Recovery of left ventricular systolic function in peripartum cardiomyopathy: an observational study from rural Tanzania.

BACKGROUND: The aim of this study was to evaluate the recovery rate of the left ventricular systolic function of women diagnosed with peripartum cardiomyopathy receiving specialized care in rural Tanzania. METHODS: In this observational study, women diagnosed with peripartum cardiomyopathy at a referral center in rural Tanzania between December 2015 and September 2021 were included. Women diagnosed between February and September 2021 were followed prospectively, those diagnosed between December 2015 and January 2021 were tracked back for a follow-up echocardiography. All participants received a clinical examination, a comprehensive echocardiogram, and a prescription of guideline-directed medical therapy. The primary outcome was recovery of the left ventricular systolic function (left ventricular ejection fraction > 50%). RESULTS: Median age of the 110 participants was 28.5 years (range 17-45). At enrolment, 49 (45%) participants were already on cardiac medication, 50 (45%) had severe eccentric hypertrophy of the left ventricle, and the median left ventricular ejection fraction was 30% (range 15-46). After a median follow-up of 8.98 months (IQR 5.72-29.37), 61 (55%) participants were still on cardiac medication. Full recovery of the left ventricular systolic function was diagnosed in 76 (69%, 95% CI 59.6-77.6%) participants. In the multivariate analysis, a higher left ventricular ejection fraction at baseline was positively associated with full recovery (each 5% increase; OR 1.7, 95% CI 1.10-2.62, p = 0.012), while higher age was inversely associated (each 10 years increase; OR 0.40, 95% CI 0.19-0.82, p = 0.012). CONCLUSION: Left ventricular systolic function recovered completely in 69% of study participants with peripartum cardiomyopathy from rural Tanzania under specialized care.

Haemorrhagic stroke in pregnancy.

OBJECTIVE: To present a case of acute haemorrhagic stroke during 3rd trimester of pregnancy and to describe management and successful delivery of healthy baby. CASE REPORT: Haemorrhagic stroke is responsible for significant morbidity and mortality. Prognosis can be improved only by urgent diagnosis and care. We report a case of pregnant woman at 37th week of pregnancy with acute haemorrhagic stroke of unknown etiology with clinical appearance of thunderclap headaches and overall disorientation. We describe diagnostic approach and a successful management followed by further differential diagnosis and treatment. The foetus was delivered by acute caesarean section at 37th week of pregnancy. CONCLUSION: Occurrence of haemorrhagic stroke in pregnancy is rare. There are no specific guidelines that recommend the time and mode of delivery; therefore, each case is assessed individually.

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.

Development and validation of an electrocardiographic artificial intelligence model for detection of peripartum cardiomyopathy.

BACKGROUND: This study used electrocardiogram data in conjunction with artificial intelligence methods as a noninvasive tool for detecting peripartum cardiomyopathy. OBJECTIVE: This study aimed to assess the efficacy of an artificial intelligence-based heart failure detection model for peripartum cardiomyopathy detection. STUDY DESIGN: We first built a deep-learning model for heart failure detection using retrospective data at the University of Tennessee Health Science Center. Cases were adult and nonpregnant female patients with a heart failure diagnosis; controls were adult nonpregnant female patients without heart failure. The model was then tested on an independent cohort of pregnant women at the University of Tennessee Health Science Center with or without peripartum cardiomyopathy. We also tested the model in an external cohort of pregnant women at Atrium Health Wake Forest Baptist. Key outcomes were assessed using the area under the receiver operating characteristic curve. We also repeated our analysis using only lead I electrocardiogram as an input to assess the feasibility of remote monitoring via wearables that can capture single-lead electrocardiogram data. RESULTS: The University of Tennessee Health Science Center heart failure cohort comprised 346,339 electrocardiograms from 142,601 patients. In this cohort, 60% of participants were Black and 37% were White, with an average age (standard deviation) of 53 (19) years. The heart failure detection model achieved an area under the curve of 0.92 on the holdout set. We then tested the ability of the heart failure model to detect peripartum cardiomyopathy in an independent University of Tennessee Health Science Center cohort of pregnant women and an external Atrium Health Wake Forest Baptist cohort of pregnant women. The independent University of Tennessee Health Science Center cohort included 158 electrocardiograms from 115 patients; our deep-learning model achieved an area under the curve of 0.83 (0.77-0.89) for this data set. The external Atrium Health Wake Forest Baptist cohort involved 80 electrocardiograms from 43 patients; our deep-learning model achieved an area under the curve of 0.94 (0.91-0.98) for this data set. For identifying peripartum cardiomyopathy diagnosed ≥10 days after delivery, the model achieved an area under the curve of 0.88 (0.81-0.94) for the University of Tennessee Health Science Center cohort and of 0.96 (0.93-0.99) for the Atrium Health Wake Forest Baptist cohort. When we repeated our analysis by building a heart failure detection model using only lead-I electrocardiograms, we obtained similarly high detection accuracies, with areas under the curve of 0.73 and 0.93 for the University of Tennessee Health Science Center and Atrium Health Wake Forest Baptist cohorts, respectively. CONCLUSION: Artificial intelligence can accurately detect peripartum cardiomyopathy from electrocardiograms alone. A simple electrocardiographic artificial intelligence-based peripartum screening could result in a timelier diagnosis. Given that results with 1-lead electrocardiogram data were similar to those obtained using all 12 leads, future studies will focus on remote screening for peripartum cardiomyopathy using smartwatches that can capture single-lead electrocardiogram data.

Disease features and management of cardiomyopathies in women.

Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation. In mitochondrial disorders with a matrilinear transmission, cardiomyopathy is part of a systemic disorder affecting both men and women. Even some inherited cardiomyopathies with autosomal transmission display phenotypic and prognostic differences between men and women. Notably, female hormones seem to exert a protective role in hypertrophic cardiomyopathy (HCM) and variant transthyretin amyloidosis until the menopausal period. Women with cardiomyopathies holding high-risk features should be referred to a third-level center and evaluated on an individual basis. Cardiomyopathies can have a detrimental impact on pregnancy and childbirth because of the associated hemodynamic derangements. Genetic counselling and a tailored cardiological evaluation are essential to evaluate the likelihood of transmitting the disease to the children and the possibility of a prenatal or early post-natal diagnosis, as well as to estimate the risk associated with pregnancy and delivery, and the optimal management strategies.

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.

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.

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.

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.