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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 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.

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.

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.

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 (

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.

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.

Background Despite ongoing advances in magnetic resonance (MR) sequence development, coronary imaging in paediatric patients remains challenging. MR imaging of the coronary arteries can be difficult due to high heart rates (HR), small diameter of the coronary arteries, and poor contrast between the blood pool and extravascular structures. The use of higher relaxivity contrast agents has been shown to improve coronary imaging in adult patients significantly, but no data is available in paediatric patients.

Introduction Treatment of high risk patients with severe aortic valve stenosis has been revolutionised by Transcatheter Aortic Valve Implantation (TAVI). We hypothesise that multi-disciplinary team (MDT) assessment by cardiologists, cardiothoracic surgeons and anaesthetists facilitates appropriate case selection and achieves better than predicted outcomes in this high risk group. Methods All patients (n=111) referred to the MDT for TAVI were prospectively enrolled. Baseline demographics and outcomes were recorded. Results are median (IQR) unless stated. Results Patients (n=25) awaiting treatment/final MDT decision were excluded. Twenty patients were treated by TAVI (seven transfemoral, 13 transapical), 27 by conventional surgical AVR (csAVR), nine with balloon aortic valvuloplasty (BAV) and 30 medically. There were no differences in baseline characteristics (except for an excess of prior CABG surgery in the TAVI group vs csAVR (16/20 vs 3/27; p Thirty-day mortalities were: TAVI: 0/19; csAVR: 0/27; BAV: 1/9; medical: 8/30 (abstract 091 figure 1). Adjusting for ES, the observed/expected 30 day mortality indices were: TAVI: 0; csAVR: 0; BAV: 0.37; medical: 1.31. Patients receiving valve replacement (TAVI and csAVR) had lower 30-day mortality than those receiving palliative treatment (BAV and medical): 0% vs 23.1%, p TAVI patients had shorter intensive care unit (ICU) stays than csAVR patients: 22.0 (9.9–41.3) h vs 36.6 (23.3–169.2) h, p=0.03 and shorter durations of tracheal intubation: 2.1 (1.9–5.8) h vs 11.9 (8.6–20.1) h, p Conclusion MDT assessment of high risk patients with severe aortic valve stenosis combined with multi-modality treatment options results in lower than predicted mortality. Patients selected for TAVI have shorter ICU stays than patients selected for csAVR despite equivalent co-morbidities.

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] A 37-year-old gentleman presented with musculoskeletal chest pain. An electrocardiogram showed a left bundle branch block. Echocardiogram demonstrated hypertrophy and deep trabeculation of the ventricular septum ( A , [Online