Cardiac implantable electronic devices in pregnancy: A position statement.

The aim of this document is to provide guidance for the management of women and birthing people with a permanent pacemaker (PPM) or implantable cardioverter defibrillator (ICD). Cardiac devices are becoming more common in obstetric practice and a reference document for contemporary evidence-based practice is required. Where evidence is limited, expert consensus has established recommendations. The purpose is to improve safety and reduce the risk of adverse events relating to implanted cardiac devices during pregnancy, birth and the postnatal period.

Travel insurance: an adult CHD patient's perspective.

Many adult CHD patients encounter difficulties in obtaining affordable travel insurance. We aimed to assess their travel habits and perspectives through a questionnaire. Our results indicate that many adult CHD patients use travel insurance but incur a premium due to their condition. There is an urgent need to provide better guidance to these patients on travel insurance options available to them.

Direct current cardioversion in pregnancy: a multicentre study.

OBJECTIVE: Direct current cardioversion (DCCV) in pregnancy is rarely required and typically only documented in single case reports or case series. A recent UK confidential enquiry reported on several maternal deaths where appropriate DCCV appeared to have been withheld. DESIGN: Retrospective cohort study. SETTING: Seventeen UK and Ireland specialist maternity centres. SAMPLE: Twenty-seven pregnant women requiring DCCV in pregnancy. MAIN OUTCOME MEASURES: Maternal and fetal outcomes following DCCV. RESULTS: Twenty-seven women had a total of 29 DCCVs in pregnancy. Of these, 19 (70%) initial presentations were to Emergency Departments and eight (30%) to maternity settings. There were no maternal deaths. Seventeen of the women (63%) had a prior history of heart disease. Median gestation at DCCV was 28 weeks, median gestation at delivery was 35 weeks, with a live birth in all cases. The abnormal heart rhythms documented at the first cardioversion were atrial fibrillation in 12/27 (44%) cases, atrial flutter in 8/27 (30%), supraventricular tachycardia in 5/27 (19%) and atrial tachycardia in 2/27 (7%). Fetal monitoring was undertaken following DCCV on 14/29 (48%) occasions (10 of 19 (53%) at ≥26 weeks) and on 2/29 (7%) occasions, urgent delivery was required post DCCV. CONCLUSIONS: Direct current cardioversion in pregnancy is rarely required but should be undertaken when clinically indicated according to standard algorithms to optimise maternal wellbeing. Once the woman is stable post DCCV, gestation-relevant fetal monitoring should be undertaken. Maternity units should develop multidisciplinary processes to ensure pregnant women receive the same standard of care as their non-pregnant counterparts.

Cardiac resynchronization therapy in adults with structural congenital heart disease and chronic heart failure.

AIMS: Evidence for CRT in adults with congenital heart disease (ACHD) and chronic heart failure is limited, with recommendations for its use extrapolated from the population with structurally normal hearts. This retrospective observational study investigates the efficacy of CRT in this heterogenous group, discussing factors predicting response to CRT. METHODS: Twenty-seven patients with structural ACHD who underwent CRT insertion or upgrade at a tertiary center in the United Kingdom were retrospectively studied. The primary outcome measure was clinical response to CRT, defined as improvement of NYHA class and/or improvement in systemic ventricular ejection fraction by one category. Secondary outcomes included change in QRS duration and adverse events. RESULTS: Thirty-seven percent of patients had a systemic right ventricle (sRV). RBBB was the commonest baseline QRS morphology (40.7%) despite this being an unfavorable characteristic for CRT. Overall, positive response to CRT was demonstrated in 18 patients (66.7%). NYHA class improved in 55.5% following CRT (p = .001) and 40.7% showed improvement in systemic ventricular ejection fraction (p = .118). There were no baseline characteristics that predicted response to CRT, and electrocardiographic measures such as QRS shortening post-CRT was not associated with positive response. Good response rates (60.0%) were demonstrated in those with sRV. CONCLUSION: CRT is efficacious in structural ACHD including in those who do not meet conventional criteria. Extrapolation of recommendations from adults with structurally normal hearts may be inappropriate. Future research should focus on improving patient selection for CRT, for example using techniques to better quantify mechanical dysynchrony and intra-procedural electrical activation mapping in these complex patients.

Greyzone myocardial fibrosis and ventricular arrhythmias in patients with a left ventricular ejection fraction >35.

AIMS: To determine whether myocardial fibrosis and greyzone fibrosis (GZF) on cardiovascular magnetic resonance (CMR) is associated with ventricular arrhythmias in patients with coronary artery disease (CAD) and a left ventricular ejection fraction (LVEF) >35%. METHODS AND RESULTS: In this retrospective study of CAD patients, GZF mass using the 3SD method (GZF3SD) and total fibrosis mass using the 2SD method (TF2SD) on CMR were assessed in relation to the primary, combined endpoint of sudden cardiac death, ventricular tachycardia, ventricular fibrillation, or resuscitated cardiac arrest. Among 701 patients [age: 65.8 ± 12.3 years (mean ± SD)], 28 (3.99%) patients met the primary endpoint over 5.91 years (median; interquartile range 4.42-7.64). In competing risks analysis, a GZF3SD mass ≥5.0 g was strongly associated with the primary endpoint [subdistribution hazard ratio (sHR): 17.4 (95% confidence interval, CI 6.64-45.5); area under receiver operator characteristic curve (AUC): 0.85, P < 0.001]. A weaker association was observed for TF2SD mass ≥23 g [sHR 10.4 (95% CI 4.22-25.8); AUC: 0.80, P < 0.001]. The range of sHRs for GZF3SD mass (1-527) was wider than for TF2SD mass (1-37.6). CONCLUSIONS: In CAD patients with an LVEF >35%, GZF3SD mass was strongly associated with the arrhythmic endpoint. These findings hold promise for its use in identifying patients with CAD and an LVEF >35% at risk of arrhythmic events.

Pre-conceptual counselling in cardiology patients: still work to do and still missed opportunities. A comparison between 2015 and 2019 in women with cardiac disease attending combined obstetric cardiology clinics. Should the European Guidelines change anything?

Women with underlying cardiac conditions have an increased risk of adverse pregnancy outcomes. Counselling reproductive age women with heart disease is important to assist them in deciding whether to pursue pregnancy, to ensure their best cardiovascular status prior to pregnancy, and that they understand the risks of pregnancy for them and baby. This also provides an opportunity to explore management strategies to reduce risks. For this growing cohort of women, there is a great need for pre-conceptual counselling.This retrospective comparative audit assessed new referrals and pre-conceptual counselling of women attending a joint obstetric-cardiology clinic at a tertiary maternity centre in a 12-month period of 2015-2016 compared with 2018-2019. This reflected the timing of the introduction of a multidisciplinary meeting prior to clinics and assessed the impact on referrals with the introduction of the European Society of Cardiology guidelines.Data were reviewed from 56 and 67 patients in respective audit periods. Patient's risk was stratified using modified World Health Organization classification.Less than 50% of women with pre-existing cardiac conditions had received pre-conceptual counselling, although half of them had risks clearly documented. The majority of patients had a recent electrocardiograph and echocardiogram performed prior to counselling, and there was a modest improvement in the number of appropriate functional tests performed between time points. One-third of patients in both cohorts were taking cardiac medications during pregnancy.There was a significant increase in the number of pregnant women with cardiac disease and in complexity according to modified World Health Organization risk classification. While there have been improvements, it is clear that further work to improve availability and documentation of pre-pregnancy counselling is needed.

The impact of the COVID-19 pandemic on application of European Society of Cardiology (ESC) guidelines for exercise in adults with CHD: a data-based questionnaire.

INTRODUCTION: Regular physical activity is safe and effective therapy for adults with CHD and is recommended by European Society of Cardiology guidelines. The COVID-19 pandemic poses enormous challenges to healthcare teams and patients when ensuring guideline compliance. We explored the implications of COVID-19 on physical activity levels in adult CHD patients. MATERIALS AND METHODS: A data-based questionnaire was distributed to adult CHD patients at a regional tertiary centre from October to November 2020. RESULTS: Prior to the COVID-19 pandemic, 96 (79.3%) of 125 respondents reported participating in regular physical activity, with 66 (52.8%) meeting target levels (moderate physical activity for at least 150 minutes per week). Commonest motivations for physical activity were general fitness (53.6%), weight loss (36.0%), and mental health benefits (30.4%). During the pandemic, the proportion that met target levels significantly decreased from 52.8% to 40.8% (p = 0.03). The commonest reason was fear of COVID-19 (28.0%), followed by loss of motivation (23.2%) and gym/fitness centre closure (15.2%). DISCUSSION: The COVID-19 pandemic has negatively impacted exercise levels of adult CHD patients. Most do not meet recommended physical activity levels, mainly attributable to fear of COVID-19. Even before the pandemic, only half of respondents met physical activity guidelines. Availability of online classes can positively impact exercise levels so could enhance guideline compliance. This insight into health perceptions and behaviours of adult CHD patients may help develop quality improvement initiatives to improve physical activity levels in this population.

COVID-19 and adult congenital heart disease services: impact on support and advice from nurse specialists.

Although the focus on service provision in response to the COVID-19 pandemic has mainly been on acute and particularly intensive care, it is important to consider other services that are still needed. This is especially the case for vulnerable patients with long-term conditions, such as those under the care of an adult congenital heart disease (ACHD) service. The authors conducted a survey of ACHD nurse specialists in centres across the UK to acertain what they were planning in terms of the redeployment of nurse specialists. The results showed a range of plans with an average of half of nurses per centre being moved, but with 65% of the workforce planned to be redeployed to deal with the pandemic. The telephone advice service at the authors' own level 1 centre showed a significant increase in patients seeking advice when compared with the previous year, with large peaks following major Government announcements. Access to specialist advice for patients with complex conditions is of vital interest, for those in a wide range of specialities.

Prognosis of incidental left bundle branch block.

AIMS: Incidental left bundle branch block (iLBBB) is a frequent cause for cardiology referrals. In such instances, there is uncertainty as to its prognosis. We sought to determine the utility of cardiovascular magnetic resonance (CMR) in the risk stratification of patients with iLBBB. METHODS AND RESULTS: Clinical events were collected in patients with iLBBB who had CMR. Controls had no cardiac symptoms or cardiac disease, a normal CMR scan and electrocardiogram. Amongst patients with iLBBB [n = 193, aged 62.7 ± 12.6 years (mean ± SD)], 110/193 (56.9%) had an abnormal phenotype (iLBBBCMR+) and 83/110 (43.0%) had a normal phenotype (iLBBBCMR-). Over 3.75 years (median; inter-quartile range: 2.7-5.5), iLBBBCMR+ had a higher total mortality [adjusted hazard ratio (aHR) 6.49, 95% confidence interval (CI) 1.91-22.0] and total mortality or major adverse cardiac events (MACEs; aHR 9.15, 95% CI 2.56-32.6) than controls (n = 107). In contrast, iLBBBCMR- had a similar risk of total mortality compared with controls, but total mortality or MACEs was higher (aHR 4.24, 95% CI 1.17-15.4; P = 0.028). Amongst iLBBB patients, both myocardial fibrosis (aHR 5.15, 95% CI 1.53-17.4) and left ventricular ejection fraction (LVEF) ≤ 50% (aHR 3.88, 95% CI 1.67-9.06) predicted total mortality. Myocardial fibrosis plus LVEF ≤50% was associated with the highest risk of total mortality (aHR: 9.87, 95% CI 2.99-32.6) and total mortality or MACEs (aHR 3.98, 95% CI 1.73-9.11). CONCLUSIONS: Outcomes in iLBBBCMR+ were poor whereas survival in iLBBBCMR- was comparable with controls. Myocardial fibrosis and LVEF <50% had an additive effect on the risk of clinical outcomes. A CMR scan is pivotal in risk-stratifying patients with iLBBB.

Variation in cardiovascular magnetic resonance myocardial contouring: Insights from an international survey.

LEVEL OF EVIDENCE: 5 Technical Efficacy Stage: 2 J. Magn. Reson. Imaging 2019;50:1336-1338.

Eligibility for subcutaneous implantable cardioverter defibrillators in the adult congenital heart disease population.

BACKGROUND: Patients with adult congenital heart disease (ACHD) have an increased risk of arrhythmic and sudden cardiac death. The subcutaneous implantable cardioverter defibrillator (S-ICD) provides a potentially safer alternative to transvenous ICDs in ACHD. Suitability for S-ICD depends on the surface electrocardiogram (ECG), which is often abnormal in ACHD patients. This study investigates the proportion of ACHD patients who meet the screening criteria for S-ICD implantation. METHODS: A standard screening ECG was performed in 102 patients with complex ACHD (Tetralogy of Fallot, Fontan circulation, and transposition of the great arteries). This process was repeated postexercise for patients who also had an exercise test. RESULTS: Three quarters (75.4%) of ACHD patients meet screening criteria for an S-ICD with at least one suitable vector. The most common number of acceptable vectors in the eligible group was two (35% of total population). In only 12% of total population, all three vectors were suitable while only one vector was suitable in 28% of total population. The primary vector (equivalent of ECG lead III) was the most common suitable vector, found in 62% of participants who had appropriate sensing vectors. Twenty-five (24.5%) patients failed to meet the S-ICD screening criteria. Of these, 14 had repaired tetralogy of Fallot. A total of 92% of patients with a Fontan circulation met ECG screening criteria. Of those who had the protocol repeated following their cardiopulmonary exercise test (n = 14), only one additional patient failed to meet the eligibility criteria. CONCLUSIONS: A quarter of ACHD patients do not meet the eligibility criteria for the S-ICD. However, more than 90% of patients with a Fontan circulation are suitable for an S-ICD.

Long-term outcomes of cardiac resynchronization therapy in adult congenital heart disease.

BACKGROUND AND AIMS: Randomized, controlled trials of cardiac resynchronization therapy (CRT) excluded patients with adult congenital heart disease (ACHD). We sought to explore long-term clinical outcomes. METHODS AND RESULTS: In this single-center, observational study, events were collected from hospital records on patients with structural ACHD (sACHD) and adults with ischemic (ICM) or nonischemic (NICM) cardiomyopathy undergoing CRT. Patients with sACHD (n = 23, age: 41.6 ± 13.5 years [mean ± standard deviation]) and adults with ICM (n = 533) or NICM (n = 458) were followed-up for 4.1 years (median; interquartile range: 2.2-6.1). Total mortality was 5/23 (21.7%; 4.4 per 100 person-years) in sACHD, 221/533 (41.5%; 11.8 per 100 person-years) in ICM, and 154/458 (33.6%; 9.7 per 100 person-years) in NICM. In univariate analyses, total mortality in sACHD was lower than in ICM (hazard ratio [HR]: 0.38; 95% confidence interval [CI] 0.15-0.91), but similar to NICM (HR: 0.48, 95% CI 0.20-1.16). Cardiac mortality in sACHD was similar to ICM (HR: 0.78, 95% CI 0.32-1.92) and NICM (HR: 1.12, 95% CI 0.45-2.78). Heart failure (HF) hospitalization rates were similar to ICM (HR: 0.44, 95% CI 0.11-1.77) and NICM (HR: 0.75, 95% CI 0.18-3.08). In multivariate analyses, no differences emerged in total mortality, cardiac mortality, or HF hospitalization between sACHD and NICM or ICM, after adjustment for age, sex, New York Heart Association class, diabetes, atrial rhythm, QRS duration, QRS morphology, systemic ventricular ejection fraction, and medical therapy. CONCLUSION: Total mortality, cardiac mortality, and HF hospitalization after CRT in patients with sACHD was similar to adults with ICM or NICM.

Estimated blood loss in pregnant women with cardiac disease compared with low risk women: a restrospective cohort study.

BACKGROUND: Women with cardiac disease are thought to be at increased risk of post-partum haemorrhage. We sought to assess the estimated blood loss (EBL) in our cohort of women with and without cardiac disease (CD) in a quaternary hospital in the UK. Our population consisted of both congenital and acquired CD; and low risk women who delivered in our unit between 01/01/2012-30/09/2016. METHODS: Data were collected using computerised hospital records. CD was classified according to the modified WHO classification (mWHO). The primary outcome measure was estimated blood loss (mL). RESULTS: A total of 5413 women with a singleton fetus in the cephalic presentation delivered during the study period (159 women with CD and 5254 controls). In the CD group, active management of the third stage of labour was consistent with that used in low risk women in 98% (152/155) of cases. Multivariable analyses demonstrated no significant difference in EBL between women with CD vs controls. The adjusted average blood losses were 247.2 ml, 241.8 ml and 295.9 ml in the control group, mWHO 1-2 and 3-4, respectively (p = 0.165). CONCLUSIONS: Women with CD have comparable EBL to low risk women when management of the active third stage of labour is the same.

Long-term follow-up of implantable cardioverter-defibrillators in adult congenital heart disease patients: indications and outcomes.

AIMS: Ventricular arrhythmias are a major cause of mortality in adult congenital heart disease (ACHD) patients. The European Society of Cardiology guidelines state that implantable cardioverter-defibrillators (ICD) should be considered in patients with congenital heart disease following spontaneous sustained ventricular tachycardia (VT) or cardiac arrest and in patients at presumed high risk. This study sought to analyse the circumstances in which ACHD patients received ICD and to assess outcomes of ICD implantation, including therapies delivered and the rate of complications. METHODS AND RESULTS: A retrospective review was performed of all adult patients with congenital heart disease undergoing ICD implant between 2000 and 2014, in a large quaternary referral centre with over 4000 adults with congenital heart disease under active follow-up. Demographics: 42 patients with congenital heart disease had ICD implants: 55% male; age range 21-71 years and mean age 45 years. Mean age at implantation of ICD was 41 years. Mean follow-up was 5 years. Diagnosis: 50% of patients had repaired tetralogy of Fallot (TOF). Twelve per cent of patients had repaired transposition of the great arteries. Reason for ICD: 15 patients (35.7%) received ICD after sustained VT. Eleven patients (26.2%) received ICD after cardiac arrest. Sixteen (38%) had ICD implanted as primary prophylaxis. Outcome: Since implantation, six patients received an appropriate full-output shock for VT from ICD. Nineteen (45%) patients suffered significant complications (inappropriate shocks 11, inappropriate anti-tachycardia pacing resulting in VF 1, infection requiring extraction 3, lead abnormalities 3, and pneumothorax 1). Equal proportions of primary and secondary prevention patients received appropriate shocks. CONCLUSIONS: Most patients had ICD for secondary prevention (62%), and the majority had repaired TOF. There was a 2.9% annual appropriate shock rate. However, there was a high incidence of complications with more than a third suffering a major complication (9% per annum). The risks and benefits of ICD implantation are patient and disease specific, and must be clearly discussed prior to implantation. Further research is warranted into the use of primary prevention ICD in ACHD and in alternatives to ICD such as ablation in specific patient groups.

Maternal mental health: a key area for future research among women with congenital heart disease.

In this viewpoint, we respond to the recently published national priorities for research in congenital heart disease (CHD) among adults, established through the James Lind Alliance Priority Setting Partnership, with specific attention to priority 3 (mental health) and priority 5 (maternal health). Our recent policy impact project explored how maternal mental health is currently addressed in adult congenital heart disease (ACHD) services in the National Health Service, identified gaps and discussed possible ways forward. Our multidisciplinary discussion groups, which included women with lived experience of CHD and pregnancy, cardiology and obstetrics clinicians and medical anthropologists, found that while pregnancy and the postnatal period increase the mental health challenges faced by women with CHD, current services are not yet equipped to address them. Based on this work, we welcome the prioritisation of both mental health and maternal health in ACHD, and suggest that future research should focus on the overlaps between these two priority areas.

Pregnancy outcomes in women following the Ross procedure.

INTRODUCTION: The Ross procedure, where a pulmonary autograft (neoaorta) replaces the aortic valve, has excellent long-term outcomes in patients with congenital aortic valve disease. However, there are reports of neoaortic dilatation and dissection. An increasing number of women are wishing to become pregnant following the Ross procedure, but little is known about the occurrence and risks of neoaortic dilatation and complications in pregnancy. We investigated neoaorta function and outcomes in pregnancy following the Ross procedure. METHODS: This retrospective study investigated women post-Ross procedure at a tertiary ACHD unit between 1997 and 2021. Imaging evaluated neoaortic root dimensions and regurgitation pre-, and post- pregnancy, compared with matched non-pregnant controls. Primary endpoints were change in neoaortic dimensions, degree of regurgitation and adverse maternal outcomes. RESULTS: Nineteen pregnancies in 12 women were included. The mean change in neoaortic root diameter post-pregnancy was 1.8 mm (SD 3.4) (p = 0.017). There was no significant change in neoaortic dimensions in matched controls during follow-up. There were no cases of dissection, arrhythmia, acute coronary syndrome, or maternal mortality. Three deliveries were pre-term, including one emergency Caesarean section due to maternal cardiac decompensation, requiring aortic root replacement post-partum but there were no neonatal deaths. CONCLUSIONS: Pregnancy following the Ross procedure is associated with neoaortic dilatation, and pregnancy is generally well tolerated. Although adverse maternal outcomes are uncommon, there are still rare cases of cardiac complications in and around the time of pregnancy. These findings emphasise the need for accessible pre-pregnancy counselling, risk stratification and careful surveillance through pregnancy by specialist cardio-obstetric multi-disciplinary teams.

Echocardiographically guided catheter closure of arterial ducts in small preterm infants on the neonatal intensive care unit.

OBJECTIVE: To describe closure of haemodynamically significant arterial ducts in preterm infants using an echocardiographically guided cardiac catheter technique in selected infants in the neonatal nursery and in preference to cardiac surgery. BACKGROUND: Persistently patent arterial ducts are common in preterm infants and are associated with significant morbidity and mortality. Cardiac catheter techniques continue to improve and occlusion of arterial ducts in preterm infants is becoming technically feasible. Closure of arterial ducts by cardiac catheter techniques would enable selected infants to avoid surgery and a lateral thoracotomy, as well as potentially obviating the need for transfer of sick preterm infants between units for duct closure. METHODS AND RESULTS: This brief report describes placement of coils or Amplatzer duct devices to occlude arterial ducts in small premature infants exclusively under echocardiographic guidance in the Neonatal Intensive Care Unit. CONCLUSIONS: Closing arterial ducts in the neonatal nursery by an echocardiographically guided cardiac catheter technique with minimal morbidity is becoming achievable and is a significant advance in neonatal care.