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

Aims The true incidence of life‐threatening ventricular tachyarrhythmic events and the risk of sudden cardiac death in the early stage of peripartum cardiomyopathy (PPCM) are still unknown. We aimed to assess the usefulness of the wearable cardioverter/defibrillator (WCD) to bridge a potential risk for life‐threatening arrhythmic events in patients with early PPCM, severely reduced left ventricular ejection fraction (LVEF) and symptoms of heart failure. Methods and results Twelve consecutively admitted women with PPCM were included in this single‐centre, prospective observational study between September 2012 and September 2013. Patients with LVEF ≤35% were considered to use the WCD for 3 months or even 6 months when considered necessary for LVEF recovery. Nine of the 12 women had a severely reduced LVEF (mean 18.3%) at the time of study enrolment; seven women received a WCD, while two patients refused to wear a WCD. During a median WCD follow‐up of 81 days (range 25–345 days), we observed a total of four events of ventricular fibrillation with appropriate and successful WCD shocks in three of the seven women receiving a WCD. No syncope or sudden arrhythmic deaths occurred in women not using the WCD during a median follow‐up of 12 months (range 5–15 months). All women showed impressive improvement of LVEF during follow‐up. Conclusion PPCM patients with severely reduced LVEF have an elevated risk for ventricular tachyarrhythmias early after diagnosis. Therefore, use of the WCD should be considered in all women with early‐stage PPCM and severely reduced LVEF during the first 6 months after initiation of heart failure therapy.

BackgroundOptimal timing of implantation of an implantable cardioverter/defibrillator (ICD) after newly diagnosed heart failure is unclear given that late reverse remodelling may occur. We aimed to analyze left ventricular ejection fraction (LVEF) after diagnosis of an LVEF ≤35% during optimization of heart failure drug therapy.Methods and ResultsOne hundred fifty‐six patients with newly diagnosed LVEF ≤35% receiving a wearable cardioverter/defibrillator (WCD) were analyzed. WCD was prescribed for 3 months until first re‐evaluation. Indications for prolongation of WCD wearing period instead of ICD implantation were: (1) LVEF at 3‐month visit 30% to 35%; (2) increase in LVEF of ≥5% compared to the last visit; and (3) nonoptimized heart failure medication. Mean LVEF was 24±7% at diagnosis and 39±11% at last follow‐up (mean, 12±10 months). Whereas 88 patients presented a primary preventive ICD indication (LVEF ≤35%) at 3‐month follow‐up, only 58 showed a persistent primary preventive ICD indication at last follow‐up. This delayed improvement in LVEF was related to nonischemic origin of cardiomyopathy, New York Heart Association functional class at baseline, heart rate, better LVEF after 3 months, and higher dosages of mineralocorticoid receptor antagonist. Twelve appropriate WCD shocks for ventricular tachycardia/ventricular fibrillation occurred in 11 patients. Two patients suffered from ventricular tachycardia/ventricular fibrillation beyond 3 months after diagnosis.ConclusionsA relevant proportion of patients with newly diagnosed heart failure shows recovery of LVEF >35% beyond 3 months after initiation of heart failure therapy. To avoid untimely ICD implantation, prolongation of WCD period should be considered in these patients to prevent sudden cardiac death while allowing left ventricular reverse remodeling during intensified drug therapy.

AimsVentricular arrhythmias (VA) occur frequently after permanent left ventricular assist device (LVAD) implantation in end stage heart failure. Left ventricular assist device patients require rhythm control in contrast to patients with biventricular support. However, the rationale for implantable cardioverter-defibrillator (ICD) utilization in LVAD patients remains unclear. This study investigated the safety and efficacy of primary prevention ICD therapy and the rate of appropriate ICD interventions in LVAD patients. Methods and resultsWe prospectively collected data from patients receiving LVADs. Patients without previous ICD received an ICD after LVAD implantation for primary prevention. Sixty-one patients with LVAD and ICD were followed prospectively for 365 + 321 days. Nine patients died from thromboembolism or haemorrhage. Overall, the rate of appropriate ICD interventions was 34%, mostly for treatment of monomorphic VT in 52%, polymorphic VT in 13%, and VF in 35%. Seventy-one percent of VA were terminated by overdrive pacing, 29% by shock. Patients with a history of VA before LVAD implantation had a significantly higher 1-year rate for ICD therapy compared with LVAD patients with a primary prevention ICD indication LVAD patients (50 vs. 24%). Similarly, patients with non-ischaemic cardiomyopathy had a significantly higher risk for ICD therapy than patients with ischaemic heart disease (50 vs. 22%). ConclusionImplantable cardioverter-defibrillator therapy is safe and effective in LVAD patients. Ventricular arrhythmias leading to ICD intervention occur frequently in 34% of LVAD patients after 1 year, with large variations depending on the underlying cardiac disease and previous arrhythmia history. Primary prevention ICD indication after LVAD implantation yields high rates of ICD intervention. A history of previous VA strongly predicts future use of ICD treatment after LVAD implantation.--

Mechanical circulatory support in patients with refractory cardiogenic shock complicating peripartum cardiomyopathy was associated with a 30-day survival of 100% and a favourable outcome. Notably, early left ventricular unloading combined with bromocriptine therapy was associated with left ventricular recovery. Therefore, an immediate transfer to a tertiary hospital experienced in mechanical circulatory support in combination with bromocriptine treatment seems indispensable for successful treatment of peripartum cardiomyopathy complicated by cardiogenic shock.