Assessment and Prediction of Cardiovascular Contributions to Severe Maternal Morbidity.

Severe maternal morbidity (SMM) refers to any unexpected outcome directly related to pregnancy and childbirth that results in both short-term delivery complications and long-term consequences to a women's health. This affects about 60,000 women annually in the United States. Cardiovascular contributions to SMM including cardiac arrest, arrhythmia, and acute myocardial infarction are on the rise, probably driven by changing demographics of the pregnant population including more women of extreme maternal age and an increased prevalence of cardiometabolic and structural heart disease. The utilization of SMM prediction tools and risk scores specific to cardiovascular disease in pregnancy has helped with risk stratification. Furthermore, health system data monitoring and reporting to identify and assess etiologies of cardiovascular complications has led to improvement in outcomes and greater standardization of care for mothers with cardiovascular disease. Improving cardiovascular disease-related SMM relies on a multipronged approach comprised of patient-level identification of risk factors, individualized review of SMM cases, and validation of risk stratification tools and system-wide improvements in quality of care. In this article, we review the epidemiology and cardiac causes of SMM, we provide a framework of risk prediction clinical tools, and we highlight need for organization of care to improve outcomes.

Transthoracic Echocardiographic Assessment of the Heart in Pregnancy-a position statement on behalf of the British Society of Echocardiography and the United Kingdom Maternal Cardiology Society.

Pregnancy is a dynamic process associated with profound hormonally mediated haemodynamic changes which result in structural and functional adaptations in the cardiovascular system. An understanding of the myocardial adaptations is important for echocardiographers and clinicians undertaking or interpreting echocardiograms on pregnant and post-partum women. This guideline, on behalf of the British Society of Echocardiography and United Kingdom Maternal Cardiology Society, reviews the expected echocardiographic findings in normal pregnancy and in different cardiac disease states, as well as echocardiographic signs of decompensation. It aims to lay out a structure for echocardiographic scanning and surveillance during and after pregnancy as well as suggesting practical advice on scanning pregnant women.

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.

Pregnancy Considerations in Tetralogy of Fallot.

The majority of women with repaired tetralogy of Fallot are able to tolerate pregnancy with a low risk of cardiovascular events. However, proactive contraceptive advice, prepregnancy counselling, and care by a pregnancy heart team with expertise in congenital heart disease are key to ensuring a good outcome for mother and baby. Maternal and fetal risks are increased in the presence of severe valvular stenosis, poorly tolerated arrhythmia, significant ventricular dysfunction, and cyanosis. It is unusual to see cyanotic adults with tetralogy of Fallot, whether unoperated or shunt palliated; pregnancy risks are greatly reduced by completing their repair before pregnancy is undertaken. The multidisciplinary pregnancy heart team should make a risk-stratified pregnancy care plan using a combination of published scoring systems and an individualized assessment of the patient's comorbidities. Low-risk patients may have the majority of their care and give birth in local units, whereas those at high risk should be managed and give birth in a tertiary centre with high-level expertise and intensive care facilities. Age-appropriate conversations about future childbearing and safe and reliable contraception should be part of routine follow-up from teenage years, so that women with tetralogy of Fallot can control their own fertility and make informed decisions about having children.

La majorité des femmes présentant une tétralogie de Fallot réparée tolèrent une grossesse et sont exposées à un faible risque d'événements cardiovasculaires. Cependant, des conseils sur la contraception, des consultations avant la grossesse et des soins prodigués par une équipe spécialisée en cardiopathies congénitales permettront de garantir de bons résultats pour la mère et l'enfant. Les risques pour la mère et l'enfant sont accrus en cas de sténose valvulaire sévère, d'arythmie mal tolérée, de dysfonction ventriculaire importante ou de cyanose. Les cas de cyanose chez les adultes atteintes de la tétralogie de Fallot sont toutefois rares, qu'elles aient subi ou non une anastomose palliative, et les risques associés à la grossesse sont considérablement réduits si la réparation est effectuée avant la grossesse. Pendant la grossesse, l'équipe multidisciplinaire spécialisée en cardiologie et obstétrique doit élaborer un plan de soins stratifié en fonction des risques, à l'aide de systèmes d'évaluation reconnus et une évaluation personnalisée des maladies concomitantes de la patiente. Les patientes à faible risque peuvent normalement recevoir leurs soins et accoucher dans des établissements locaux, tandis que celles à risque élevé doivent être prises en charge et accoucher dans un centre de soins tertiaire doté d'un haut niveau d'expertise et d'installations de soins intensifs. Dès l'adolescence, lorsque des patientes atteintes de la tétralogie de Fallot se présentent pour une consultation de routine, il est important de discuter de la procréation et de l'utilisation de méthodes de contraception sûres et fiables en utilisant un langage adapté à leur âge afin qu'elles sachent ce qu'il faut faire pour éviter une grossesse et puissent prendre une décision éclairée quant à l'éventualité d'avoir un enfant.

Coagulation and Anticoagulation in Fontan Patients.

Patients with a Fontan circulation for single-ventricle physiology are at increased risk of developing thromboembolic events. Thromboembolic events can lead to failure of the Fontan circulation, chronic sequelae in case of stroke, and early mortality. Controversies exist regarding the substrates, risk factors, and optimal detection methods for thromboembolic events. Despite the major clinical implications, there is currently no consensus regarding the optimal antithrombotic therapy to prevent or treat thromboembolic events after the Fontan procedure. In this review we aimed to untangle the available literature regarding antithrombotic prophylaxis and treatment for pediatric and adult Fontan patients. A decision-tree algorithm for thromboprophylaxis in Fontan patients is proposed. Additionally, the current state of knowledge is reviewed with respect to the epidemiology, pathophysiology, and detection of thromboembolic events in Fontan patients, and important evidence gaps are highlighted.

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.

Outcome and right ventricle remodelling after valve replacement for pulmonic stenosis.

BACKGROUND: Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR). METHODS: We performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed. RESULTS: After a median follow-up of 38.6 (30.9-49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m2 for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m2 for RV end-systolic volume indexed(sensitivity 80%, specificity 56%). CONCLUSIONS: Previous RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.

Pregnancy Outcomes in Women With Rheumatic Mitral Valve Disease: Results From the Registry of Pregnancy and Cardiac Disease.

BACKGROUND: Cardiac disease is 1 of the major causes of maternal mortality. We studied pregnancy outcomes in women with rheumatic mitral valve disease. METHODS: The Registry of Pregnancy and Cardiac Disease is an international prospective registry, and consecutive pregnant women with cardiac disease were included. Pregnancy outcomes in all women with rheumatic mitral valve disease and no prepregnancy valve replacement is described in the present study (n=390). A maternal cardiac event was defined as cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, and hospitalization for other cardiac reasons or cardiac intervention. Associations between patient characteristics and cardiac outcomes were checked in a 3-level model (patient-center-country). RESULTS: Most patients came from emerging countries (75%). Mitral stenosis (MS) with or without mitral regurgitation (MR) was present in 273 women, isolated MR in 117. The degree of MS was mild in 20.9%, moderate in 39.2%, severe in 19.8%, and severity not classified in the remainder. Maternal death during pregnancy occurred in 1 patient with severe MS. Hospital admission occurred in 23.1% of the women with MS, and the main reason was heart failure (mild MS 15.8%, moderate 23.4%, severe 48.1%; P<0.001). Heart failure occurred in 23.1% of patients with moderate or severe MR. An intervention during pregnancy was performed in 16 patients, 14 had percutaneous balloon mitral commissurotomy, and 2 had surgical valve replacement (1 for MS, 1 for MR). In multivariable modeling, prepregnancy New York Heart Association class >1 was an independent predictor of maternal cardiac events. Follow-up at 6 months postpartum was available for 53%, and 3 more patients died (1 with severe MS, 1 with moderate MS, 1 with moderate to severe MR). CONCLUSIONS: Although mortality was only 1.9% during pregnancy, ≈50% of the patients with severe rheumatic MS and 23% of those with significant MR developed heart failure during pregnancy. Prepregnancy counseling and considering mitral valve interventions in selected patients are important to prevent these complications.

Implementation of guidelines for multidisciplinary team management of pregnancy in women with pre-existing diabetes or cardiac conditions: results from a UK national survey.

BACKGROUND: Despite numerous publications stating the importance of multidisciplinary care for women with pre-existing medical conditions, there is a lack of evidence regarding structure or processes of multidisciplinary working, nor impact on maternal or infant outcomes. This study aimed to evaluate the implementation of guidelines for multidisciplinary team (MDT) management in pregnant women with pre-existing diabetes or cardiac conditions. These conditions were selected as exemplars of increasingly common medical conditions in pregnancy for which MDT management is recommended to prevent or reduce adverse maternal and fetal outcomes. METHODS: National on-line survey sent to clinicians responsible for management or referral of women with pre-existing diabetes or cardiac conditions in UK National Health Service (NHS) maternity units. The survey comprised questions regarding the organisation of MDT management for women with pre-existing diabetes or cardiac conditions. Content was informed by national guidance. RESULTS: One hundred seventy-nine responses were received, covering all health regions in England (162 responses) and 17 responses from Scotland, Wales and Northern Ireland. 132 (74%) related to women with diabetes and 123 (69%) to women with cardiac conditions. MDT referral was reportedly standard practice in most hospitals, particularly for women with pre-existing diabetes (88% of responses vs. 63% for cardiac) but there was wide variation in relation to MDT membership, timing of referral and working practices. These inconsistencies were evident within and between maternity units across the UK. Reported membership was medically dominated and often in the absence of midwifery/nursing and other allied health professionals. Less than half of MDTs for women with diabetes met the recommendations for membership in national guidance, and although two thirds of MDTs for women with cardiac disease met the core recommendations for membership, most did not report having the extended members: midwives, neonatologists or intensivists. CONCLUSIONS: The wide diversity of organisational management for women with pre-existing diabetes or cardiac conditions is of concern and merits more detailed inquiry. Evidence is also required to support and better define the recommendations for MDT care.

Global cardiac risk assessment in the Registry Of Pregnancy And Cardiac disease: results of a registry from the European Society of Cardiology.

AIMS: To validate the modified World Health Organization (mWHO) risk classification in advanced and emerging countries, and to identify additional risk factors for cardiac events during pregnancy. METHODS AND RESULTS: The ongoing prospective worldwide Registry Of Pregnancy And Cardiac disease (ROPAC) included 2742 pregnant women (mean age ± standard deviation, 29.2 ± 5.5 years) with established cardiac disease: 1827 from advanced countries and 915 from emerging countries. In patients from advanced countries, congenital heart disease was the most prevalent diagnosis (70%) while in emerging countries valvular heart disease was more common (55%). A cardiac event occurred in 566 patients (20.6%) during pregnancy: 234 (12.8%) in advanced countries and 332 (36.3%) in emerging countries. The mWHO classification had a moderate performance to discriminate between women with and without cardiac events (c-statistic 0.711 and 95% confidence interval (CI) 0.686-0.735). However, its performance in advanced countries (0.726) was better than in emerging countries (0.633). The best performance was found in patients with acquired heart disease from developed countries (0.712). Pre-pregnancy signs of heart failure and, in advanced countries, atrial fibrillation and no previous cardiac intervention added prognostic value to the mWHO classification, with a c-statistic of 0.751 (95% CI 0.715-0.786) in advanced countries and of 0.724 (95% CI 0.691-0.758) in emerging countries. CONCLUSION: The mWHO risk classification is a useful tool for predicting cardiac events during pregnancy in women with established cardiac disease in advanced countries, but seems less effective in emerging countries. Data on pre-pregnancy cardiac condition including signs of heart failure and atrial fibrillation, may help to improve preconception counselling in advanced and emerging countries.

Long-term outcome following pregnancy in women with a systemic right ventricle: is the deterioration due to pregnancy or a consequence of time?

INTRODUCTION: The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous studies have reported deterioration in RV function following pregnancy. However, conditions with a systemic RV are also associated with progressive RV dysfunction over time. To date, no study has examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients. METHODS: Women who had undergone pregnancy under the care of the Adult Congenital Heart Disease Unit at the Queen Elizabeth Hospital were retrospectively identified and matched to separate male and nulliparous female controls. Functional status (New York Health Association [NYHA]), RV function, and systemic atrioventricular valve regurgitation were recorded for each group at baseline, postpregnancy (or at 1 year for control groups) and at latest follow-up. RESULTS: Eighteen women had 31 pregnancies (range 1-4) resulting in 32 live births. There were no maternal but one neonatal death. At baseline, there was no significant difference in NYHA class or RV function between pregnancy and control groups. In postpregnancy, there was a significant deterioration in the pregnant group alone for both NYHA class (P = 0.004) and RV function (P = 0.02). At latest follow-up, there was a significant deterioration in RV function in all three groups. There was still a reduction from baseline in NYHA of women who had undergone pregnancy (P = 0.014), which again was not seen in the controls groups. CONCLUSION: This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. This study will allow this cohort of women to be more accurately counseled as to the potential long-term risks of pregnancy.

End-of-life care in adults with congenital heart disease: now is the time to act.

PURPOSE OF REVIEW: There are increasing numbers of adults with congenital heart disease (CHD) and these patients remain at long-term risk of complications and premature death. This review focuses on the changing picture of adult CHD with more complex patients surviving, the challenges of balancing life-prolonging intervention, the barriers to discussing the end-of-life (EOL) issues and draws on the experience of other specialities in managing young patients. RECENT FINDINGS: The prevalence of adults with the most severe forms of CHD has increased, especially those with a Fontan circulation. The eventual decline is inevitable with limited treatment options. There should be a parallel palliative care approach in patients who are being considered for high-risk, life-prolonging interventions. Oncologists caring for the young patients with cancer and cystic fibrosis specialists have demonstrated the unique needs of young patients with chronic diseases that may be applicable to adult CHD patients and help with their EOL planning. SUMMARY: These patients require an early and proactive approach to EOL discussions, and the unique needs of young patients should be recognized. Further research is needed to develop local and national guidelines for the palliative care approach in these patients.

The safety and effects of bosentan in patients with a Fontan circulation.

OBJECTIVE: Adult patients with a Fontan circulation tend to have diminished exercise capacity. The principal objective of this study was to investigate the safety of the endothelin receptor antagonist bosentan in Fontan patients, and, secondarily, to assess effects on cardiovascular performance, New York Heart Association functional classification (NYHA FC), and ventricular function. DESIGN: A 6-month prospective, single-center, pilot, safety study of bosentan in Fontan patients. Setting. Adult Congenital Heart Disease referral center. PATIENTS: All patients ≥18 years old with a Fontan circulation and in NYHA FC ≥II were invited to enroll. Interventions. Patients started on 62.5 mg bid of bosentan, uptitrating to 125 mg bid after 2 weeks. OUTCOME MEASURES: Safety was assessed by the incidence of anticipated and unanticipated adverse events during the 6-month study period; specifically those relating to hepatic, renal, or hematological dysfunction as measured by monthly blood tests. Other outcome measures included cardiopulmonary exercise test, 6-minute walk distance test, Borg dyspnea index, NYHA FC, and ventricular function parameters using transthoracic echocardiography. RESULTS: Of the eight patients enrolled, six completed the study. Two patients withdrew from the study (one for non-trial related reasons, one due to adverse events). No clinically significant adverse events relating to bosentan therapy occurred during this study and, in particular, no significant abnormalities in hepatic function tests were observed. Three patients reported transient adverse events. Improvements in NYHA FC and systolic ventricular function were observed after 6 months of bosentan treatment. CONCLUSIONS: The small number of patients with a Fontan circulation in our study was able to tolerate bosentan for 6 months. The safety and tolerability of bosentan in a larger patient population remains unknown. The results presented here justify further investigation in larger studies.

An unusual treatment for protein losing enteropathy.

We present the case of a 22-year-old man with a Fontan circulation who suffered from intractable protein losing enteropathy for an 8-year period necessitating several hospital admissions for recurrent pleural effusions and edema. Despite trying several recognized medical therapies to alleviate his protein losing enteropathy, his condition did not improve until the introduction of loperamide for troublesome diarrhea. Following this, his symptoms and serum albumin improved dramatically and he has not required any further hospital admissions at 22 months follow up.

Treatment of dynamic subvalvar muscular obstruction in the native right ventricular outflow tract by percutaneous stenting in adults.

Percutaneous techniques are being increasingly used in adult congenital heart disease but there is limited experience in the treatment of native nonvalvar right ventricular outflow tract obstruction. We describe two cases of percutaneous stenting of the subpulmonary region where surgery was not an option.

Acquired thoracic aortic interruption: percutaneous repair using graft stents.

Two adult patients with isolated, aortic interruption were successfully treated by percutaneous insertion of graft stents. Prior to the intervention, both patients were hypertensive and on medication. In both cases, an ascending aortogram demonstrated a blind ending of the thoracic aorta distal to the left subclavian artery with a large gradient across the interruption and with multiple collaterals. A graft stent was successfully deployed across the interrupted segment in both cases. We believe that this is one of the first reported cases of percutaneous stenting of aortic interruption and represents a promising new therapeutic option for these adult patients.

Infected thrombus compression of the right ventricular outflow tract in transposition of the great arteries.

We describe the first reported case of infected thrombus compression of the right ventricular outflow tract in a Rastelli patient as a complication of pregnancy. This case emphasizes the importance of cardiac magnetic resonance imaging and echocardiography in the assessment of patients with adult congenital heart disease prior to and during pregnancy.