Adult Congenital Heart Disease Centers Research Articles

Fenestrated Transcatheter ASD Closure in Adults with Diastolic Dysfunction and/or Pulmonary Hypertension: Case Series and Review of the Literature.

This study aims to evaluate the safety and efficacy of transcatheter fenestrated ASD closure and to summarize the literature regarding the published techniques and outcomes of transcatheter partial ASD closure. Patients with left ventricular diastolic dysfunction (LVDD) or right ventricular (RV) dysfunction and/or pulmonary hypertension (PHT) may suffer untoward consequences of complete closure of an ostium secundum atrial septal defect (ASD). Therefore, for patients that fall under these categories we suggest partial occlusion of the defect, which may be better tolerated than complete defect closure. After obtaining IRB approval, a search for patients that have undergone percutaneous ASD closure was performed in the Ahmanson/UCLA Adult Congenital Heart Disease Center database to identify which patients received a fenestrated ASD closure device. Eight consecutive patients ranging between 22 and 83 years of age (mean 48 years) with PHT and/or LVDD or RV dysfunction who underwent fenestrated transcatheter ASD closure at UCLA were identified. None of the subjects experienced complications related to the procedure. Postprocedure clinical evaluation showed improvement in symptoms and exercise capacity. Available follow-up transthoracic echocardiography data (mean 4 months, range 0-20 months) demonstrated patent fenestrations in four of eight patients. None of the patients had thromboembolic or infectious complications and there were no device migrations, erosions or embolizations. Partial ASD occlusion in patients with diastolic dysfunction or RV dysfunction and/or PHT is safe and may be better tolerated than complete ASD closure in selected patients.

P1 Diagnosis and stroke risk of atrial fibrillation in patients with atrial septal defects: A single centre experience

Background Atrial fibrillation (AF) is one of the most common sustained tachyarrhythmias. It increases risk of developing stroke by up to five times. There is an increased incidence in patients with adult congenital heart disease (ACHD). The CHA 2 DS 2 VASC scoring system is validated for assessing the risk of ischaemic stroke in AF in the general population. Its role in ACHD is unclear. This study assesses the burden of AF in patients with uncomplicated atrial septal defects (ASD). Methods A retrospective analysis of the ACHD database of all patients at a large quaternary specialist ACHD centre. Results 135 (3%) patients with a diagnosis of ASD and co-existing AF were identified out of 4500 patients under regular follow up at Queen Elizabeth Hospital Birmingham. 101 (75%) secundum ASD, 12 (9%) sinus venosus ASD, 1 (1%) coronary sinus ASD and 21 (15%) unclear. 47 (35%) surgical closures, 28 (21%) percutaneous closure, 57 (42%) unrepaired, 3 (2%) both surgical and percutaneous. There were 7 residual defects post closure. -The mean age was 67.8(+/-12.8) years, range 36–90 years, 65 male (48.1%). -The mean CHA 2 DS 2 VASC score was 2.21 (Range 0–6). -The mean age of onset of AF was 56(+/-12.3) years. In 40% the AF was asymptomatic. -90% of patients with a CHA 2 DS 2 VASC score of 2 or more were anticoagulated with either warfarin or a novel oral anticoagulant (n = 75). 11 patients had suffered a stroke (8.15%). -7 presented initially with a stroke and asymptomatic AF; of these 6 were also found to have ASDs after the stroke. The mean age stroke occurred was 58.7(+/-20) years. The mean CHA 2 DS 2 VASC score for patients who had a stroke was 4.18 (Range 2–6). Discussion The incidence of AF in ASDs is high and is frequently asymptomatic. AF and strokes occur at a young age in this population. A significant proportion of our patients presented for the first time with a stroke and asymptomatic AF. Expert consensus currently advocates screening for arrhythmias in certain ACHD groups but not ASD. This data suggest that patients with ASD should be actively screened for AF. The role of the CHA 2 DS 2 VASC score if unclear in these patients but a high CHA 2 DS 2 VASC does appear to correlate with a higher incidence of stroke.

Nationwide Survey of the Transfer of Adults With Congenital Heart Disease From Pediatric Cardiology Departments to Adult Congenital Heart Disease Centers in Japan.

Current Japanese transfer practices for adult congenital heart disease (ACHD) patients in pediatric departments are elucidated in this study. The focus was on 149 facilities (from the Japanese Society of Pediatric Cardiology and Cardiac Surgery Subspecialty Board and the Japanese Association of Children's Hospitals and Related Institutions). One hundred and thirteen facilities were surveyed and the response rate was 75.8%. Twenty-six facilities (23.0%) treated ≥200 outpatients annually; 48 facilities (42.9%) treated <50 outpatients. Only eight facilities admitted ≥50 inpatients; 61 facilities (54.0%) admitted <10 inpatients. Nine facilities fulfilled international regional ACHD center criteria. The estimated median number of patients receiving outpatient pediatric department follow up was 33,806. Sixty facilities (53.6%) treated patients in pediatric departments after they reached adulthood. Of 49 facilities that transferred patients, the transfer was most commonly to another department in the same facility (20 facilities; 40.8%), typically the adult cardiology department (29 facilities; 59.2%). In future, 55 facilities (48.7%) desired the transfer of patients to regional ACHD centers, while 34 facilities (30.1%) preferred to continue treating patients in the pediatric department. The number of regional ACHD centers offering sufficient outpatient and inpatient care is limited; transfer from pediatric departments is not standard in Japan. Role division clarification between regional ACHD centers and other facilities and cooperative network establishment including transitional care programs is necessary. (Circ J 2016; 80: 1242-1250).

Abstract 12047: Quality of Life in Adults With Congenital Heart Disease Worldwide: A Multilevel Study in 15 Countries

Objectives: Health care should be evaluated not only on the basis of saving lives, but also in terms of improving the quality of life (QOL). However, results regarding QOL in adults with congenital heart disease (CHD) are inconsistent and vary across the world. Methodological differences and limitations hamper the interpretation of these results. Therefore, we aimed (1) to describe QOL in an international sample of adults with CHD using a uniform method; (2) to investigate the association between patient characteristics and variation in QOL; and (3) to explore international variation in QOL and the relationship with country-specific characteristics. Methods: For this cross-sectional multilevel study, patients were recruited from adult CHD centers or national registries from 15 countries. QOL was assessed by a linear analog scale (0-100). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity and patient-reported New York Heart Association (NYHA) class. Country-specific characteristics included national happiness score and six cultural dimensions. Multilevel generalized linear mixed models were conducted and Empirical Bayes estimates for QOL were calculated to account for the sample size in each country. Results: In total, 4,028 adults with CHD were enrolled (median age=32 y; 53% women; 26% mild, 49% moderate, 26% complex CHD). Median QOL for the total sample was 80. Age, marital status, employment status and NYHA class were significantly associated with variation in QOL (p&lt;.001). Australia had the highest QOL estimate (82) and Japan the lowest (72). Four countries had a QOL estimate of ≥80 (i.e., Australia, Switzerland, USA, Malta). After adjusting for patient characteristics, happiness and cultural dimensions were not significantly associated with variation in QOL. Conclusions: This is the first international study comprehensively assessing QOL in any subgroup of cardiac patients. We found that QOL in adults with CHD varies across countries and is related to patient characteristics but not to country-specific characteristics. The identification of significant patient characteristics can support the development of interventions targeting the QOL of adults with CHD around the world.

Dextrocardia in Adults with Congenital Heart Disease

Dextrocardia is rare in the general population, and may be associated with significant additional cardiac malformations. We aimed to identify the prevalence and patterns of additional cardiac defects, as well as the associated long-term morbidity and mortality, in adult patients with dextrocardia, in a specialised Adult Congenital Heart Disease (ACHD) service. A retrospective study of patients with dextrocardia managed by our tertiary ACHD service, since January 2000, was performed. Medical records were reviewed and the National Death Index was consulted to confirm survival status. Of 3698 adults in our ACHD Service, 19 (0.5%) had dextrocardia. Mean follow-up duration was 7±7.5 years. The mean age at last review was 36.8±10.5 years (range 20-63 years). Situs was solitus in 14 (74%) and inversus in five (26%). Eleven patients (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four patients with two ventricles had AV-VA discordance. All patients had at least one additional cardiac malformation. Fourteen patients (74%) required surgical intervention. Eleven patients (58%) underwent a Fontan-type operation. Five patients (26%) required ablation procedures for arrhythmia. One patient had infective endocarditis and two deaths occurred, both in patients who also had AV-VA discordance. Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease. Those with associated congenital heart abnormalities are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that patients surviving to adulthood and then managed in an ACHD centre may have good medium-term survival.

Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre.

Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. We included 6969 adult patients (age 29.9 ± 15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased. ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.

ICD Therapy after Atrial Switch Repair for d-Transposition of the Great Arteries—German Multicenter Experience

Sudden cardiac death is the most common cause of late mortality after atrial switch repair for d-transposition of the great arteries. Experience with ICD therapy in these patients is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in atrial switch patients at adult congenital heart disease centers. Biometrical and clinical data as well as data on device implantation, programming, ICD discharges and complications of 33 patients from four centers were analyzed retrospectively. ICDs were implanted for primary/secondary prevention in 29 (88%) and 4 (12%) individuals, respectively. Median age at implantation was 27.5 years. 30 patients (91%) presented with NYHA ≤II at implantation. All patients with secondary prevention indication were classified as NYHA I. 21 (64%) individuals suffered from atrial reentrant tachycardia (ART). During median follow-up of 4.7 years, 5 appropriate ICD therapies were delivered in 2 (6%) patients with primary prevention indication. 12 inappropriate ICD discharges occurred in 8 patients (24%) due to ART (n = 6) or lead failure (n = 2). ICD related complications occurred in 9 patients (27%): lead dislodgment/failure in 7 patients, ICD infection in 2 (6%) patients. At implantation, heart failure as defined by NYHA classification was not advanced in the majority of patients. Appropriate discharges were only occasionally observed. Lead failure and ART were present in >20% of the patients and were frequent reasons for inappropriate ICD discharges. Facing these results, rigorous treatment of ART seems mandatory.

Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation

ObjectivesTo assess the frequency, anatomic characteristics, and associations of systemic to pulmonary venous collaterals in adult patients undergoing cardiac catheterization after a Fontan operation. Additionally, the embryologic basis for the presence of venous collaterals is reviewed. MethodsCardiac catheterization data was reviewed for 66 adults with single ventricle physiology and a Fontan palliation. ResultsThere were a total of 66 patients that underwent catheterization between 2004 and 2014 at the Ahmanson/UCLA Adult Congenital Heart Disease Center. There were 24 males and 42 females. Systemic venous to pulmonary venous collaterals were present in 38 patients (58%), most commonly originating from the right brachiocephalic vein (35%), azygous vein (20%) and superior vena cava (13%). Trans-catheter interventional closure was performed in 27/38 (71%) of patients with venous collaterals. At baseline these patients had lower oxygen saturation when compared to those not requiring intervention, 85.6%±6.1% vs 89.9%±5.4%, p<0.05. At 6months, the ambulatory systemic saturation improved from 85.6%±6.1% to 91.8%±6.4%, p<0.05. At two years follow-up, the ambulatory systemic saturation had decreased to 90.5%±4.1% (p<0.05). ConclusionIn adults with single ventricle physiology and prior Fontan surgery, systemic venous collaterals are common and can be percutaneously occluded at minimal risk with resultant improvement in systemic oxygen saturation on long term follow up. When evaluated from a developmental standpoint, 85% of collaterals are above the diaphragm and could be secondary to recanalization of the collateral veins, an embryological connection between systemic and pulmonary veins.

Ebstein's Anomaly in Those Surviving to Adult Life – A Single Centre Experience

Ebstein's anomaly (EA) occurs in about one to five per 200 000 live births. Long-term follow-up data of adults with EA is scarce due to the relatively low frequency of the disease and the variation of its anatomic and haemodynamic severity. Since 1995, in our adult congenital heart disease (ACHD) centre, we have practised a uniform approach to management of adults with EA, with surgery reserved for those with refractory arrhythmia (failed medical and/or catheter-based treatment) or worsening symptoms of breathlessness. A retrospective review of medical records of all such patients with EA and normal cardiac connections was performed. Fifty-one EA patients (17 males) were identified. Mean age at diagnosis was 21+/-21 years and mean follow-up time at our centre was 21±14 years. During this time, 18 patients (35%) had documented supraventricular arrhythmia. Sixteen patients (30%) underwent ablation therapy with long-term relief from arrhythmia in nine (56%). Nine patients (18%) underwent tricuspid valve (TV) surgery (four repair and five replacement), with seven patients having undergone a tricuspid valve surgery prior to referral to our unit. Three patients died, one of cardiogenic shock after redo surgery (58 years), one of progressive heart failure (45 years) and one with malignancy. Overall survival was 100% to age 40 years, 95% to age 50 years and 81% to age 60 years. Ebstein's Anomaly in adulthood often has severe morphological abnormalities but is compatible with good medium-term survival, with a generally symptom driven approach to the indications for interventions.

Global Longitudinal Strain May Identify Preserved Systolic Function of the Systemic Right Ventricle

BackgroundAssessment of systemic right ventricle (RV) function is a key point in the follow-up of patients with dextroposition of the great arteries (D-TGA) after the atrial switch procedure. Cardiac magnetic resonance (CMR) is considered the reference standard but is not feasible for a considerable number of patients. Experience with the application of 2-dimensional speckle tracking echocardiography for the assessment of systemic RV function in such patients is very limited, and the cutoff strain value differentiating normal and subnormal RV function is missing. MethodsWe prospectively examined, with echocardiography and CMR, 40 patients with D-TGA (mean age, 25.6 ± 5 years; 25 men; 33 after the Senning, 7 after the Mustard procedure) who attended a tertiary adult congenital heart disease centre. ResultsThere was a linear correlation between global longitudinal strain (GLS) and CMR systemic RV ejection fraction (EF) values (r = 0.4; P = 0.01), systemic RV fractional area change and CMR systemic RV EF (r = 0.41; P = 0.008). There was no correlation between systemic RV s', tricuspid annular plane systolic excursion or the rate of change of the systemic RV pressure with time and CMR systemic RV EF. Only 1 of 19 patients with GLS < −14.2% had CMR systemic RV EF < 45%. The GLS cutoff value of −14.2% predicted the CMR systemic RV EF of ≥ 45% with 90% specificity and 83% sensitivity. Its positive predictive value was 96%. ConclusionsThe systemic RV GLS is able to discriminate between D-TGA patients after the atrial switch with, and those without, a CMR systemic RV EF of at least 45%.

Trends in Utilization of Specialty Care Centers in California for Adults With Congenital Heart Disease

The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

Experiences and Outcomes of Transition from Pediatric to Adult Health Care Services for Young People with Congenital Heart Disease: A Systematic Review.

This review synthesizes the empirical literature on outcomes and experiences of transfer and transition from pediatric to adult care for young people with congenital heart disease. A systematic review of papers published between January 2001 and May 2013 that examined outcomes or experiences of transfer and transition among young people with congenital heart disease was conducted. Data were extracted by two independent reviewers with the outcomes data combined using narrative synthesis and the experiences data integrated using thematic synthesis. Thirteen papers were included in the review: six reported outcomes following transfer, six reported experiences of transfer and transition, and one reported both outcomes and experiences. The review data indicate that high proportions of young people were lost to follow-up or experienced long gaps in care after leaving pediatric cardiology. Factors that protected against loss to follow-up or lapse in care included: beliefs that specialized adult care was necessary; poorer health status; attendance at pediatric appointments without parents; and pediatric referral to an adult congenital heart disease center. Data on experiences highlighted that many young people were unconcerned about transition, but lacked knowledge about their condition and were insufficiently prepared for transfer. In terms of adult services, many young people desired continuity in the quality of care, youth-oriented facilities, a personalized approach, and for their parents to remain involved in their care, but in a secondary, supportive capacity. In conclusion, the high proportions of young people lost to follow-up highlight the need for formal transition programs, which ensure a planned and coordinated transfer. Patients with congenital heart disease need education throughout adolescence about the implications of their condition, the differences between pediatric and adult services, and self-care management.

Abstract 20514: Adults With Hypoplastic Left Heart Syndrome: Outcomes in a New Cohort of Patients

Background: Surgical palliation of hypoplastic left heart syndrome (HLHS) has only been possible for the past few decades. Prior to this, without heart transplantation, HLHS was universally fatal in infancy. The oldest survivors of palliated HLHS are only now entering adulthood and limited data are available regarding their welfare. Methods: For this multi-center, cross-sectional, observational study, 6 adult congenital heart disease (ACHD) centers contributed data regarding all HLHS patients aged &gt;18 years followed at their institution. HLHS was defined as a dominant right ventricle (RV) and diminutive left ventricle with a combination of mitral valve disease (stenosis [MS] or atresia [MA]) and aortic valve disease (stenosis [AS] or atresia [AA]). Patients with single RV physiology without hypoplasia of left heart inlet and outlet structures were excluded. All available clinical data, including cardiac imaging, cardiac catheterization results and exercise tests were reviewed. Results: The study included 53 HLHS adults (65% male) with mean age 21.8±3.6 years. Underlying cardiac anatomy was AA&amp;MA (n=21, 41%), AS&amp;MS (n=19, 37%), AS&amp;MA (n=10, 20%), and AA&amp;MS (n=1, 2%). Stage 1 Norwood was completed at age 6.0±4.4 days, Glenn shunt at age 10.8±8.7 months and Fontan at age 3.3±2 years. Stage 1 cardiopulmonary bypass and circulatory arrest times were 94±46 and 59±2 minutes respectively. The mean duration of follow-up in an ACHD center was 3.4±2.5 years. After age 18 years, major adverse events had occurred in 15/53 patients (28%). These mutually exclusive events were: death (n=3, 6%), transplant (n=1, 2%), protein losing enteropathy (n=2, 4%), stroke (n=2, 4%), symptomatic ventricular tachycardia (n=1, 2%), heart failure requiring admission for intravenous therapy (n=3, 6%) and major cardiac surgery (n=3, 6% [aortic valve replacement n=1, tricuspid valve replacement n=2]). Conclusions: Young adults with a Fontan palliation for HLHS are at high risk of major adverse cardiac events. It is essential that close attention is paid to successful transition of this vulnerable population into adult care and that these patients remain under vigilant specialist follow-up through adult life.

Abstract 11317: Outcomes of Atrial Arrhythmia Surgery for Atrial Tachyarrhythmias in Patients With Repaired Tetralogy of Fallot

Background: Atrial tachyarrhythmia is common in adults with tetralogy of Fallot (TOF) due to surgical scarring from repairs and atrial enlargement. The maze procedure refers to surgical ablation within the right atrium to disrupt arrhythmogenic circuits and is sometimes performed concomitantly during reoperation on repaired TOF patients. Our study aims to evaluate the effectiveness of maze in TOF patients. Methods: We performed a retrospective chart review that identified 30 TOF patients who underwent a pulmonary valve replacement (PVR) with maze and 38 TOF patients who underwent a PVR without maze from 1994 to 2011 and had at least 2 years of post-surgical follow-up at the Ahmanson/UCLA Adult Congenital Heart Disease Center. Preoperative and postoperative arrhythmia status and management were compared in maze and non-maze groups. Results: Before the procedure, the most common pre-operative arrhythmias in the maze group were a history of atrial fibrillation (AFib)(n=16), atrial flutter (AFL)(n=10), and other supraventricular tachycardia (SVT)(n=6). Isolated right atrial maze was performed in 26 patients, and combined right and left atrial maze-cox procedure was performed in 4 patients. Of the 16 patients in the maze group with pre-op Afib, 6 had recurrent Afib within the first 2 years of follow-up (62.5% relative reduction, p=0.012). Of the 10 patients with pre-op AFL, only 1 had recurrence (90% relative reduction, p=0.012). Of the 6 patients with pre-op SVT, 4 had recurrence (33.3% relative reduction, p=0.727). There was no significant arrhythmia status change in the non-maze group at 2 years. Comparing patients with and without maze, the average cardiopulmonary bypass times were 155 minutes and 97 minutes, respectively (p=0.064), and aortic cross clamp times were 122 minutes and 64 minutes, respectively (p=0.004). On average, patients with maze spent 3.7 more days in the hospital compared to those without maze (p=0.001). Conclusions: Performing a concomitant maze procedure in patients undergoing TOF repair was associated with a moderate improvement in atrial tachyarrhythmia burden over 2 years. TOF patients who had the concomitant maze procedure required longer cardiopulmonary bypass time, aortic cross clamp time, and total hospital stay.

Circulation : Clinical Summaries

<i>Circulation</i> : Clinical Summaries

Specialized Adult Congenital Heart Disease Care

Background— Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality. Methods and Results— We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case–control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, −5.0%;95% CI, −10.8% to −0.8%). In exploratory post hoc cohort and case–control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65–0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08–0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22–0.67). Conclusions— A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.

Long-term natural history and postoperative outcome of double-chambered right ventricle—Experience from two tertiary adult congenital heart centres and review of the literature

BackgroundDouble-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature. Results50 patients (32 female, median age 39years [IQR: 27;53]) with DCRV under follow-up could be identified. A retrospective review of medical records was performed. Almost all patients (96%) had ventricular septal defects as underlying diagnosis. Eight patients remained completely asymptomatic during follow-up and have been managed conservatively thus far. The remaining patients developed symptoms at a median age of 26years. Surgical correction was performed in 33 patients (median age at operation 27years). No residual intraventricular gradient was present at the latest follow-up in 91% of operated patients and functional class improved significantly with only 6 patients remaining in NYHA class 2. There was no early or late operative mortality and no patient required re-operation for DCRV during a median follow-up of 8years. ConclusionsContemporary adult DCRV patients have good survival prospects and low long-term morbidity. Despite occasionally presenting with considerable intraventricular gradients we could not identify any case of sudden death in our unoperated adult sub-population. Although asymptomatic adults may be encountered even with severe obstruction, symptom development is common during adult life. Cardiac surgery in this cohort is inherently low risk and offers good long-term haemodynamic and functional results justifying early intervention in consideration of the progressive nature of this rare congenital lesion.

Choice and Competition Between Adult Congenital Heart Disease Centers

Although concentrating adult congenital heart disease services at high-volume centers has been widely advocated, the potential beneficial effects of competition and patient choice have received relatively little attention. We aimed to assess the degree of patient choice and competition between adult congenital heart disease units and to investigate whether competition indices correlate with clinical quality or research output. Competition between the 10 major adult congenital heart disease units in England was evaluated based on the Herfindahl-Hirschman Index, representing the sum of squared market shares of individual units. In addition, to account for geography and feasible access, we calculated spatial indices of competition based on travel time by road. These indices were correlated with 30-day mortality postpulmonary valve replacement in adult patients (as obtained from the National Central Cardiac Audit Database) and the aggregate research impact factors of individual centers. On a national level, a high level of competition without obvious dominant players was found (Herfindahl-Hirschman Index between 0.107 and 0.013). When accounting for geography, however, important disparities in patient choice and competition faced by individual centers emerged. The degree of local competition was correlated significantly with clinical outcomes and research output. In contrast, no association between center volume and outcome could be established. Beyond the usual focus on concentrating services at high-volume centers, the potentially beneficial effects of competition should not be ignored. Therefore, policymakers should consider fostering a competitive environment for adult congenital heart disease centers or at least avoiding creating government-granted monopolies in the field.

Use of Supine Bicycle Stress Echocardiography in Quantitating Right Ventricular Reserve and Exercise Capacity in Adults with Repaired Tetralogy of Fallot

Objective: This study aimed to examine exercise hemodynamic parameters and exercise capacity in adults with repaired tetralogy of Fallot (rTOF) obtained during supine bicycle echocardiography. Background: In addition to volumetric MRI parameters, exercise capacity in adults with rTOF is often used to determine timing for pulmonic valve replacement (PVR). Supine bicycle echocardiography is useful in measuring right ventricular (RV) hemodynamic parameters and quantifying exercise capacity and myocardial workload. Methods: Supine bicycle echocardiograms performed since 2002 were retrospectively reviewed among 33 adults with rTOF followed at a single adult congenital heart disease center. Echocardiographic and hemodynamic parameters were measured before and at peak exercise. Results: Mean age was 36 ± 12 yrs and severe pulmonic regurgitation (PR) was present in 56%. Mean exercise duration was 15 ± 5 minutes and peak double product was 24,461 ± 6779 units. There was no correlation between resting right ventricular volumetric measurements or tricuspid regurgitant velocity with exercise duration or peak double product. Symptomatic patients in this study had significantly shorter exercise times (17.1 ± 4 vs 12.1 ± 4 minutes, p=0.01) and a trend toward a significantly lower change in the mean pulmonary gradient with exercise (13 ± 8 mmHg vs. 7 ± 2 mmHg, p=0.07). After multivariable linear regression analysis adjusting for age, sex, and severity of PR, a 7 mmHg increase in the ΔPVGp was independently associated with an additional minute of exercise duration (p=0.01). Conclusion: Obtaining exercise right heart hemodynamics with supine bicycle echocardiography is feasible among adult patients with rTOF. Change in transpulmonary valvular gradient with exercise, may reflect RV contractile reserve and provide a physiologic correlate to symptoms and exercise capacity in patients with rTOF

Usefulness of the Seattle Heart Failure Model to Identify Adults With Congenital Heart Disease at High Risk of Poor Outcome

Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at high versus low risk for cardiovascular outcomes and poor exercise capacity. The ACHD population is growing and presents increasingly for care in the community and at tertiary centers. Few strategies exist to identify the patients with ACHD at high risk for heart failure and mortality.We studied 153 adults with transposition of the great arteries, Ebstein anomaly, tetralogy of Fallot, double outlet right ventricle, and single ventricle from 2 ACHD centers. The primary outcome was cardiovascular death, with a secondary composite outcome of death, transplant, ventricular assist device, cardiovascular admission, and treatment for arrhythmia. We defined risk groups based on SHFM 5-year predicted survival: high (predicted survival <70%), intermediate (70% to 85%), and low risk (>85%). Ten patients had the primary outcome of death, and 46 the combined end point. The hazard of death in the SHFM high- versus the intermediate-risk group was 7.09 (95% confidence interval 1.5 to 33.4, p = 0.01; no deaths in the low-risk group) and the hazard of the composite outcome between the high- versus low-risk group was 6.64 (95% confidence interval 2.5 to 17.6, p = 0.0001). Kaplan-Meier survival analysis showed greater probability of all-cause mortality (p = 0.003) in the high-risk group. In conclusion, the SHFM can help identify subjects with ACHD at risk for adverse outcome and poor cardiopulmonary efficiency. This may add to the care of patients with ACHD in the community and streamline care at tertiary centers.