High-Risk Congenital Heart Disease in Pregnancy.

High-risk congenital heart disease (CHD) in pregnancy presents a complex clinical challenge. With improved medical care and increased survival rates, a growing population of adults with complex CHD are surviving to adulthood, including women of reproductive age. This chapter focuses on risk stratification and management of pregnant women with high-risk CHD, emphasizing the importance of considering both anatomical and physiological complexity. Maternal physiological changes, such as blood volume increase, cardiac output changes, and alterations in vascular resistance, can significantly impact high-risk CHD patients. Management of high-risk CHD in pregnancy necessitates a multidisciplinary approach and individualized care.

Valvular Heart Disease in Pregnancy.

Valvular heart disease is a common cause of peripartum cardiovascular morbidity and mortality. The hemodynamic changes of pregnancy and their impact on preexisting valvular lesions are described in this paper. Tools for calculation of maternal and fetal risk during pregnancy are also discussed. The pathophysiology and management of valvular lesions, both obstructive and regurgitant, are then described, followed by discussion of mechanical and bioprosthetic valve complications during pregnancy.

Peripartum cardiomyopathy unveiled: Etiology, diagnosis, and therapeutic insights.

Peripartum cardiomyopathy (PPCM) remains a significant challenge in maternal health, marked by its unpredictable onset and varied clinical outcomes. With rising incidence rates globally, understanding PPCM is vital for improving maternal care and prognosis. This review aims to consolidate current knowledge on PPCM, highlighting recent advancements in its diagnosis, management, and therapeutic approaches. This comprehensive review delves into the epidemiology of PPCM, underscoring its global impact and demographic variations. We explore the complex etiology of the condition, examining known risk factors and discussing the potential pathophysiological mechanisms, including oxidative stress and hormonal influences. The clinical presentation of PPCM, often similar yet distinct from other forms of cardiomyopathy, is analyzed to aid in differential diagnosis. Diagnostic challenges are addressed, emphasizing the role of advanced imaging and biomarkers. Current management strategies are reviewed, focusing on the absence of disease-specific treatments and the application of general heart failure protocols. The review also discusses the prognosis of PPCM, factors influencing recovery, and the implications for future pregnancies. Finally, we highlight emerging research directions and the urgent need for disease-specific therapies, aiming to provide a roadmap for future studies and improved patient care. This review serves as a crucial resource for clinicians and researchers, contributing to a deeper understanding and better management of PPCM.

Peripartum cardiomyopathy in low- and middle-income countries.

Peripartum cardiomyopathy (PPCM) causes pregnancy-associated heart failure, typically during the last month of pregnancy, and up to 6 months post-partum, in women without known cardiovascular disease. PPCM is a global disease, but with a significant geographical variability within and between countries. Its true incidence in Africa is still unknown because of the lack of a PPCM population-based study. The variability in the epidemiology of PPCM between and within countries could be due to differences in the prevalence of both genetic and non-genetic risk factors. Several risk factors have been implicated in the aetiopathogenesis of PPCM over the years. Majority of patients with PPCM present with symptoms and signs of congestive cardiac failure. Diagnostic work up in PPCM is prompted by strong clinical suspicion, but Echocardiography is the main imaging technique for diagnosis. The management of PPCM involves multiple disciplines - cardiologists, anaesthetists, intensivists, obstetricians, neonatologists, and the prognosis varies widely.

Socio-economic factors determine maternal and neonatal outcomes in women with peripartum cardiomyopathy: A study of the ESC EORP PPCM registry.

BACKGROUND: Peripartum cardiomyopathy (PPCM) is a global disease with substantial morbidity and mortality. The aim of this study was to analyze to what extent socioeconomic factors were associated with maternal and neonatal outcomes. METHODS: In 2011, >100 national and affiliated member cardiac societies of the European Society of Cardiology (ESC) were contacted to contribute to a global PPCM registry, under the auspices of the ESC EORP Programme. We investigated the characteristics and outcomes of women with PPCM and their babies according to individual and country-level sociodemographic factors (Gini index coefficient [GINI index], health expenditure [HE] and human developmental index [HDI]). RESULTS: 739 women from 49 countries (Europe [33%], Africa [29%], Asia-Pacific [15%], Middle East [22%]) were enrolled. Low HDI was associated with greater left ventricular (LV) dilatation at time of diagnosis. However, baseline LV ejection fraction did not differ according to sociodemographic factors. Countries with low HE prescribed guideline-directed heart failure therapy less frequently. Six-month mortality was higher in countries with low HE; and LV non-recovery in those with low HDI, low HE and lower levels of education. Maternal outcome (death, re-hospitalization, or persistent LV dysfunction) was independently associated with income. Neonatal death was significantly more common in countries with low HE and low HDI, but was not influenced by maternal income or education attainment. CONCLUSIONS: Maternal and neonatal outcomes depend on country-specific socioeconomic characteristics. Attempts should therefore be made to allocate adequate resources to health and education, to improve maternal and fetal outcomes in PPCM.

Outcomes at one year in women with peripartum cardiomyopathy: Findings from the ESC EORP PPCM Registry.

AIMS: There are few prospective reports of 1-year outcomes for women with peripartum cardiomyopathy (PPCM). We report findings from the European Society of Cardiology EURObservational Research Programme PPCM Registry. METHODS AND RESULTS: The registry enrolled women from 51 countries from 2012 to 2018. Eligibility included: (i) a peripartum state, (ii) signs or symptoms of heart failure, (iii) left ventricular (LV) ejection fraction ≤45%, (iv) exclusion of alternative causes of heart failure. We report mortality, thromboembolism, stroke, rehospitalization, LV recovery and remodelling at 1 year. Differences between regions were compared. One-year mortality data were available in 535 (71%) women and follow-up differed across regions. At 1 year, death from any cause occurred in 8.4% of women, with regional variation (Europe 4.9%, Africa 6.5%, Asia-Pacific 9.2%, Middle East 18.9%; p < 0.001). The frequencies of thromboembolism and stroke were 6.3% and 2.5%, respectively, and were similar across regions. A total of 14.0% of women had at least one rehospitalization and 3.5% had recurrent rehospitalizations (i.e. two or more). Overall, 66.1% of women had recovery of LV function (22% between 6 months and 1 year), with a mean LV ejection fraction increase from baseline of 21.2% (±13.6). Recovery occurred most frequently in Asia-Pacific (77.5%) and least frequently in the Middle East (32.7%). There were significant regional differences in the use of heart failure pharmacotherapies. CONCLUSIONS: Approximately 1 in 12 women with PPCM had died by 1 year and thromboembolism and stroke occurred in 6.3% and 2.5%, respectively. Around 1 in 7 women had been rehospitalized and, in 1 in 3, LV recovery had not occurred. PPCM is associated with substantial mortality and morbidity globally.

Proteomic Profiling in Patients With Peripartum Cardiomyopathy: A Biomarker Study of the ESC EORP PPCM Registry.

BACKGROUND: Peripartum cardiomyopathy (PPCM) remains an important cause of maternal morbidity and mortality globally. The pathophysiology remains incompletely understood, and the diagnosis is often missed or delayed. OBJECTIVES: This study explored the serum proteome profile of patients with newly diagnosed PPCM, as compared with matched healthy postpartum mothers, to unravel novel protein biomarkers that would further an understanding of the pathogenesis of PPCM and improve diagnostic precision. METHODS: Study investigators performed untargeted serum proteome profiling using data-independent acquisition-based label-free quantitative liquid chromatography-tandem mass spectrometry on 84 patients with PPCM, as compared with 29 postpartum healthy controls (HCs). Significant changes in protein intensities were determined with nonpaired Student's t-tests and were further classified by using the Boruta algorithm. The proteins' diagnostic performance was evaluated by area under the curve (AUC) and validated using the 10-fold cross-validation. RESULTS: Patients with PPCM presented with a mean left ventricular ejection fraction of 33.5% ± 9.3% vs 57.0% ± 8.8% in HCs (P < 0.001). Study investigators identified 15 differentially up-regulated and 14 down-regulated proteins in patients with PPCM compared with HCs. Seven of these proteins were recognized as significant by the Boruta algorithm. The combination of adiponectin, quiescin sulfhydryl oxidase 1, inter-α-trypsin inhibitor heavy chain, and N-terminal pro-B-type natriuretic peptide had the best diagnostic precision (AUC: 0.90; 95% CI: 0.84-0.96) to distinguish patients with PPCM from HCs. CONCLUSIONS: Salient biologic themes related to immune response proteins, inflammation, fibrosis, angiogenesis, apoptosis, and coagulation were predominant in patients with PPCM compared with HCs. These newly identified proteins warrant further evaluation to establish their role in the pathogenesis of PPCM and potential use as diagnostic markers.

Validation of Risk Stratification for Cardiac Events in Pregnant Women With Valvular Heart Disease.

BACKGROUND: Most risk stratification tools for pregnant patients with heart disease were developed in high-income countries and in populations with predominantly congenital heart disease, and therefore, may not be generalizable to those with valvular heart disease (VHD). OBJECTIVES: The purpose of this study was to validate and establish the clinical utility of 2 risk stratification tools-DEVI (VHD-specific tool) and CARPREG-II-for predicting adverse cardiac events in pregnant patients with VHD. METHODS: We conducted a cohort study involving consecutive pregnancies complicated with VHD admitted to a tertiary center in a middle-income setting from January 2019 to April 2022. Individual risk for adverse composite cardiac events was calculated using DEVI and CARPREG-II models. Performance was assessed through discrimination and calibration characteristics. Clinical utility was evaluated with Decision Curve Analysis. RESULTS: Of 577 eligible pregnancies, 69 (12.1%) experienced a component of the composite outcome. A majority (94.7%) had rheumatic etiology, with mitral regurgitation as the predominant lesion (48.2%). The area under the receiver-operating characteristic curve was 0.884 (95% CI: 0.844-0.923) for the DEVI and 0.808 (95% CI: 0.753-0.863) for the CARPREG-II models. Calibration plots suggested that DEVI score overestimates risk at higher probabilities, whereas CARPREG-II score overestimates risk at both extremes and underestimates risk at middle probabilities. Decision curve analysis demonstrated that both models were useful across predicted probability thresholds between 10% and 50%. CONCLUSIONS: In pregnant patients with VHD, DEVI and CARPREG-II scores showed good discriminative ability and clinical utility across a range of probabilities. The DEVI score showed better agreement between predicted probabilities and observed events.

Post-COVID-19 Peripartum Cardiomyopathy: Experience from a Large Tertiary Referral Center.

BACKGROUND: Peripartum cardiomyopathy (PPCM) is a rare but potentially devastating complication of pregnancy. Although the pathophysiology of PPCM is not fully understood, there are known risk factors for developing PPCM, which are maternal and gestation related. In the first wave of the coronavirus disease 2019 (COVID-19) pandemic, we witnessed an elevated incidence of PPCM among COVID-19 survivors. OBJECTIVES: To present a single-center case series of three patients diagnosed with peripartum cardiomyopathy after recovered from COVID-19 during the index pregnancy. METHODS: In this single center case study, all patients diagnosed with PPCM at our institute during the examined time frame were included. Electronic medical records were studied. RESULTS: Three patients previously diagnosed with asymptomatic or mildly symptomatic COVID-19 disease during pregnancy presented with PPCM before or shortly after delivery. Patients underwent testing to rule out residual COVID-19 myocarditis, were treated pharmacologically and with wearable defibrillators as needed, and were examined in follow-up 1-9 months after delivery. CONCLUSIONS: Residual endothelial damage due to COVID-19 disease, even if originally mild in presentation, could predispose pregnant patients to PPCM and should be considered as a risk factor when assessing patients with new onset symptoms of heart failure. Further research is needed to confirm this hypothesis and fully determine the underlying pathophysiology. These preliminary findings warrant a high index of suspicion for PPCM in COVID-19 recoverers.

Successful management of peripartum cardiomyopathy in a Kenyan setting: a case series.

Peripartum cardiomyopathy is a rare life-threatening condition occurring in previously healthy women with symptoms mimicking those of normal pregnancy and is associated with a high mortality rate. A high index of suspicion coupled with a sound understanding of the disease is crucial to correctly diagnose and manage the patients to improve final maternal outcomes. In this report, we present a total of five cases of peripartum cardiomyopathy in women aged 22 to 38 years who presented between 3 and 21 days postpartum. All patients presented with severely reduced ejection fractions indicative of heart failure and were immediately admitted to our facility. A timely diagnosis was made and patients started on a combination of antibiotics, anticoagulants, and anti-heart failure medication. Despite the severity of the disease upon presentation, early diagnosis and precise management of the disease were essential in achieving favorable patient outcomes. Therefore, this report provides crucial knowledge about the presentation and progression of peripartum cardiomyopathy and presents a treatment protocol from a Kenyan perspective that was successfully employed in the management of all five cases.

Cardiovascular Complications of Pregnancy.

The physiologic changes in pregnancy predispose the pregnant patient to a variety of potential cardiovascular complications. In this article, we discuss the major cardiovascular disorders of pregnancy and their management, highlight specific diagnostic challenges, and discuss new developments in the field. Topics covered in this article include venous thromboembolism, acute myocardial infarction, peripartum cardiomyopathy, and aortic dissection.

Peripartum cardiomyopathy: risk factors and predictors of outcome.

PURPOSE OF REVIEW: Peripartum cardiomyopathy (PPCM) contributes significantly to maternal morbidity and mortality worldwide. In this review, we describe the present-day epidemiology and current understanding of the pathogenesis of PPCM. We provide an updated approach to diagnosis and management of PPCM, and discuss risk factors and predictors of outcome. RECENT FINDINGS: The highest incidences of PPCM have been reported in African, Asian, and Caribbean populations. Contemporary literature supports a 'two-hit' hypothesis, whereby the 'first hit' implies a predisposition, and the 'second hit' refers to an imbalanced peripartal hormonal milieu that results in cardiomyopathy. Whereas a half of patients will have left ventricular (LV) recovery, a tenth do not survive. Clinical findings and special investigations (ECG, echocardiography, cardiac MRI, biomarkers) can be used for risk stratification. Frequent prescription of guideline-directed medical therapy is associated with improved outcomes. SUMMARY: Despite advances in elucidating the pathogenesis of PPCM, it remains unclear why only certain women develop the disease. Moreover, even with better diagnostic work-up and management, it remains unknown why some patients with PPCM have persistent LV dysfunction or die. Future research should be aimed at better understanding of the mechanisms of disease and finding new therapies that could improve survival and LV recovery.

Peripartum Cardiomyopathy: Current Understanding of Pathophysiology, Diagnostic Workup, Management, and Outcomes.

Peripartum cardiomyopathy (PPCM) is a relatively rare, potentially life-threatening, idiopathic form of cardiomyopathy that affects previously healthy young women during late pregnancy or in the early postpartum period and is characterized by left ventricular systolic dysfunction in the absence of any other identifiable cardiac causes. Morbidity and mortality with PPCM are remarkably high and it continues to be one of the leading causes of maternal death. Although remarkable advances have been made in our understanding of PPCM in the last few decades, unanswered questions remain regarding its pathophysiology, diagnostic workup, and management options. In this article, we will complete an updated, comprehensive review of PPCM, including the epidemiology and risk factors, proposed etiology, presentation and complications, management, prognostic indicators and outcomes. In addition, we will identify current challenges and gaps in knowledge.

Aortic Dissection and COVID-19 Pneumonia in a Pregnant Woman at 34 Weeks of Gestation.

Between 0.1% and 0.3% of all aortic dissections occur during pregnancy. Arterial hypertension, connective tissue disorders, and congenital cardiovascular anomalies-including bicuspid aortic valves-are well-known risk factors. The causality between pregnancy and aortic dissection is unclear, but there have been some observations that COVID-19 illness may increase the risk. This report describes a pregnant woman at 34 weeks of gestation who had a bicuspid aortic valve and experienced an acute aortic dissection while ill with COVID-19 pneumonia. Computed tomography confirmed a type A aortic dissection and bilateral patchy pulmonary opacities. Cesarean delivery was performed, followed by replacement of the aortic valve with a mechanical aortic prosthesis and reconstruction of the ascending aorta and hemiarch. The intraoperative course was uneventful, and the patient was successfully weaned from mechanical ventilation after 51 hours. COVID-19 during pregnancy seems to increase the risk for aortic dissection, although there is no evidence base for an association. Because guidelines for diagnosis and treatment in such complex cases are lacking, care from a multidisciplinary team is crucial for successful outcomes.