Mechanical circulatory support in patients with congenital heart disease: a European Registry for Patients with Mechanical Circulatory Support (EUROMACS) study.

OBJECTIVES: This study aims to explore characteristics and clinical outcomes of patients with congenital heart disease (CHD) in the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). METHODS: This is a retrospective study of EUROMACS participants receiving MCS as bridge-to-transplant, possible bridge-to-transplant, or rescue therapy/bridge-to-recovery from 2011 to 2023 (n = 5340). Adult and paediatric cohorts were analysed separately. The primary outcome was mortality on MCS; secondary outcomes included recovery, transplant and complications including bleeding, cerebrovascular events, and sepsis. RESULTS: Among adult patients, mortality at 1-year was 33.3% among the CHD cohort vs 22.1% in the non-CHD cohort. Adult CHD patients had higher hazards of mortality within the first year after MCS implantation [hazard ratios 1.98, 95% confidence interval (CI) 1.35-2.91, P < 0.001] and bleeding events (subdistribution hazard ratios 2.10, 95% CI 1.40-3.16, P < 0.001) compared with non-CHD patients. Both associations remained significant after accounting for multiple mediators. Among paediatric patients, mortality at 1 year was 22.1% in the CHD cohort vs 17.3% in the non-CHD cohort (hazard ratios 1.39, 95% CI 0.83-2.32, P = 0.213). CONCLUSIONS: Adult and paediatric patients with CHD on MCS have higher adverse event risk compared with non-CHD MCS patients, though children did not have greater risk of mortality. As the number of CHD patients requiring advanced heart failure management continues to grow, these findings can enhance informed decision-making. CLINICAL TRIAL REGISTRATION NUMBER: Registry name: EUROMACS.

Informing preconception counseling: Outcomes among female heart transplant recipients in the ISHLT registry.

BACKGROUND: The numbers of women of child-bearing age undergoing heart transplantation (HT) and female pediatric HT recipients surviving to child-bearing age have increased, along with improvements in post-transplant survival. Data regarding life expectancy and comorbidities in reproductive-aged female HT recipients are needed to inform shared decision-making at the time of preconception counseling. METHODS: The International Society for Heart and Lung Transplantation (ISHLT) Thoracic Organ Transplant Registry was investigated for HT recipients between January 1, 2000 and June 30, 2017. Women of childbearing age were defined as those aged 15-45 years, either at transplant, or at the respective post-transplant follow-up. Characteristics and outcomes of female recipients of childbearing age at transplant, 5-, 10-, and 15-year follow-up were compared to females > 45 years of age, males 15-45 years and males > 45 years of age at the corresponding time intervals. Outcomes included survival, development of diabetes (DM), severe renal dysfunction (CKD), and cardiac allograft vasculopathy (CAV). RESULTS: During the study period, 71,585 HT recipients were included: 24% (n = 17,194) were female and 9.2% (n = 6602) were of childbearing age at HT. A pre-transplant diagnosis of peripartum cardiomyopathy was associated with significantly worse post-transplant survival, a finding that remained independent of panel reactive antibody levels. The presence of pre-transplant DM and/or severe CKD was significantly associated with lower survival as were the presence of CAV, DM, and CKD post-HT. CONCLUSION: Knowledge of the impact of pre-existing comorbidities and complications post-HT on survival are important for risk stratification for preconception counseling post-HT.

Cardio-Obstetrics and Heart Failure: JACC: Heart Failure State-of-the-Art Review.

Heart failure and cardiomyopathy are significant contributors to pregnancy-related deaths, as maternal morbidity and mortality have been increasing over time. In this setting, the role of the multidisciplinary cardio-obstetrics team is crucial to optimizing maternal, obstetrical and fetal outcomes. Although peripartum cardiomyopathy is the most common cardiomyopathy experienced by pregnant individuals, the hemodynamic changes of pregnancy may unmask a pre-existing cardiomyopathy leading to clinical decompensation. Additionally, there are unique management considerations for women with pre-existing cardiomyopathy as well as for those women with advanced heart failure who may be on left ventricular assist device support or have undergone heart transplantation. The purpose of this review is to discuss: 1) preconception counseling; 2) risk stratification and management strategies for pregnant women extending to the postpartum "fourth trimester" with pre-existing heart failure or "pre-heart failure;" 3) the safety of heart failure medications during pregnancy and lactation; and 4) management of pregnancy for women on left ventricular assist device support or after heart transplantation.

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.

Maternal left ventricular function and adverse neonatal outcomes in women with cardiac disease.

PURPOSE: To evaluate the relationship between maternal left ventricular systolic function, utero-placental circulation, and risk of adverse neonatal outcomes in women with cardiac disease. METHODS: 119 women managed in the pregnancy heart clinic (2019-2021) were identified. Women were classified by their primary cardiac condition. Adverse neonatal outcomes were: low birth weight (< 2500 g), small-for-gestational-age (< 10th birth-weight centile), pre-term delivery (< 37 weeks' gestation), and fetal demise (> 20 weeks' gestation). Parameters of left ventricular systolic function (global longitudinal strain, radial strain, ejection fraction, average S', and cardiac output) were calculated and pulsatility index was recorded from last growth scan. RESULTS: Adverse neonatal outcomes occurred in 28 neonates (24%); most frequently in valvular heart disease (n = 8) and cardiomyopathy (n = 7). Small-for-gestational-age neonates were most common in women with cardiomyopathy (p = 0.016). Early pregnancy average S' (p = 0.03), late pregnancy average S' (p = 0.02), and late pregnancy cardiac output (p = 0.008) were significantly lower in women with adverse neonatal outcomes than in those with healthy neonates. There was a significant association between neonatal birth-weight centile and global longitudinal strain (p = 0.04) and cardiac output (p = 0.0002) in late pregnancy. Pulsatility index was highest in women with cardiomyopathy (p = 0.007), and correlated with average S' (p < 0.0001) and global longitudinal strain (p = 0.03) in late pregnancy. CONCLUSION: Women with cardiac disease may not tolerate cardiovascular adaptations required during pregnancy to support fetal growth. Adverse neonatal outcomes were associated with reduced left ventricular systolic function and higher pulsatility index. The association between impaired systolic function and reduced fetal growth is supported by insufficient utero-placental circulation.

Reproductive health after thoracic transplantation: An ISHLT expert consensus statement.

Pregnancy after thoracic organ transplantation is feasible for select individuals but requires multidisciplinary subspecialty care. Key components for a successful pregnancy after lung or heart transplantation include preconception and contraceptive planning, thorough risk stratification, optimization of maternal comorbidities and fetal health through careful monitoring, and open communication with shared decision-making. The goal of this consensus statement is to summarize the current evidence and provide guidance surrounding preconception counseling, patient risk assessment, medical management, maternal and fetal outcomes, obstetric management, and pharmacologic considerations.

Pregnancy in a woman with a chronic descending thoracic aortic dissection.

Pregnancy increases aortic wall stress and, for a woman with a chronic dissection, this can lead to extension of the dissection, aortic rupture, and death. We report a pregnancy in a woman with a history of a chronic type B aortic dissection. As a child, she had repeat balloon dilation of aortic coarctation, and one of the procedures was complicated by an iatrogenic dissection at the dilation site. At the age of 27 years, she had a planned pregnancy.

Maternal and Fetal Hemodynamic Adaptations to Pregnancy and Clinical Outcomes in Maternal Cardiac Disease.

BACKGROUND: Although insufficient maternal cardiac output (CO) has been implicated in poor outcomes in mothers with heart disease (HD), maternal-fetal interactions remain incompletely understood. We sought to quantify maternal-fetal hemodynamics with the use of magnetic resonance imaging (MRI) and explore their relationship with adverse events. METHODS: Pregnant women with moderate or severe HD (n = 22; mean age 32 ± 5 years) were compared with healthy control women (n = 21; 34 ± 3 years). An MRI was performed during the third trimester at peak output (maternal-fetal) and 6 months postpartum with return of maternal hemodynamics to baseline (reference). Phase-contrast MRI was used for flow quantification and was combined with T1/T2 relaxometry for derivation of fetal oxygen delivery/consumption. RESULTS: Third-trimester CO and cardiac index (CI) measurements were similar in HD and control groups (CO 7.2 ± 1.5 vs 7.3 ± 1.6 L/min, P = 0.79; CI 4.0 ± 0.7 vs 4.3 ± 0.7 L/min/m,2P = 0.28). However, the magnitude of CO/CI increase (Δ, peak pregnancy - reference) in the HD group exceeded that in the control group (CO 46 ± 24% vs 27 ± 16% [P = 0.007]; CI 51 ± 28% vs 28 ± 17% [P = 0.005]). Fetal growth and oxygen delivery/consumption were similar between groups. Adverse cardiovascular outcomes (nonmutually exclusive) in 6 HD women included arrhythmia (n = 4), heart failure (n = 2), and hypertensive disorder of pregnancy (n = 1); premature delivery was observed in 2 of these women. The odds of a maternal cardiovascular event were inversely associated with peak CI (odds ratio 0.10, 95% confidence interval 0.001-0.86; P = 0.04) and Δ,CI (0.02, 0.001-0.71; P = 0.03). CONCLUSIONS: Maternal-fetal hemodynamics can be well characterised in pregnancy with the use of MRI. Impaired adaptation to pregnancy in women with HD appears to be associated with development of adverse outcomes of pregnancy.

A review of the management of patients with advanced heart failure in the intensive care unit.

Despite progress in the medical and device therapy for heart failure (HF), the prognosis for those with advanced HF remains poor. Acute heart failure (AcHF) is the rapid development of, or worsening of symptoms and signs of HF typically leading to hospitalization. Whilst many HF decompensations are managed at a ward-based level, a proportion of patients require higher acuity care in the intensive care unit (ICU). Admission to ICU is associated with a higher risk of in-hospital mortality, and in those who fail to respond to standard supportive and medical therapy, a proportion maybe suitable for mechanical circulatory support (MCS). The optimal pre-operative management of advanced HF patients awaiting durable MCS or cardiac transplantation (CTx) is vital in improving both short and longer-term outcomes. This review will summarize the clinical assessment, hemodynamic profiling and management of the patient with AcHF in the ICU. The general principles of pre-surgical optimization encompassing individual systems (the kidneys, the liver, blood and glycemic control) will be discussed. Other factors impacting upon post-operative outcomes including nutrition and sarcopenia and pre-surgical skin decolonization have been included. Issues specific to durable MCS including the assessment of the right ventricle and strategies for optimization will also be discussed.

Cardiovascular and abdominal flow alterations in adults with morphologic evidence of liver disease post Fontan palliation.

BACKGROUND: Although morphologic abnormalities in the liver are commonly encountered post Fontan palliation, the relationships between hepatic morphology, vascular flows, and clinical status remain incompletely understood. We therefore aimed to explore flow characteristics in hepatic and intestinal vessels and to examine cardiovascular associations with liver disease. METHODS: This was a retrospective study of adults post Fontan palliation undergoing clinically indicated cardiovascular magnetic resonance imaging (MRI). Patients were included if MRI flow quantification was available for cardiac, hepatic and intestinal vessels; patients were excluded if phase-contrast flow imaging was insufficient for analysis. RESULTS: Thirty patients were studied (median age at MRI 28.5 years [range 19-47]). Eighteen subjects (60%) were classified as having morphologic liver disease according to validated criteria based on available MRI imaging. Abdominal and cardiovascular flows were quantified. Patients with morphologic liver disease had a 41% reduction in superior mesenteric artery (211 ± 124 versus 358 ± 181 mL/min/m2, p = .004), a 36% reduction in hepatic vein (496 ± 247 versus 778 ± 220 mL/min/m2, p = .01), a 31% reduction in portal vein (399 ± 133 versus 580 ± 159 mL/min/m2, p = .004), and an 18% reduction in Fontan pathway flows (1358 ± 429 versus 1651 ± 270 mL/min/m2, p = .04) compared with the remaining population. Adverse cardiovascular events were not associated with morphologic liver disease. CONCLUSION: Morphologic liver disease appears to be associated with flow alterations within the heart, liver and intestine post Fontan palliation. These novel observations suggest that a potential relationship exists between morphologic disease and vascular flows thereby providing further insights into the pathophysiology of liver disease in this high-risk population.

A myeloperoxidase precursor, pro-myeloperoxidase, is present in human plasma and elevated in cardiovascular disease patients.

Myeloperoxidase (MPO)-derived oxidants have emerged as a key contributor to tissue damage in inflammatory conditions such as cardiovascular disease. Pro-myeloperoxidase (pro-MPO), an enzymatically active precursor of myeloperoxidase (MPO), is known to be secreted from cultured bone marrow and promyelocytic leukemia cells, but evidence for the presence of pro-MPO in circulation is lacking. In the present study, we used a LC-MS/MS in addition to immunoblot analyses to show that pro-MPO is present in human blood plasma. Furthermore, we found that pro-MPO was more frequently detected in plasma from patients with myocardial infarction compared to plasma from control donors. Our study suggests that in addition to mature MPO, circulating pro-MPO may cause oxidative modifications of proteins thereby contributing to cardiovascular disease.

Pregnancy Outcomes Following Cardiac Transplantation.

OBJECTIVE: This study sought to report pregnancy outcomes in women following cardiac transplantation. METHODS: This was a descriptive retrospective cohort study of women with pregnancies following cardiac transplantation managed at two large tertiary centres in Canada and Belgium between 2001 and 2017. RESULTS: Sixteen women had 17 singleton pregnancies following cardiac transplantation. The mean maternal age was 28 ± 5.8, and the transplant-to-pregnancy interval was 7.3 ± 4.0 years. There were two first trimester terminations, one for teratogenicity concerns and the other because of a maternal cardiac condition. There was one spontaneous miscarriage. All women had normal left ventricular function at the start of pregnancy. Graft rejection occurred in two women. Other maternal complications included anemia requiring blood transfusion (n = 5), renal failure or deterioration (n = 4), preeclampsia (n = 2), and urine infections (n = 2). The mean GA at delivery was 35 ± 3.5 weeks. Six infants were born preterm, and two were small-for-gestational-age. Fetal anomalies were identified in two pregnancies. Women were followed after pregnancy for a median of 5.6 years (range, 10 months to 15 years). Although there were no deaths during pregnancy, two women died at 10 and 18 months after delivery. CONCLUSION: With appropriate multidisciplinary care, women with cardiac transplants can have successful pregnancies. Although rates of fetal loss are low, these women continue to be at risk for graft rejection, preterm birth, other pregnancy-related complications, and cardiovascular death.

Concurrent maternal and fetal tachyarrhythmia in pregnancy.

The occurrence of a maternal and fetal tachyarrhythmia together in pregnancy is exceedingly rare. We report a case of a persistent fetal atrial ectopic tachycardia occurring in conjunction with a maternal atrial tachycardia with left ventricular systolic dysfunction. Amiodarone was effective in treating both maternal and fetal arrhythmias.

Pregnancy in Women With Repaired Truncus Arteriosus: A Case Series.

Pregnancy in women with repaired truncus arteriosus (TA) is rare. We report the outcomes of 8 pregnancies in 4 women with surgically repaired TA. None of the women had adverse cardiac events during pregnancy. Two pregnancies, both in the same woman, were complicated by hypertensive disorders of pregnancy. The birth weight was less than the 10th percentile for gestational age in 3 pregnancies. Although women with repaired TA can do well during pregnancy, they require careful preconception assessment and surveillance.

Pulmonary Valve Procedures Late After Repair of Tetralogy of Fallot: Current Perspectives and Contemporary Approaches to Management.

Few topics in adult congenital heart disease have approached the level of scrutiny bestowed on pulmonary valve replacement (PVR) strategies late after tetralogy of Fallot (TOF) repair. Despite the successes of primary surgery for TOF, there is a growing group of adults with residual right ventricular outflow tract and pulmonary valve dysfunction. Patients with residual chronic pulmonic regurgitation as a consequence of earlier surgery can later develop symptoms of exercise intolerance and complications including heart failure, tachyarrhythmias, and sudden cardiac death. Optimal timing of PVR has sparked debate, which has catalyzed increasing research efforts over the past decade. Although performance of PVR in the absence of symptoms is currently on the basis of the rationale that achievement of complete reverse remodelling is highly desirable, whether this approach results in improvement in patient outcomes in the long-term has yet to be shown. Surgical PVR and percutaneous pulmonary valve intervention are different techniques with specific advantages and disadvantages that require careful consideration for each individual patient, alongside the need for requisite reinterventions over the course of a patient's lifetime. Criteria pertaining to referral strategies are ever being refined as newer technologies for percutaneous therapies continue to evolve. In this article we review the literature surrounding the indications for, the optimal timing of, and the approaches to pulmonary valve procedures in adults with previously repaired TOF.

Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

OBJECTIVES: Individuals with childhood-onset coronary artery anomalies are at increased risk of lifelong complications. Although pregnancy is thought to confer additional risk, a few data are available regarding outcomes in this group of women. We sought to define outcomes of pregnancy in this unique population. METHODS: We performed a retrospective survey of women with paediatric-onset coronary anomalies and pregnancy in our institution, combined with a systematic review of published cases. We defined paediatric-onset coronary artery anomalies as congenital coronary anomalies and inflammatory arteriopathies of childhood that cause coronary aneurysms. Major cardiovascular events were defined as pulmonary oedema, sustained arrhythmia requiring treatment, stroke, myocardial infarction, cardiac arrest, or death. RESULTS: A total of 25 surveys were mailed, and 20 were returned (80% response rate). We included 46 articles from the literature, which described cardiovascular outcomes in 82 women (138 pregnancies). These data were amalgamated for a total of 102 women and 194 pregnancies; 59% of women were known to have paediatric-onset coronary artery anomalies before pregnancy. In 23%, the anomaly was unmasked during or shortly after pregnancy. The remainder, 18%, was diagnosed later in life. Major cardiovascular events occurred in 14 women (14%) and included heart failure (n=5, 5%), myocardial infarction (n=7, 7%), maternal death (n=2, 2%), cardiac arrest secondary to ventricular fibrillation (n=1, 1%), and stroke (n=1, 1%). The majority of maternal events (13/14, 93%) occurred in women with no previous diagnosis of coronary disease. CONCLUSIONS: Women with paediatric-onset coronary artery anomalies have a 14% risk of adverse cardiovascular events in pregnancy, indicating the need for careful assessment and close follow-up. Prospective, multicentre studies are required to better define risk and predictors of complications during pregnancy.

Pregnancy in cardiac transplant recipients.

PURPOSE: Successful pregnancy following cardiac transplantation has been described, although outcome data from individual centers are relatively sparse. We investigated maternal and fetal outcomes including change in left ventricular (LV) function and calcineurin inhibitor (CNI) dose in women who became pregnant from our institution. METHODS: We identified every female patient <49 years at the time of transplant who survived >3 months post-surgery, between 1985 and 2014. Those who conceived had a review of their medical records and transplant charts. Those currently alive were interviewed. RESULTS: There were 22 pregnancies in 17 women with 20 live births (91%). Mean time from transplantation was 98±62.4 months. Rejection complicated one pregnancy, and LV function remained normal in all others. Hypertension complicated 3 (13.6%), preeclampsia 3 (13.6%), and cholestasis 1 (4.5%). Mean birthweight was 2447±608 grams at 34.1±3.6 weeks. Four women died following pregnancy. A significant increase in total daily dose of tacrolimus and cyclosporine A was required to maintain therapeutic levels through pregnancy (CyA, P<.001; and Tac, P=.001), with no deterioration in serum creatinine. CONCLUSIONS: We report a 91% live birth rate post-cardiac transplantation. Meticulous individualized care with frequent monitoring of CNI levels and LV function is necessary to optimize the maternal and fetal outcomes.