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OBJECTIVES To evaluate the ability of non-contrast enhanced magnetic resonance imaging (MRI) techniques to characterize Fontan associated liver disease (FALD) in adolescent and adult Fontan patients. METHODS Fontan patients (n = 29) and healthy controls (n = 13) underwent an MRI protocol with T1, T2 and Apparent Diffusion Coefficient (ADC) mapping. Routine FALD screening included abdominal ultrasound and laboratory testing. RESULTS Median follow-up after Fontan operation was 15.1 (IQR 12.0-16.8) years. Distinct differences in tissue characteristics were visualized. T1 and T2 relaxation times were prolonged in Fontan patients, particularly of the right lobe (T1: 745 (IQR 715-784) ms vs. 586 (IQR 555-602) ms, p

Measures of left ventricular (LV) systolic and diastolic function are known predictors of mortality after repair of tetralogy of Fallot. We aimed to characterise LV reverse remodelling achievable with ramipril therapy.A blinded post-hoc analysis of baseline and 6-month follow-up echocardiograms from the APPROPRIATE (ISRCTN: 97515585) randomised double-blinded placebo-controlled trial of ramipril therapy was performed in 64 patients: 32 in ramipril and 32 in placebo group. Tissue Doppler systolic and diastolic myocardial velocities, mitral inflow velocities and time intervals were measured. Left atrial area and left atrial emptying fraction were calculated. There was significant increase in long axis shortening mean (standard deviation); MAPSE [1.9 (4.2) mm vs -0.2 (3.7) mm; p = 0.030], peak lateral systolic velocity; S' lateral [1.0 (2.0) cm/s vs -0.3 (2.2) cm/s; p = 0.025], peak lateral early diastolic velocity; E' lateral [0.57 (2.4) cm/s vs -3.3 (3.9) cm/s; p 0.001], transmitral to lateral mitral annular early diastolic velocity ratio; E/E' lateral [-0.7 (1.9) vs 1.5 (1.9); p 0.001] over the study period in the ramipril compared to the placebo group. Significantly higher measurements were observed in the ramipril arm of the subgroup of patients with right ventricular restrictive physiology in terms of peak late diastolic velocity; A [5.9 (13.5) cm/s vs -5.8 (12.5) cm/s; p = 0.041] and early to late diastolic transmitral velocity ratio; E/A [-0.18 (0.42) vs 0.23 (0.48); p = 0.037].Six months' ramipril treatment appears to limit progression of both diastolic and systolic LV function in adults late after tetralogy of Fallot repair. With increased appreciation that even subtle LV disease predicts tetralogy of Fallot outcomes, further clinical trials of drug therapies are justified.

Introduction The descending aorta (DAo) in patients with hypoplastic left heart syndrome (HLHS) after Norwood procedure is frequenty enlarged and exhibits increased stiffness. Such findings of previous studies still remain unexplained given the fact that the DAo is not involved in the Norwood operation. Methods We studied five HLHS patients with DAo dilatation (aged: 2.9–15.1 years (y), median 9.0 y), four HLHS patients without DAo dilatation (aged: 3.5–9.7 years, median 6.45 y) and 7 healthy controls (aged 6.3–41.6 y, median 26.0 y) using cardiovascular magnetic resonance imaging with acquisition of cine images, contrast-enhanced angiograms and 4D flow. 4D flow data were analyzed based on in-house developed analysis software to quantify vortical flow patterns in terms of vorticity. Results All patients with DAo dilatation presented with a caliber reduction between the proximal and distal aortic arch of more than 40% (median reduction 71%, range 43%–79%) and with increased z-scores of the DAo. Vorticity in the DAo of patients with DAo dilatation (median: −24 s−1, range: −26 s−1 - −8 s−1) was significantly increased in magnitude compared to controls (median: 0 s−1, range: −2 – +2) (p Conclusions The findings of signficiantly increased vorticity and its association with increased DAo z-scores can potentially explain vascular alterations in the DAo of HLHS patients. This study gives motivation for further investigations and may ultimately lead to future Norwood procedure modifications.

Background Implantable cardioverter-defibrillator (ICD) systems are established therapy for prevention of sudden cardiac death. Long-term data on ICD systems in children and adolescents is rare. The present study displays a long-term single-center follow-up of children and adolescents with ICD systems. Methods and results The present study represents a single-center experience of patients younger than 18 years who received an ICD (n = 58). Follow-up data included in-house follow-up as well as examinations of collaborating specialists. Mean age at implantation was 14.0 ± 3.3 years and 33 patients (56.9%) were male. A transvenous ICD system was implanted in 54 patients (93.1%). In 33 patients (56.9%) electrical heart disease or idiopathic ventricular fibrillation represented the underlying condition of ICD implantation. Median follow-up duration was 70 months (45; 94). 3 patients (5.2%) died during the observation period. None of these deaths was associated with ICD failure. Appropriate shocks occurred in 32 patients (55.2%). Inappropriate shock delivery was recorded in 17 patients (29.3%). Supraventricular tachycardia represented the most frequent cause of inappropriate shock delivery (9 patients, 52.9%). T-wave oversensing led to inappropriate shock delivery in 3 patients (17.6%). In 5 patients (29.4%), lead failure caused inappropriate shock delivery. Of note, during follow-up lead failure was reported in 15 patients (25.9%) leading to surgical revision. Conclusion ICD therapy in children and adolescents is effective for prevention of sudden cardiac death. The rate of appropriate shock deliveries was significantly higher as compared with large ICD trials. Inappropriate therapies occurred frequently. In particular supraventricular tachycardia, T-wave oversensing and lead failures were responsible for these episodes.

Background Echocardiographic assessment of diastolic function in the setting of Fontan physiology is not well validated. We recently demonstrated that atrioventricular systolic to diastolic duration ratio (AVV S/D ratio) independently predicts mortality in Fontan-adults and that a value >1.1 was associated with poor prognosis. Purpose To correlate echocardiographic measures of diastolic function with direct measurement of ventricular end-diastolic pressure (VEDP). Methods A retrospective analysis was undertaken of Fontan-adults who had transthoracic echocardiography (TTE) within 12 months of direct measurement of VEDP during cardiac catheterisation. Results Fifteen Fontan adults (3 males, mean age 29 ± 9 years) were evaluated. Thirteen patients had dominant morphologic left ventricle and 2 had morphologic right ventricle. Four had atriopulmonary connection and 11 had total cavopulmonary connection. Twelve patients were NYHA Class I/II and 3 were Class III. Time between TTE and cardiac catheter was 46 ± 113 days; VEDP was 8 ± 5 mmHg. Ten patients had preserved ventricular function, 3 had mild and 2 had moderate systolic impairment by subjective TTE assessment. AVV S/D ratio had the strongest correlation with VEDP (r = 0.8, p = 0.001). AVV S/D ratio ≥ 1.1 had 100% positive predictive value and 92% negative predictive value for detecting VEDP >10 mmHg. The only conventional echocardiographic measure of diastolic function that correlated with VEDP was pulmonary vein A wave - atrioventricular A wave duration difference (r = 0.8, p = 0.02). Conclusions TTE measures reflect VEDP in adults with a Fontan circulation. AVV S/D ratio is a simple parameter yet to enter standard practice that can be used to identify elevated VEDP.

Background Pregnancy in Marfan Syndrome (MFS) is associated with increased maternal risk of cardiovascular events. Given the maternal and genetic risks, pre-conception counselling is essential to facilitate informed choices. Multidisciplinary antenatal care with regular imaging is mandatory and best delivered through a Joint Cardiac Obstetric Service (JCOS). The aim of this study was to compare the care delivered in a JCOS against recognised international standards (European Society of Cardiology (ESC)). Methods Pregnancies in women with MFS from 2005 to 2015 were identified from our institutional database. Patient records were reviewed and practice assessed against pre-determined standards based on ESC guidelines. Results There were 23 pregnancies in 15 women with MFS. 13/23 (57%) occurred in women with aortic dilatation at baseline. There were 3 important maternal cardiac events (type A dissection; deterioration in left ventricular function; significant left ventricular and progressive aortic dilatation). Four women did not have access to expert pre-conception counselling. These women were all referred to the JCOS late in established pregnancy. Imaging was often delayed and only 7/23 cases (30%) met the standard for minimum frequency of echocardiographic surveillance. Only 12/23 (52%) had pre-conception imaging of the whole aorta with CT/MRI. Distal aortic dilatation was identified in 7/23 cases but none of these underwent further MRI evaluation during pregnancy. Conclusion Despite having a dedicated JCOS, our data show that facilitating complete obstetric and cardiac care for this group remains challenging. Education of local care providers and timely referral for expert pre-conception counselling in a JCOS are key.

Objective All women with CHD, especially those with more severe disease, should be offered preconception counseling (PCC), to discuss the risk of complications and to plan a future pregnancy. Several scoring system have been devised to estimate the risk of adverse events in pregnancies complicated by maternal heart disease (HD) and while comparisons have been made across the whole population, none have focused on the high-risk population. Methods Retrospective cohort study that included women classed as modified WHO (mWHO) 3 and 4 who had a pregnancy from at least 20weeks gestation between 1994 and 2015 managed within our institution. We assessed how well the quoted risk (at PCC) of an adverse event (maternal or fetal) related to the actual rate of occurrence. We calculated NYHA and CARPREG scores for all patients, and the clinician assessment of percentage risk, to predict the occurrence of an adverse outcome. Results We identified 76 mWHO 3 and 4 women who had a total of 102 pregnancies. However, only in 63 pregnancies had the woman attended PCC. Both maternal and fetal adverse events were common. NYHA did not significantly predict any adverse events, whilst a CARPREG score of >3 score predicted heart failure and mWHO4 score predicted maternal death. However, the best prediction of adverse outcomes was a composite quoted risk (percent) given at PCC. Conclusions Women must have access to PCC as those with worse CARPREG and mWHO scores encounter greater adverse events.

Conceptually the right ventricle (RV) is less suitable to support the Fontan circulation than the left (LV). After palliation of hypoplastic left heart syndrome (HLHS) involving aortic reconstruction during the Norwood procedure the RV is exposed to abnormal afterload. We studied ventricular function and ventriculo-arterial coupling in HLHS patients (RV) and Fontan patients with single LV morphology that did (LV+N) and did not (LV-N) undergo Norwood-type aortic reconstruction.Eighty patients (55 RV, 8 LV+N, 17 LV- N) were simultaneously studied with the conductance-catheter and echocardiography 4.8 (0.9-22.9)years after Fontan completion.Ejection fraction (EF) was lowest in the HLHS group (RV 60.9±11.0 vs. LV+N 68.4±10.5 vs. LV-N 69.7±8.0, P=0.003) whereas end systolic elastance (Ees), i.e. ventricular contractility, and end diastolic stiffness (Eed) were highest (Ees: RV 3.38±2.2 vs. LV+N 2.3.±13.8 vs. LV-N 1.92±1.37mmHg/ml, P=0.02; Eed: RV 0.59±0.36 vs. LV+N 0.48±0.29 vs. LV-N 0.32±0.17mmHg/ml, P0.02). Arterial elastance, a measure of afterload, was highest in HLHS patients and correlated positively with Ees and Eed and inversely with EF in the study cohort. Only long axis function analysis suggested superior ventricular function in HLHS patients whereas all other echocardiographic measures did not reveal any group differences.Ventricular contractility of the RV of HLHS patients is higher than that of the ventricle of Fontan patients with LV morphology. This likely reflects a physiological response to higher arterial elastance resulting from aortic arch reconstruction. Increased arterial elastance negatively impacts diastolic stiffness, which is higher in the systemic RV than LV.

Introduction Arrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation. Methods Five consecutive patients (3 female, mean age 47.8 ± 12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients. Results Patients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4–1375) from their last ablation. During a median follow up of 31 months (range 1–70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing. Conclusions Catheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.

© 2016 Elsevier Ireland LtdBackground Systemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity. Methods and results Twenty-two consecutive patients, mean age 28 ± 8 years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P = 0.002 & P = 0.049), prolonged systemic RV isovolumic contraction time (P = 0.024) and reduced systemic RV free wall and septal excursion (P = 0.027 & P = 0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r = 0.73 & P = 0.004 and r = 0.62 & P = 0.041, respectively, and QRS duration r = 0.68, P

Objective To identify the factors associated with an increased post-partum blood loss in women with congenital heart disease (CHD). Methods The study was a retrospective cohort study, which included 366 nulliparous women with CHD and a singleton pregnancy cared for in a single tertiary centre (Chelsea and Westminster Hospital) between 1994 and 2014. The women were classified into one of 12 different functional groups and univariate and multivariate regression analysis were used to identify factors associated with increased blood loss at delivery. Results The average volume of blood loss in women with CHD was twice that expected. Univariate analysis showed that White European women had the lowest blood loss. Women who had been on anticoagulants, had a forceps delivery, emergency Caesarean section or general anaesthesia lost more blood than those having a spontaneous vaginal birth under regional analgesia. Higher CARPREG scores were associated strongly with increased blood loss. Women with a Fontan circulation had the highest blood loss and the difference remained significant after correcting for other significant variables. Conclusions Women with CHD are at increased risk of PPH. We have identified several potentially modifiable risk factors that may be targeted to reduce this risk. In addition, women with a Fontan circulation were most prone to PPH, independent of other risk factors, suggesting the existence of lesion-specific abnormalities and the need for extra vigilance in this group of women at the time of birth.

Background: Adult congenital heart disease (ACHD) guidelines advise life-long, regular, follow up in predefined intervals for ACHD patients. However, limited data exist to support this position. We examine, herewith, compliance to scheduled outpatient clinic appointments and its impact on outcome. Methods and results: We examined 4461 ACHD patients (median age at entry 26.4 years, 51% female) and their follow up records at our tertiary centre between 1991 and 2008. Clinic attendance was quantified from electronic hospital records. For survival analysis we employed the last clinic attendance before 2008 as starting of follow-up. Overall 23% of scheduled clinic appointments were not attended. The main predictors of clinic non-attendance (CNA) were younger age, non-Caucasian ethnicity, lower socioeconomic status, number of previous CNAs and the lack of planned additional investigation/s (e.g. echocardiography) scheduled on the same day. During a cumulative follow-up time of 48,828 patient-years, 366 (8.2%) patients died. Both, the number of CNAs (HR = 1.08, 95% CI 1.05–1.12 per CNA, P < 0.001) and the ratio of CNA to follow up period (HR = 1.23, 95% CI 1.04–1.44 per CNA/year, P = 0.013) emerged as predictors of mortality independent of adjustment for patients' age, disease complexity, functional class and socioeconomic status. Conclusions: Patient adherence to scheduled ACHD outpatient-clinics is associated with better survival. Identifying patients at an increased risk of CNA in a single tertiary centre is feasible. Our data provides previously lacking evidence supporting the practice of periodic assessment of ACHD patients at tertiary clinics. Non-attenders should be specifically targeted and receive counselling to modulate their increased risk of death.

Objectives To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. Background Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. Methods Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). Results Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. Conclusions Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Background Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. Methods and results CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0±12.8years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR 150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end–end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133±20/73±10mmHg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9years was low (0.69% per year, 95% CI: 0.33–1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43–5.72, p Conclusion Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

Background Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received. Methods Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders. Results 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic. Conclusions There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

Patent foramen ovale (PFO) has long been implicated with cryptogenic stroke, migraine and decompression illness. PFO is common and its implicated pathologies cause devastating neurological sequelae; and hence have drawn the attention of medical practitioners across disciplines. The pathogenesis is hypothesized to be caused by micro-emboli or neuro-hormones which would otherwise being filtered by the lungs, astraying into the systemic circulation via the atrial communication especially during Valsalva maneuver. Treatment options have been proposed; among others are medical therapy, PFO closure or both. While medical therapy as secondary prevention is being adopted by most centers in the world, PFO closure is performed in selected patients only. The reason being is that most studies linking PFO to these pathologies are observational in nature. And these associations do not equate to a firm cause and effect relationship. For causal relationship to be established, good quality prospective data is required. Recently, there has been emergence of new prospective trials which improve the understanding of PFO closure in these pathologies. This article reviews the associations between PFO and the three main implicated pathologies as well as the evidence for PFO closure in the current era.

Aims Repaired tetralogy of Fallot (rtoF) patients are at risk of atrial or ventricular tachyarrhythmia and sudden cardiac death. Risk stratification for arrhythmia remains difficult. We investigated whether cardiac anatomy and function predict arrhythmia. Methods One-hundred-and-fifty-four adults with rtoF, median age 30.8 (21.9–40.2) years, were studied with a standardised protocol including cardiovascular magnetic resonance (CMR) and prospectively followed up over median 5.6 (4.6–7.0) years for the pre-specified endpoints of new-onset atrial or ventricular tachyarrhythmia (sustained ventricular tachycardia/ventricular fibrillation). Results Atrial tachyarrhythmia (n = 11) was predicted by maximal right atrial area indexed to body surface area (RAAi) on four-chamber cine-CMR (Hazard ratio 1.17, 95% Confidence Interval 1.07–1.28 per cm2/m2; p = 0.0005, survival receiver operating curve; ROC analysis, area under curve; AUC 0.74 [0.66–0.81]; cut-off value 16 cm2/m2). Atrial arrhythmia-free survival was reduced in patients with RAAi ≥ 16 cm2/m2 (logrank p = 0.0001). Right ventricular (RV) restrictive physiology on echocardiography (n = 38) related to higher RAAi (p = 0.02) and had similar RV dilatation compared with remaining patients. Ventricular arrhythmia (n = 9) was predicted by CMR RV outflow tract (RVOT) akinetic area length (Hazard ratio 1.05, 95% Confidence Interval 1.01–1.09 per mm; p = 0.003, survival ROC analysis, AUC 0.77 [0.83–0.61]; cut-off value 30 mm) and decreased RV ejection fraction (Hazard ratio 0.93, 95% Confidence Interval 0.87–0.99 per %; p = 0.03). Ventricular arrhythmia-free survival was reduced in patients with RVOT akinetic region length > 30 mm (logrank p = 0.02). Conclusion RAAi predicts atrial arrhythmia and RVOT akinetic region length predicts ventricular arrhythmia in late follow-up of rtoF. These are simple, feasible measurements for inclusion in serial surveillance and risk stratification of rtoF patients.

Background Systemic right ventricular (RV) function is a major determinant of long-term outcome in patients with palliated hypoplastic left heart syndrome (HLHS). Abnormal elastic properties of the reconstructed aorta may negatively impact on ventricular function. We therefore aimed to assess arterial elastance and its relationship to systemic RV function in these patients. Methods Fifty-six HLHS patients (median age 5.4 years; range 2.9–14.2 years) were studied at a median of 2.6 years (range 0.8–12.7 years) after completion of the Fontan circulation with the pressure–volume conductance system. Results Arterial elastance (Ea) was abnormally high and correlated inversely with RV ejection fraction (r = − 0.42, P = 0.001). However, end systolic elastance (Ees) – a load independent measure of intrinsic systolic ventricular function – and more so end diastolic stiffness (Eed) were positively correlated with Ea (Ees vs. Ea: r = 0.44, P = 0.001: Eed vs. Ea: r = 0.62, P Patients who were treated for significant aortic arch obstruction after surgical palliation showed higher Ea and Eed even four years after successful treatment compared to the remainder of the group (Ea: 3.4 ± 1.2 vs. 2.8 ± 1.0 mm Hg/ml, P = 0.04 and Eed: 0.67 ± 0.44 vs. 0.45 ± 0.3 mm Hg/ml, P = 0.04). Conclusions Arterial elastance is abnormally high in palliated HLHS patients and negatively impacts on ejection fraction but not on intrinsic systolic RV function early after completion of the Fontan circulation. Increased arterial elastance, however, is associated with increased RV diastolic stiffness with potential adverse effects on long-term outcome. Furthermore, arterial elastance and diastolic stiffness are particularly high in patients who needed treatment for aortic arch obstruction.

Objectives To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome. Methods All adult patients with Eisenmenger syndrome treated with DTT at our institution were included. Functional class (FC), oxygen saturation and 6-minute walk test distance (6MWTd) were analysed retrospectively. Results Between 2002 and 2010, 79 Eisenmenger patients (21 males, 16 with Down syndrome) aged 34±10years (range 17–68years) were included. Median follow-up was 3.3years (range 0.2 to 8.9years). 6MWTd increased early after initiation of DTT, with a plateau after approximately 3years and no obvious trend towards a deterioration on average during longer-term follow-up. Two patients died during follow-up and escalation of treatment was required in 18 patients after a median period of 2.5years. Escalation of therapy was also associated with an increase in 6MWTd. In addition, FC improved on DTT and oxygen saturation, increased, both at rest and peak exercise. This effect was more pronounced in the patients with the lowest baseline oxygen saturation at rest. Conclusions Long-term DTT is safe and improves objective exercise capacity and subjective symptoms. Response to DTT was typically observed early after initiation of DTT and was, on average, maintained longer-term. However, 1 in 5 patients required escalation of DTT, with time, due to symptomatic deterioration and this was associated with an afresh improvement in 6MWTd.

BACKGROUND: The size of the remnant left ventricle (LV) may influence right ventricular function and thus long-term outcome in palliated hypoplastic left heart syndrome (HLHS). We therefore sought to assess the impact of the size of the hypoplastic LV on intrinsic RV function in HLHS patients after Fontan surgery. METHODS: Fifty-seven HLHS patients were studied 2.5 (range: 0.8-12.6) years after Fontan-type palliation with the pressure-volume conductance system. The patient cohort was divided into two groups according to the median LV area index (group 1: LV area index≤1.33cm(2)/m(2), n=29; group 2: LV area index>1.33cm(2)/m(2), n=28). RESULTS: The slopes of the end systolic elastance (Ees) and the preload recruitable stroke work relation (Mw) were not different between group 1 and 2 (Ees: 2.70±1.92 vs. 3.68±2.68mmHg/ml; Mw: 52.75±14.98 vs. 51.09±16.63mmHg x ml; P=NS for all). Furthermore, the systolic responses to dobutamine were not statistically different between groups. However, the slope of the end diastolic stiffness (Eed) was higher in group 2 and catecholaminergic stimulation resulted in a decrease in Eed in group 2 (group 1: 0.40±0.26 vs. 0.52±0.45; group 2: 0.68±0.44 vs. 0.47±0.38mmHg/ml, P