate CHD, and 23.7% had severe CHD (3.2% Fontan). Most (66%) had a CHA2DS2-VASc score ≥ 2 and 82% HAS-BLED ≤ 2. In a median follow-up of 41 (IQR 21) months (400.4 patient-years), there were TE in two patients. The annual risk for TE was 0.49%/patient/year. The cardiovascular mortality was 2% and all-cause mortality 5%; there were no fatal TE or bleeding events. Minor (n = 6, 6.5%) and major (n = 3, 3.2%) bleeding events were observed, a median of 12 (IQR 15) months after starting NOAC therapy. The annual risk for bleeding was 2.2%/patient/year. Renal disease (HR 14.6 [95% CI 1.23-73.6], p = 0.033) and the HAS-BLED score were predictors of major (adjusted HR 6.97 [95% CI 1.69-28.78], p = 0.007) and minor (adjusted HR 3.80 [95% CI 1.48-9.78], p = 0.006) bleeding complications. In this real-life cohort of selected ACHD, the use of NOACs was safe and effective, with a low incidence of bleeding events.

Catheter ablation in adult congenital heart disease on uninterrupted oral anticoagulation: Is it safe? Data from a large single-center study.

Catheter ablation in adult congenital heart disease (ACHD) patients is a critical treatment strategy for complex arrhythmias including atrial fibrillation (AF) and atrial tachycardia (AT). In addition to vitamin K antagonists (VKAs), direct oral anticoagulants (DOACs) are increasingly used in this patient population. The purpose of this study was to assess the safety of catheter ablation in ACHD patients on uninterrupted oral anticoagulation with VKA or DOAC, examining thromboembolic, bleeding, and vascular access complications. Retrospective analysis of 234 ACHD patients with simple (n = 83), moderate (n = 66), or complex (n = 85) CHD (mean age 46 years) undergoing 368 ablation procedures on uninterrupted oral anticoagulation with VKA (45.4%) or DOAC (54.6%) was undertaken. Arrhythmias were AF in 97, right AT in 181, left AT in 65, or a combination of AF and AT in25. No thromboembolic complications occurred. Major complications occurred in 4 patients (1.1%; 1 VKA, 3 DOAC), including retroperitoneal hematoma in 2 and arteriovenous (AV) fistula requiring surgical treatment in 2. Minor bleeding or vascular access complications occurred in 46 cases (12.5%), including hematomas >5 cm in 26, AV fistulas (not requiring surgical intervention) in 13, and pseudoaneurysms in 7 (thrombin injection in 3/7). Overall, no significant difference was found between DOAC (14.9%) and VKA groups (12.0%; P = .411). Catheter ablation in ACHD patients on uninterrupted oral anticoagulation with VKA or DOAC is feasible and safe. No thromboembolic events occurred, and major bleeding or vascular access complications were rare. No significant differences regarding minor bleeding or vascular access complications between patients on DOAC or VKA were found.

Pregnancy outcomes in women with severe congenital heart disease – a specialized centre experience

Abstract Introduction Progress in pediatric cardiology and cardiac surgery has dramatically raised the number of women with severe complex congenital heart disease (SC-CHD) that reach reproductive age. Pregnancy (P) in this group of women has an increased risk of adverse cardiac and neonatal events and its predictive factors are not fully defined. Our purpose was to assess the experience of our center regarding P and neonatal outcomes in women with SC-CHD. Methods Retrospective analysis of obstetric data in women with CHD followed at our institution. P and neonatal outcomes were evaluated regarding patients presenting SC-CHD and mild and moderate complexity CHD (MMC-CHD), according to ESC guidelines CHD complexity classification. Results The study enrolled 680 women with CHD (median age, 27.2 years) who experienced 1262 pregnancies, resulting in 998 live births. A previous corrective procedure was found in 334 women (49.1%). CHD complexity was considered mild, moderate and severe in 263 (38.7%), 359 (52.8%) and 58 (8.5%), respectively. SC-CHD included 38 women with cyanotic CHD and 18 with pulmonary vascular disease. The most common heart defect in SC-CHD pts was cyanotic tetralogy of Fallot (19.0%), followed by dextro-transposition of the great arteries with atrial switch /palliative procedure (13.8%) – Figure 1. Pts with SC-CHD had successful deliveries in 56.1% comparing with 82.1% in MMC-CHD. Women with SC-CHD had significantly higher incidence of preterm birth (24.6% vs 7.4%, p&amp;lt;0.001), spontaneous abortion (26.3% vs 12.3%, p=0.004) and neonatal mortality (10.3% vs 2.3%) comparing with MMC-CHD. Low birth weight was also extremely more frequent in the SC-CHD group (44.7% vs 8.5%, p&amp;lt;0.001). No difference was found relating to the presence of CHD in infants from SC-CHD mothers compared to off-spring from MMC-CHD (8.4 vs 5.3%, p=0.407). Cesarian deliveries had similar rates independently of increased CHD complexity (34.2% vs 32.1%). Overall, pregnancy was quite well tolerated, although cardiac complications were more common in SC-CHD P (0.4% vs 4.3%, p 0.013). Only one maternal death was registered, during 1st trimester, in a woman with a large unrepaired ventricular septal defect and cyanosis. Conclusion Severe complex CHD remains a challenging condition for pregnancy with increased maternal and neonatal morbimortality. This emphasizes the importance of extensive prepregnancy counselling and centralization of care to address specific risks and requirements of the condition. Funding Acknowledgement Type of funding sources: None. Figure 1. Severe CHD

Impact of atrial tachycardia management on outcomes in adult patients with congenital heart disease

BackgroundAtrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with high morbi-mortality. Atrial tachycardia (AT) including intra-atrial reentrant tachycardia and focal atrial tachycardia are the most prevalent arrhythmias in ACHD. We aimed to compare mid-term outcomes according to arrhythmia management. MethodsAll hospitalizations for AT in ACHD were retrospectively identified from medico-administrative database from September 2006 to September 2016 in two centers. Outcomes of patients with AT managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, stroke, admission for heart failure and death were analyzed during follow-up. ResultsOne hundred and twenty-one ACHD with AT (median age 41 [IQR 32–52] years, male 62%, 41% moderate CHD and 38% complex CHD) were included. Median follow-up was 3.3 years [IQR 1.4–6.2]. Thirty-seven (30.5%) patients underwent DCCV and 84 (69.5%) CA. No significant difference in age, CHD complexity, history of atrial arrhythmia, heart failure, and CHA2DS2-VASC score were observe. Patients undergoing DCCV had higher NYHA class. Atrial arrhythmia recurrence was lower in CA group (Log-rank test, p ​= ​0.045). Most recurrences were AT, but atrial fibrillation was recorded in 35.1% after DCCV and 9.5% after CA (p ​= ​0.001). At the end of follow-up, patients who were initially treated by CA had a significantly lower incidence of severe cardiovascular events (48.6% in DCCV group vs. 16.7% in CA group, HR: 0.46, CI 95%: 0.22–0.93, p ​= ​0.031). ConclusionsA proactive management of AT by CA is associated with better long-term outcomes in ACHD.

Patient evaluation of a virtual visit program for adults with congenital heart disease

Improved longevity for adults with congenital heart disease (ACHD) necessitates regular, longitudinal care for this population. Telemedicine has emerged as a strategy to increase access to subspecialty care. We evaluated patient experience with a virtual visit program in the pre-COVID era to identify patient-centered benefits and limitations. We enrolled patients for 30-minute synchronous videoconferencing virtual visits at our institution between October 2013 and March 2019. All patients were Massachusetts residents. Patients were surveyed and their characteristics were abstracted from electronic medical records. A total of 264 virtual visits were conducted among 174 patients with a median age of 40 years. Patients traveled a median of 70 miles for in-person visits. Many visits were to review patient data (47%), and most individuals had moderate complexity CHD (45%). Patients reported very high satisfaction with a median visit rating of 10. Patients mostly preferred virtual visits when considering convenience and cost. No difference in preference to in-person visits was reported when considering sharing private information, confidence that concerns would be addressed, and overall visit quality. In-person visits were still preferred for personal connections and showing a physical problem. We find that patients are highly satisfied with virtual visits. ACHD programs should consider blended virtual and in-person care. Long-term regulatory provisions will further improve care through the expansion of telemedicine in the post-COVID era.

Abstract 16134: A Multicenter Cross-sectional Study of Dyslipidemia Among Adults in New England With Congenital Heart Disease

Background: Improved survival of individuals living with congenital heart disease (CongHD) has highlighted the importance of acquired atherosclerosis. Hypertension and diabetes are known to affect those with CongHD, compared to the general population. However, the prevalence of dyslipidemia among adults with CongHD is not well described. Objective: To describe the prevalence of dyslipidemia according to 2018 Multisociety Guideline on the Management of Blood Cholesterol and the frequency of lipid screening among adults with CongHD. Methods: We performed a prospective, cross-sectional study of individuals ≥18 years of age seen at four ambulatory congenital cardiology centers within the New England Congenital Cardiology Association regional referral network. Consenting subjects were administered a survey regarding prevalence of cardiovascular risk factors and previous screening. A non-fasting fingerprick sample of capillary blood was obtained from each subject for immediate analysis using a point-of-care lipid analyzer. Results: There were 186 subjects recruited with one subject excluded from analysis due to self-reported fever. Of the remaining 185 subjects, 93 (50.3%) were male. The median age was 30 years (range 18-71). CHD anatomy was classified as simple for 21 (11.4%) subjects, moderate for 101 (54.6%) subjects, and complex for 63 (34.1%) subjects. Of those who responded, 67.9% recalled having their cholesterol levels checked previously. Only 14.8% reported a history of high cholesterol or triglycerides. However, 85 (45.9%) subjects met NCEP definition of dyslipidemia, with 60 (32.4%) having HDL &lt;40 mg/dL and 37 (20.0%) having TC ≥200 mg/dL. TC was significantly higher in the simple CHD group than in the moderate and complex groups (178.4 vs. 170.1 vs. 157.6, p=0.03). Conversely, HDL tended to be lower in complex CongHD compared to simple and moderate CHD (44.1 vs. 46.9 vs. 49.8, p=0.05), although this did not reach statistical significance. Conclusion: Dyslipidemia is highly prevalent among our cohort of adults with CongHD despite less than 15% reporting a prior diagnosis of high cholesterol. Low HDL, was most common, and predominated in those with complex CHD, while high TC more often affected individuals with simple and moderate CHD.

Characteristics of patients with adult congenital heart disease treated by non-specialized doctors: The potential loss of follow-up

BackgroundIn the treatment of adult congenital heart disease (ACHD), the transfer of patients from pediatric cardiologists to ACHD cardiologists is of relevance. However, little is known about the clinical courses of ACHD patients that have been referred by non-CHD-specialized doctors (n-CSDs). MethodsThis retrospective cohort study included 230 patients (average age: 37 ± 15.2 years, male: 97) who were referred to a single specialized ACHD center between April 2016 and July 2019. We compared the characteristics and clinical courses between patients referred by n-CSDs and those referred by CHD-specialized-doctors (CSDs). ResultsOverall, 121 (53%) patients were referred by n-CSDs. Among them, 91 (75%) patients were referred by adult cardiologists. Univariate analysis showed that the patients referred by n-CSDs were older than those referred by CSDs (41.6 ± 16.3 vs. 32.0 ± 12.0 years, p < 0.01), were more likely to have simple CHD, and less likely to have severe CHD (27.0% vs. 12.8% and 16.5% vs. 40.4%, respectively, p < 0.01). Patients referred by n-CSDs were also more likely to have a history of loss of follow-up (16.5% vs. 3.7%, p < 0.01) and to require invasive treatments after referral, including cardiac surgeries and transcatheter interventions (47.9% vs. 26.6 %, p < 0.01). Notably, unintended invasive treatments that were not designated by the referring doctors were more frequently required in patients with moderate complexity referred by n-CSDs (50.0% vs. 23.3%, p = 0.02). ConclusionsPatients with moderate CHD complexity referred by n-CSDs are more likely to require unintended invasive treatments. Referrals to specialized ACHD centers may be most beneficial for these patients.

Health-related quality of life in adult CHD surgical patients in a low middle-income country: a mixed-methods study.

This mixed-methods study aimed to assess health-related quality of life in young adults with CHD following surgery in a low middle-income country, Pakistan. Despite the knowledge that geographic, cultural and socio-economic factors may shape the way health and illness is experienced and managed and consequently determine a person's health-related quality of life, few health-related quality of life studies are conducted in low middle-income countries. This deficit is pronounced in CHD, so there is little guidance for patient care. The study utilised concurrent, mixed methods. Adults with CHD (n = 59) completed health-related quality of life surveys (PedsQLTM 4.0 Generic Core Scale, PedsQLTM Cognitive Functioning Scale and PedsQLTM 3.0 Cardiac Module). Semi-structured interview data were collected from a nested sub-sample of 17 participants and analysed using qualitative content analysis, guided by the revised Wilson-Cleary model of health-related quality of life. The lowest health-related quality of life domain was emotional with the mean score (71.61 ± 20.6), followed by physical (78.81 ± 21.18) and heart problem (79.41 ± 18.05). There was no statistical difference in general or cardiac-specific health-related quality of life between mild, moderate or complex CHD. Qualitative findings suggested low health-related quality of life arose from a reduced capacity to contribute to family life including family income and gender. A sense of reduced marriageability and fear of dependency were important socio-cultural considerations. CHD surgical patients in this low-income country experience poor health-related quality of life, and contributing factors differ to those reported for high-income countries. Socio-cultural understandings should underpin assessment, management and care-partnering with young adults with CHD following surgical correction.

Impact of intra-atrial reentrant tachycardia management on outcomes in adult patients with congenital heart disease

Atrial arrhythmias are common complications in adults with congenital heart disease (ACHD) and are associated with a high morbidity and mortality. Intra-atrial reentrant tachycardia (IART) is the most prevalent arrhythmia in this population. This study aimed to compare outcomes according to arrhythmia management. All hospitalisations for IART in ACHD patients were retrospectively identified from medico-administrative database from September 2006 to September 2016. Outcomes of patients with IART managed with direct-current cardioversion (DCCV) were compared with patients referred for catheter ablation (CA). Atrial arrhythmia recurrence, death, stroke and first admission for heart failure (HF) were analyzed during follow-up. Ninety-one ACHD patients with IART (baseline median age 38 years [IQR 29-50], male 64%, 45% moderate CHD and 41% complex CHD) were included. Median follow-up was 3 years [IQR 1.6-6.7]. Twenty-six (28.6%) patients underwent DCCV and 65 (71.4%) CA. No significant difference in CHD complexity, past medical history of atrial arrhythmia and HF were observed between the 2 groups. Patients who underwent DCCV were significantly older (43 years [IQR 34–51] vs. 36 years [IQR 26–44], P = 0.03), with a higher CHADS-VASC score (CHADS-VASC ≥ 1 in 61% in DCCV group vs. 44% in CA group, P = 0.001). Atrial arrhythmia recurrence was observed in 52% after CA and 65% after DCCV ( Fig. 1 A, P = 0.28). Most of recurrence were IART, but atrial fibrillation was recorded in 37.5% after DCCV and 25.8% after CA ( P = 0.4). At the end of follow-up, patients who were initially treated by CA were more frequently in sinus rhythm (84% vs. 27%, P = 0.02), with a significant lower incidence of severe cardiovascular events ( Fig. 1 B, P < 0.01). A proactive management of IART by catheter CA is associated with better mid-term outcomes in adult patients with CHD.

Management and Outcome of Infective endocarditis in adults with congenital heart disease

Causes, epidemiology and microbiology of infective endocarditis (IE) have evolved in recent decades. Although novel tools for diagnosis and therapeutic strategies have emerged, mortality and morbidity remained high. These trends may particularly concern the growing population of adults with congenital heart disease (ACHD) who have an increased risk for IE. We aimed to describe causes, management and outcome of IE in ACHD, and to analyze the risk factors for in-hospital death. From 1997 to 2017, 628 consecutive episodes of IE in adults were recorded in our center. Among them, 118 (18.7%) concerned 117 ACHD (mean age, 40 ± 17.7 years, 80.5% male). Endocarditis was classified according to the modified Duke criteria. Overall, 50.8% of patients had a simple CHD, 12,7% a moderate, and 36,4% a complex. A total of 37,3% were right sided IE (97,3% pulmonary site and 2,3% tricuspid valve). It was at least the second episode of IE for 18 patients (15.3%) and a predisposing event could be identified in only 30.3% cases. Oral streptococci and Staphylococcus aureus were the most frequently microorganisms isolated (31.1% and 25,4%, respectively). Surgical intervention was performed in 76 episodes (64,5%). In-hospital mortality was 10.2% and was directly related to IE in 9/12 cases. On univariate analysis, risk factors for death were complexity of CHD (OR = 7.2, 95% CI[1.1–48.2] and OR = 5.3 95% CI[1.0–27.1] for moderate and complex CHD respectively, P ≤ 0.04), numbers of sternotomies ≥ 2 (OR = 5.1, 95% CI[1.2–21.2], P = 0.02), and leukocytes number (OR = 1.1, 95% CI[1.0–1.2], P < 0.01). On multivariate analysis complex CHD lesions were the strongest predictor for death (0.02). Mortality associated with IE in ACHD patients is lower than reported in acquired heart disease. Multidisciplinary approach by IE team and GUCH specialists may have improved management and outcome in this setting. However, risk for death remains high in complex lesions.

Abstract 16935: Severity of Congenital Heart Disease, Not Risk of Acute Coronary Syndrome,Drives Extent of Ischemic Evaluation for Chest Pain in the Emergency Department

Introduction: Population growth and increasing complexity of disease among adults with congenital heart disease (ACHD) has led to an increased burden on U.S. emergency services. Assessment of chest pain (CP) within this population poses a challenge to emergency department (ED) providers; and as this population ages, their cardiovascular risk profile continues to change. There is a paucity of literature describing appropriate assessment of CP among ACHD patients within the ED setting. Hypothesis: Despite very low probabilities for acute coronary syndrome (ACS), ACHD patients are likely to undergo extensive evaluations for CP within the ED, possibly correlating with ACHD complexity. Methods: Retrospective review of ED visits for primary complaint of acute CP among ACHD patients aged 18-50 years (n=182) presenting to a single tertiary center between 1/1/1998 and 4/1/2018. CP quality was classified according to Diamond-Forrester criteria and severity of ACHD according to the Bethesda Classification. Results: The mean age of the cohort was 32.8±8.7 years, 88 were women, 102 were Caucasian, 74 had simple CHD, 58 had moderate severity CHD, and 50 had high complexity CHD. Although 94.5% of patients were identified as having a low 30-day risk of major adverse cardiovascular events according to Heart Score, 86.3% had ≥1 set cardiac biomarkers drawn. Only 6 patients (3%) had an initial ECG with changes concerning for possible ischemia. 3 patients (2%) had troponin levels above the upper limit of normal and only 1 case required urgent catheterization. Among ACHD complexity groupings (simple, moderate severity, and high complexity), there were no differences seen with regard to, gender, frequency of ECG changes suggesting ischemia, chest pain quality or coronary disease risk factors (hypertension, hypercholesterolemia, diabetes, smoking, BMI or predefined cardiovascular disease). Despite this fact, complexity of ACHD was associated with increased ED length of stay (p=0.02), likelihood of an urgent echocardiogram being performed (p&lt;0.001), ordering of cardiology consultation (p &lt;0.001) and hospital admission (p =0.009), without concurrent increase in 30-day overall mortality. Conclusion: ACHD patients undergo extensive evaluation for ACS despite having low pre-test probability, with complexity of underlying congenital disease correlating with increased ED resource utilization despite similar clinical outcomes.

Complex assessment of severity and clinical functional status in adults with congenital heart disease

The aim — to estimate the severity and clinical functional status in adults with congenital heart disease (ACHD).Materials and methods. The study included 456 consecutive adults with CHD who were admitted to Scientific and Practical Medical Center of Pediatric Cardiology and Cardiac Surgery of Health Ministry of Ukraine (Kyiv, Ukraine) from January 1, 2014 to December 31, 2015. The average age was 27.4 ± 0.5 years (from 18 to 67 years), men were 225 (49.3 %), women — 231 (50.7 %). The severity of CHD was assessed using 2 scales: the severity of primary diagnosis (scale 1), and Disease severity index (scale 2); the functional status — by Ability index, (scale 3), Congenital heart disease functional index (scale 4) and NYHA functional class (FC) of heart failure (scale 5).Results and discussion. There were statistically significant differences in FC I, III and IV between complex and simple CHD, in FC II, III and IV between complex and moderate CHD and in FC I, II between moderate and simple CHD by Scale 3. There were statistically significant differences in FC II, IV and V between complex and simple CHD, in FC II­V between complex and moderate CHD and in FC I — III between moderate and simple CHD by Scale 4. The comparative analysis of scale 5 and scale 1 presented the same statistically significant differences compared to scale 3.Conclusions. The preserved functional clinical status of adults with congenital heart disease, absence of limitation of physical activity in everyday life, the opportunity to be engaged in work or study full day should be the main criteria for assessing the health in this cohort of patients, despite the complexity of congenital heart disease, previous surgical and/or percutaneous interventions.