Psychosocial Risk Factors for Health-Related Quality of Life in Adult Congenital Heart Disease.

BACKGROUND: There is variability in the impact of adult congenital heart disease (ACHD) on health-related quality of life (HRQoL). A greater insight into the impact of ACHD may be gained from investigating HRQoL in various diagnostic groups and considering the importance of psychosocial risk factors for poor HRQoL. OBJECTIVE: We compared the HRQoL of people with ACHD with normative data from the general population and among 4 diagnostic groups and identified risk factors for poor HRQoL in ACHD from a comprehensive set of sociodemographic, clinical, and psychosocial factors. METHODS: We conducted a cross-sectional study with 303 participants from 4 diagnostic groups Simple, Tetralogy of Fallot, Transposition of the Great Arteries, Single Ventricle who completed measures of illness perceptions, coping, social support, mood, and generic and disease-specific HRQoL. Data were analyzed using 1-sample t tests, analysis of variance, and hierarchical multiple regressions. RESULTS: There was diminished psychosocial HRQoL in the Simple group compared with the general population. Consistently significant risk factors for poor HRQoL included younger age, a perception of more severe symptoms due to ACHD, depression, and anxiety. Clinical factors were poor predictors of HRQoL. CONCLUSIONS: The findings highlight the need to develop intervention studies aiming to improve HRQoL in people with ACHD and the routine assessment of illness perceptions and mood problems during key periods in people's lives. This will help address patient misconceptions that could be tackled by clinicians or specialist nurses during routine outpatient appointments and identify people in need of psychological support.

Cognitive dysfunction in adult CHD with different structural complexity.

OBJECTIVE: We carried out a cross-sectional study to assess cognitive function in a sample of adult CHD patients, within the Functioning in Adult Congenital Heart Disease study London. The association between cognitive functioning and disease complexity was examined. METHODS: A total of 310 patients participated in this study. Patients were classified into four structural complexity groups - tetralogy of Fallot, transposition of the great arteries, single ventricle, and simple conditions. Each patient underwent neuropsychological assessment to evaluate cognitive function, including memory and executive function, and completed questionnaires to assess depression and anxiety. RESULTS: Among all, 41% of the sample showed impaired performance (>1.5 SD below the normative mean) on at least three tests of cognitive function compared with established normative data. This was higher than the 8% that was expected in a normal population. The sample exhibited significant deficits in divided attention, motor function, and executive functioning. There was a significant group difference in divided attention (F=5.01, p=0.002) and the mean total composite score (F=5.19, p=0.002) between different structural complexity groups, with the simple group displaying better cognitive function. CONCLUSION: The results indicate that many adult CHD patients display impaired cognitive function relative to a healthy population, which differs in relation to disease complexity. These findings may have implications for clinical decision making in this group of patients during childhood. Possible mechanisms underlying these deficits and how they may be reduced or prevented are discussed; however, further work is needed to draw conclusive judgements.

Radiofrequency ablation of atrial tachyarrhythmias in adults with tetralogy of Fallot - predictors of success and outcome.

BACKGROUND: Adults with tetralogy of Fallot experience atrial tachyarrhythmias; however, there are a few data on the outcomes of radiofrequency ablation. We examined the characteristics, outcome, and predictors of recurrence of atrial tachyarrhythmias after radiofrequency ablation in tetralogy of Fallot patients. Methods/results Retrospective data were collected from 2004 to 2013. In total, 56 ablations were performed on 37 patients. We identified two matched controls per case: patients with tetralogy of Fallot but no radiofrequency ablation and not known to have atrial tachyarrhythmias. Acute success was 98%. Left atrial arrhythmias increased in frequency over time. The mean follow-up was 41 months; 78% were arrhythmia-free. Number of cardiac surgeries, age, and presence of atrial fibrillation were predictors of recurrence. Lone cavo-tricuspid isthmus-dependent flutter reduced the likelihood of atrial fibrillation. Right and left atria in patients with tetralogy of Fallot were larger in ablated cases than controls. NYHA class was worse in cases and improved after ablation; baseline status predicted death. Of matched non-ablated controls, a number of them had atrial fibrillation. These patients were excluded from the case-control study but analysed separately. Most of them had died during follow-up, whereas of the matched ablated cases all were alive and the majority in sinus rhythm. CONCLUSION: Patients with tetralogy of Fallot and atrial tachyarrhythmias have more dilated atria than those without atrial tachyarrhythmias. Radiofrequency ablation improves functional status. Left atrial ablation is more commonly required with repeat procedures. There is a high prevalence of atrial tachyarrhythmias, particularly atrial fibrillation, in patients with tetralogy of Fallot; early radiofrequency ablation may have a protective effect against this.

Haemodynamic consequences of targeted single- and dual-site right ventricular pacing in adults with congenital heart disease undergoing surgical pulmonary valve replacement.

AIMS: The purpose of this study was to create an epicardial electroanatomic map of the right ventricle (RV) and then apply post-operative-targeted single- and dual-site RV temporary pacing with measurement of haemodynamic parameters. Cardiac resynchronization therapy is an established treatment for symptomatic left ventricular (LV) dysfunction. In congenital heart disease, RV dysfunction is a common cause of morbidity-little is known regarding the potential benefits of CRT in this setting. METHODS AND RESULTS: Sixteen adults (age = 32 ± 8 years; 6 M, 10 F) with right bundle branch block (RBBB) and repaired tetralogy of Fallot (n = 8) or corrected congenital pulmonary stenosis (n = 8) undergoing surgical pulmonary valve replacement (PVR) for pulmonary regurgitation underwent epicardial RV mapping and haemodynamic assessment of random pacing configurations including the site of latest RV activation. The pre-operative pulmonary regurgitant fraction was 49 ± 10%; mean LV end-diastolic volume (EDV) 85 ± 19 mL/min/m(2) and RVEDV 183 ± 89 mL/min/m(2) on cardiac magnetic resonance imaging. The mean pre-operative QRS duration is 136 ± 26 ms. The commonest site of latest activation was the RV free wall and DDD pacing here alone or combined with RV apical pacing resulted in significant increases in cardiac output (CO) vs. AAI pacing (P < 0.01 all measures). DDDRV alternative site pacing significantly improved CO by 16% vs. AAI (P = 0.018), and 8.5% vs. DDDRV apical pacing (P = 0.02). CONCLUSION: Single-site RV pacing targeted to the region of latest activation in patients with RBBB undergoing PVR induces acute improvements in haemodynamics and supports the concept of 'RV CRT'. Targeted pacing in such patients has therapeutic potential both post-operatively and in the long term.

What do we know about cognitive functioning in adult congenital heart disease?

With the advent of improved medical and surgical care in congenital heart disease, there has been an increase in the number of patients who survive into adulthood, giving rise to a new patient population 'Adults with congenital heart disease'. In the past, morbidity and mortality were the primary concerns for this group. However, with improvements in outcome attention has shifted to other factors such as psychosocial and cognitive functioning. This paper reviews the literature on the cognitive functioning in adult congenital heart disease patients. A total of five relevant articles were retrieved via electronic searches of six databases, including MEDLINE, EMBASE, CINAHL, AMED, PsychINFO, and PubMed. The results displayed a consensus on the presence of some cognitive difficulties in adult congenital heart disease patients. The aetiology of cognitive dysfunctions appears to be multifactorial. The literature is limited by the very small number of studies looking at adults with congenital heart disease, with the majority focusing on cognitive functioning among children with congenital heart disease. However, the presence of cognitive dysfunctions and the resulting impact on the patient's day to day lives warrant for a more detailed and prospective research to enhance the understanding of its aetiology and impact.

Quality of life of adult congenital heart disease patients: a systematic review of the literature.

AIMS: This review explores the quality of life of adult congenital heart disease patients and the relationship between disease severity and quality of life. METHODS: We searched seven electronic databases and the bibliography of articles. The 31 selected studies fulfilled the following criteria: adult population; quantitative; assessment of quality of life and/or impact of disease severity on quality of life using validated measures; English language. Data extraction forms were used to summarise the results. RESULTS: There are evident methodological limitations within the reviewed studies such as heterogeneous populations, designs, and quality of life conceptualisations and measurements. Despite these problems, findings suggest that the quality of life of adult congenital heart disease patients is compromised in the physical domain compared with their healthy counterparts, whereas no differences were found in relation to the psychosocial and environmental/occupational domain. Some severity variables appear to be significant correlates of quality of life and could be considered in a future standardised classification of disease severity. CONCLUSION: The methodological limitations of past research in relation to the definition and measurement of quality of life, the study designs, and disease severity classifications need to be addressed in future studies in order to provide robust evidence and valid conclusions in this area of study. This will enable the development of targeted interventions for the improvement of quality of life in the adult population of congenital heart disease patients.

Successful percutaneous recanalization of thrombosed major aortopulmonary collateral artery in palliated tetralogy of fallot with pulmonary atresia.

We report the case of a 25-year-old male with palliated tetralogy of Fallot and pulmonary atresia presenting with thrombotic occlusion of a major aortopulmonary collateral artery to the right lung. Percutaneous intervention was successful in recanalizing this vessel, resulting in symptomatic improvement.

Postcardiotomy syndrome: recurrent cardiac tamponade and an exquisite steroid response.

A 26-year-old woman presented moribund with fever and pleuritic chest pain 3 times in 4 months following elective aortic root surgery. She was admitted 41 days after surgery with cardiac tamponade requiring surgical drainage twice within 1 week. Despite this, she was re-admitted for a second time 4 days after discharge with persistent pericardial effusion. High fevers and an incidental regurgitant murmur were extensively investigated for and treated as possible endocarditis or graft infection without conclusive results. The patient spent a total of 61 days in hospital during this period, receiving seven different antibiotic courses. Her third admission, with most severe clinical features, nearly led to further surgery and removal of her aortic graft but instead culminated in a multidisciplinary team decision to initiate steroid therapy for postcardiotomy syndrome. A short course of oral prednisolone saw her pericardial effusion and symptoms resolve completely.

The surgical history, management, and outcomes of subaortic stenosis in adults.

BACKGROUND: Subaortic stenosis (SAS) is a curtain of tissue involving the subaortic region, the aortic and mitral valves, the septum, and the fibrous trigones. Little is known of its course or the outcomes of its surgical management in adults. METHODS: We reviewed our experience of the surgical management of SAS in adults from 1999 to 2010. We divided patients into three groups: (1) those presenting for first-time SAS resection (6 patients, 4 male, median age of 46.9 ± 17 years, mean follow-up of 5 ± 2.7 years); (2) those requiring redo resection of SAS without organic aortic valve dysfunction (8 patients, 3 male, median age of 25.3 ± 5 years, mean follow-up of 8 ± 3.08 years); and (3) those with SAS and aortic valve dysfunction (8 patients, 4 males, median age of 34.8 ± 12 years, mean follow-up of 4.5 ± 2.5 years; 5 had previous SAS surgery). RESULTS: Patients underwent extensive excision of the SAS, release of the fibrous trigones, and a septal myectomy if required. There was 1 early death in group 2 and 1 in group 3. In group 3, 1 patient underwent the Ross procedure and 7 patients had mechanical valve implantation. No patient required permanent pacemaker implantation. Overall follow-up was 3.3 ± 3 years (range, 6 months to 10 years). The preoperative left ventricular outflow tract gradient ranged from 40 to 120 mm Hg, and the postoperative left ventricular outflow tract gradient ranged from 0 to 16 mm Hg. At latest follow-up, no patient in groups 1 or 2 had greater than mild native aortic regurgitation. CONCLUSIONS: Subaortic stenosis resection in adults can successfully relieve left ventricular outflow tract obstruction, with low mortality. The complexity of SAS increases with time; therefore a longer duration of follow-up is needed to further validate our conclusions.

Echocardiographic assessment of diastolic biventricular properties in patients operated for severe pulmonary regurgitation and association with exercise capacity.

AIMS: We aimed to assess the impact of surgical pulmonary valve replacement (PVR) for severe pulmonary regurgitation (PR) on biventricular function and its effect on exercise capacity. METHODS AND RESULTS: Seventy-three patients (mean age 23.6±11.5 years, 47 females) underwent surgical PVR for PR. Echocardiogram and magnetic resonance imaging to assess ventricular size and function, and a cardiopulmonary exercise test were performed before, and 1-year post-surgery. Median New York Heart Association class improved from 2 to 1 but peak oxygen uptake (VO2) did not change. Left ventricular (LV) cardiac output increased from 3.2±0.9 to 3.5±0.7 L/min (P=0.003). However, this was not associated with increased trans-mitral velocities (▵E=-0.13, P=0.004; ▵A=0.03, P=0.395), or increased heart rate (-0.002%, P=0.993). Trans-tricuspid rapid right ventricular (RV) filling increased significantly, whereas early diastolic myocardial velocity in RV wall decreased (E velocity: 0.57±0.14-0.65±0.21, P=0.034; and E/e' from 6.7±1.9 to 14.8±7.0, P<0.0001). RV and LV late diastolic velocities and their ratio to early velocities (A, a', E/A, and e'/a') correlated with pre- and/or post-PVR peak VO2. No correlations were found between indexes of systolic function and peak VO2, either before or after surgery. Doppler evidence of restrictive RV physiology resolved after elimination of PR. CONCLUSION: Surgical PVR for PR improves RV filling and increases left ventricular stroke volume, however, this could not be demonstrated by conventional Doppler echocardiography. Diastolic ventricular function was associated with exercise capacity. Because of its load dependency, E/e' ratio failed in assessing diastolic function. Pre-systolic flow in pulmonary trunk in presence of severe PR does not determine intrinsic myocardial stiffness.

Extraction of pacing leads jailed by a stent in a mustard circulation.

A case of successful lead extraction of atrial and ventricular pacing leads "jailed" by a stent in the superior limb of a Mustard baffle in a 40-year-old man with surgically corrected transposition of the great arteries and pacemaker pocket infection. Manual traction alone was sufficient to remove the jailed leads with no subsequent complications.

Transplantation for adults with congenital heart disease.

Heart transplantation is a recognised treatment for end-stage heart failure of any cause including congenital heart disease. Congenital heart disease has contributed relatively little to the adult heart transplant activities in the past two decades. However, this is likely to change as an increasing number of children with congenital heart disease reach adulthood because of the advances in paediatric cardiology and surgery. Some of these grown-ups with congenital heart disease (GUCH patients) will need transplantation for late myocardial dysfunction either secondary to uncorrected lesions, or despite previous repair or palliative surgery. These patients are managed along the same clinical principles as those with cardiac failure of other aetiologies, despite the lack of any evidence to support this approach. Nevertheless, they introduce new challenges. First, some may have pulmonary vascular disease and require heart-lung transplantation, or lung transplantation combined with repair of their cardiac defects. Second, those with failing Fontan circulation are usually much sicker than other transplant candidates, with protein-losing enteropathy along with renal and hepatic dysfunction. Third, a suitable donor organ may not be found due to elevated levels of antibodies in response to previous blood transfusions and possibly the previous implantation of homografts. Fourth, the operation may be technically difficult because of the presence of adhesions secondary to previous operations, collaterals, and unusual anatomy. Fifth, postoperative care may be complicated because of predisposition to bleeding, infection and pulmonary hypertension, and the presence of residual aortopulmonary collaterals resulting in a significant left-to-right shunt. Despite a higher early mortality, the overall results of heart transplantation so far have been encouraging with survivals similar to that of adults with acquired heart disease and that of the paediatric population. However, this may change as the proportion of high-risk patients (failing Fontans) increases. GUCH patients with Eisenmenger's syndrome may be offered lung transplantation with repair of the cardiac defect or heart-lung transplantation. However, because of the limited success of these approaches, and improved management of pulmonary hypertension, patient selection remains difficult.

The potential impact of percutaneous pulmonary valve stent implantation on right ventricular outflow tract re-intervention.

OBJECTIVE: To assess the impact of a percutaneous technique for pulmonary valve implantation on the conventional surgical valve/conduit approach to right ventricular outflow tract re-intervention. METHODS: We have retrospectively reviewed our results following surgical or percutaneous re-intervention to the right ventricular outflow tract in both paediatric and adult groups. Between November 1998 and March 2004, 94 patients underwent surgical re-intervention to the right ventricular outflow tract. Percutaneous pulmonary valve implantation was introduced in October 2002 and 35 procedures were performed to March 2004. The median age was 26 years (6-65 years) in the surgical group and 16 years (9-39 years) in the percutaneous group. Tetralogy of Fallot was the commonest original diagnosis (64.9 and 62.9%, respectively). The predominant indication for re-intervention in the surgical group was pulmonary regurgitation (64.9%) compared to the percutaneous group in which it was homograft/conduit stenosis or a mixed lesion (68.6%). RESULTS: There has been one (1.1%) early death reported in the surgical series and none in the percutaneous group. In the surgical group 9 (9.6%) experienced a procedural complication whilst 3 (8.5%) of those undergoing a percutaneous valve experienced a significant procedural event necessitating urgent surgery. Important early morbidity was 8 (8.5%) in the surgical group and 2 (5.7%) in the percutaneous group. Freedom from re-operation at 1 year was 100% in the surgical group and 86.1% in the percutaneous group due to late restenosis. Median hospital stay in the surgical group was 7 (4-114) days and 2 (2-22) days in the percutaneous group. CONCLUSIONS: Preliminary data suggests that percutaneous pulmonary valve implantation provides a promising additional and complementary approach to a successful surgical programme. Both approaches are safe with acceptable levels of morbidity and low mortality. With current technology the aneurysmal outflow tract remains a problem for the percutaneous approach. Follow-up remains too short, at present, to prove longevity of the percutaneous conduit.