scholarly journals Clinical presentation, management, and 6-month outcomes in women with peripartum cardiomyopathy: an ESC EORP registry

2020 ◽  
Vol 41 (39) ◽  
pp. 3787-3797 ◽  
Author(s):  
Karen Sliwa ◽  
Mark C Petrie ◽  
Peter van der Meer ◽  
Alexandre Mebazaa ◽  
Denise Hilfiker-Kleiner ◽  
...  

Abstract Aims  We sought to describe the clinical presentation, management, and 6-month outcomes in women with peripartum cardiomyopathy (PPCM) globally. Methods and results  In 2011, >100 national and affiliated member cardiac societies of the European Society of Cardiology (ESC) were contacted to contribute to a global registry on PPCM, under the auspices of the ESC EURObservational Research Programme. These societies were tasked with identifying centres who could participate in this registry. In low-income countries, e.g. Mozambique or Burkina Faso, where there are no national societies due to a shortage of cardiologists, we identified potential participants through abstracts and publications and encouraged participation into the study. Seven hundred and thirty-nine women were enrolled in 49 countries in Europe (33%), Africa (29%), Asia-Pacific (15%), and the Middle East (22%). Mean age was 31 ± 6 years, mean left ventricular ejection fraction (LVEF) was 31 ± 10%, and 10% had a previous pregnancy complicated by PPCM. Symptom-onset occurred most often within 1 month of delivery (44%). At diagnosis, 67% of patients had severe (NYHA III/IV) symptoms and 67% had a LVEF ≤35%. Fifteen percent received bromocriptine with significant regional variation (Europe 15%, Africa 26%, Asia-Pacific 8%, the Middle East 4%, P < 0.001). Follow-up was available for 598 (81%) women. Six-month mortality was 6% overall, lowest in Europe (4%), and highest in the Middle East (10%). Most deaths were due to heart failure (42%) or sudden (30%). Re-admission for any reason occurred in 10% (with just over half of these for heart failure) and thromboembolic events in 7%. Myocardial recovery (LVEF > 50%) occurred only in 46%, most commonly in Asia-Pacific (62%), and least commonly in the Middle East (25%). Neonatal death occurred in 5% with marked regional variation (Europe 2%, the Middle East 9%). Conclusion  Peripartum cardiomyopathy is a global disease, but clinical presentation and outcomes vary by region. Just under half of women experience myocardial recovery. Peripartum cardiomyopathy is a disease with substantial maternal and neonatal morbidity and mortality.

Author(s):  
Rahul Goli ◽  
Jian Li ◽  
Jeff Brandimarto ◽  
Lisa D. Levine ◽  
Valerie Riis ◽  
...  

Background: Peripartum cardiomyopathy (PPCM) occurs in approximately 1:2000 deliveries in the US and worldwide. The genetic underpinnings of PPCM remain poorly defined. Approximately 10% of women with PPCM harbor truncating variants in TTN (TTNtvs). Whether mutations in other genes can predispose to PPCM is not known. It is also not known if the presence of TTNtvs predicts clinical presentation or outcomes. Nor is it known if the prevalence of TTNtvs differs in women with PPCM and preeclampsia, the strongest risk factor for PPCM. Methods: Women with PPCM were retrospectively identified from several US and international academic centers, and clinical information and DNA samples were acquired. Next-generation sequencing was performed on 67 genes, including TTN , and evaluated for burden of truncating and missense variants. The impact of TTNtvs on severity of clinical presentation, and on clinical outcomes, was evaluated. Results: 469 women met inclusion criteria. 10.4% of women with PPCM bore TTNtvs (Odds ration [OR]=9.4 compared with 1.2% in reference population; Bonferroni-corrected P [P*] =1.2x10 -46 ). We additionally identified overrepresentation of truncating variants in FLNC (OR=24.8, P*=7.0x10 -8 ), DSP (OR=14.9, P*=1.0x10-8), and BAG3 (OR=53.1, P*=0.02), genes not previously associated with PPCM. This profile is highly similar to that found in non-ischemic dilated cardiomyopathy (DCM). Women with TTNtvs had lower left ventricular ejection fraction (LVEF) on presentation than did women without TTNtvs (23.5% vs 29%, P=2.5x10 -4 ), but did not differ significantly in timing of presentation after delivery, in prevalence of preeclampsia, or in rates of clinical recovery. Conclusions: This study provides the first extensive genetic and phenotypic landscape of PPCM, and demonstrates that predisposition to heart failure is an important risk factor for PPCM. The work reveals a degree of genetic similarity between PPCM and DCM, suggesting that gene-specific therapeutic approaches being developed for DCM may also apply to PPCM, and that approaches to genetic testing in PPCM should mirror those taken in DCM. Finally, the clarification of genotype/phenotype associations has important implications for genetic counseling.


Author(s):  
Kris Kawamoto ◽  
Elizabeth Langen ◽  
Elizabeth A Jackson ◽  
Melinda Davis

Background: Women diagnosed with peripartum cardiomyopathy (PPCM) are frequently advised not to breastfeed their infants because of concern that the nursing hormone prolactin drives the pathogenesis of PPCM. However, this remains controversial. We sought to determine whether women with PPCM who breastfed had lower rates of recovery. Methods: Patients with PPCM at the University of Michigan (2000-2011) were retrospectively reviewed. Demographics, clinical data, and outcomes were collected. Questionnaires requesting information about breastfeeding status and duration were mailed to women with PPCM. This data was correlated with recovery status, defined as left ventricular ejection fraction ≥ 50% at 1 year after diagnosis. Results: Of 27 women with PPCM with known breastfeeding status, 13 women breastfed (48%) and 14 did not (52%). Among women who breastfed, 8 recovered (62%) and 5 did not (38%). Among women who did not breastfeed, 11 recovered (79%) and 3 did not (21%). This was not statistically different (p=0.29). There were no significant differences between breastfeeding and non-breastfeeding women related to their mean age or treatment with heart failure medications. Of the 16 patients who completed additional survey questionnaires, 11 (69%) reported their physician had instructed them not to breastfeed. Conclusions: Contrary to some expert opinions, breastfeeding was not associated with lack of recovery. While larger prospective studies are needed to clarify this conclusion, it appears that a diagnosis of PPCM should not be an absolute contraindication to breastfeeding.


Medicina ◽  
2019 ◽  
Vol 55 (6) ◽  
pp. 246
Author(s):  
Elizabeth Richard ◽  
Pierre Yves Turgeon ◽  
Michelle Dubois ◽  
Mario Sénéchal

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure that develops during the last month of pregnancy or within first months of delivery. We report the case of a 40-year-old woman diagnosed with severely symptomatic PPCM characterized by left ventricular ejection fraction (LVEF) of 10% and significant dyssynchrony secondary to a left bundle branch block (LBBB). Early cardiac resynchronization therapy (CRT) was used to achieve remarkable functional and LVEF recovery. This case suggests that early CRT must be considered for patients suffering from severely symptomatic PPCM despite optimal medical therapy for whom advanced heart failure therapies are proposed.


2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
D L Villanueva ◽  
M C Villanueva ◽  
E J Llanes

Abstract Background Peripartum cardiomyopathy is a rare, pregnancy associated cause of left ventricular heart failure in previously healthy women. It remains an important cause of cardiac-related maternal morbidity and mortality worldwide. Half of the patients will recover left ventricular function after 6 months. However, in the remainder of patients who do not recover cardiac function, they will require advanced heart failure therapies. Bromocriptine, a dopamine agonist which inhibits prolactin release, has demonstrated improvement in left ventricular recovery and clinical outcome. We sought to determine the effect of adding Bromocriptine to standard heart failure therapy on the improvement and recovery of left ventricular function of these patients. Inclusion Criteria. Studies were included if they satisfied the following criteria:1) Randomized Controlled Trials; 2) Pregnant patients who fulfilled the criteria for diagnosis of peripartum cardiomyopathy and 3) Reported data on improvement in left ventricular ejection fraction and clinical outcomes. Methods   Using PUBMED, Clinical Key, Science Direct, Scopus, and Cochrane databases, a search for eligible studies was conducted from June to December 31, 2018. The quality of each study was evaluated using the Cochrane Risk of Bias Tool. The primary outcome of interest is on the effect of Bromocriptine on the improvement of left ventricular function and clinical outcomes among these patients. Review Manager 5.3 was utilized to perform analysis of random effects for continuous outcomes. Results We identified 2 randomized controlled trials of 58 pregnant patients diagnosed with peripartum cardiomyopathy, showing that among those who received Bromocriptine on top of standard heart failure therapy, there is a significant improvement in the left ventricular ejection fraction at 6 months [mean difference 15.14 (95% CI, 6.53 to 23.75) p <0.05] compared to standard heart failure therapy alone. It was also observed that those who received Bromocriptine had better clinical outcomes. Conclusion The addition of Bromocriptine on top of standard heart failure therapy significantly improved the left ventricular ejection fraction of patients with peripartum cardiomyopathy at 6 months post-partum. This novel therapy may be considered to improve the management of these patients.


2021 ◽  
Vol 4 (1) ◽  
pp. 55-62
Author(s):  
Rafidya Indah Septica ◽  
Isngadi Isngadi

Kardiomiopati peripartum (KMPP) atau Peripartum cardiomyopathy (PPCM) adalah kelainan jantung idiopatik dengan karakteristik disfungsi sistolik dan simptom gagal jantung pada akhir masa kehamilan atau beberapa bulan setelah kehamilan tanpa sebab lain yang mengancam jiwa maternal dengan risiko morbiditas dan mortalitas postpartum cukup tinggi. Penelitian terbaru dalam pemahaman tentang patofisiologi PPCM menunjukkan proses yang melibatkan faktor endotel dan faktor toksik kardio, seperti sFlt-1 dan 16 kDa prolaktin, sehingga kemampuan jantung beradaptasi terhadap kehamilan normal terlampaui pada ibu yang sudah rentan terhadap serangan jantung. Terapi spesifik PPCM belum dapat ditentukan. Bromokriptin yang bekerja memblok pelepasan prolaktin dari glandula pituitaria, pada beberapa penelitian awal menghasilkan perbaikan fraksi ejeksi ventrikel kiri secara bermakna. Penelitian lebih lanjut dengan jumlah sampel yang lebih besar masih harus dilakukan untuk terapi ini. Prinsip manajemen direkomendasikan sesuai dengan patofisiologi yang terjadi. Optimalisasi atau reduksi preload baik dengan reduksi natrium maupun cairan dan penggunaan diuretika, menurunkan afterload dengan vasodilator, dan memperbaiki kontraktilitas jantung dengan inotropik, dromotropik, atau inodilator adalah strategi utama yang direkomendasikan. Tidak ada perubahan strategi dalam manajemen terapi ini, tetapi pilihan teknik anestesi saat ini lebih berkembang ke analgesi/anestesi regional. Pemahaman penggunaan dosis dan konsentrasi anestetika lokal menjadi penting untuk mencapai target dalam strategi yang direkomendasikan.   Peripartum Cardiomyopathy: Update in Anesthesia Management Abstract Peripatum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure secondary to left ventricle systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause for heart failure is identified, life-threatening, and postpartum high morbidity and mortality risk. Recent studies in the understanding of PPCM pathophysiology indicate that there’s processes involving endothelial and cardio-toxic factors such as e.g. sFlt-1 and 16 kDa prolactin, leading the heart’s capacity to adapt to a normal pregnancy may be exceeded in some women already susceptible to cardiac insult. Spesific therapy for PPCM can not be determined. Bromocriptine that blocks the release of a hormone called prolactine from the pituitary gland in some preliminary studies improved left ventricular ejection fraction significantly. Further research with larger sample size remains to be done for this therapy. Management principles for PPCM are recommended in accordance with the pathophysiology. Depending on the volume status, preload has to be optimized by either fluid administration or sodium restriction and diuretics, decrease afterload using vasodilator, and improve contractility by using inotropic, dromotropic, or inodilator are the main strategies. There is no change in management strategy for PPCM, but regional analgesia/anesthesia preferably for now. Understanding the dose and concentration administration of local anesthethic drugs are important to achieve targets recommendation.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J.P Sousa ◽  
L Puga ◽  
J Lopes ◽  
C Saleiro ◽  
R Gomes ◽  
...  

Abstract Background Peripartum cardiomyopathy (PPCM) is a rare but serious condition that affects childbearing women. Dopamine agonists (DAs) may represent a specific therapy, potentially facilitating left ventricular recovery, through inhibition of prolactin secretion. However, their therapeutic value in this setting has not been fully demonstrated. Purpose To perform a meta-analysis aimed at evaluating the extent to which DAs are able to interfere with the natural history of PPCM. Methods We systematically searched MEDLINE, Embase, Web of Science, Cochrane Library, Google Scholar, Scopus and DARE for both randomized controlled trials (RCTs) and observational studies addressing the impact of DAs on main outcomes of PPCM patients, published up until February 1, 2020. Endpoints were those of mortality, recovery from heart failure and, likewise, the degree to which left ventricular ejection fraction (LVEF) was restored. All analyses were conducted under a DA plus optimized medical therapy (OMT) vs. OMT alone design, while results were pooled using traditional meta-analytic techniques, under a random-effects model. Odds ratios (ORs) were computed for the first two outcomes, whereas mean difference (MD) was calculated to quantify LVEF restoration. Results 2 RCTs, 2 prospective cohort, 1 prospective case-control and 2 retrospective cohort studies, encompassing 452 patients, were regarded as eligible for quantitative evaluation. 180 patients were allocated to the DA arm, which was mostly represented by bromocriptine; in fact, only 1 study, including 24 patients, specified cabergolin utilization. Overall, 5 papers including 295 patients reported 42 deaths, whereas 5 papers comprising 305 patients detailed 220 heart failure recoveries, thus unveiling that LVEF restoration was the norm. The addition of a DA to OMT provided no signal of a survival benefit (OR 0.71, 95% CI 0.27–1.87, p=0.49, i2=27%). On the other hand, the incorporation of a DA into the therapeutic regimen narrowly missed significance for the heart failure recovery endpoint (OR 2.68, 95% CI 0.98–7.31, p=0.05, i2=56%). Furthermore, DAs were demonstrated to incrementally improve LVEF by 15% (MD 15.00, 95% CI 10.24–19.76, p<0.00001, i2=77%). Adverse events, including thromboembolic ones, were rare, though adjunct anticoagulation was broadly reported. Conclusion In PPCM patients, the addition of a DA to OMT seems to be both effective at incrementally improving LVEF and safe, even though not reaching survival benefit status. These findings appear to corroborate the so-called prolactin hypothesis for PPCM pathophysiology. Funding Acknowledgement Type of funding source: None


2018 ◽  
Author(s):  
Kendra M Gray ◽  
Michael R Foley

Peripartum cardiomyopathy (PPCM) is a serious and rare disease of late pregnancy or the early postpartum period. It is defined as idiopathic, nonfamilial, nongenetic, heart failure occurring in the absence of any other identifiable causes of heart disease within the last month of pregnancy or within the first 5 months postdelivery in otherwise previously healthy woman. The incidence in the United States is 1 per 3,000 to 4,000 live births. Left ventricular systolic dysfunction develops, almost always leading to a left ventricular ejection fraction of less than 45%. PPCM is unique in its rapid medical course and propensity to spontaneously resolve within 3 to 6 months of disease onset. The mortality rate is high, up to 10%, and the risk of relapse in subsequent pregnancies is also elevated. Treatment for PPCM varies slightly based on whether the woman is pregnant or postpartum. Conventional pharmacologic treatment includes diuretics, angiotensin-converting enzyme inhibitors (postpartum only), vasodilators such as hydralazine, digoxin, β-blockers, and anticoagulants. This review contains 5 figures, 5 tables, and 36 references. Key Words: critical care obstetrics, ejection fraction, heart failure, left ventricular systolic dysfunction, management, maternal mortality, peripartum cardiomyopathy, preeclampsia, pregnancy


2020 ◽  
Vol 90 (1-2) ◽  
pp. 49-58 ◽  
Author(s):  
Wang Chunbin ◽  
Wang Han ◽  
Cai Lin

Abstract. Vitamin D deficiency commonly occurs in chronic heart failure. Whether additional vitamin D supplementation can be beneficial to adults with chronic heart failure remains unclear. We conducted a meta-analysis to derive a more precise estimation. PubMed, Embase, and Cochrane databases were searched on September 8, 2016. Seven randomized controlled trials that investigated the effects of vitamin D on cardiovascular outcomes in adults with chronic heart failure, and comprised 592 patients, were included in the analysis. Compared to placebo, vitamin D, at doses ranging from 2,000 IU/day to 50,000 IU/week, could not improve left ventricular ejection fraction (Weighted mean difference, WMD = 3.31, 95% confidence interval, CL = −0.93 to 7.55, P < 0.001, I2 = 92.1%); it also exerts no beneficial effects on the 6 minute walk distance (WMD = 18.84, 95% CL = −24.85 to 62.52, P = 0.276, I2 = 22.4%) and natriuretic peptide (Standardized mean difference, SMD = −0.39, 95% confidence interval CL = −0.48 to 0.69, P < 0.001, I2 = 92.4%). However, a dose-response analysis from two studies demonstrated an improved left ventricular ejection fraction with vitamin D at a dose of 4,000 IU/day (WMD = 6.58, 95% confidence interval CL = −4.04 to 9.13, P = 0.134, I2 = 55.4%). The results showed that high dose vitamin D treatment could potentially benefit adults with chronic heart failure, but more randomized controlled trials are required to confirm this result.


2011 ◽  
Vol 7 (1) ◽  
pp. 29
Author(s):  
Charlotte Eitel ◽  
Gerhard Hindricks ◽  
Christopher Piorkowski ◽  
◽  
◽  
...  

Cardiac resynchronisation therapy (CRT) is an efficacious and cost-effective therapy in patients with highly symptomatic systolic heart failure and delayed ventricular conduction. Current guidelines recommend CRT as a class I indication for patients with sinus rhythm, New York Heart Association (NYHA) functional class III or ambulatory class IV, a QRS duration ≥120ms, and left ventricular ejection fraction (LVEF) ≤35%, despite optimal pharmacological therapy. Recent trials resulted in an extension of current recommendations to patients with mild heart failure, patients with atrial fibrillation, and patients with an indication for permanent right ventricular pacing with the aim of morbidity reduction. The effectiveness of CRT in patients with narrow QRS, patients with end-stage heart failure and cardiogenic shock, and patients with an LVEF >35% still needs to be proved. This article reviews current evidence and clinical applications of CRT in heart failure and provides an outlook on future developments.


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