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 2016 May;4(5):380-8. doi: 10.1016/j.jchf.2016.01.004. Epub 2016 Mar 9.

Relaxin-2 and Soluble Flt1 Levels in Peripartum Cardiomyopathy: Results of the Multicenter IPAC Study.

Abstract

OBJECTIVES: 

This study explored the association of vascular hormones with myocardial recovery and clinical outcomes in peripartum cardiomyopathy (PPCM).

BACKGROUND: 

PPCM is an uncommon disorder with unknown etiology. Angiogenic imbalance may contribute to its pathophysiology.

METHODS: 

In 98 women with newly diagnosed PPCM enrolled in the Investigation in Pregnancy Associated Cardiomyopathy study, serum was obtained at baseline for analysis of relaxin-2, prolactin, soluble fms-like tyrosine kinase 1 (sFlt1), and vascular endothelial growth factor (VEGF). Left ventricular ejection fraction (LVEF) was assessed by echocardiography at baseline and 2, 6, and 12 months.

RESULTS: 

Mean age was 30 ± 6 years, with a baseline of LVEF 0.35 ± 0.09. Relaxin-2, prolactin, and sFlt1 were elevated in women presenting early post-partum, but decreased rapidly and were correlated inversely with time from delivery to presentation. In tertile analysis, higher relaxin-2 was associated with smaller left ventricular systolic diameter (p = 0.006) and higher LVEF at 2 months (p = 0.01). This was particularly evident in women presenting soon after delivery (p = 0.02). No relationship was evident for myocardial recovery and prolactin, sFlt1 or VEGF levels. sFlt1 levels were higher in women with higher New York Heart Association functional class (p = 0.01) and adverse clinical events (p = 0.004).

CONCLUSIONS: 

In women with newly diagnosed PPCM, higher relaxin-2 levels soon after delivery were associated with myocardial recovery at 2 months. In contrast, higher sFlt1 levels correlated with more severe symptoms and major adverse clinical events. Vascular mediators may contribute to the development of PPCM and influence subsequent myocardial recovery. (Investigation in Pregnancy Associate Cardiomyopathy [IPAC]; NCT01085955).

Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

KEYWORDS: 

cardiomyopathy; heart failure; hormones; pregnancy and post-partum 2016 Mar;9(3):e002683. doi: 10.1161/CIRCHEARTFAILURE.115.002683.

GNB3 C825T Polymorphism and Myocardial Recovery in Peripartum Cardiomyopathy: Results of the Multicenter Investigations of Pregnancy-Associated Cardiomyopathy Study.

Abstract

BACKGROUND: 

Black women are at greater risk for peripartum cardiomyopathy (PPCM). The guanine nucleotide-binding proteins β-3 subunit (GNB3) has a polymorphism C825T. The GNB3 TT genotype more prevalent in blacks is associated with poorer outcomes. We evaluated GNB3 genotype and myocardial recovery in PPCM.

METHODS AND RESULTS: 

A total of 97 women with PPCM were enrolled and genotyped for the GNB3 T/C polymorphism. Left ventricular ejection fraction (LVEF) was assessed by echocardiography at entry, 6 and 12 months postpartum. LVEF over time in subjects with the GNB3 TT genotype was compared with those with the C allele overall and in black and white subsets. The cohort was 30% black, age 30+6, LVEF 0.34+0.10 at entry 31+25 days postpartum. The % GNB3 genotype for TT/CT/CC=23/41/36 and differed markedly by race (blacks=52/38/10 versus whites=10/44/46, P<0.001). In subjects with the TT genotype, LVEF at entry was lower (TT=0.31+0.09; CT+CC=0.35+0.09, P=0.054) and this difference increased at 6 (TT=0.45+0.15; CT+CC=0.53+0.08, P=0.002) and 12 months (TT=0.45+0.15; CT+CC=0.56+0.07, P<0.001.). The difference in LVEF at 12 months by genotype was most pronounced in blacks (12 months LVEF for GNB3 TT=0.39+0.16; versus CT+CC=0.53+0.09, P=0.02) but evident in whites (TT=0.50++0.11; CT+CC=0.56+0.06, P=0.04).

CONCLUSIONS: 

The GNB3 TT genotype was associated with lower LVEF at 6 and 12 months in women with PPCM, and this was particularly evident in blacks. Racial differences in the prevalence and impact of GNB3 TT may contribute to poorer outcomes in black women with PPCM.

© 2016 American Heart Association, Inc.

KEYWORDS: 

cardiomyopathy; heart failure; peripartum period; polymorphism genetics; pregnancy 2016 Jan 21;374(3):233-41. doi: 10.1056/NEJMoa1505517. Epub 2016 Jan 6.

Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

Abstract

Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. 

Methods In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. 

Results We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P=1.3×10(-7)) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P=0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P=2.7×10(-10)); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P=0.005). 

Conclusions The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder. 



Clinical Outcomes for

Peripartum Cardiomyopathy

in North America

VOL. 66, NO. 8, 2015 ISSN 0735-1097/$36.00 

Results of the IPAC Study
(Investigations of Pregnancy-Associated Cardiomyopathy)

DenniEs M. McNamara, MD, MS,* Uri Elkayam, MD,y Rami Alharethi, MD,z Julie Damp, MD,xileen Hsich, MD,k Gregory Ewald, MD,{ Kalgi Modi, MD,# Jeffrey D. Alexis, MD,** Gautam V. Ramani, MD,yy Marc J. Semigran, MD,zz Jennifer Haythe, MD,xx David W. Markham, MD,kk Josef Marek, MD,* John Gorcsan III, MD,* Wen-Chi Wu, PHD,{{ Yan Lin, PHD,{{ Indrani Halder, PHD,## Jessica Pisarcik, BSN,* Leslie T. Cooper, MD,*** James D. Fett, MD,*

for the IPAC Investigators

ABSTRACT

BACKGROUND Peripartum cardiomyopathy (PPCM) remains a major cause of maternal morbidity and mortality. 

OBJECTIVES This study sought to prospectively evaluate recovery of the left ventricular ejection fraction (LVEF) and clinical outcomes in the multicenter IPAC (Investigations of Pregnancy Associated Cardiomyopathy) study.

METHODS We enrolled and followed 100 women with PPCM through 1 year post-partum. The LVEF was assessed
by echocardiography at baseline and at 2, 6, and 12 months post-partum. Survival free from major cardiovascular events (death, transplantation, or left ventricular [LV] assist device) was determined. Predictors of outcome, particularly race, parameters of LV dysfunction (LVEF), and remodeling (left ventricular end-diastolic diameter [LVEDD]) at pre- sentation, were assessed by univariate and multivariate analyses.

RESULTS The cohort was 30% black, 65% white, 5% other; the mean patient age was 30 ` 6 years; and 88% were receiving beta-blockers and 81% angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. The LVEF at study entry was 0.35 ` 0.10, 0.51 ` 0.11 at 6 months, and 0.53 ` 0.10 at 12 months. By 1 year, 13% had experienced major events or had persistent severe cardiomyopathy with an LVEF <0.35, and 72% achieved an LVEF $0.50. An initial LVEF <0.30 (p 1⁄4 0.001), an LVEDD $6.0 cm (p < 0.001), black race (p 1⁄4 0.001), and presentation after 6 weeks post- partum (p 1⁄4 0.02) were associated with a lower LVEF at 12 months. No subjects with both a baseline LVEF <0.30 and an LVEDD $6.0 cm recovered by 1 year post-partum, whereas 91% with both a baseline LVEF $0.30 and an LVEDD <6.0 cm recovered (p < 0.00001).

CONCLUSIONS In a prospective cohort with PPCM, most women recovered; however, 13% had major events or persistent severe cardiomyopathy. Black women had more LV dysfunction at presentation and at 6 and 12 months post-partum. Severe LV dysfunction and greater remodeling at study entry were associated with less recovery. (Investigations of Pregnancy Associated Cardiomyopathy [IPAC]; NCT01085955) (J Am Coll Cardiol 2015;66:905–14) © 2015 by the American College of Cardiology Foundation. 


 2015 Jan;17(1):354. doi: 10.1007/s11936-014-0354-x.

Why do some recovered peripartum cardiomyopathy mothers experience heart failure with a subsequent pregnancy?

Author information

  • 1Investigations of Pregnancy Associated Cardiomyopathy (IPAC), Peripartum Cardiomyopathy Network of North America (PCN), Cardiovascular Institute, University of Pittsburgh Medical Center, Pittsburgh, PA, USA, [email protected]

Abstract

OPINION STATEMENT: 

After concerns about survival and recovery from peripartum cardiomyopathy (PPCM), the question commonly asked is, "Is it safe to have another pregnancy?" While important advances have been made in the past decade in the recognition and treatment of PPCM, we still do not know why some apparently recovered PPCM mothers have a relapse of heart failure in a subsequent pregnancy. Knowing that some risk for relapse is always present, careful monitoring of the post-PPCM pregnancy is currently the best way to enable earlier diagnosis with institution of effective evidence-based treatment. In that situation it is reassuring to observe that when a subsequent pregnancy begins with recovered left ventricular systolic function to echocardiographic ejection fraction ≥0.50, even with relapse, the response to treatment is good with much more favorable outcomes. On the other hand, beginning the subsequent pregnancy with echocardiographic ejection fraction <0.50 greatly increases the risk for less favorable outcomes. This article summarizes the current state of knowledge; addresses the important questions facing patients, their families, and caregivers; and identifies the need for a prospective multi-center study of women with post-PPCM pregnancies. The reality is that an estimated 10 % to 20 % of apparently recovered PPCM mothers are going to relapse in a post-PPCM pregnancy; but we do not yet know why. Nevertheless, the lowest risk for relapse is experienced by those who (1) recover to left ventricular ejection fraction 0.55 prior to another pregnancy; (2) have no deterioration of left ventricular ejection fraction after phasing out angiotensin-converting enzyme inhibitor/angiotensin-receptor blocker treatment following recovery; and perhaps, (3) demonstrate adequate contractile reserve on exercise echocardiography.



 2014 Oct 31. pii: S1050-1738(14)00197-2. doi: 10.1016/j.tcm.2014.10.019. [Epub ahead of print]

Discoveries in peripartum cardiomyopathy.

Author information

  • 1Cardiovascular Institute, University of Pittsburgh Medical Center, Pittsburgh, PA; Department of Adult Medicine, Hospital Albert Schweitzer, Deschapelles, Haiti. Electronic address: [email protected]
  • 2Division of Cardiology, Emory University, Atlanta, GA.

Abstract

The past decade has seen remarkable gains for outcomes in peripartum cardiomyopathy (PPCM), one of the leading causes of maternal mortality and morbidity in the USA and many other countries, including the high-incidence areas of Haiti and South Africa. This review article emphasizes the importance of continuing the process of increasing awareness of PPCM and presents details of this evolving picture, including important discoveries that point the way to full recovery for almost all PPCM subjects. In addition, new interventions will be highlighted, which may facilitate recovery. Numerous studies have demonstrated that when the diagnosis of PPCM is made with LVEF > 0.30, the probability is that recovery to LVEF ≥ 0.50 will occur in the overwhelming majority of subjects. PPCM patients diagnosed with severely depressed systolic function (LVEF < 0.30) and a remodeled left ventricle with greater dilatation (LVEDd ≥ 60mm) are least likely to reach the outcome recovery goals. These are the patients with the greatest need for newer interventional strategies.


 2015 Jan;17(1):354. doi: 10.1007/s11936-014-0354-x.

Why do some recovered peripartum cardiomyopathy mothers experience heart failure with a subsequent pregnancy?

Author information

  • 1Investigations of Pregnancy Associated Cardiomyopathy (IPAC), Peripartum Cardiomyopathy Network of North America (PCN), Cardiovascular Institute, University of Pittsburgh Medical Center, Pittsburgh, PA, USA, [email protected]

Abstract

OPINION STATEMENT: 

After concerns about survival and recovery from peripartum cardiomyopathy (PPCM), the question commonly asked is, "Is it safe to have another pregnancy?" While important advances have been made in the past decade in the recognition and treatment of PPCM, we still do not know why some apparently recovered PPCM mothers have a relapse of heart failure in a subsequent pregnancy. Knowing that some risk for relapse is always present, careful monitoring of the post-PPCMpregnancy is currently the best way to enable earlier diagnosis with institution of effective evidence-based treatment. In that situation it is reassuring to observe that when a subsequent pregnancy begins with recovered left ventricular systolic function to echocardiographic ejection fraction ≥0.50, even with relapse, the response to treatment is good with much more favorable outcomes. On the other hand, beginning the subsequent pregnancy with echocardiographic ejection fraction <0.50 greatly increases the risk for less favorable outcomes. This article summarizes the current state of knowledge; addresses the important questions facing patients, their families, and caregivers; and identifies the need for a prospective multi-center study of women with post-PPCM pregnancies. The reality is that an estimated 10 % to 20 % of apparently recovered PPCM mothers are going to relapse in a post-PPCM pregnancy; but we do not yet know why. Nevertheless, the lowest risk for relapse is experienced by those who (1) recover to left ventricular ejection fraction 0.55 prior to another pregnancy; (2) have no deterioration of left ventricular ejection fraction after phasing out angiotensin-converting enzyme inhibitor/angiotensin-receptor blocker treatment following recovery; and perhaps, (3) demonstrate adequate contractile reserve on exercise echocardiography.



 2014 Mar 26;6(3):87-99.

Peripartum cardiomyopathy: A puzzle closer to solution.

Abstract

Peripartum cardiomyopathy (PPCM) represents new heart failure in a previously heart-healthy peripartum patient. It is necessary to rule out all other known causes of heart failure before accepting a diagnosis of PPCM. The modern era for PPCM in the United States and beyond began with the report of the National Institutes of Health PPCM Workshop in 2000, clarifying all then-currently known aspects of the disease. Since then, hundreds of publications have appeared, an indication of how devastating this disease can be to young mothers and their families and the urgent desire to find solutions for its cause and better treatment. The purpose of this review is to highlight the important advances that have brought us nearer to the solution of this puzzle, focusing on what we have learned about PPCM since 2000; and what still remains unanswered. Despite many improvements in outcome, we still do not know the actual triggers that initiate the pathological process; but realize that cardiac angiogenic imbalances resulting from complex pregnancy-related immune system and hormonal changes play a key role.

KEYWORDS:

Heart failure, Peripartum cardiomyopathy, Pregnancy

PMID:
 
24669290
 
[PubMed - as supplied by publisher] 
PMCID:
 
PMC3964190
 
Free PMC Article

See summary of recent AHA Annual Scientific Sessions at Dallas for IPAC study findings:

Abstract 12898: Myocardial Recovery at Six Months in Peripartum Cardiomyopathy: Results of the NHLBI Multicenter IPAC study (Circulation 2013, Supplement):

Dennis McNamara1; Julie Damp2; Uri Elkayam3; Eileen Hsich4; Gregory Ewald5; Leslie Cooper6; Kalgi Modi7; Gautam Ramani8; Jeffrey Alexis9; Marc Semigran10; Mark Drazner11; Jennifer Haythe12; Jessica Pisarcik13; Josef Marek1; John Gorcsan1; James Fett14

Introduction: Peripartum cardiomyopathy (PPCM) is a rare complication of pregnancy which remains a major cause of maternal morbidity and mortality. We sought to prospectively define contemporary outcomes and predictors of recovery at 6 months post-partum in the multicenter IPAC investigation.

Method: IPAC (Investigation of Pregnancy Associated Cardiomyopathy) is an NHLBI sponsored multicenter investigation of myocardial recovery in PPCM. Women presenting with an LVEF≤0.45 within two months of delivery were enrolled at 30 centers. LVEF was assessed by transthoracic echocardiography at entry and at 6 months postpartum. Status at 6 months (alive, dead, on LVAD or transplanted) as well as the degree of recovery was compared by race and presenting LVEF.

Results: For the cohort of 100 women the mean age=30 ± 6, Gravida= 2.8 ± 1.9, Para=2.2 ± 1.3, % NYHA class 1-4=12/46/24/17, and % race= 66 white, 30 black, 4 other. Medical therapy at entry included beta blockers in 89% and ACE inhibitors in 80%. Bromocriptine was utilized in only one subject. Mean LVEF was 0.34 ± 0.11 at entry and 0.51 ± 0.11 at 6 months. Only 9% of subjects had an LVEF≤0.35 at 6 months, while 65% had an LVEF> 0.50. Major events by six months included 3 LVADs, 3 deaths, and one transplant in 5 total patients (5%). LVEF was lower at entry and 6 month in blacks compared to whites (LVEF at entry B/W=0.28±0.12/0.36±0.10, p=0.002; 6 month 0.47±0.14/ 0.53±0.08, p=0.02). Outcomes were poorer in subjects with an LVEF≤0.30 at entry (n=30) as 4 of 5 subjects with events were in this subset and the LVEF at 6 months was significantly lower (mean EF at 6 months 0.42±0.13 versus 0.54±0.08, p=0.001).

Conclusions: The majority of women with PPCM treated with standard therapy recover, however the rate of death, transplant or LVAD remains unacceptably high. Outcomes differ by race and initial LVEF. Women with severe dysfunction at presentation have the poorest outcomes and may represent a target for future interventional trials


Released 5 November 2013:   2013 Nov;9(6):809-16. 

Earlier detection can help avoid many serious complications of peripartum cardiomyopathy.

Source

Hospital Albert Schweitzer, Deschapelles, Haiti, c/o 2331 Mt Hood Ct SE, Lacey, WA 98503, USA and Peripartum Cardiomyopathy Network, USA, Investigations in Pregnancy Associated Cardiomyopathy, Central Office, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. [email protected]

Abstract

Peripartum cardiomyopathy (PPCM) has a remarkable potential for recovery. It may be within our capability to help almost all women with PPCM not only to survive, but also to completely recover heart function. Time-of-diagnosis left ventricular ejection fraction (LVEF) ≥0.35 is associated with better survival rates and higher full recovery rates. Increased mortality, chronic cardiomyopathy, thromboembolic complications and serious ventricular tachyarrhythmias are associated with diagnostic LVEF <0.30. Delays in diagnosis may result in lower LVEF at diagnosis and subsequent lower recovery rates. Greater awareness of the possibility of heart failure developing in previously healthy young women, with no history of heart disease, will contribute to earlier diagnosis, with potentially better preserved heart function. Women of African descent may be at higher risk for poorer outcomes. Recent investigations suggest newer biomarkers may help with earlier detection of PPCM.

New information about use of bromocriptine in treatment of PPCM:    These investigators indicate that bromocriptine “may not be sufficiently effective in all patients, especially in PPCM patients with very low baseline EF.”   Basic Res Cardiol (2013) 108:366

Phenotyping and outcome on contemporary management in a German cohort of patients with peripartum cardiomyopathy

A. Haghikia • E. Podewski • E. Libhaber • S. Labidi • D. Fischer • P. Roentgen • D. Tsikas • J. Jordan • R. Lichtinghagen • C. S. von Kaisenberg • I. Struman • N. Bovy • K. Sliwa • J. Bauersachs • Denise Hilfiker-Kleiner

Received: 15 May 2013 / Revised: 7 June 2013 / Accepted: 12 June 2013 Ó The Author(s) 2013. This article is published with open access at Springerlink.com

ORIGINAL CONTRIBUTION

Abstract Peripartum cardiomyopathy (PPCM) is a life- threatening heart disease developing towards the end of pregnancy or in the months following delivery in previ- ously healthy women in terms of cardiac disease. Enhanced oxidative stress and the subsequent cleavage of the nursing hormone Prolactin into an anti-angiogenic 16 kDa sub- fragment emerged as a potential causal factor of the dis- ease. We established a prospective registry with confirmed PPCM present in 115 patients (mean baseline left ven- tricular ejection fraction, LVEF: 27 ± 9 %). Follow-up data (6 ± 3 months) showed LVEF improvement in 85 % and full recovery in 47 % while 15 % failed to recover with death in 2 % of patients. A positive family history of

cardiomyopathy was present in 16.5 %. Pregnancy-asso- ciated hypertension was associated with a better outcome while a baseline LVEF B 25 % was associated with a worse outcome. A high recovery rate (96 %) was observed in patients obtaining combination therapy with beta- blocker, angiotensin-converting enzyme (ACE) inhibitors/ angiotensin-receptor-blockers (ARBs) and bromocriptine. Increased serum levels of Cathepsin D, the enzyme that generates 16 kDa Prolactin, miR-146a, a direct target of 16 kDa Prolactin, N-terminal-pro-brain-natriuretic peptide (NT-proBNP) and asymmetric dimethylarginine (ADMA) emerged as biomarkers for PPCM. In conclusion, low baseline LVEF is a predictor for poor outcome while pregnancy-induced hypertensive disorders are associated with a better outcome in this European PPCM cohort. The high recovery rate in this collective is associated with a treatment concept using beta-blockers, ACE inhibitors/ ARBs and bromocriptine. Increased levels of Cathepsin D activity, miR-146a and ADMA in serum of PPCM patients.  

PPCM Patient Page can be found in:  
 2013 May 21;127(20):e622-e626.


Peripartum Cardiomyopathy.
Givertz MM.
 

Source

Cardiovascular Division, Brigham and Women's Hospital, 75 Francis St, Boston, MA 02115. [email protected]

[Note:   this provides very good current information for PPCM patients and their families.   Individual PPCM patients may e-mail me at [email protected] for more details.---JDF] 

 2012 Nov;120(5):1013-9. doi: http://10.1097/AOG.0b013e31826e46a1.

Peripartum cardiomyopathy: population-based birth prevalence and 7-year mortality.

Source

Department of Obstetrics and Gynecology, Mountain Area Health Education Center, Asheville, and the North Carolina Birth Defects Monitoring Program, State Center for Health Statistics, North Carolina Division of Public Health, Raleigh, North Carolina; and the Division of Reproductive Health, Centers for Disease Control and Prevention, Atlanta, Georgia.

Abstract

OBJECTIVE:

: To estimate the birth prevalence and 7-year case-fatality rate of peripartum cardiomyopathy for a statewide population by applying the National Institutes of Health Workshop on Peripartum Cardiomyopathy definition, including echocardiographic criteria for left ventricular dysfunction.

METHODS:

: This was an epidemiologic study of residents of North Carolina experiencing an obstetric delivery or a pregnancy-related death before delivery in 2002 through 2003 including 235,599 live births. Potential cases were identified from International Classification of Disease, Ninth Revision, Clinical Modification (ICD-9-CM), pregnancy and cardiovascular codes followed by medical record review, and from the state pregnancy-related mortality file. Only women meeting the established definition including echocardiographic criteria for left ventricular dysfunction and women with diagnoses at autopsy were included. The state death file and the U.S. Social Security Death Index were searched for the years 2002 through 2010 for all cases.

RESULTS:

: A total of 740 potential cases from 70 hospitals were identified from discharge ICD-9-CM codes. The medical records for 698 (94.3%) were located and reviewed. Seventy-eight met inclusion criteria. An additional seven women had diagnoses only at autopsy. The birth prevalence was 1 case for every 2,772 live births or 3.61 cases per 10,000 live births (95% confidence interval 2.88-4.46). The 7-year case-fatality rate was 16.5% (95% confidence interval 10--25.9%). Black non-Hispanic women experienced an almost fourfold increased prevalence and fatality compared with white women. Women older than age 35 years had the highest prevalence.

CONCLUSIONS:

: The racial disparity in both birth prevalence and case-fatality is striking; one in six women died within 7 years.

LEVEL OF EVIDENCE:

: II.


 2012 Sep 25. [Epub ahead of print]

Management of Peripartum Cardiomyopathy.

Source

Division of Cardiovascular Medicine, Brigham and Women's Hospital, 75 Francis Street, Boston, MA, 02115, USA, [email protected]

Abstract

OPINION STATEMENT: Peripartum cardiomyopathy is diagnosed in women who develop systolic heart failure in the last month of pregnancy or within 5 months of delivery. This diagnosis should not be made in women with pre-existing cardiac disease or another cause of cardiac dysfunction. To prevent delay in diagnosis and treatment, a high index of suspicion is required given the overlapping symptoms of late pregnancy and heart failure. Traditional heart failure medical therapies are indicated, although drugs must be carefully reviewed for safety during pregnancy and lactation. Long-term prognosis is largely determined by the degree of ventricular recovery. Patients with acute or persistent hemodynamic compromise despite medical therapy should be considered for mechanical circulatory support and evaluated promptly for cardiac transplantation. A multidisciplinary team is required to care for patients with peripartum cardiomyopathy through parturition and beyond. All peripartum cardiomyopathy patients should be counseled that repeat pregnancy can negatively impact cardiac function and lead to recurrent heart failure or even death. Patients with persistent ventricular dysfunction should be strongly advised against another pregnancy.


Nature. 2012 May 9;485(7398):333-8.

Cardiac angiogenic imbalance leads to peripartum cardiomyopathy.

Source

Cardiovascular Institute, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, Massachusetts 02115, USA.

Abstract

Peripartum cardiomyopathy (PPCM) is an often fatal disease that affects pregnant women who are near delivery, and it occurs more frequently in women with pre-eclampsia and/or multiple gestation. The aetiology of PPCM, and why it is associated with pre-eclampsia, remain unknown. Here we show that PPCM is associated with a systemic angiogenic imbalance, accentuated by pre-eclampsia. Mice that lack cardiac PGC-1α, a powerful regulator of angiogenesis, develop profound PPCM. Importantly, the PPCM is entirely rescued by pro-angiogenic therapies. In humans, the placenta in late gestation secretes VEGF inhibitors like soluble FLT1 (sFLT1), and this is accentuated by multiple gestation and pre-eclampsia. This anti-angiogenic environment is accompanied by subclinical cardiac dysfunction, the extent of which correlates with circulating levels of sFLT1. Exogenous sFLT1 alone caused diastolic dysfunction in wild-type mice, and profound systolic dysfunction in mice lacking cardiac PGC-1α. Finally, plasma samples from women with PPCM contained abnormally high levels of sFLT1. These data indicate that PPCM is mainly a vascular disease, caused by excess anti-angiogenic signalling in the peripartum period. The data also explain how late pregnancy poses a threat to cardiac homeostasis, and why pre-eclampsia and multiple gestation are important risk factors for the development of PPCM.


J Card Fail. 2012 Jan;18(1):28-33. Epub 2011 Nov 9.

Myocardial recovery in peripartum cardiomyopathy: prospective comparison with recent onset cardiomyopathy in men and nonperipartum women.

Source

Mayo Clinic, Rochester, MN.

Abstract

BACKGROUND:

Whether myocardial recovery occurs more frequently in peripartum cardiomyopathy (PPCM) than in recent onset cardiomyopathiesin men and nonperipartum women has not been prospectively evaluated. This was examined through an analysis of outcomes in the Intervention in Myocarditis and Acute Cardiomyopathy 2 (IMAC2) registry.

METHODS AND RESULTS:

IMAC2 enrolled 373 subjects with recent onset nonischemic dilated cardiomyopathy. Left ventricular ejection fraction (LVEF) was assessed at entry and 6 months, and subjects followed for up to 4 years. Myocardial recovery was compared between men (group 1), nonperipartum women (group 2) and subjects with PPCM (group 3). The cohort included 230 subjects in group 1, 104 in group 2, and 39 in group 3. The mean LVEF at baseline in groups 1, 2, and 3 was 0.23 ± 0.08, 0.24 ± 0.08, and 0.27 ± 0.07 (P = .04), and at 6 months was 0.39 ± 0.12, 0.42 ± 0.11, and 0.45 ± 0.14 (P = .007). Subjects in group 3 had a much greater likelihood of achieving an LVEF >0.50 at 6 months than groups 1 or 2 (19 %, 34%, and 48% respectively, P = .002).

CONCLUSIONS:

Prospective evaluation confirms myocardial recovery is greatest in women with PPCM, poorest in men, and intermediate in nonperipartum women. On contemporary therapy, nearly half of women with PPCM normalize cardiac function by 6 months.   [My comment:   we know from other observations that many PPCM patients continue to improve after the first 6 months post-diagnosis; more who reach these recovery levels at 12, 18, 24 + months.---JDF]



J Am Coll Cardiol. 2011 Aug 9;58(7):659-70.

Clinical characteristics of peripartum cardiomyopathy in the United States: diagnosis, prognosis, and management.

Source

Division of Cardiology, Department of Medicine, University of Southern California Keck School of Medicine, Los Angeles, California, USA. [email protected]

Abstract

Peripartum cardiomyopathy is a pregnancy-associated myocardial disease characterized by the development of heart failure due to marked left ventricular systolic dysfunction. Although the disease is relatively uncommon, its incidence is increasing, and it can be associated with important and lasting morbidity and with mortality. Peripartum cardiomyopathy seems to affect women in different parts of the world but with considerable differences in clinical presentation. The purposes of this review are to describe the clinical profile of peripartum cardiomyopathy in the United States and to provide recommendations for the diagnosis and the management of this disease.



Obstet Gynecol Clin North Am. 2010 Jun;37(2):283-303.

Update on peripartum cardiomyopathy.

Source

Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, University of Illinois at Chicago, 840 South Wood Street, M/C 808, Chicago, IL 60612, USA. [email protected]

Abstract

Although multiple mechanisms have been postulated, peripartum cardiomyopathy (PPCM) continues to be a cardiomyopathy of unknown cause. Multiple risk factors exist and the clinical presentation does not allow differentiation among potential causes. Although specific diagnostic criteria exist, PPCM remains a diagnosis of exclusion. Treatment modalities are dictated by the clinical state of the patient, and prognosis is dependent on recovery of function. Randomized controlled trials of novel therapies, such as bromocriptine, are needed to establish better treatment regimens to decrease morbidity and mortality. The creation of an international registry will be an important step to better define and treat PPCM. This article discusses the pathogenesis, risk factors, diagnosis, management, and prognosis of this condition.


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