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A middle aged male with history of Fallot type Double outlet right ventricle and coarctation of aorta who underwent multiple surgeries since childhood. With RV to pulmonary artery conduit and end to end coarctation repair initially, followed by aortic valve, pulmonary valve and RV-PA conduit replacements later. He subsequently developed significant flow through a fistula between a previously coiled aortic pseudoaneurysm and LVOT. This was percutaneously closed with a vascular plug and additionally a PFO, detected, was closed with a device. Patient returned to the clinic with decompensated heart failure. Updated imaging demonstrated that the plug had migrated and together with the coil, which was impinging on the AVR, resulting in severe supra-valvar AS and continuous significant persistent residual flow across the Aorta- LVOT fistula via false aneurysm. Our case highlights the importance of using multimodality imaging for complex congenital heart diseases. In view of high surgical risks associated with previous multiple sternotomies and patient’s preference, percutaneous approaches were adopted initially: however, it was unfortunate to see the migration of vascular plug. After successful deployment of AVP, migration or recanalization is rare, but should not be completely discounted.

Infective endocarditis (IE) is a potentially lethal disease for adults with congenital heart disease (ACHD). Staphylococcus aureus (SA) is one of the commonest pathogens, identified in patients with ACHD and IE. This study aims to analyse the epidemiology, clinical characteristics and in-hospital management of IE due to SA (SAIE) in ACHD.This is a single center retrospective study on documented episodes of SAIE in ACHD patients, treated at the Royal Brompton Hospital between 1999 and February 2020.Thirty-seven ACHD patients had an episode of SAIE (mean age 35.5 ± 14.9 years). More than half (51.3%), had a cardiac lesion of at least moderate complexity, with left ventricular outflow tract lesions being the most frequent. Twenty-four (64.9%) patients had a history of at least one previous open-heart surgery, and prosthetic material was used in 20 patients. A predisposing event was identified in 11 episodes (29.7%). Surgery was performed during the same admission in 24 (64.9%) patients, with early surgery (≤ 7 days from admission) performed in 12 patients. Early operation was associated with a shorter in-hospital stay compared to a later operation during the same admission or medical management [28.5 (IQR 23) vs. 43.5 (IQR 30) days, P = 0.028]. In-hospital mortality was 8.1%. Renal dysfunction at admission was associated with in-hospital multiorgan complications and/or death (OR 7.50, 95% CI, 1.18-47.67, P = 0.03).SAIE remains a life-threatening complication, necessitating cardiac surgery in the majority of ACHD patients. Ongoing educational investment on prevention and timely SAIE detection is warranted.

Aims Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021. Methods and results This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate Conclusion Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.

BackgroundCoarctation of the aorta (CoA) is one of the more common congenital heart defects affecting up to 5% of patients with congenital heart disease. Pregnant patients with unrepaired or severe re-coarctation are considered to be modified World Health Organisation (mWHO) IV, have the highest risk of maternal mortality and morbidity. The management of unrepaired CoA during pregnancy is influenced by a variety of factors which include the extent of the coarctation and coarctation characteristics, but due to paucity of data, it largely relies on expert opinion.Case summaryA 27 year old multi-gravid woman underwent successful percutaneous stent implantation for severe native CoA due to maternal resistant hypertension and foetal cardiac compromise on echocardiography. After intervention, the remainder of her pregnancy was uneventful with improved arterial hypertension control. The foetal cardiac structures, namely left ventricular size, improved after intervention. This case demonstrates the importance of CoA intervention during pregnancy to optimise both maternal and foetal outcome.ConclusionCoarctation of the aorta should be considered in pregnant women with poorly controlled hypertension. This case also highlights that, despite associated risks, percutaneous intervention can lead to improved maternal haemodynamics and fetal growth.

AimsLess than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition.Methods and resultsOverall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition.ConclusionA transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses.

Background As the ACHD population ages, an increasing number of patients are being admitted for heart failure. Management is complicated by the limited evidence on pharmacological and other therapies and lack of risk stratification tools. Improved surgical and medical treatment options in this patient cohort has improved life expectancy. Purpose Assess the mortality and rate of rehospitalisation in ACHD patients following admission with decompensated HF and identify clinical predictors of outcome. Methods All ACHD patients admitted for HF requiring IV diuresis from February 2016 to December 2020 in a single tertiary centre were included. Cox analysis was used to identify predictors of death after the index admission. Results 91 patients were included: 44.0% female, 45.8±14.2 years. The most frequent underlying diagnoses were: 15.4% with primary valve or left ventricular outflow tract disease, 12.1% with transposition of the great arteries post-atrial switch, 9.9% with tetralogy of Fallot and 30.8% had a systemic right ventricle. Most (56.7%) patients had CHD of “great” complexity according to the Bethesda classification, whereas no patients had “simple” CHD. Cyanosis was present in 26.4%. Heart failure was predominantly related to systemic ventricular dysfunction in 35.2%, subpulmonary ventricular dysfunction in 25.3%, biventricular dysfunction in 24.2% and univentricular dysfunction in 15.4%. Pulmonary hypertension was present in most (58.2%) patients, only half (46.0%) of these had pre-capillary pulmonary hypertension. On admission, approximately half of patients were on standard heart failure therapy: 48.4% ACE-inhibitors or angiotensin receptor blockers, 54.9% beta-blockers, 54.9% mineralocorticoid receptor antagonists and 2% on sacubitril/valsartan. In-hospital mortality was only 3.3% in this high-risk population. During a median follow-up of 22.1 [0.2–58.4] months, 37.5% patients were rehospitalised for HF and 37.5% patients died (22.7% mortality at 1 year). On univariable Cox analysis, the following parameters were predictors of mortality: pulmonary hypertension, cyanosis, unoperated or palliated CHD, chronic kidney disease (CKD), hyponatraemia, mineralocorticoid receptor antagonist use on admission, admission BNP>350ng/L and a maximum daily inpatient loop diuretic requirement >160mg (furosemide equivalents). On multivariable Cox analysis, pulmonary hypertension, cyanosis, hyponatraemia and CKD were independent predictors of mortality; patients with ≥2 of these risk factors had an 8-fold higher mortality (figure). Conclusion ACHD patients hospitalised for decompensated HF have a poor outlook with a quarter of patients dying within a median Funding Acknowledgement Type of funding sources: None. Figure 1

Background In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment. Objectives To assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery. Methods We assessed perioperative survival in patients >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable derived PErioperative ACHd (PEACH) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. Results The development cohort comprised 1782 procedures performed during the study period. Re-sternotomy was undertaken in 897(50.3%). There were 31(1.7%) in-hospital deaths. The PErioperative ACHd (PEACH) score showed excellent discrimination ability (AUC 0.88, 95%CI:0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC 0.69, 95%CI:0.6-0.78, p=0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC 0.75, 95%CI:0.72-0.77) and was well calibrated (Hosmer Lemishow 2 goodness-of-fit p=0.55). There was agreement in expected and observed perioperative mortality between cohorts. Conclusions The PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. Condensed abstract: The PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. In this study, we assessed the performance of components of the existing EuroSCORE II and ACHS score in predicting in-hospital mortality after ACHD surgery and combined them with other predictors of peri-operative mortality into a single PErioperative ACHd (PEACH) score. This novel score showed good discrimination ability in both development (AUC 0.88, 95%CI:0.82-0.94) and external validation (ACHS score AUC 0.69, 95%CI:0.6-0.78, p=0.0003) cohorts, with good agreement between expected and observed perioperative mortality.

BackgroundTo date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.MethodsData on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding.ResultsAmong the 359 patients with an sRV (32.2 (IQR 26.4–38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0–9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV.ConclusionsDespite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

Background The survival rate after Fontan procedure is improving and many of these patients now survive well into adulthood. Data on the late morbidity and mortality of Fontan patients in their fourth decade of life are lacking. Purpose To evaluate the late outcome of adult patients with Fontan circulation who have survived beyond 35 years. Methods Data were collected retrospectively on consecutive patients with a Fontan circulation ≥35 years between 2005 and 2019. Baseline (at 35th birthday) and follow-up data includes functional class, blood tests, history of arrhythmia, heart failure (HF), thromboembolism, Fontan related liver disease (FLD), protein losing enteropathy (PLE) and plastic bronchitis. Echocardiographic data on systemic atrioventricular valve (SAVV) regurgitation and ventricular function were also analysed. Results 61 patients were included (29, 47.5% female). Tricuspid atresia was the most common underline anatomy (29,47.5%) and the median age at Fontan procedure was 12 [8.0–19.0] years. 34 (56%) patients had an atrio-pulmonary connection Fontan and 15 (24.5%) total cavo-pulmonary connection (86.7% lateral tunnel and 13.3% extra-cardiac conduit), 10 (16%) a Bjork Fontan and 2 (3%) a Kawashima procedure. At baseline 40 (66%) patients were in NYHA class II or III, 7 (11%) had a moderate or severely impaired ventricular function and 12 (20%) had at least moderate SSAV regurgitation. At baseline, 35 (57.4%) patients had experienced at least one arrhythmia, 7 (11.4%) patients had a history of atrial thrombosis or thromboembolic events and 2 (3.3%) of PLE. At a median follow-up of 6.4 [2.9–11.3] years, 11 (18%) patients died, 7 of chronic HF, 1 hepatic carcinoma, 1 sepsis, 1 pulmonary embolism, 1 malignancy (1 cause unknown). During follow-up, 28 (46%) patients had a new episode of atrial arrhythmia, 23 (38%) developed FLD and 2 (3.3%) PLE. 9 (15%) required at least one admission for HF. 2 (3.3%) patients experienced worsening of their ventricular function and 7 (11.4%) of their SAVV regurgitation. On univariable analysis, a baseline diagnosis of PLE (HR 15.23, 95% CI:2.77–83.86, p=0.002), HF (HR 6.18, 95% CI:1.74–21.99, p=0.005), atrial arrhythmia (HR 4.99, 95% CI:1.07–23.21, p=0.04), lower serum albumin (HR 1.14, 95% CI:1.06–1.23, p=0.0006), lower iron levels (HR 1.31, 95% CI:1.02–1.68, p=0.04) and a higher urea (HR 1.18, 95% CI:1.04–1.34, p=0.01) were associated with death or heart transplantation. On bivariable analysis, lower serum albumin was stronger than all other univariable predictors. Conclusions Adult Fontan survivors are a high risk population with significant morbidity and mortality driven primarily by HF and other long-term complications of the Fontan operation. The predictors of outcome in this cohort reflects the chronic low cardiac output state and systemic venous hypertension. Vigilance is required to identify patients who may benefit of haemodynamic optimization and close monitoring for Fontan-related complications. Funding Acknowledgement Type of funding source: None

Introduction The Fontan operation has revolutionized the outcome of patients with univentricular hearts. However, long-term morbidity and mortality remain high and exercise intolerance in common in this population. Previous studies have failed to demonstrate a clear relation between exercise capacity, expressed as peak oxygen consumption (pVO2), and the risk of death in contrast with other congenital and non-congenital cohorts. Aims To investigate the correlates of pVO2 in the Fontan population and its relation to mortality. Methods Data were collected retrospectively on consecutive patients with a Fontan circulation who underwent a cardiopulmonary exercise test (CPET) between 2005–2019. Clinical and exercise data were collected at the time of CPET and patients were followed thereafter. Cox regression analysis was used to assess the association between exercise parameters and mortality. Different methods of estimating predicted pVO2 were compared in their impact on the prognostic value of pVO2. Results A total of 152 patients were included. Mean age at CPET was 28.9±9.3 years and 74 (48.7%) were female. The majority of patients had a total cavo-pulmonary connection (TCPC; 53.3%) followed by atrio-pulmonary (2.1%) and other Fontan variants 4.6%. The majority of patients reported no limitation on physical activity [AC1] (NYHA class I, 91, 62.3%). Baseline oxygen saturation was 93±5% and 26 (17.6%) patients had a persistent fenestration. On exercise, pVO2 was 21.9±7.4ml/min and was significantly reduced in the majority of asymptomatic patients (56.1±17.4% predicted according to the Wasserman/Hansen (WH) formula). The VE/VCO2 slope was raised (>33) in 92 (60.9%) patients. A total of 88 (58.7%) patients had an impaired heart rate reserve, and were unable to achieve a heart rate of at least 70% predicted. Over a median follow-up of 4.6 [2.5–8.9] years, 25 (16.4%) patients died. pVO2 expressed as ml/min was the only exercise parameter related to mortality in this cohort (HR 0.93, 95% CI: 0.87–0.99, p=0.03).pVO2 was even more strongly related to mortality when expressed as percentage of predicted using the WH formula (HR 0.76, 95% CI: 0.59–0.98, p=0.03), but not the Jones formula for predicted pVO2 (HR 0.83, 95% CI: 0.67–1.02, p=0.08). Additional predictors of outcome included NYHA class (no asymptomatic patients died), use of loop diuretics and a non-TCPC circulation, but not age or sex were not (Figure 1). On multivariable analysis, percent predicted pVO2 (WH) remained a predictor of outcome (HR per 10 unit increase 0.65, 95% CI: 0.46–0.93, p=0.017) with NYHA class and diuretic treatment, and with the type of Fontan operation. Conclusion A clear relation between pVO2 and mortality could be demonstrated in this Fontan population by using unadjusted pVO2, or appropriate estimates of predicted pVO2. CPET can thus be used for the risk stratification of Fontan patients, providing that care is taken in the way that pVO2 is reported and interpreted. Figure 1 Funding Acknowledgement Type of funding source: None

Background The arterial switch operation (ASO) has replaced atrial switch procedures for D-transposition of the great arteries (D-TGA), with 90% of patients now reaching adulthood. However, patients may have residual lesions and/or sequelae, some of which may remain unrecognized, necessitating lifelong specialist surveillance. We examined the cardiac outcomes of a large number of contemporary ASO patients under tertiary adult congenital heart disease (ACHD) care. Methods We examined late major adverse cardiovascular events (MACE) in adult TGA patients (>16 years) who underwent an ASO between 1981 and 2003 and continued their follow-up in 2 tertiary ACHD centers. MACE were defined as death, re-intervention, myocardial ischemia, arrhythmia, stroke/TIA, infective endocarditis and heart failure. Results Overall, 199 patients (66% male, mean age 27±5 years) were followed in adult life for a median of 10 years [IQR 7–15] and were included in this study. Overall survival during this period was 99.5% (95% confidence interval [CI]: 94.4%-99.8%). Sixty-two (31.2%) patients experience MACE, including 52 reinterventions. MACE and reintervention-free survival at 20, 30 and 35 years were 87.6%, 58.6%, 50.6% and 89.5%, 69.1, 61%, respectively. Atrial arrhythmia was the most frequent cardiac event with an incidence of 5.5 cases per 1000 patient-years, whereas incidence of ventricular tachycardia and sudden cardiac death was 1.8‰ and 0.9‰ patient-years, respectively. Coronary artery disease was diagnosed in 6 (3%) patients, of whom 4 had symptoms, 1 had ST depression on ECG at rest and 3 had abnormal wall motion on echocardiography. The most frequent indication for reoperation was right ventricular outflow tract obstruction (n=35/52, 63.7%), whereas left ventricular outflow tract (LVOT) re-interventions rate increased significantly during adulthood compared to childhood from 1% to 5%, p=0.03 (Figure 1). On multivariate analysis, history of cardiac complications during infancy (HR 2.3, 95% CI:1.3–4.0, p Conclusion Adult patients with ASO for TGA have a low late mortality. However, MACE are common requiring reintervention, particularly for RVOT obstruction and neo-aortic valve dysfunction, the latter with rising rates during adulthood. Patients with cardiovascular complications during childhood are at the highest risk for MACE. All patients merit life-long tertiary care. Figure 1 Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Assistance Publique des Hôpitaux de Paris

Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

Introduction Patients with transposition of the great arteries (TGA) who underwent atrial switch repair (ASR, Senning or Mustard) and patients with congenitally corrected TGA (ccTGA) have a morphological right ventricle in the systemic position. Patient with a systemic right ventricle (SRV) are prone to exercise intolerance and have a significantly worse survival compared to the general population. Risk stratification is an important component of their management and routine clinical assessment. Purpose To describe the severity and mechanisms of exercise limitation in patients with a SRV, and its relation to mortality. Methods All SRV patients who underwent a cardiopulmonary exercise test (CPET) in a tertiary centre from 2000–2019 were included. Demographics, clinical and imaging data were collected around the time of CPET. Cox survival analysis was used to assess the association between exercise parameters and mortality. Results A total of 262 patients underwent at least one CPET during the study period. Mean age at CPET was 37.4±12 and 154 (58.8%) were male. The majority of patients had TGA with ASR (150, 57.3%), while 112 (42.7%) had ccTGA. The majority of patients were in NYHA class I (36%) or II (36%). Moderate or severe right ventricular dysfunction was present in 188 (78.0%), while 59 (26.6%) patients had moderate or severe TR. Baseline oxygen saturation was 96±4% and median BNP was 71 [37–140] ng/L. Co-existent congenital lesions were present in almost one half (48.8%) of patients, including pulmonary stenosis (31,7, 12.1%) and ventricular septal defect (61,3, 23.4%). Average peak VO2 (pVO2) was 22.3±8.1ml/kg/min, (66±22% of predicted). The VE/VCO2 slope was raised (>33) in 128 (49%) patients. Patients who were cyanotic at rest and/or desaturated during exercise had a significantly higher VE/VCO2 slope (40.3±15.9 vs. 33.6±9.0, p=0.0004) and lower pVO2 (20.7±8.0 vs. 24.3±7.7, p=0.0007). A total of 119 (46.1%) patients achieved target heart rate, while 36 (14.0%) patients were unable to achieve a heart rate of at least 60% predicted. Over a median follow up of 3.0 [1.1–5.9] years, 36 (13.7%) patients died. Univariable predictors of mortality from CPET included pVO2, VE/VCO2 slope, heart rate reserve (HRR), anaerobic threshold, peak systolic blood pressure and exercise time (Figure). On bivariable analysis using pVO2 with each of the other exercise parameters, only peak systolic blood pressure remained in the model, even when adding age. In ccTGAs, peak VO2 was the only parameter remaining in the multivariable models. In ASR patients, HRR was stronger than all other parameters. Conclusion In this large cohort of patients with systemic RVs, older than existing reports in this area, peak VO2, peak systolic BP and HRR appear to be stongly related to mortality. These findings reflect the underlying physiology and consequences of previous surgery and should be used in clinical practice to risk stratify patients. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None

Background To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV. Methods Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding. Results Among the 359 patients with an sRV (32.2 (IQR 26.4–38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0–9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV. Conclusions Despite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.

Background Pulmonary vascular resistance (PVR) is an essential parameter assessed during cardiac catheterization. It is used to confirm pulmonary vascular disease, to assess response to targeted pulmonary hypertension (PH) therapy and to determine the possibility of surgery, such as closure of intra-cardiac shunt or transplantation. While PVR is believed to mainly reflect the properties of the pulmonary vasculature, it is also related to blood viscosity (BV). Objectives We aimed to assess the relationship between measured (mPVR) and viscosity-corrected PVR (cPVR) and its impact on clinical decision-making. Methods We assessed consecutive PH patients undergoing cardiac catheterization. BV was assessed using the Hutton method. Results We included 465 patients (56.6% female, median age 63y). The difference between mPVR and cPVR was highest in patients with abnormal Hb levels (anemic patients: 5.6 [3.4–8.0] vs 7.8Wood Units (WU) [5.1–11.9], P2.0WU) difference between mPVR and cPVR, and this was more pronounced in those with anemia (52.9%) or raised Hb (77.6%). In patients in the upper quartile for this difference, mPVR and cPVR differed by 4.0WU [3.4–5.2]. Adjustment of PVR required Conclusions We report, herewith, a clinically significant difference between mPVR and cPVR in a third of contemporary patients assessed for PH. This difference is most pronounced in patients with anemia, in whom mPVR significantly underestimates PVR, whereas in most patients with raised Hb, mPVR overestimates it. Our data suggest that routine adjustment for BV is necessary.

Background Adults with congenital heart disease (ACHD) have an increased risk of infective endocarditis (IE), which is associated with significant morbidity and mortality. This risk is further compounded by patient-related factors such as education and awareness of IE. The onus of patient education falls on both patient and physician, and is paramount to successful outcomes. Our study sought to evaluate patients' understanding of the risks, preventative measures and symptoms of IE, and to identify high-risk ACHD patients who would benefit from targeted education. Methods A cross-sectional study was conducted using a pre-tested questionnaire to assess knowledge of and attitudes towards IE. Patients attending the outpatient department of a tertiary referral centre completed the questionnaires independently. Baseline demographics and clinical data were collected from electronic patient records. Results 132 questionnaires were completed (median age 38 years, 50% male). 106 patients (80%) had previous surgical or percutaneous interventions and 7 patients (5%) had suffered with infective endocarditis in the past. 37% were able to accurately define IE. Out of a range of symptoms, most patients chose temperature (47%) and tiredness (39%) as classical symptoms of IE, however none correctly identified all listed symptoms as potential signs of IE. The majority of patients knew tooth abscess (58%) and body piercings (50%) were risk factors for IE. A fifth of patients (20%) were failing to have annual dental check-ups. 22% thought that IE would only require a few days stay as an inpatient and only 20% of patients were aware of the requirement for prolonged antibiotic treatment and the majority (63%) were unaware of the potential need for open heart surgery. 1 in 4 patients could recall having received information regarding IE. A third of patients reported that they would have made lifestyle changes had they known that IE required prolonged intravenous antibiotic treatment and could result in open heart surgery and death. Discussion Our study highlights key issues in the management of ACHD. Moving forward with the continuously growing population of patients we need to focus on the multi-disciplinary approach including specialist clinical nurses and increasing awareness online and through meetings and patient days as well as the importance of transition services as paediatric patients move across to adult specialists. General physcians will also encounter ACHD, therefore it is important to ensure awareness is widespread in the form online platforms and leaflets. We must also acknowledge the impact of guidelines and ensure there is still a significant focus on IE within them. To conclude, despite the significant morbidity and mortality associated with IE in ACHD, patient awareness of symptoms, risk factors and consequences is limited. Promotion of IE awareness is a cost-effective intervention, which can reduce the incidence and complications of IE.

Background Surgical repair of transposition of the great arteries (TGA) is most commonly via the arterial switch operation (ASO). This involves translocation of the aorta and pulmonary trunk, typically with anastomosis of the branch pulmonary arteries anteriorly (LeCompte manoeuvre) and re-implantation of the coronary arteries onto the posterior neo-aorta. As such, the position of the coronary ostia may differ from their expected locations. Purpose To use ECG-gated CT angiography to describe the anatomic position of coronary ostia in post-switch TGA patients guiding potential catheter interventions in this population. Methods All post ASO patients who underwent CT imaging between 2008–2018 were identified. Patients with complex anatomy such as double outlet right ventricle were excluded. The positions of the coronary ostia were measured in degrees from vertical on a double-oblique reconstruction in the aortic valve plane. Ostium positions were compared to those of patients with no congenital heart disease via Watson's two-sample test of homogeneity for circular data. Angular dispersion was compared between groups via the Wallraff test. P Results Of 206 adult patients with TGA and ASO followed in our adult congenital heart disease centre, 38 (18.4%) had CT imaging available for analysis during the study period (mean age 24±6.8, 75% male). The control group consisted of 15 patients investigated for chest pain (mean age 54±15.1, 73% male). In the control group, the right and left coronary ostia arose at a mean angle of −19 and +125 degrees from vertical (figure 1a). This was significantly different to the mean ASO coronary ostia clustered at mean angles of −70 and +29 degrees from vertical (Watson p There was no significant difference in spread of left ostia (Rho 0.9 vs 0.99, p=0.12), but right ostia were significantly more variable in ASO patients than controls (0.71 vs 0.96, p=0.003). Figure 1 Conclusions Coronary ostial positions in the neo-aorta of post-ASO patients differ significantly from those of normal controls, with considerable variability, especially in right coronary position. CT can demonstrate coronary ostia in 3D space and derive appropriate tube angles to guide catheterisation in post-operative congenital cardiac patients, optimise catheter selection, reduce catheterisation tome, radiation and contrast dose.

Introduction Adult patients with congenital heart disease (ACHD) are a heterogeneous population and a variety of procedures are performed in ACHD centers, which differ significantly from those performed in the general adult population. Therefore, ACHD patients have not been included in the development of risk stratification models, such as the EuroSCORE. We assessed the utility of the components of the EuroSCORE in predicting in-hospital mortality around surgery in a large cohort of ACHD patients and tested a modified risk score for the ACHD population. Methods Data were collected retrospectively on all consecutive patients >16 years who underwent congenital heart disease surgery in a large tertiary center in 2015–18. Preoperative characteristics and perioperative outcomes were collected from clinical records and databases. Wilcoxon rank sum test, Fisher's test and Logistic regression analysis were used to identify predictors of in-hospital death. Variables significant on univariate linear regression were used to create a risk score for each patient, either attributing 1 point for each risk factor (unweighted model), or weighing this by its log(Odds ratio) in the logistic regression model (weighted score). Receive operator characteristic (ROC) analysis with calculation of the areas under the curve (AUC) was used to assess the performance of each scoring system in predicting in-hospital mortality. Results A total of 476 operations occurred in 459 patients who underwent cardiac surgery during the study period. Age at surgery was 35.9±14.7 years, 258 (56.2%) were male, 231 (51.3%) patients had a previous sternotomy. There were 19 (4.1%) in-hospital deaths. Certain components of EuroSCORE were very rarely observed in our ACHD patients and were not included in the analysis. Of components of the EuroSCORE, female sex (OR 3.79, 95% CI: 1.42–11.89, p=0.01), functional NYHA class>2 (OR 7.65, 95% CI: 2.35–29.26, p=0.001), left ventricle dysfunction (OR 3.14, 95% CI: 1.22–8.01, p=0.02), previous surgery (OR 5.33, 95% CI: 1.41–34.68, p=0.03), emergency or urgent surgery (OR 7.96, 95% CI: 3.1–21.99, p Conclusions While several components of the EuroSCORE are relevant to ACHD patients, an ACHD-specific scoring system for predicting perioperative mortality is needed. In this analysis, we propose a simplified risk score for ACHD patients undergoing surgery, which performs well in this population.

Background Establishing surgical indication for aortic valve replacement or repair (AVR) in the setting of severe aortic regurgitation (AR) can be challenging in young patients. Current guidelines state patients should be operated based on symptoms, presence of left ventricular (LV) dysfunction (Ejection Fraction, EF ≤50%) or severely LV dilatation (LV end diastolic diameter, LVEDD ≥70mm or LV end systolic diameter, LVESD ≥50mm) 1. Purpose Our goal was to study the change of the LV size and function in a cohort of young adults with severe AR after surgery and relate this to pre-operative ventricular characteristics. Methods We reviewed all patients who underwent AVR in our centre between 2013 and 2018. The echocardiographic data was collected prior to, pre discharge and at 6–12 months after surgery. A ROC analysis was used to determinate the discriminative power of baseline LV diameters in predicting normalization of LV size pre discharge. Normal values were considered as per the guidelines3. Results A total of 75 adult patients were included: mean age 25±10.5 years, 64% male. The majority (61%) had a bicuspid valve, 17% an autograft (previous Ross procedure), 10% developed AR after a VSD was closed, 6% had an arterial switch procedure, 3% a truncal valve and 3% Tetralogy of Fallot. 10% went for a Ross procedure, 60% received a bioprosthetic and 30% a mechanical aortic valve. The majority (61%) were completely asymptomatic. The vast majority (88%) were operated with LVEDD ≤70mm (mean 60±6.9mm), 84% had a LVESD ≤50mm and 80% had an LVEF >50%. A significant reduction in LVEDD occurred within a few days from surgery: mean reduction 9.2±6.2mm, p On ROC analysis the baseline LVEDD and LVESD were excellent predictors of lack of normalization of LV size post surgery (AUC=0.82, 95% CI: 0.70–0.94 and AUC=0.79, 95% CI 0.66–0.92, respectively). Sensitivity and specificity for normalization of LVEDD were 87% and 73%, with a preoperative LVEDDD cut-off value of ≥61mm. A cut off point of ≥42mm on the LVESD achieved a sensitivity of 83% and specificity of 65%. Conclusions In our practice we tend to operate on young patients with severe AR earlier than stated in the guidelines. Our data supports this approach, demonstrating that patients with significantly increased LV diameters prior to surgery do not tend to remodel in the first postoperative echo. Despite operating early, patients with LV EF

Pulmonary vascular resistance (PVR) is an essential parameter assessed during cardiac catheterization. It is used to assess response to targeted pulmonary hypertension (PH) therapy; determine the possibility of surgery, such as closure of intracardiac shunt or heart transplantation; and according to

Background Most of the patients who underwent arterial switch operation (ASO) for D-transposition of the great arteries (D-TGA) reach adulthood. However, they may have residual lesions necessitating lifelong surveillance. We aimed to evaluate the long-term outcomes of a large number of contemporary ASO patients. Methods We examined late major adverse cardiovascular events (MACE) in adult TGA patients (> 16 years) who underwent an ASO between 1980 and 2002 and continued their follow-up in 2 major tertiary ACHD centers. MACE were defined as death, re-intervention, myocardial ischemia, arrhythmia, stroke/transient ischemic attack, infective endocarditis and heart failure. Results Overall, 306 patients (67% male, mean age 25 ± 5years) were followed in adult life for a median of 8 years [IQR 6–13]. Seventy-three (23.8%) patients experience MACE, including 40 medical events and 60 reinterventions. MACE -free survival at 2, 5, and 10 years of follow-up in adulthood was 93.0%, 85.1%, 70.7%, respectively. Atrial arrhythmia was the most frequent cardiac event with an incidence of 3.8‰ patient-years, whereas the incidence of life-threatening ventricular tachyarrhythmia was 1.7‰. Coronary artery disease was diagnosed in 11 (3.5%) patients. The most frequent indication for cardiac intervention was right ventricular outflow tract obstruction (n = 40/60, 66.7%) followed by coronary revascularisation procedure (n = 11/60, 18.3%), Fig. 1 . On multivariate analysis, history of cardiac complications during infancy (HR 2.7, 95%CI:1.6–4.5, P Conclusion MACE are common in adult patients with ASO for TGA, particularly those with a history of cardiovascular complications during childhood and uncommon coronary pattern. Consequently, all patients with an anatomic repair for TGA merit life-long tertiary care.

Non-invasive assessment of haemodynamic data, such as pressure and flow profiles, is helpful in detecting cardiac disease at an early stage. However, current methods lack spatial accuracy and do not take local variations into account. This paper presents a software tool that extracts the arterial geometry and blood inflow profiles from MR images, which are subsequently used to run a 1D haemodynamic simulation model, and displays its output. The workflow is highly automated but allows user-interaction to correct inaccuracies. The tool was evaluated for inter-observer agreement on one healthy volunteer, and results are shown for one patient with an aortic coarctation. The resulting haemodynamic parameters show high agreement between di ff erent users and reveal local changes within a coarctation patient.

Background In patients with acquired heart failure, hypoalbuminaemia is associated with increased risk of death. The prevalence of hypoproteinaemia and hypoalbuminaemia and their relation to outcome in adult patients with congenital heart disease (ACHD) remains, however, unknown. Methods Data on patients with ACHD who underwent blood testing in our centre within the last 14 years were collected. The relation between laboratory, clinical or demographic parameters at baseline and mortality was assessed using Cox proportional hazards regression analysis. Results A total of 2886 patients with ACHD were included. Mean age was 33.3 years (23.6–44.7) and 50.1% patients were men. Median plasma albumin concentration was 41.0 g/L (38.0–44.0), whereas hypoalbuminaemia (

BACKGROUND: Pulse wave velocity (PWV) is a biomarker for the intrinsic stiffness of the aortic wall, and has been shown to be predictive for cardiovascular events. It can be assessed using cardiovascular magnetic resonance (CMR) from the delay between phase-contrast flow waveforms at two or more locations in the aorta, and the distance on CMR images between those locations. This study aimed to investigate the impact of different distance measurement methods on PWV. We present and evaluate an algorithm for automated centreline tracking in 3D images, and compare PWV calculations using distances derived from 3D images to those obtained from a conventional 2D oblique-sagittal image of the aorta.METHODS: We included 35 patients from a twin cohort, and 20 post-coarctation repair patients. Phase-contrast flow was acquired in the ascending, descending and diaphragmatic aorta. A 3D centreline tracking algorithm is presented and evaluated on a subset of 30 subjects, on three CMR sequences: balanced steady-state free precession (SSFP), black-blood double inversion recovery turbo spin echo, and contrast-enhanced CMR angiography. Aortic lengths are subsequently compared between measurements from a 2D oblique-sagittal plane, and a 3D geometry.RESULTS: The error in length of automated 3D centreline tracking compared with manual annotations ranged from 2.4 [1.8-4.3] mm (mean [IQR], black-blood) to 6.4 [4.7-8.9] mm (SSFP). The impact on PWV was below 0.5m/s (CONCLUSIONS: Automatic aortic centreline tracking in three commonly used CMR sequences is possible with good accuracy. The 3D length obtained from such sequences can differ considerably from lengths obtained from a 2D oblique-sagittal plane, depending on aortic curvature, adequate planning of the oblique-sagittal plane, and patient motion between acquisitions. For accurate PWV measurements we recommend using 3D centrelines.

Introduction: Patients with Tetralogy of Fallot (TOF) and pulmonary insufficiency and a dilated right ventricle (RV) may suffer from a reduction in left ventricular (LV) performance. It is not clear whether timely pulmonary valve replacement (PVR) preserves LV mechanics. Methods: Ten TOF patients who underwent PVR were identified from hospital records, and pre- and postoperative CMR images were post-processed with a semi-automatic tissue tracking software. LV circumferential strain, time to peak strain, and torsion were compared before and after PVR. A control group of ten age-matched normal volunteers was assessed as a comparison. Results: LV circumferential strain did not change before versus after PVR (basal -18.3 +/- 3.7 % vs. -20.5 +/- 3 %, p 0.082; mid-ventricular -18.4 +/- 3.6 % vs. -19.1 +/- 2 %, p 0.571; apical -22.7 +/- 5.2 % vs. -22.1 +/- 4 %; p 0.703). There was also no difference seen between the baseline strain and normal controls (control basal -18.2 +/- 3.3 %, p 0.937; mid -18 +/- 3.2 %, p 0.798; apex -24.1 +/- 5 %, p 0.552). LV torsion remained unchanged from baseline to post PVR (systolic 2.75 (1.23 – 9.51) °/cm vs. 2.3 +/- 1.2 °/cm, p 0.285; maximum 5.5 +/- 3.5 °/cm vs. 2.34 (1.37 – 8.07) °/cm, p 0.083). There was no difference in time to measured peak LV circumferential strain before versus after PVR (basal 0.44 +/- 0.1 vs. 0.43 +/- 0.05, p 0.912; mid-ventricular 0.42 +/- 0.08 vs. 0.38 +/- 0.06, p 0.186; apical 0.40 +/- 0.08 vs. 0.40 +/- 0.06, p 0.995). At the same time, pulmonary regurgitation and RV end-diastolic and end-systolic volume indices decreased and LV end-diastolic volume increased after PVR. RV and LV ejection fractions remained constant. Conclusion: PVR allows for favorable remodeling of both ventricular volumes for TOF patients with significant pulmonary regurgitation. In this cohort, LV myocardial functional parameters such as circumferential strain, time to peak strain, and LV torsion were normal at baseline and remain unchanged after PVR.

Background Left atrium (LA) size and function are powerful biomarkers of cardiovascular outcomes in many diseases. We sought to determine if the expected age-associated increase in arterial stiffness (AS) and left ventricular (LV)-LA afterload leads to corresponding effects on LA function and this can be measured with cardiovascular magnetic resonance (CMR). Additionally, we investigated the significance of these markers in asymptomatic individuals with cardiovascular risk factors (CRF).

A 42-year-old man presented to his local hospital with sepsis. He was cyanotic and had finger clubbing with oxygen saturation of 85% to 90%. A computed tomographic pulmonary angiogram led to an initial diagnosis of left-sided superior vena cava and partial anomalous pulmonary venous drainage. He had been generally well but always had limited exercise capacity (New York Heart Association class 2) and mild cyanosis and finger clubbing had been noted previously but had never been investigated. He was reviewed at an adult congenital center, and his symptoms were thought to be inconsistent with partial anomalous pulmonary venous drainage and left-sided superior vena cava. An echocardiogram raised suspicion for abnormal drainage of the SVC, and bubble echocardiography showed early and complete opacification of the left atrium and left ventricle after contrast injection in the left arm. There was late but significant filling of the right atrium and right ventricle, but this was less dense, signifying a right to left shunt. A cardiac magnetic resonance imaging demonstrated complete occlusion of the right SVC (Figure [A]). The brachiocephalic vein and other head and neck veins drained via tortuous venous collaterals to the left upper and right upper pulmonary veins (Figure [B]; …

First case of infective endocarditis on a percutaneous aortic valve replacement due to Moraxalla nonliquefaciens in 64 year old woman. It was successfully treated medically with antibiotics. She had not suitable for surgical aortic valve replacement due to 3 sternotomies for thymoma resection and subsequent radiotherapy with blocked major thoracic veins. Due to her azathioprine immunosuppresion (myasthenia) she may have been at increased endocarditis risk. We suggest prophylactic antibiotics at implant for this group in future.

To the Editor We are intrigued by the article by Priest and Ashley.1 The importance of genomics has been clearly identified and the article beautifully describes the role of genetics in cardiovascular medicine but concentrates on inherited arrhythmias, cardiomyopathies and occasional pharmacogenomic profiling.1 We suggest they have failed to consider the large, unrecognised need for genomics in adults patients with congenital heart conditions. More than 90% of patients with congenital heart conditions now survive to adulthood and yet as neonates/children there was restricted availability of genetic testing and limited techniques. When such patients attend the adult clinic they want to know the …

A 36-year-old man born with pulmonary atresia intact ventricular septum, atrial septal defect and Ebstein anomaly with hypoplastic right heart (figure 1 and online supplementary video). Waterson shunt had been performed in childhood, but over a period of time it had occluded with pulmonary circulation being dependent on bronchopulmonary collaterals. He is on targeted therapy for pulmonary hypertension. He was symptomatic with intermittent focal atrial tachycardia, despite amiodarone therapy. Coumarin therapy was avoided due …

Background Ventricular septal defect are the most common congenital defect but only accounts for 10% of congenital defects in the adults due to spontaneous closure. The objective of this study is to review the natural course and outcomes of the repaired and unrepaired VSD in this large follow up cohort in non surgical (CHD) centre. Methods We reviewed the NORPAP database (n = 2322) and found 300 patients with ventricular septal defect (12.9% incidence) Results 137/300 were male, 163/300 (54%) were female. Age range 17–83, median age at FU was 39 years. Spectrum of type of ventricular septal defect is described Table 1 and associated concomitant cardiac lesions. Surgical closure of VSD occurred in 122 and percutaneous closure in 3 (total 125). 24/125 had redo surgery and 10/24 had 3 rd operation. 1/10 had 4 th , 5 th and 6 th procedure for repeated tricuspid valve obstruction (thrombus and pannus). 175 patients were unoperated at transition to adult clinic, 26 have since had spontaneous closure, 6 partial closure; 1 is waiting VSD closure. Arrhythmia incidence was low (33) and equal in both operated and unoperated. Atrial arrhythmias in 21, ventricular in 9, bradycardia requiring pacemaker in 3; overall incidence of device therapy was low (7). 22 patients had endocarditis – 9 had had previous surgery, 13 unoperated (1 twice). 12/163 (7.3%) females and 10 males/137 (7.3%) was against male preponderance. IHD disease was not a common associated feature of VSD n = 4. 10 patients had RVOT – obstruction associated VSD out of which 6 required resection and repair of VSD- 1 patient required further intervention due to LVOT-obstruction caused by aneurysmal septal patch 38 patients developed pulmonary hypertension out of which 21 patients had Eisenmenger syndrome. 32 patients were unoperated in this group while 6 patients developed pulmonary hypertension post repair Conclusion Ventricular septal defects associated with concomitant lesions require long term follow up in dedicated adult CHD clinics. Small unoperated VSD’s and repaired VSD’s can be followed in physiologist led echo clinics with ACH-CSN review as required to watch for late outflow tract obstruction or other sequelae. No death was reported in our cohort up to date. Over 50% of endocarditis in this cohort occurred in unoperated VSD’s

Background Trisomy 21 is frequently associated with congenital heart disease (CHD). Survival and life expectancy has increased as a result of surgical intervention and medical therapies. The NORPAP GUCH database, started in 1993, has 2668 patients. Methodology Patients with Trisomy 21 were selected from the NORPAP database. Population demographics, underlying diagnosis, comorbidities and mortality were analysed. Results 110 patients had T21; Mean 34 years, Median 30 years, M:F 1.1:1 (Figure 1). Underlying CHD was AV Canal defect 57 (51.8%), VSD 30 (27.3%), Fallot Repair 10 (9.1%), Secundum ASD 6 (5.5%), Bicuspid Aortic Valve 3 (2.7%), Mitral Regurgitation 1 (0.9%), Single Ventricle 1 (0.9%), Pulmonary Stenosis 1 (0.9%), PDA 1 (0.9%). 61 (55%) patients had had surgery. Of those under 40, 51% were unoperated. Death occurred in 9 patients (9%). Average age at death 46 years. 78% of deaths were in patients with AV Canal defect. Mode of death was cardiac in 8 and PAH-CHD was present in all but one. Figure 2 shows associated comorbidities. 35% have PAH -– 59% receivetargeted therapy. Conclusion AV Canal defect was the most frequent underlying CHD in the T21 NORPAP cohort and was associated with the highest mortality. 1/3 of all patients had PAH and 1/4 of these died during follow up. Survival into the 4th and 5th decade is frequent. Patients in the 3rd decade now may expect to survive for another 25–30 years. Ongoing cardiac and non-cardiac morbidity requires dedicated CHD clinics, frequently with PAH clinicians given that 50% of those under 40 have not had surgery and may need targeted therapy.

Background Coarctation of the aorta (CoA) is associated with premature mortality with a mean survival age of 35 year in spite of early repair. Treatment paradigms are continually changing and contemporary data on CoA are needed. Methods The Norwich and Papworth (NORPAP) dataset was set up in 1993 to record demographics, treatments and outcomes of adult congenital heart disease (ACHD) patients in the east of England. We report on the CoA subset. Results In total 2322 patients were included in the dataset and of these 223 had CoA (9%): 146 males and 77 females, mean age 40 years (range 16–94). Hypertension was documented in 34% and coronary heart disease 6%. 91% received a corrective procedure (80% surgical and 11% percutaneous) and of these 38% required more than one procedure. Percutaneous intervention made up about half of all re-do procedures. 13% of second procedures were not related to CoA and were mostly aortic valve replacement. There were two case of subarachnoid haemorrhage and 2 deaths (0.8%) over approximately 10 years follow up. Conclusion There is a high initial intervention rate in CoA with about 40% requiring additional intervention. The serious complication rate was low in our cohort suggesting that prognosis is improving over time.

Results: Patients (n=26) awaiting treatment / fnal MDT decision were excluded. 17 patients were treated by TAVI (7 transfemoral, 10 transapical), 27 by conventional surgical AVR (csAVR), 9 with balloon valvuloplasty (BAV) and 30 medically. There were no differences in baseline characteristics (but for an excess of prior CABG in the TAVI group vs. csAVR (13/17 vs. 3/27; p

Total anomalous venous drainage is a rare congenital cardiac defect seen in 1% of the population; there is usually a concomitant atrial septal defect. We discuss such a diagnostic challenge in a patient who presented on a number of occasions with acute respiratory distress and haemoptysis. True understanding of her underlying congenital heart disease was not appreciated until review in a congenital heart disease clinic - in which she presented for prepregnancy counselling. She was found to have a stricture at the site of anastomosis of the common chamber with the left atrium, resulting in pulmonary venous hypertension leading to acute presentation of pulmonary haemorrhages. She underwent successful full balloon dilatation of the site, leading to full recovery. Most patients are diagnosed at birth and undergo surgical repair. The outcome is good, and historically it has been regarded that it is unusual for them to have long-term complications, although dysrhythmias remain a recognized long-term complication. Surgery for total anomalous pulmonary venous drainage is nevertheless a palliation, and it is vital that the possible role of late complications is considered in all cases.

A case of Takayasu aortitis associated with sarcoidosis presenting with recurrent angina is reported. This association has been called ‘Takayasu syndrome’, which reflects what is likely a shared etiology. Myocardial perfusion abnormalities have recently been documented in sarcoidosis, but this case clarifies for the first time that the angina in Takayasu syndrome is likely due to small vessel coronary arteritis. Corticosteroids and cytotoxic therapy have been shown to be beneficial in all forms of sarcoidosis related to vasculitis. Initiation of steroid therapy may provide relief of angina in patients with evidence of reversible ischemia in normal coronary arteries.

Background Adults with CHD now outnumber the paediatric population due to greater than 90% survival to the age of 18. About 400 syndromic types account for 25% of the CHD population; some of these are associated with high inheritance rates about which the adult CHD need counselling. The NORPAP database is derived from a dedicated adult CHD service started in 1993. Methods We reviewed the NORPAP database (n = 2322) of which 264 patients (144M, 120F) with syndromic appearance had been genotyped. Mean age 35 years (16–89). 224 genotype positive and 40 genotype negative were identified. Spectrums of syndromes are described below in the Figure 1. The highest incidence of CHD with genotype was AV canal defects, but also VSD, conotruncal abnormalities and aortopathies (Marfan, L-D, E. D. IV). Phenotypes and cardiac defects were studied as most patients had more than one associated lesions. Table 2 describes the association. Importantly 144 (55%) patients had cardiac surgery of which 32 (27%) required a second intervention required surgical intervention (higher incidence in Trisomy 21). Incidence of arrhythmias was low n = 19 (7%); 11 (4%) had device therapy. 47 (18%) patients had PAH-CHD of whom 11 were on targeted therapy. Endocarditis occurred in 4 patients only, surprising given the number with 22q11deletion. The incidence of IHD to date is low (3). Conclusion Clinical syndromes with CHD are seen frequently seen in adult CHD clinics. Only 10% of the NORPAP database has been genotyped despite increasingly close involvement with clinical geneticists. Due limited availability of genetic testing in previous eras, many adult CHD survivors should have phenotype-genotype assessment not only to enhance parenthood counselling and cascade screening, but, increasingly, risk stratification of recognised comorbidities.