Successful treatment of peripartum cardiomyopathy with mechanical assist devices and cardiac transplantation

In the severe form of peripartum cardiomyopathy short and long-term continuous flow ventricular assist devices offer a safe bridge to transplant where cardiac transplantation seems to be the only hope and treatment end point for most of these patients. In this report we described the outcome of a 33 years old patient on the 32nd gestational week with peripartum cardiomyopathy who was successfully treated with biventricular mechanical assist devices and cardiac transplantation.     

Pathophysiology,diagnosis and management of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a potentially life‐threatening condition typically presenting as heart failure with reduced ejection fraction (HFrEF) in the last month of pregnancy or in the months following delivery in women without another known cause of heart failure. This updated position statement summarizes the knowledge about pathophysiological mechanisms, risk factors, clinical presentation, diagnosis and management of PPCM. As shortness of breath, fatigue and leg oedema are common in the peripartum period, a high index of suspicion is required to not miss the diagnosis. Measurement of natriuretic peptides, electrocardiography and echocardiography are recommended to promptly diagnose or exclude heart failure/PPCM. Important differential diagnoses include pulmonary embolism, myocardial infarction, hypertensive heart disease during pregnancy, and pre‐existing heart disease. A genetic contribution is present in up to 20% of PPCM, in particular titin truncating variant. PPCM is associated with high morbidity and mortality, but also with a high probability of partial and often full recovery. Use of guideline‐directed pharmacological therapy for HFrEF is recommended in all patients respecting contraindications during pregnancy/lactation. The oxidative stress‐mediated cleavage of the hormone prolactin into a cardiotoxic fragment has been identified as a driver of PPCM pathophysiology. Pharmacological blockade of prolactin release using bromocriptine as a disease‐specific therapy in addition to standard therapy for heart failure treatment has shown promising results in two clinical trials. Thresholds for devices (implantable cardioverter‐defibrillators, cardiac resynchronization therapy and implanted long‐term ventricular assist devices) are higher in PPCM than in other conditions because of the high rate of recovery. The important role of education and counselling around contraception and future pregnancies is emphasised.     

A fatal case of peripartum cardiomyopathy

Abstract

Peripartum cardiomyopathy is a life-threatening cardiac condition affecting pregnant women either late in pregnancy or early in the post-partum period. The latest studies show a dramatic improvement in the mortality rates of women affected with this disorder, which has been correlated with advances in medical therapy for heart failure. However, patients continue to die of this condition. The following case report describes a typical patient with peripartum cardiomyopathy diagnosed on clinical grounds, along with echocardiogram findings of severe systolic dysfunction and global hypokinesis consistent with dilated cardiomyopathy. Emergency cesarean delivery had to be performed for fetal distress. There was significant improvement of the patient's condition with standard pharmacological management for heart failure at the time of discharge. However, five weeks after discharge, fatal cardiac arrest occurred. It is hoped that this article will raise awareness about this rare but potentially fatal condition and promote understanding of its main clinical features, diagnostic criteria, and conventional pharmacological management.     

Peripartum cardiomyopathy: A systematic review of the literature

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure. It is defined as cardiomyopathy that develops in the last month of pregnancy or within 5 months of the postpartum period without an identifiable cause. We conducted a systematic review of literature of prospective studies with a focus on echocardiographic and long‐term clinical outcomes in PPCM. We searched MEDLINE and Embase up to October 1, 2017. Prospective studies (sample size ≥20) reporting all‐cause mortality and follow‐up duration of ≥1 year were included. Of the 956 studies identified, 7 met the inclusion criteria. A total of 445 patients with a mean age of 30 years (range, 27–32 years) were included. The mean follow‐up duration was 41 months (range, 12–61 months). The majority of patients had New York Heart Association class III or IV symptoms at the time of diagnosis. Only 3 studies reported data on ethnicity where the majority of patients were non‐Caucasian. Most of the patients (81%–93%) were on guideline‐directed medical therapy, except 1 study (41%). Left ventricular ejection fraction at baseline ranged from 24% to 35% (mean, 28%) and at follow‐up from 31% to 53% (mean, 44%). Recovery in systolic function was noted in 20% to 82% (mean, 50%) of patients. All‐cause mortality ranged from 0% to 28% (mean, 16%). This systematic review summarizes the evidence to date on the clinical characteristics and outcomes of patients with PPCM. Multicenter registries with long‐term follow‐up will help shed further light on characteristics and outcomes of patients with this rare disease.     

Ventricular arrhythmias originating from the epicardial ventricular outflow tract complicated with peripartum cardiomyopathy

We report two cases undergoing electrophysiological studies for ventricular arrhythmias (VAs) associated with peripartum cardiomyopathy. Those two cases demonstrated that subsequent pregnancies might result in deterioration of VAs even though they exhibit no symptoms of heart failure. Those findings may clinically impact the decision making when women with a history of peripartum cardiomyopathy desire to become pregnant again. The VA foci in both cases were determined or suggested to be in the epicardium of the ventricular outflow tract from the results of the catheter ablation and electrophysiological study. Therefore, catheter ablation of those VAs may be feasible but challenging.     

Current state of knowledge on aetiology,diagnosis, management,and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy

Peripartum cardiomyopathy (PPCM) is a cause of pregnancy‐associated heart failure. It typically develops during the last month of, and up to 6 months after, pregnancy in women without known cardiovascular disease. The present position statement offers a state‐of‐the‐art summary of what is known about risk factors for potential pathophysiological mechanisms, clinical presentation of, and diagnosis and management of PPCM. A high index of suspicion is required for the diagnosis, as shortness of breath and ankle swelling are common in the peripartum period. Peripartum cardiomyopathy is a distinct form of cardiomyopathy, associated with a high morbidity and mortality, but also with the possibility of full recovery. Oxidative stress and the generation of a cardiotoxic subfragment of prolactin may play key roles in the pathophysiology of PPCM. In this regard, pharmacological blockade of prolactin offers the possibility of a disease‐specific therapy.     

Successful pregnancy, delivery and puerperium in a heart transplant patient with previous peripartum cardiomyopathy.

A 25-year-old female who had orthotopic cardiac transplantationfor peripartum cardiomyopathy, became pregnant and had a successfulpregnancy and delivery, and remains asymptomatic after 2 yearsof follow-up.     

The role of MRI in peripartum cardiomyopathy

Cardiac magnetic resonance imaging may be useful in the evaluation of peripartum cardiomyopathy, an inflammatory cardiomyopathy associated with pregnancy. Late gadolinium enhancement may be an indicator of ongoing inflammation and serve as a guide for endomyocardial biopsy for those patients not improving as expected with effective heart failure treatment.     

Peripartum cardiomyopathy

In 1971, Demakis and colleagues established the term peripartum cardiomyopathy (PPCM) and defined it by criteria based on the clinical profile of their patients. With the recognition that these criteria are arbitrary and that PPCM often presents earlier in pregnancy, the definition of PPCM has been recently updated by a working group on PPCM of the European Society of Cardiology. This article discusses the cause, clinical presentation, prognosis, and treatment of PPCM, as well as other related topics.     

Reversal of IFN-gamma, oxLDL and prolactin serum levels correlate with clinical improvement in patients with peripartum cardiomyopathy

AIM: Peripartum cardiomyopathy (PPCM) is characterized by acute onset of heart failure of unknown aetiology. We aimed to identify mechanisms involved in initiation and progression of the disease. METHODS AND RESULTS: Serum markers related to cardiac function, apoptosis, oxidative stress, remodelling, inflammation and the nursing hormone prolactin were analyzed in PPCM patients and healthy controls. The kinetics of these markers were compared between patients who improved cardiac function (IMP) and those patients who did not improve (NIMP), over 6 months follow-up. All patients received ACE-inhibitors, beta-blockers and diuretics. Baseline levels of TGF-beta-1 were significantly lower, MMP-9 and VEGF were not different; all other markers were significantly higher in PPCM compared with controls. Only baseline NT-proBNP levels were higher in NIMP compared with IMP. After 6 months, NT-proBNP, oxLDL and IFN-gamma were significantly lower in IMP and the decrease in oxLDL, IFN-gamma and prolactin was significant in IMP but not in NIMP. Significant correlations were observed between the kinetics of NT-proBNP, oxLDL, prolactin and IFN-gamma in PPCM patients. CONCLUSION: Baseline NT-proBNP and the failure to decrease oxLDL, IFN-gamma and prolactin are associated with poor outcome in PPCM, suggesting a potential role of these factors in the pathophysiology of PPCM and for risk stratification of PPCM patients.