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.

EPidemiology Of Cardiogenic sHock in Scotland (EPOCHS): A multicentre, prospective observational study of the prevalence, management and outcomes of cardiogenic shock in Scotland.

Background: Despite high rates of cardiovascular disease in Scotland, the prevalence and outcomes of patients with cardiogenic shock are unknown. Methods: We undertook a prospective observational cohort study of consecutive patients with cardiogenic shock admitted to the intensive care unit (ICU) or coronary care unit at 13 hospitals in Scotland for a 6-month period. Denominator data from the Scottish Intensive Care Society Audit Group were used to estimate ICU prevalence; data for coronary care units were unavailable. We undertook multivariable logistic regression to identify factors associated with in-hospital mortality. Results: In total, 247 patients with cardiogenic shock were included. After exclusion of coronary care unit admissions, this comprised 3.0% of all ICU admissions during the study period (95% confidence interval [CI] 2.6%-3.5%). Aetiology was acute myocardial infarction (AMI) in 48%. The commonest vasoactive treatment was noradrenaline (56%) followed by adrenaline (46%) and dobutamine (40%). Mechanical circulatory support was used in 30%. Overall in-hospital mortality was 55%. After multivariable logistic regression, age (odds ratio [OR] 1.04, 95% CI 1.02-1.06), admission lactate (OR 1.10, 95% CI 1.05-1.19), Society for Cardiovascular Angiographic Intervention stage D or E at presentation (OR 2.16, 95% CI 1.10-4.29) and use of adrenaline (OR 2.73, 95% CI 1.40-5.40) were associated with mortality. Conclusions: In Scotland the prevalence of cardiogenic shock was 3% of all ICU admissions; more than half died prior to discharge. There was significant variation in treatment approaches, particularly with respect to vasoactive support strategy.

Pregnancy and its management in women with GSD type III - a single centre experience.

We present a review of our experience and pregnancy outcome in patients with GSD III managed by our centre. Between 1997 and 2010 there were 15 pregnancies in seven women with GSD III. Four women had GSD IIIb (nine pregnancies) and three GSD IIIa (six pregnancies). There was a successful outcome in all 15 pregnancies with delivery of 15 liveborn infants. Four infants were of low birthweight (<2nd centile) but all have developed normally apart from one with behavioural/psychiatric problems. Three women had pre-existing cardiomyopathy prior to pregnancy. One of these women had deterioration of her cardiomyopathy during pregnancy and again in the post-partum period. Women with GSD III do not seem to have any issues with fertility. Overall the outcome of pregnancy for both mother and child is good. Care needs to be taken to avoid maternal hypoglycemia which may be associated with intrauterine growth restriction and low birth weight. Cardiac function should be monitored carefully particularly in those with pre-existing cardiomyopathy.

Pregnancy outcome and management of women with an implantable cardioverter defibrillator: a single centre experience.

AIMS: With improved survival of patients with congenital and inherited heart disease, there is now a younger cohort of patients with an implantable cardioverter defibrillator (ICD) for the prevention and treatment of ventricular dysrrhythmias. Young women with such disorders often wish to embark on pregnancy, but pregnancy outcome data for this group is sparse. We therefore evaluated pregnancy outcome in patients with heart disease and an ICD in situ. METHODS AND RESULTS: A retrospective analysis was performed on all women with an ICD in situ, who had pregnancy care provided by the specialist maternal cardiology service at University College London Hospitals. Data for 19 pregnancies in 14 women were collected. The underlying cardiac diagnoses were congenital heart disease (one), familial hypertrophic cardiomyopathy (eight), familial dilated cardiomyopathy (one), inherited long QT syndrome (one), and idiopathic cardiac arrest (one). Three women had moderate impairment of the left ventricular systolic function (ejection fraction <45%), in the remainder it was normal. Nine ICD implants were for primary prevention of sudden cardiac death (64%) and five for secondary prevention (36%). Of the 19 pregnancies, 18 continued beyond 24 weeks gestation with 18 live births. In eight pregnancies there were medical or device-related complications (42.9%) as follows: arrhythmias (four) (21.1%), heart failure (two) (9.1%), ICD shocks (one) (5.3%), atrial lead fracture (one) (5.3%), and lead-related thrombus (one) (5.3%). There were no inappropriate device shocks or therapies. CONCLUSIONS: Women with heart disease and an ICD implant can have a good outcome during pregnancy but medical and device complications are not uncommon.

Messages focused on the effect of alcohol on the immune system boosted intention to adhere to alcohol intake guidelines during Covid-19 lockdown.

Objective: Research shows that personal relevance may affect the impact of alcohol-related health information. This study explored alcohol consumption during the UK Covid-19 lockdown, and whether a message emphasising the effect of alcohol on the immune system was more effective in altering intentions to follow low-risk drinking guidelines than other messages about the effects of alcohol on health.Methods & Measures: From April to June 2020, 953 drinkers completed an online questionnaire, and were randomly allocated to exposure to a control condition or one of three messages emphasising the impact of alcohol on: the immune system; mental health; or physical health. Outcome variables were: concern about alcohol intake, and intention to adhere to low-risk drinking guidelines.Results: Pre-post ANCOVAs revealed that participants in the immunity message group had significantly stronger intention to adhere to low-risk guidelines than the control group (after controlling for initial intention). Concern for the effect of alcohol on health was not significantly affected.Conclusion: During Covid-19 lockdown, a message emphasising the impact of alcohol on the immune-system had a greater effect on intention to observe low-risk drinking guidelines than other messages. Contextually relevant messages could be used for alcohol health campaigns and for improving alcohol labelling.

Risk factors for complicated grief among family members bereaved in intensive care unit settings: A systematic review.

BACKGROUND: Families of intensive care unit (ICU) decedents are at increased risk of experiencing complicated grief. However, factors associated with complicated grief in ICU and bereavement needs assessment are not available routinely. We aimed to conduct a systematic review identifying risk factors associated with complicated grief among family members of ICU decedents. MATERIALS AND METHODS: MEDLINE, EMBASE, CINAHL, PsycINFO, the Cochrane Library and Web of Science were searched to identify relevant articles. Observational studies and randomised and non-randomised controlled trials were included. Studies were screened and quality appraised in duplicate. Risk of bias was assessed using Newcastle-Ottawa Scale. A narrative synthesis was undertaken. RESULTS: Seven studies conducted across three continents were eligible. Four studies were of high quality. 61 risk factors were investigated across the studies. Factors associated with a decreased risk of complicated grief included age, patient declining treatment and involvement in decision-making. Factors associated with increased risk included living alone, partner, dying while intubated, problematic communication, and not having the opportunity to say goodbye. CONCLUSION: This systematic review has identified risk factors which may help identify family members at increased risk of complicated grief. Many of the studies has small sample sizes increasing the risk of erroneously reporting no effect due to type II error. Some factors are specific to the ICU setting and are potentially modifiable. Bereavement services tailored to the needs of bereaved family members in ICU settings are required. (PROSPERO registration ID 209503).

Repopulation of decellularised porcine pulmonary valves in the right ventricular outflow tract of sheep: Role of macrophages.

The primary objective was to evaluate performance of low concentration SDS decellularised porcine pulmonary roots in the right ventricular outflow tract of juvenile sheep. Secondary objectives were to explore the cellular population of the roots over time. Animals were monitored by echocardiography and roots explanted at 1, 3, 6 (n = 4) and 12 months (n = 8) for gross analysis. Explanted roots were subject to histological, immunohistochemical and quantitative calcium analysis (n = 4 at 1, 3 and 12 months) and determination of material properties (n = 4; 12 months). Cryopreserved ovine pulmonary root allografts (n = 4) implanted for 12 months, and non-implanted cellular ovine roots were analysed for comparative purposes. Decellularised porcine pulmonary roots functioned well and were in very good condition with soft, thin and pliable leaflets. Morphometric analysis showed cellular population by 1 month. However, by 12 months the total number of cells was less than 50% of the total cells in non-implanted native ovine roots. Repopulation of the decellularised porcine tissues with stromal (α-SMA+; vimentin+) and progenitor cells (CD34+; CD271+) appeared to be orchestrated by macrophages (MAC 387+/ CD163low and CD163+/MAC 387-). The calcium content of the decellularised porcine pulmonary root tissues increased over the 12-month period but remained low (except suture points) at 401 ppm (wet weight) or below. The material properties of the decellularised porcine pulmonary root wall were unchanged compared to pre-implantation. There were some changes in the leaflets but importantly, the porcine tissues did not become stiffer. The decellularised porcine pulmonary roots showed good functional performance in vivo and were repopulated with ovine cells of the appropriate phenotype in a process orchestrated by M2 macrophages, highlighting the importance of these cells in the constructive tissue remodelling of cardiac root tissues.

Pregnancy outcome in women with congenital heart disease and residual haemodynamic lesions of the right ventricular outflow tract.

AIMS: To determine pregnancy outcome and risk factors for adverse events in women with congenital heart disease (CHD) and residual haemodynamic right ventricular (RV) outflow tract (RVOT) lesions. METHODS AND RESULTS: Pregnancy outcome data for women with CHD and residual RVOT lesions have been recorded since 2001. There were 76 pregnancies in 47 women that continued beyond 24 weeks gestation. At conception 20% had RVOT obstruction, 32% had pulmonary regurgitation (PR) and 49% had mixed RVOT obstruction and PR. Moderate-to-severe PR was present in 30 (39%) and RVOT obstruction > or =30 mmHg in 12 (16%) of pregnancies. Seven pregnancies (9%) were complicated by right heart failure (RHF). No arrhythmias were documented. Predictors for RHF were moderate-to-severe PR in combination with at least one additional risk factor (twin pregnancy, branch pulmonary artery stenosis, RV systolic dysfunction, RV hypertrophy). All patients responded to diuretic treatment and had a good pregnancy outcome without foetal complications. CONCLUSION: In patients with CHD and residual RVOT lesions, the outcome of pregnancy is good. Patients with moderate-to-severe PR were at risk for symptomatic RHF only if additional risk factors were present. When treated by a multidisciplinary team, maternal and foetal outcome was good. The general recommendation that pulmonary valve replacement should be undertaken prior to pregnancy in patients with moderate-to-severe PR and RV dilatation needs to be reconsidered.

Pregnancy in patients with implantable cardiac defibrillators.

The number of patients of reproductive age with inherited and congenital heart disease receiving implantable cardiac defibrillators (ICD) is steadily increasing. Safely and effectively coordinating pregnancy in this high-risk cohort is important to optimise maternal-foetal outcomes. As members of the multidisciplinary team caring for pregnant patients with indications for ICD, cardiologists and electrophysiologists should be aware of the considerations and nuances involved in managing these patients. This article reviews the pathophysiology of arrhythmias, ICD implantation considerations, novel minimal fluoroscopy techniques and subcutaneous ICD. In addition, antenatal and device management during pregnancy and delivery are discussed.

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.

A novel and unique treatment of right ventricular inflow obstruction in a patient with a Bjork modification of the Fontan palliation before pregnancy.

INTRODUCTION: Patients with complex congenital cardiac disease are increasingly surviving to adulthood and many are keen to consider pregnancy. Haemodynamic status should be optimal prior to embarking on pregnancy and for some this may mean surgical intervention to alleviate haemodynamic residua. METHODS: We report the successful implantation of a percutaneous pulmonary stent valve into a right atrial to right ventricular conduit in a young woman with a Bjork modification of the Fontan palliation to improve haemodynamics prior pregnancy. DISCUSSION: Catheter interventions offer a low-risk option for the treatment of haemodynamic residua and innovative use of new technologies such as the pulmonary stent valve presents a novel, safe, and effective treatment for such conduit problems.

Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A "Hidden" Burden of Early Outcome.

BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. Although 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome. METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day and 6-month mortality and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into 4 time intervals. RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. During the study period, 30-day mortality improved significantly, with an overall 30-day mortality of 1.5%; 6-month mortality and prolonged ICU stay were 2.4% and 6.7%, respectively. Despite a decreased number of preoperative patients in New York Heart Association Functional Classification III or higher, prolonged ICU stay increased over the eras. Predictors of adverse outcome were New York Heart Association class III or higher, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures. CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

Maternal and neonatal outcomes in women with history of coronary artery disease.

BACKGROUND: Pregnancy outcomes in women with pre-existing coronary artery disease (CAD) are poorly described. There is a paucity of data therefore on which to base clinical management to counsel women, with regard to both maternal and neonatal outcomes. METHOD: We conducted a retrospective multicentre study of women with established CAD delivering at 16 UK specialised cardiac obstetric clinics. We included pregnancies of 24 weeks' gestation or more, delivered between January 1998 and October 2018. Data were collected on maternal cardiovascular, obstetric and neonatal events. RESULTS: 79 women who had 92 pregnancies (94 babies including two sets of twins) were identified. 35.9% had body mass index >30% and 24.3% were current smokers. 18/79 (22.8%) had prior diabetes, 27/79 (34.2%) had dyslipidaemia and 21/79 (26.2%) had hypertension. The underlying CAD was due to atherosclerosis in 52/79 (65.8%), spontaneous coronary artery dissection (SCAD) in 11/79 (13.9%), coronary artery spasm in 7/79 (8.9%) and thrombus in 9/79 (11.4%).There were six adverse cardiac events (6.6% event rate), one non-ST elevation myocardial infarction at 23 weeks' gestation, two SCAD recurrences (one at 26 weeks' gestation and one at 9 weeks' postpartum), one symptomatic deterioration in left ventricular function and two women with worsening angina. 14% of women developed pre-eclampsia, 25% delivered preterm and 25% of infants were born small for gestational age. CONCLUSION: Women with established CAD have relatively low rates of adverse cardiac events in pregnancy. Rates of adverse obstetric and neonatal events are greater, highlighting the importance of multidisciplinary care.

Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction: is age a predictor of outcome?

BACKGROUND: The timing of pulmonary valve replacement (PVR) for free pulmonary incompetence in patients with congenital heart disease remains a dilemma for clinicians. We wanted to assess the determinants of improvement after PVR for pulmonary regurgitation over a wide range of patient ages and to use any identified predictors to compare clinical outcomes between patient groups. METHODS AND RESULTS: Seventy-one patients (mean age 22+/-11 years; range, 8.5 to 64.9; 72% tetralogy of Fallot) underwent PVR for severe pulmonary regurgitation. New York Heart Association class improved after PVR (median of 2 to 1, P<0.0001). MRI and cardiopulmonary exercise testing were performed before and 1 year after intervention. After PVR, there was a significant reduction in right ventricular volumes (end diastolic volume 142+/-43 to 91+/-18, end systolic volume 73+/-33 to 43+/-14 mL/m(2), P<0.0001), whereas left ventricular end diastolic volume increased (66+/-12 to 73+/-13 mL/m(2), P<0.0001). Effective cardiac output significantly increased (right ventricular: 3.0+/-0.8 to 3.3+/-0.8 L/min, P=0.013 and left ventricular: 3.0+/-0.6 to 3.4+/-0.7 L/min, P<0.0001). On cardiopulmonary exercise testing, ventilatory response to carbon dioxide production at anaerobic threshold improved from 35.9+/-5.8 to 34.1+/-6.2 (P=0.008). Normalization of ventilatory response to carbon dioxide production was most likely to occur when PVR was performed at an age younger than 17.5 years (P=0.013). CONCLUSIONS: A relatively aggressive PVR policy (end diastolic volume <150 mL/m(2)) leads to normalization of right ventricular volumes, improvement in biventricular function, and submaximal exercise capacity. Normalization of ventilatory response to carbon dioxide production is most likely to occur when surgery is performed at an age

Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome.

BACKGROUND: Percutaneous pulmonary valve implantation was introduced in the year 2000 as a nonsurgical treatment for patients with right ventricular outflow tract dysfunction. METHODS AND RESULTS: Between September 2000 and February 2007, 155 patients with stenosis and/or regurgitation underwent percutaneous pulmonary valve implantation. This led to significant reduction in right ventricular systolic pressure (from 63+/-18 to 45+/-13 mm Hg, P<0.001) and right ventricular outflow tract gradient (from 37+/-20 to 17+/-10 mm Hg, P<0.001). Follow-up ranged from 0 to 83.7 months (median 28.4 months). Freedom from reoperation was 93% (+/-2%), 86% (+/-3%), 84% (+/-4%), and 70% (+/-13%) at 10, 30, 50, and 70 months, respectively. Freedom from transcatheter reintervention was 95% (+/-2%), 87% (+/-3%), 73% (+/-6%), and 73% (+/-6%) at 10, 30, 50, and 70 months, respectively. Survival at 83 months was 96.9%. On time-dependent analysis, the first series of 50 patients (log-rank test P<0.001) and patients with a residual gradient >25 mm Hg (log-rank test P=0.01) were associated with a higher risk of reoperations. CONCLUSIONS: Percutaneous pulmonary valve implantation resulted in the ability to avoid surgical right ventricular outflow tract revision in the majority of cases. This procedure might reduce the number of operations needed over the total lifetime of patients with right ventricle-to-pulmonary artery conduits.

Use of high intensity adjusted dose low molecular weight heparin in women with mechanical heart valves during pregnancy: a single-center experience.

The use of standard dose low molecular weight heparin (LMWH) to anticoagulate women with mechanical valves in pregnancy is associated with morbidity and mortality. We conducted a prospective audit of the use of adjusted dose high intensity LMWH in 12 pregnancies in 11 women with prosthetic heart valves. LMWH +/- low-dose aspirin was started at therapeutic-dose with monitoring of anti-Xa levels to achieve a target level of 1.0-1.2 IU/mL (0.8-1.2 in the first 3/12 pregnancies). This necessitated a mean increase in the dose of LMWH of 54.4% (SD+/-33.2) over initial dose. Eleven of 12 pregnancies resulted in live births, with one intrauterine fetal death at 37 weeks. One non-fatal valve thrombosis occurred at 26 weeks gestation associated with subtherapeutic anti-Xa levels. Three patients experienced major bleeding. This regime provides a therapeutic option for women with mechanical heart valves during pregnancy, provided anti-Xa levels are kept within the target range. These patients require close surveillance for bleeding and thrombotic complications within a multi-disciplinary setting.