S Bowater

Employment characteristics of a complex adult congenital heart disease cohort

Background Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. Aims To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. Methods Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). Results A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). Conclusions The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.

Management of advanced adult congenital heart disease

There are increasing numbers of adults with congenital heart disease. These patients remain at lifelong risk of complications including heart failure, arrhythmias and premature death. This review examines the management of those patients with advanced disease, with particular reference to patients with either a systemic right ventricle or a univentricular circulation. Drugs used to treat left ventricular dysfunction in acquired heart disease have been shown to have little benefi t in this setting. There are, however, promising results from small trials looking at selective pulmonary vasodilators in patients with a previous Fontan operation. Whilst there is evidence of a benefi t with implantable cardiac defi brillators and cardiac resynchronisation, there remains a lack of clear guidelines as to which patients will benefi t from these invasive therapies. Cardiac transplantation in these patients is associated with an increased early mortality but the long term outcome is similar to those with acquired heart failure. Transplantation however, is limited by both the patient’s suitability and the availability of a matched organ. End of life care should be discussed with all patients with advanced disease and ideally this should be done early on and in parallel to other therapies.

Pregnancy outcomes in patients with a fontan circulation and proposal for a risk-scoring system: single centre experience

BackgroundPregnancy in women with Fontan physiology poses a significant management challenge and is deemed high risk. The aim of this study is to describe short and long-term pregnancy outcomes in women who have undergone Fontan palliation and propose a novel risk-stratification model specific to women with a Fontan heart.MethodsWe undertook a single-centre, retrospective cohort study of all female Fontan patients (n = 78) from 1991–2015. We recorded pregnancy outcome, maternal cardiovascular and obstetric complications and fetal outcome. We propose a risk stratification model to identify those women who might be at highest risk of adverse outcomes during pregnancy.ResultsTwenty-one women had 55 pregnancies, with 13 (24%) live births and 38 (69%) spontaneous miscarriages (p < 0.001). Eight (62%) out of 13 live birth pregnancies incurred maternal cardiovascular complications and six (46%) experienced maternal obstetric complications. Median gestational age at delivery was 32(27–39) weeks with 12 out of 13 (92%) pregnancies resulting in pre-term delivery. There were more pregnancies (OR 4.90, 95% CI 1.46–16.42, p ≤ 0.01) and a trend towards a higher proportion of live births (OR 7.60, 95% CI 1.81–31.97, p = 0.06), in the ‘lower risk’ compared to those women in the ‘very high risk’ group. There were no maternal deaths.ConclusionsWe observed a high first trimester miscarriage rate, significant maternal cardiovascular and obstetric complication rates and a high rate of pre-term births in pregnant Fontan women. Our risk stratification model requires further investigation but may identify those women at particularly high risk of a poor outcome, and inform realistic pre-pregnancy counselling.

9 Long-term outcomes of adults with alcapa repair

Background Anomalous left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare congenital abnormality, which, if untreated, can cause complications such as myocardial infarction, heart failure and death; only few untreated patients survive to adulthood. We sought to determine long-term outcomes of these patients in our Adult Congenital Heart Disease service. Methods A retrospective review of case notes of patients with ALCAPA was performed, reviewing clinic letters, operation details, cardiac imaging and exercise tests. Results We identified 8 patients (6 female) with mean age 26.3±6.2 years. Mean follow-up was 4.1 years (range, 18 months to 12 years). All patients have had surgical repair; one patient required concomitant mitral valve repair. Mean age at operation was 9.7 years (range, 3 months to 34 years old); 3 patients (38%) were operated as adults. At last clinic review, one patient had NYHA III symptoms, the rest were well (NYHA I). Mean peak oxygen consumption on cardiopulmonary exercise testing was 36±2.4 ml/kg/min (range 33.4 to 38.8 ml/kg/min, mean 93%±15.9% predicted, range 78% to 112%). All patients were in sinus rhythm and no ischaemia or arrhythmias were identified. 7 patients (88%) had good left ventricular function (mean EF 61%); 1 patient had mildly impaired function (EF 50%) due to an apical transmural infarction. Moderate mitral regurgitation was seen in 3 patients (38%) and all had normal aortic root size. Conclusion We describe long-term outcomes of patients with ALCAPA syndrome. Postoperatively, the majority remain asymptomatic with good exercise capacity. Their left ventricular systolic function is good. Life-long follow up is warranted.

74 Non-invasive Assessment of Pulmonary Haemodynamics in Fontan Patients

Introduction The Fontan circulation relies on a low pulmonary vascular resistance. At present the gold standard method of measuring this is with invasive cardiac catheterisation which can be difficult in this group. Previous work has shown that pulmonary vascular resistance is inversely correlated with pulmonary capacitance which can be approximated non-invasively using data derived from a maximal cardiopulmonary exercise test (CPEX). We have determined the non-invasive pulmonary artery capacitance (Ventilatory Product) in a large cohort of adult Fontan patients. Methods 220 patients under regular follow up at University Hospital Birmingham were identified and CPEX results were available for 131 patients. We subdivided these into two groups, those with an atriopulmonary Fontan (AP) and combined those with lateral tunnel and total cavo-pulmonary connexion (TCPC). Parameters obtained were: NYHA functional class, maximum workload, ventilatory product, peak oxygen consumption, peak end tidal CO2 and VE/VCO2 slope. Results 77 patients with TCPC/lateral tunnel versus 54 AP Fontan were included. In the AP Fontan group, mean ventilatory product correlated positively with pVO2 (r2=0.344) and maximum workload (r2=0.515) and negatively with VE/VCO2 slope (r2=-–0.366). In the combined TCPC/lateral tunnel Fontan group, mean ventilatory product correlated positively with pV02 (r2=0.484) and maximum workload (r2=0.485) and negatively with VE/VCO2 slope (r2=-0.127). When determined for each functional class ventilatory product was as follows in TCPC/lateral tunnel group: NYHA I 355.8 +/-±100, NYHA II 272.14 ± 105, NYHA III 241.76 ± 90.3. In the AP Fontan group mean ventilatory product was as follows: NYHA I 349.2 ± 131.8, NYHA II 271.5 121.5, NYHA III 273.9 +/- 140.6. The mean VO2 peak was significantly higher in the combined TCPC/lateral tunnel group (26.64 vs 20.56, p = 0.00). Further sub analysis within each functional class between both groups also showed statistical significance. TCPC/lateral NYHA class I mean V02 peak 29.56 vs 25.11 vs (p = 0.01), class II mean V02 peak 22.72 vs 18 (p = 0.038) and class III mean 22.14 vs 15.31 (p = 0.018). Discussion Invasive measurements of PVR are difficult in the Fontan patient. We have demonstrated that a non-invasive indirect measurement of pulmonary artery capacitance -the ventilatory product, correlates with measures of performance, and negatively correlates with VE/VCO2, which is known to be of prognostic significance in heart failure. Furthermore our data shows that ventilatory product falls with increasing functional class. From this, we propose that the ventilatory product may be used to identify Fontan patients suitable for pulmonary vasodilator therapy. Our hypothesis however will require testing in prospective studies of pulmonary vasodilator therapy in Fontan patients.

20 Do atriopulmonary (AP) fontan dimensions using CMR influence the development of atrial tachyarrhythmia

Introduction Atrial tachyarrhythmia is regarded as a late consequence of the Fontan circulation, particularly in those with an AP Fontan and is associated with significant morbidity and mortality. Developing methods to recognise those at the highest risk will allow early intervention, improving patient outcomes. Purpose To establish relationship between AP Fontan chamber (FC) dimensions, ventricular volumes and function and the development of atrial arrhythmia (AR). Methods Modified AP Fontan patients with CMR (1.5T, Avanto, Siemens) were reviewed (Figure 1). Horizontal long axis (HLA, TrueFISP cine, flip angle 80, TR/TE 50/1.26ms, retrospective ECG gating) and transaxial 3D MRA non-contrast (Modified Siemens Whole Heart coronary artery sequence, respiratory navigator, ECG-triggered, free breathing, slice thickness 2.00mm) of the FC and the left atrium were examined. Abstract 20 Figure 1 HLA of 48 year old male with 5 ablations. End-diastolic area of 69cm2. Slow flow can be seen in the FC Results 55 patients 58% female and 42% male, mean age 33yrs (SD 7.9). 32 patients suffered a documented AR, most commonly atrial flutter (79%). There was a significant difference in age between the two groups with those having an AR tending to be older (36yrs vs 29yrs p < 0.05) the age of procedure was also younger in the AR group (6yrs vs. 10yrs p < 0.05) (Table 1). There was no difference in ventricular ejection fraction (p > 0.05). There were no significant differences in atrial parameters between the two groups (p > 0.05), however all measures of the FC were statistically different with larger measures occurring in the AR group (p < 0.05). Receiver operator characteristic curves were constructed for each of the atrium and FC variables. Only end diastolic area and FC longitudinal dimension produced statistically significant results (ROC 0.745 P < 0.05 and 0.719 p < 0.05). Using an upper limit of 27cm2 for end diastolic FC area odds ratio of AR was 8.57 (p < 0.05) with a sensitivity of 0.596 and a specificity of 0.870. Abstract 20 Table 1 Patient demographics and CMR parameters Non-arrhythmia group N=23 (Standard deviation) Arrhythmia Group N=32 (Standard Deviation) Age (yrs) 29(5) 36(9) P < 0.05 * Age of Fontan Procedure (yrs) 6(4) 1 (9) P < 0.05 * Ejection fraction (%) 55(11) 55(12) P > 0.05 * End Diastolic Area of Fontan chamber (cm 2 ) 19(8) 30(16) P < 0.05 * End Diastolic Longitudinal Value of Fontan Chamber (cm 2 ) 4(1) 6(2) P < 0.05 * End Systolic Area of Fontan Chamber (cm 2 ) 23(11) 32(16) P < 0.05 * End Systolic longitudinal Value Fontan Chamber (cm 2 ) 5(1) 6(2) P < 0.05 * * P values represent significance level of between group t-tests Conclusions Increasing AP FC size is associated with increased risk of AR.

75 The Burden of AF and Stroke in Adult Congenital Heart Disease

Introduction Atrial arrhythmias are strongly associated with thromboembolism with 1 in 5 strokes being attributed to atrial fibrillation (AF). Patients with adult congenital heart disease (ACHD) are at an increased risk of developing atrial arrhythmias compared to those with structurally normal hearts either due to the inherent cardiac anomaly or due to scar formation from surgery. The CHA2DS2VASC score is validated for stroke risk stratification for those with acquired heart disease. Its role in adult congenital heart disease however, is unclear. Methods A retrospective review of the electronic ACHD database of all patients at a large quaternary specialist ACHD Centre with approximately 4500 patients under regular follow up. Data spanned from year 2000–2015 and data collected from referral letters, clinic letters, ECG and echocardiograms. Results 376 patients with atrial arrhythmias were identified. Mean age was 57.7 ± 15.8 years with 51% female. 88 (23%) of patients had complex disease (including those with a Fontan circulation) with the remainder having simple or moderate forms of congenital heart disease. In this cohort, 52 (13.8%) patients had at least one thrombo-embolic event with 77% being either transient ischaemic attacks or strokes. The remainder of events were embolic peripheral artery occlusions, deep vein thromboses and pulmonary emboli. The mean age of first occurrence of thromboembolic event was 44.2 ± 19 years and mean CHA2DS2VASC score was 1.04 ± 0.7 prior to the event. In 46% of cases the thromboembolic event occurred after the atrial arrhythmia had been diagnosed and 71% of patients were not anticoagulated at the time of their first event. Of those patients anticoagulated at the time of their event (n = 9), 2 patients had a documented subtherapeutic INR, 2 were non-compliant with their medication and 1 was on a Novel Oral Anticoagulant (NOAC). It was unclear in 11% of cases whether they were anticoagulated before the event. 88% of patients with a CHA2DS2VASC score of 2 or more were anticoagulated, with 93% on warfarin and 5% on NOACs and 2% on low molecular weight heparin. Of those with a CHA2DS2VASC score of 0–1, 74% were on either warfarin or a NOAC. There were 4 known deaths from complications related to anticoagulation (2 gastrointestinal bleeds and 2 from massive haemoptysis). Discussion Atrial arrhythmias are common in ACHD and there is a high incidence of stroke in those with AF. In those groups with higher incidence of AF, loop recorders may be beneficial for detecting asymptomatic disease. The CHA2DS2VASC scoring system does not appear to be applicable for stroke risk stratification in ACHD. The mean pre- event CHA2DS2VASC was 1. Another marker for patients with ACHD is required to differentiate low risk from high risk patients. The role of NOACs in this group of patients is unclear and needs further evidence. 1 patient had a stroke on a NOAC despite being compliant.

P30 Early outcomes in adults with palliated hypoplastic left heart syndrome

Background Patients with palliated hypoplastic left heart syndrome (HLHS) are now surviving into adulthood. We anticipate that these patients may develop significant morbidity related to their Fontan circulation. We sought to determine long-term complications including cardiac symptoms, structural pathology, such as ventricular failure, atrio-ventricular valve regurgitation, and rhythm disturbances. Methods We performed a retrospective review of the case notes of all patients with HLHS under our care, reviewing clinic letters, cardiac imaging and exercise tests. Results We identified 27 HLHS patients (75% male) with mean age 20.4 ± 2.9 years. The majority of patients (74%) had undergone a stage III (Fontan) definitive palliation. At their last clinic review, only 37% (n = 10) of patients were breathless on exertion of which 6 had NYHA III symptoms. Mean arterial saturations were 90% (range of 71–97%) and mean peak oxygen consumption (VO2 max) was 25.3 ml/kg/min (mean 60% predicted). 70% of patients (n = 19) had evidence of systemic right ventricular impairment ranging from mild (n = 12, 44%) to severe (n = 2, 7%). 74% (n = 20) had evidence of mild or moderate systemic atrio-ventricular valve regurgitation. No patients developed significant heart failure requiring advanced therapy or cardiac transplantation. All patients were in sinus rhythm, 30% (n = 8) had previous rhythm disorders; 3 atrial flutter requiring electrical or medical cardioversion and ablations, 1 non-sustained ventricular tachycardia and 4 bradyarrhythmias requiring permanent pacemaker insertion. 89% (n = 24) of patients were anti-coagulated with Warfarin. 1 patient suffered an ischaemic stroke. No other complications of Fontan circulation, such as hepatic dysfunction or protein losing enteropathy, were seen. Conclusion We describe our experience of HLHS patients following transition to adult care. In general, HLHS patients reach early adulthood without any significant complications. The majority have a mild degree of systemic ventricular impairment. Rhythm disturbances are, so far, rare. Ongoing longitudinal follow up is necessary to establish the long term consequences of Fontan palliation for HLHS.