Peripartum Cardiomyopathy Patients Research Articles

Ventricular Tachycardia in Patients With Peripartum Cardiomyopathy: Prevalence, Predictors, and Associated In-Hospital Adverse Events.

Introduction The purpose of this study was to determine the prevalence of ventricular tachycardia (VT) among patients admitted with peripartum cardiomyopathy (PPCM) as well as to analyze the independent association of VT with in-hospital outcomes among PPCM patients. Methods Data were obtained from the National Inpatient Sample from January 2016 to December 2019. We assessed predictors of VT in patients admitted with PPCM. We also assessed the independent association of VT with clinical outcomes among patients admitted with PPCM. Results From 2016 to 2019, 4730 patients with PPCM were reported to the national inpatient sample database, 309 of which developed VT (6.5%). Using multivariate analysis, we found predictors of VT to include patient characteristics and factors such as age (adjusted OR (aOR)=1.020, p=0.023), chronic kidney disease (aOR=1.440, p=0.048), coagulopathy (aOR=1.964, p=0.006), and atrial fibrillation (aOR=3.965,p<0.001). Conversely, pre-eclampsia was significantly associated with a decreased risk of VT in PPCM patients (aOR=0.218,p=0.001). Conclusion In a large cohort of patients admitted with peripartum cardiomyopathy, we found the prevalence of VT to be 6.5%. Risk factors for VT in this patient population included conditions such as coagulopathy and atrial fibrillation.

Clinical Presentation and 6-Month Outcomes of Patients with Peripartum Cardiomyopathy in Indonesia.

Due to the rarity of peripartum cardiomyopathy (PPCM) globally, baseline characteristic data for PPCM patients are still scarce. Therefore, this study aims to determine the baseline characteristics and 6-month outcomes of PPCM patients in Indonesia. From January 2014 to December 2021, all PPCM patients aged ≥18 years who were admitted to Dr. Hasan Sadikin General Hospital in Bandung, Indonesia, participated in this single-center, prospective cohort study. All patients were re-evaluated within 6 months of PPCM diagnosis. A total of 138 patients with PPCM were admitted to Dr. Hasan Sadikin General Hospital in Bandung. The mean age of all patients was 30.4 ± 6.4 years old. Approximately 60% patients were multipara and had preeclampsia. All guideline-directed medical therapy for heart failure was received by most patients, excluding mineralocorticoid receptor antagonists (25.2%) and bromocriptine (14.1%). The neonatal mortality rate was 5.1%. Among those who survived, 61.2% had normal weight, 31.8% had low birth weight, and 7% had very low birth weight. At the 6-month follow-up, 6.7% of the patients died, 63.3% recovered, and 1.9% were rehospitalized. The present study found a high incidence of PPCM in Indonesia. Our patients frequently had preeclampsia, which contributed to the higher rate of miscarriage and low birth weight. Our liberal use of beta-blockers and ACEi/ARB may have contributed to the higher 6-month recovery rate than that in other countries.

Thromboembolism in peripartum cardiomyopathy: a systematic review.

Women with peripartum cardiomyopathy (PPCM) are at an increased risk of arterial and venous thromboembolic events. The review summarizes the evidence on the incidence of thromboembolic complications in women with PPCM, diagnostic approaches, related outcomes, and effects of therapies that have been used. English articles were retrieved from Web of Science and PubMed using search terms to capture studies related to PPCM (or postpartum cardiomyopathy) and all combinations of thrombosis- and embolism-related keywords. A total of 347 articles from PubMed and 85 from Web of Science were obtained, and after removing duplicates, 327 articles were screened for original data and classified into four domains: epidemiology, risk factors, diagnosis, and therapy of thromboembolism in PPCM. Ultimately, 30 articles were included. Data were synthesized in summary tables for each domain. Studies in the United States and Europe reported varying incidence for thromboembolism in PPCM, up to 14% in 6 months. Risk factors include elevated levels of coagulation factors, decreased protein C and S activity, decreased fibrinolysis, and a low left ventricular ejection fraction (LVEF). Cesarean delivery and post-operative status were correlated with a higher incidence of thromboembolic complications. Diagnosis relied mostly on ultrasonography and magnetic resonance and depended on the suspected location of thrombus. Anticoagulation has been used mostly for PPCM patients with a reduced LVEF, with the duration varying across guidelines and healthcare systems. Unfractionated heparin and low molecular weight heparin (LMWH) were considered safe choices during pregnancy, while warfarin and novel oral anticoagulants (NOACs) were used postpartum. The association of bromocriptine with risk of thromboembolic complications remains debated. There are important gaps in our understanding of the epidemiology, risk stratification, and optimal secondary prevention of thromboembolism in PPCM. Larger prospective studies with detailed phenotyping are required.

Frequency and Clinical Implications of Referrals to Heart Failure Among Patients with Peripartum Cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a rare but significant cause of new-onset heart failure (HF) during the peri- and post-partum periods. Advances in GDMT for HF with reduced ventricular function have led to substantial improvements in survival and quality of life, yet few studies examine the longitudinal care received by patients with PPCM. The aim of this research is to address this gap by retrospectively characterizing patients with PPCM across a multihospital health system and investigating the frequency of cardiology and HF specialty referrals. Understanding whether surveillance and medical management differ among patients referred to HF will help to underscore the importance of referring patients with PPCM to HF specialists for optimal care.

Rationale and design for the peripartum cardiomyopathy in Nigeria (PEACE) registry

Background: Nigeria probably has the highest burden of peripartum cardiomyopathy (PPCM) in the world. The primary objective is to describe the burden, ventricular remodelling, and outcomes (rehospitalisation rate, cardioembolic events and survival) of PPCM in Nigeria. In the substudies, we aim to describe the relationship between selenium deficiency, oxidative stress and PPCM, the impact of sodium selenite supplementation on cardiac function in PPCM, and the prevalence of selenium deficiency and its relationship with cardiac function in apparently healthy pregnant women. Methods: The main registry and the first substudy are prospective longitudinal studies, while the second substudy is an openlabel randomised trial. 36 study centres across Nigeria have been registered and 10 of them are already recruiting subjects. Patients will be recruited from June to December 2017 and followed up till December 2018. Serum selenium and glutathione peroxidase will be assayed at recruitment for consecutive PPCM patients with left ventricular ejection fraction &lt;45% at 6 months postpartum. 200 subjects with selenium deficiency will be randomised into treatment (selenium selenite 200 μg tablets daily for 3 months) and control arms. In the second substudy, 120 apparently healthy pregnant women will be recruited at 2838 weeks of gestation and reviewed at 68 weeks postpartum, and their serum selenium and glutathione peroxidas levels will be measured at recruitment. Conclusions: This will be the largest systematic evaluation of PPCM in Nigeria, and it is hoped that the information will assist in developing locally applicable treatment guidelines and policies for the disease.

Rationale and design for the peripartum cardiomyopathy in Nigeria (PEACE) registry

Background: Nigeria probably has the highest burden of peripartum cardiomyopathy (PPCM) in the world. The primary objective is to describe the burden, ventricular remodelling, and outcomes (rehospitalisation rate, cardioembolic events and survival) of PPCM in Nigeria. In the substudies, we aim to describe the relationship between selenium deficiency, oxidative stress and PPCM, the impact of sodium selenite supplementation on cardiac function in PPCM, and the prevalence of selenium deficiency and its relationship with cardiac function in apparently healthy pregnant women. Methods: The main registry and the first substudy are prospective longitudinal studies, while the second substudy is an openlabel randomised trial. 36 study centres across Nigeria have been registered and 10 of them are already recruiting subjects. Patients will be recruited from June to December 2017 and followed up till December 2018. Serum selenium and glutathione peroxidase will be assayed at recruitment for consecutive PPCM patients with left ventricular ejection fraction &lt;45% at 6 months postpartum. 200 subjects with selenium deficiency will be randomised into treatment (selenium selenite 200 μg tablets daily for 3 months) and control arms. In the second substudy, 120 apparently healthy pregnant women will be recruited at 2838 weeks of gestation and reviewed at 68 weeks postpartum, and their serum selenium and glutathione peroxidas levels will be measured at recruitment. Conclusions: This will be the largest systematic evaluation of PPCM in Nigeria, and it is hoped that the information will assist in developing locally applicable treatment guidelines and policies for the disease.

Rationale and design for the peripartum cardiomyopathy in Nigeria (PEACE) registry

Background: Nigeria probably has the highest burden of peripartum cardiomyopathy (PPCM) in the world. The primary objective is to describe the burden, ventricular remodelling, and outcomes (rehospitalisation rate, cardioembolic events and survival) of PPCM in Nigeria. In the substudies, we aim to describe the relationship between selenium deficiency, oxidative stress and PPCM, the impact of sodium selenite supplementation on cardiac function in PPCM, and the prevalence of selenium deficiency and its relationship with cardiac function in apparently healthy pregnant women. Methods: The main registry and the first substudy are prospective longitudinal studies, while the second substudy is an openlabel randomised trial. 36 study centres across Nigeria have been registered and 10 of them are already recruiting subjects. Patients will be recruited from June to December 2017 and followed up till December 2018. Serum selenium and glutathione peroxidase will be assayed at recruitment for consecutive PPCM patients with left ventricular ejection fraction &lt;45% at 6 months postpartum. 200 subjects with selenium deficiency will be randomised into treatment (selenium selenite 200 μg tablets daily for 3 months) and control arms. In the second substudy, 120 apparently healthy pregnant women will be recruited at 2838 weeks of gestation and reviewed at 68 weeks postpartum, and their serum selenium and glutathione peroxidas levels will be measured at recruitment. Conclusions: This will be the largest systematic evaluation of PPCM in Nigeria, and it is hoped that the information will assist in developing locally applicable treatment guidelines and policies for the disease.

Predicting poor left ventricular function recovery in Peripartum cardiomyopathy

Introduction Peripartum cardiomyopathy (PPCM) is a rare type of cardiomyopathy that manifests as acute heart failure associated with pregnancy. Delays in early identification result in poor recovery of left ventricular (LV) function; however, no risk prediction model exists. We sought to yield a scoring system known as the Padjadjaran Peripartum CardioMyopathy Recovery (PPCM recovery) score to predict the probability of poor LV function recovery in PPCM patients. Methods All baseline and clinical parameters were prospectively collected from a cohort of patients with PPCM admitted to Dr. Hasan Sadikin General Hospital in Bandung, Indonesia between January 2014 and December 2021. Logistic regression analyses were performed to investigate the relationship between each variable and the risk of poor LV function recovery in PPCM patients. Results This prospective cohort study included 113 patients with PPCM (84 recovered and 29 non-recovered patients). Significant mitral regurgitation (MR), left ventricular ejection fraction (LVEF) <30%, left ventricular end-diastolic diameter (LVEDD) ≥56 mm, and New York Heart Association functional class (NYHA FC) IV were all strong predictors of poor LV function recovery. These variables were integrated into the PPCM recovery score (AUC of 0.85). Patients with a score of ≥8 were nearly 18 times more likely to have poor LV function recovery (sensitivity 57%, specificity 93%). Conclusion PPCM recovery score is a convenient scoring system based on clinical and echocardiography assessment that may assist in distinguishing which patients are more likely to develop poor LV function recovery; therefore, these patients should be immediately referred to a tertiary referral hospital.

Abstract 17104: Characterization of a Novel, Clinically Relevant Mouse Model of Peripartum Cardiomyopathy

Pregnancy requires significantly increased cardiac output (~30%) achieved through peripheral vasodilation and increased stroke volume, due to increased end-diastolic volume. Most women resolve these physiological changes within 6 months of delivery; however, some women progress to heart failure late in pregnancy or in the immediate post-partum period. Peri-partum cardiomyopathy (PPCM) is a rare condition of largely unknown etiology which, at its most-severe and life-threatening, requires cardiac transplantation. Pre-eclampsia occurs at much higher rates in the PPCM population (~40%) compared with the generally pregnant population (~2-5%). We have previously shown that transgenic mice expressing the human-specific isoform of the TBXA2R gene (TPβ) spontaneously develop pre-eclampsia accompanied by all the associated hallmarks expected from the human disease. We now show TPβ transgenic (TPβ-Tg) mice are a model of PPCM. Using trans-thoracic echocardiographic imaging, TPβ-Tg mice had no basal phenotype; however, unlike C57/Bl6J (WT) mice that resolved the pregnancy-induced cardiac remodeling, TPβ-Tg mice had exaggerated cardiac dilatation at term (E18.5; P&lt;0.01) which increased in severity over the subsequent 6 months (P&lt;0.005). In addition, TP-Tg mice exhibited reduced ejection fraction (22±13% vs 67% in WT), significant LV wall thinning (1.12±0.16 mm vs 1.6±0.17 mm WT), and loss of LV mass (66.55±9 mg vs 95±11mg WT). Analysis of myocardium of human PPCM patients undergoing heart transplant (n=12) compared to healthy myocardium from non-diseased donors (n=42) by RT-PCR and IHC showed highly expressed TPβ in PPCM patients. In human PPCM samples, there was a significant (p&lt;0.01) dysregulation of genes associated with circadian rhythm as determined by RNAseq analysis. Quantitative PCR analysis of hearts from non-pregnant (WT=8; TPβ-Tg=4) and pregnant (WT=10; TPβ-Tg=13) mice found similar dysregulation of multiple genes associated with circadian rhythm compared to WT mice (p&lt;0.01). This indicates that PPCM may be associated with failure to terminate CLOCK signaling as a result of TPβ activation. Our results support dysregulation of TBXA2R splicing as part of the pathogenesis of PPCM and identify new therapeutic targets for this condition.

Breastfeeding in peripartum cardiomyopathy mothers: A systematic review

Introduction: Peripartum cardiomyopathy (PPCM) develops around the end of pregnancy or in the initial months after delivery. PPCM is induced by vasculotoxic chemicals secreted from the placenta and pituitary. Prolactin is one of these hormones that could be harmful. The maternal pituitary release prolactin in response to breastfeeding. The advantages of nursing for both mother and child must be taken seriously while deciding whether to breastfeed with PPCM. This systematic review aims to analyze the existing scientific evidence on breastfeeding in PPCM patients. Method: To ensure comprehensive retrieval of relevant research we search the following key databases: PubMed and ScienceDirect through 1000 for peer reviewed articles (in all languages) evidence related to breastfeeding in peripartum cardiomyopathy. Results: The studies suggest that PPCM influenced by the hormone prolactin which related to breastfeeding. Therapy of prolactin inhibitors is given to inhibit the worsening of PPCM patients, but with side effects patients cannot provide exclusive breastfeeding. From the study, breastfeeding has no significant effect on the LVEF of mothers with PPCM, but in PPCM conditions that are not well compensated, breastfeeding is not recommended and patients should undergo therapy with bromocriptine. Conclusion: Breastfeeding and bromocriptine go opposite but both have benefits for PPCM patients, consideration for breastfeeding or bromocriptine administration must considering the patient's condition. Administration of bromocriptine for certain conditions such as severe PPCM can accelerate LV function recovery. There will be side effects for both children and mothers who are not breastfed.

Transcriptomic Comparison of Human Peripartum and Dilated Cardiomyopathy Identifies Differences in Key Disease Pathways

Peripartum cardiomyopathy (PPCM) is a rare form of acute onset heart failure that presents in otherwise healthy pregnant women around the time of delivery. While most of these women respond to early intervention, about 20% progress to end-stage heart failure that symptomatically resembles dilated cardiomyopathy (DCM). In this study, we examined two independent RNAseq datasets from the left ventricle of end-stage PPCM patients and compared gene expression profiles to female DCM and non-failing donors. Differential gene expression, enrichment analysis and cellular deconvolution were performed to identify key processes in disease pathology. PPCM and DCM display similar enrichment in metabolic pathways and extracellular matrix remodeling suggesting these are similar processes across end-stage systolic heart failure. Genes involved in golgi vesicles biogenesis and budding were enriched in PPCM left ventricles compared to healthy donors but were not found in DCM. Furthermore, changes in immune cell populations are evident in PPCM but to a lesser extent compared to DCM, where the latter is associated with pronounced pro-inflammatory and cytotoxic T cell activity. This study reveals several pathways that are common to end-stage heart failure but also identifies potential targets of disease that may be unique to PPCM and DCM.

Association polymorphism of guanine nucleotide-binding protein β3 subunit (GNB3) C825T and insertion/deletion of the angiotensin-converting enzyme (ACE) gene with peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a potentially life-threatening pregnancy-related heart disease. Genetic roles such as gene polymorphisms may relate to the etiology of PPCM. This study analyzes the association between single nucleotide gene polymorphism (SNP) guanine nucleotide-binding protein beta-3 subunit (GNB3) C825T and insertion/deletion (I/D) of the angiotensin-converting enzyme (ACE) gene with the incidence of PPCM. An analytic observational study with a case-control design was conducted at the Integrated Cardiac Service Center of Dr. Soetomo General Hospital, Surabaya, Indonesia. PPCM patients of the case and control groups were enrolled. Baseline characteristic data were collected and blood samples were analyzed for SNP in the GNB3 C825T gene and for I/D in the ACE gene by using the polymerase chain reaction, restriction fragment length polymorphism, and Sanger sequencing. We also assessed ACE levels among different ACE genotypes using a sandwich-ELISA test. A total of 100 patients were included in this study, with 34 PPCM cases and 66 controls. There were significant differences in GNB3 TT and TC genotypes in the case group compared with that in the control group (TT: 35.3% vs. 10.6%, p = 0.003; TC: 41.2% vs. 62.5%, p = 0.022). The TT genotype increased the risk of PPCM by 4.6-fold. There was also a significant difference in the ACE DD genotype in the case group compared with that in the control group (26.5% vs. 9.1%, p = 0.021). DD genotypes increased the risk of PPCM by 3.6-fold. ACE levels were significantly higher in the DD genotype group than in the ID and II genotype groups (4,356.88 ± 232.44 pg/mL vs. 3,980.91 ± 77.79 pg/mL vs. 3,679.94 ± 325.77 pg/mL, p < 0.001). The TT genotype of GNB3 and the DD genotype of the ACE are likely to increase the risk of PPCM. Therefore, these polymorphisms may be predisposing risk factors for PPCM incidence. ACE levels were significantly higher in the DD genotype group, which certainly had clinical implications for the management of PPCM patients in the administration of ACE inhibitors as one of the therapy options.

Prevalence of HSPB6 gene variants in peripartum cardiomyopathy: Data from the German PPCM registry

BackgroundHeat shock protein family B (small) member 6 (HSPB6) mediates cardioprotective effects against stress-induced injury. In humans two gene variants of HSPB6 have been identified with a prevalence of 1% in patients with dilated cardiomyopathy (DCM). Peripartum cardiomyopathy (PPCM) is a potentially life-threatening heart disease of unknown etiology in previously healthy women of whom 16–20% of PPCM carry gene variants associated with cardiomyopathy. This study was designed to analyze the prevalence of pathogenic HSPB6 gene variants in PPCM. Methods and resultsWhole-exome sequencing was performed in whole blood samples of PPCM patients (n = 65 PPCM patients from the German PPCM registry) and screened subsequently for HSPB6 gene variants. In this PPCM cohort one PPCM patient carries a HSPB6 gene variant of uncertain significance (VUS), which was not associated with changes in the amino acid sequence and no likely pathogenic or pathogenic variants were detected. ConclusionHSPB6 gene variants did not occur more frequently in a cohort of PPCM patients from the German PPCM registry, compared to DCM patients. Genetic analyses in larger cohorts and in cohorts of different ethiologies of PPCM patients are needed to address the role of the genetic background in the pathogenesis of PPCM.

A Rare Case of Peripartum Cardiomyopathy Followed Up for Intracardiac Thrombus and Sudden Embolism of Right Coronary Artery: A Case Report and Review of the Literature

Peripartum cardiomyopathy (PPCM) is a rare disease with high mortality in which dilated heart failure occurs in the last month of pregnancy or within 5 months after delivery, without any other etiology or pre-existing heart disease. Sometimes, it may present with intracardiac thrombus and associated life-threatening embolic complications. Here, we will present the management of a 28-year-old patient who was diagnosed with PPCM and intracardiac thrombus immediately after delivery and was followed up in the intensive care unit, in which an inferior myocardial infarction was detected in the electrocardiogram taken for sudden onset chest pain. Although peripheral thromboembolism is occasionally detected in PPCM patients with intracardiac thrombus, coronary embolisms is not common. Presentation with coronary embolism in patients with PPCM has been described in only 4 cases in the literature, but to the best of our knowledge, this is the first case to describe embolism to the right coronary artery.

The outcome of peripartum cardiomyopathy patients-single center experience.

Peripartum cardiomyopathy (PPCM) diagnosis made by excluding identifiable causes of heart failure (HF) and occurs end of the pregnancy or during the postpartum period of five months. It presents a clinical HF spectrum with left ventricular systolic dysfunction. The purpose of this study is to retrospectively evaluate the clinical characteristics, cardiac magnetic resonance (CMR) imaging features, and end-points consisting of left ventricle recovery, left ventricular assist device implantation, heart transplantation, and all-cause mortality. Outpatient HF records between 2008 to 2021 were screened. Thirty-seven patients were defined as PPCM. Twenty-five patients had CMR evaluation at the time of diagnosis, and six patients were re-evaluated with CMR. The mean age was 30.5±5.6 years, and the mean LVEF was 28.2%±6.7%. In 13(35.7%) patients, LVEF recovered during the follow-up course. The median recovery time was 281(IQR [78-358]) days. LVEF on CMR was 35.3±10.5, and three patients exhibited late gadolinium enhancement(LGE) patterns. Sub-endocardial and mid-wall uptake pattern types were detected. 18(75%) patients met the Petersen left ventricle non-compaction cardiomyopathy(LVNC) criteria. Patients with NC/C ratio lower than 2.3 had lower LVEDVi and LVESVi (124.9±35.4, 86.4±7.5, p=.003; 86.8±34.6, 52.6±7.6, p=.006), respectively. The median follow-up time was 2129 (IQR [911-2634]) days. The primary endpoint-free 1-year survival was 88.9% (event rate 11.1%), and 5-year survival was 75.7% (event rate 24.3%). In a retrospective cohort of PPCM patients, 35.7% of patients' LVEF recovered, and the primary end-point of free-5-year survival was 75%. Twenty-five patients were assessed with CMR; three of four met the Petersen CMR-derived LVNC at initial evaluation.

Abstract 15353: Identifying Different Clinical Phenotypes in Peripartum Cardiomyopathy by Latent Class Analysis

Introduction: Peripartum cardiomyopathy (PPCM) is a heterogeneous disorder whose pathophysiology remains poorly understood. To better understand the heterogeneity of PPCM, we used latent class analysis (LCA) to explore the different clinical phenotypes in a large sample of PPCM patients. Methods: Using the National Inpatient Sample (NIS) database, we identified 917 patients admitted to hospitals in 2017-2019 with a primary diagnosis of PPCM. We performed LCA based on 8 risk factors: age (&lt; 30 vs &gt; 30), race (African American (AA) vs non-AA), hypertension in pregnancy (HIP) (preeclampsia, gestational hypertension, or chronic hypertension), diabetes mellitus (DM), obesity, tobacco use, cannabis use, and psychiatric disorders (anxiety, depressive, bipolar, or schizophrenia spectrum disorders). We then compared in-hospital outcomes among the different clusters identified by LCA. Data were analyzed using Stata version 17 for descriptive analysis and Mplus version 8.8 for LCA. Results: Four patient clusters were identified. C1 (n=471, 51.4%) had no AA or cannabis users and members had the lowest probability of having HIP (0.387), DM (0.27), and obesity (0.17). C2 (n=328, 35.8%) were all AA who did not use cannabis and had the lowest probability of using tobacco (0.076), and having psychiatric disorders (0.084). C3 (n=31, 3.4%) was the youngest group all of whom used tobacco and cannabis and had the largest probability of having psychiatric disorders (0.484). On the other hand, C4 (n=87, 9.5%) was the oldest group none of whom used cannabis but members had the highest probability being obese (0.664), having HIP (0.825), and DM (0.642). Among all clusters, C3 had the lowest in-hospital mortality (0.0%) and hospital charges ($57,357). C4 had the highest in-hospital mortality 1.1% and longest hospital length of stay (5.4 ± 7.0 days). Conclusions: Using LCA, we identified four clinically distinct classes PPCM with varying in-hospital outcomes. These may reflect different additional underlying mechanisms in PPCM. Our findings may help identify treatment targets and patient selection for future clinical trials.

Peripartum Cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a rare but potentially life-threatening heart disease, with onset in the last month of pregnancy or in the first months after delivery in previously heart-healthy women. PPCM patients typically present with heart failure due to left ventricular (LV) dysfunction with an LV ejection fraction (EF) < 45 %. In the last years clinical and experimental studies contributed to a better understanding of the pathophysiology and the clinical course of PPCM. In the context of oxidative stress, the nursing hormone prolactin is cleaved into a smaller antiangiogenic and proapoptotic 16k Da form, leading to myocardial dysfunction. In an animal model this can be prevented by treatment with the dopamine agonist bromocriptine, which suppresses prolactin release. This therapeutic approach was confirmed in several clinical studies. Therefore, the current guidelines recommend a treatment consisting of a heart failure treatment according to current guidelines in combination with the dopamine agonist bromocriptine. If the diagnosis is made early and the treatment is started immediately, the prognosis is good compared to other forms of cardiomyopathies, as LV function recovers in most cases.In the acute phase the severity of heart failure differs among PPCM patients. Some patients present with mild forms, whereas some PPCM patients display severely reduced LV function and cardiogenic shock. Especially the latter cases are still challenging, as treatment with β1-adrenergic receptor agonists is associated with progression of heart failure and a worse cardiac outcome. Therefore, patients with cardiogenic shock complicating PPCM should be treated in centers experienced in mechanical circulatory support in combination with bromocriptine treatment.

Cabergoline treatment promotes myocardial recovery in peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a rare heart disease, occurring in previously heart-healthy women during the last month of pregnancy or the first months after delivery due to left ventricular (LV) systolic dysfunction. A common pathomechanistic pathway of PPCM includes increased oxidative stress and the subsequent generation of a cleaved prolactin fragment (16kDa PRL), which promotes the onset of heart failure (HF) in a microRNA (miR)-146a-dependent manner. Inhibition of prolactin secretion with the dopamine D2 receptor (D2R) agonist bromocriptine combined with standard HF therapy supports cardiac recovery. This study examined whether treatment with the more selective D2R agonist cabergoline prevents HF development in an experimental PPCM mouse model and might be used as an alternative treatment regime for PPCM. Postpartum (PP) female PPCM-prone mice with a cardiomyocyte restricted STAT3-deficiency (αMHC-Cretg/+ ; Stat3fl/fl ; CKO) were treated over two consecutive nursing periods with cabergoline (CKO Cab, 0.5mg/kg/day) and were compared with bromocriptine treated CKO (CKO Br) and postpartum-matched WT and CKO mice. Cabergoline treatment in CKO PP mice preserved cardiac function [fractional shortening (FS): CKO Cab: 34.5±9.4% vs. CKO: 22.1±9%, P<0.05] and prevented the development of cardiac hypertrophy, fibrosis, and inflammation as effective as bromocriptine therapy (FS: CKO Br: 33.4±5.6%). The myocardial up-regulation of the PPCM biomarkers plasminogen inhibitor activator 1 (PAI-1) and miR-146a were prevented by both cabergoline and bromocriptine therapy. A small cohort of three PPCM patients from the German PPCM Registry was treated with cabergoline (1mg per week for 2weeks, followed by 0.5mg per week for another 6weeks) due to a temporary unavailability of bromocriptine. All PPCM patients initially presented with a severely reduced LV ejection fraction (LVEF: 26±2%). However, at 6months of follow-up, LV function (LVEF: 56±2%) fully recovered in all three PPCM patients, and no adverse events were detected. In the experimental PPCM mouse model, the selective D2R agonist cabergoline prevents the onset of postpartum HF similar to bromocriptine. In PPCM patients, cabergoline treatment was safe and effective as all patients fully recovered. Cabergoline might serve as a promising alternative to bromocriptine. However, these findings are based on experimental data and a small case series and thus have to be interpreted with caution and should be validated in a larger clinical trial.

Clinical characteristics and long-term outcomes in patients with peripartum cardiomyopathy (PPCM) receiving left ventricular assist devices (LVAD).

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure (HF), presenting with left ventricular (LV) systolic dysfunction either at the end of pregnancy or in the months following delivery. In rare cases, PPCM leads to severe impairment of LV function, refractory cardiogenic shock or advanced HF. LV assist devices (LVAD) have been shown to be a feasible treatment option in advanced HF. However, little is known about long-term outcomes and prognosis of PPCM patients undergoing LVAD implantation. A retrospective analysis of data from PPCM patients undergoing LVAD implantation in two tertiary centers with respect to long-term outcomes was performed. Twelve patients of median age 30 (18-39) years were included. Eight patients were experiencing cardiogenic shock (INTERMACS 1) at implantation. Seven patients were implanted within 1 month of their PPCM diagnosis. Median duration of LVAD support was 19 (2-92) months with median follow up of 67 (18-136) months (100% complete). In-hospital and 1-year mortality were 0% and 8.3%, respectively. Two patients died on LVAD support, four patients were successfully bridged to transplantation, two patients are still on LVAD, and four were successfully weaned due to sufficient LV recovery (one died after LV function deteriorated again). LVAD treatment of decompensated end-stage PPCM is feasible. Early LVAD provision led to hemodynamic stabilization in our cohort and facilitated safe LV recovery in one third of these young female patients.

Risk factors for intracardiac thrombus in peripartum cardiomyopathy: a retrospective study in China.

Peripartum cardiomyopathy (PPCM) are more vulnerable to intracardiac thrombus than other types of cardiomyopathies, although explicit anticoagulant strategy is not sure. Too aggressive anticoagulation therapy can lead to severe bleeding events. Hence, we want to construct a risk stratification model for intracardiac thrombus in PPCM patients. A total of 159 suspected PPCM cases were initially screened, whereas 123 confirmed cases were enrolled in the final analysis. The study population was randomly assigned as derivation group (N=83) and validation group (N=40). The derivation cohort was utilized to develop the model, and the validation cohort was used to internal validate the discriminatory ability of the model. Formation of intracardiac thrombus was detected in 22 patients. After adjusted by multivariable logistic regression analysis, left ventricle ejection fraction (LVEF, OR 0.772, 95% CI 0.665-0.897, P=0.001), haemoglobin levels (OR 1.050, 95% CI 1.003-1.099, P=0.038), and thrombocyte counts (OR 1.018, 95% CI 1.006-1.029, P=0.003) were identified as risk factors independently associated with intracardiac thrombus and were finally included in the tentative risk stratification model with a C-indexes of 0.916 (95% CI: 0.850-0.982, P<0.001). A score of ≤7 was regarded as low risk, 8-10 defined intermediate risk, and ≥11 defined high risk in our model. Internal validation showed good discriminatory ability of the model with a C-indexes of 0.790 (95% CI: 0.644-0.936, P=0.017). In our retrospective study, impaired LVEF, elevated haemoglobin levels, and high thrombocyte counts were regarded as independent risk factors for intracardiac thrombus in PPCM. A risk stratification model derived from these risk factors, which was economic and easily applicable in clinical practice, could rapidly and accurately identify PPCM patients with higher-risk of intracardiac thrombus.