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.     

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.     

Long‐term prognosis,subsequent pregnancy,contraception and overall management of peripartum cardiomyopathy: practical guidance paper from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy

Peripartum cardiomyopathy is an idiopathic cardiomyopathy presenting with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause for heart failure is identified. Outcome varies from full recovery to residual left ventricular systolic dysfunction and even death. Many women return to their physician to acquire information on their long‐term prognosis, to seek medical advice regarding contraception, or when planning a subsequent pregnancy. This position paper summarizes current evidence for long‐term outcome, risk stratification of further pregnancies and overall management. Based on the best available evidence, as well as the clinical experience of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy members, a consensus on pre‐ and postpartum management algorithms for women undergoing a subsequent pregnancy is presented.     

Heart Failure Association of the European Society of Cardiology Specialist Heart Failure Curriculum

It is well established that organized care of heart failure patients, including specialist management by cardiologists, improves patient outcomes. In response to this, other national training bodies (the UK and the USA) have developed heart failure subspecialty curricula within their Cardiology Training Curricula. In addition, European Society of Cardiology (ESC) subspecialty curricula exist for Interventional Cardiology and Heart Rhythm Management. The purpose of this heart failure curriculum is to provide a framework which can be used as a blueprint for training across Europe. This blueprint mirrors other ESC curricula. Each section has three components: the knowledge required, the skills which are necessary, and the professionalism (attitudes and behaviours) which should be attained. The programme is designed to last 2 years. The first year is devoted to the specialist heart failure module. The second year allows completion of the optional modules of advanced imaging, device therapy for implanters, cardiac transplantation, and mechanical circulatory support. The second year can also be devoted to continuation of specialist heart failure training and/or research for those not wishing to continue with the advanced modules.     

The innate immune system in chronic cardiomyopathy: a European Society of Cardiology (ESC) scientific statement from the Working Group on Myocardial Function of the ESC

Activation of the immune system in heart failure (HF) has been recognized for over 20 years. Initially, experimental studies demonstrated a maladaptive role of the immune system. However, several phase III trials failed to show beneficial effects in HF with therapies directed against an immune activation. Preclinical studies today describe positive and negative effects of immune activation in HF. These different effects depend on timing and aetiology of HF. Therefore, herein we give a detailed review on immune mechanisms and their importance for the development of HF with a special focus on commonalities and differences between different forms of cardiomyopathies. The role of the immune system in ischaemic, hypertensive, diabetic, toxic, viral, genetic, peripartum, and autoimmune cardiomyopathy is discussed in depth. Overall, initial damage to the heart leads to disease specific activation of the immune system whereas in the chronic phase of HF overlapping mechanisms occur in different aetiologies.     

Spanish Heart Transplant Registry. 29th Official Report of the Spanish Society of Cardiology Working Group on Heart Failure

Introduction and objectives

The present report updates the characteristics and results of heart transplantation in Spain, mainly focused in the 2008-2017 period.

Heart Failure Association of the European Society of Cardiology Quality of Care Centres Programme: design and accreditation document

Heart failure (HF) is the major contributor to cardiovascular morbidity and mortality. Given its rising prevalence, the costs of HF care can be expected to increase. Multidisciplinary management of HF can improve quality of care and survival. However, specialized HF programmes are not widely available in most European countries. These circumstances underlie the suggestion of the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) for the development of quality of care centres (QCCs). These are defined as health care institutions that provide multidisciplinary HF management at all levels of care (primary, secondary and tertiary), are accredited by the HFA/ESC and are implemented into existing health care systems. Their major goals are to unify and improve the quality of HF care, and to promote collaboration in education and research activities. Three types of QCC are suggested: community QCCs (primary care facilities able to provide non‐invasive assessment and optimal therapy); specialized QCCs (district hospitals with intensive care units, able to provide cardiac catheterization and device implantation services), and advanced QCCs (national reference centres able to deliver advanced and innovative HF care and research). QCC accreditation will require compliance with general and specific HFA/ESC accreditation standards. General requirements include confirmation of the centre's existence, commitment to QCC implementation, and collaboration with other QCCs. Specific requirements include validation of the centre's level of care, service portfolio, facilities and equipment, management, human resources, process measures, quality indicators and outcome measures. Audit and recertification at 4–6‐year intervals are also required. The implementation of QCCs will evolve gradually, following a pilot phase in selected countries. The present document summarizes the definition, major goals, development, classification and crucial aspects of the accreditation process of the HFA/ESC QCC Programme.     

Spanish Heart Transplantation Registry. 16th official report of the Spanish Society of Cardiology Working Group on Heart Failure, Heart Transplantation, and Associated Therapies

This paper describes the general situation regarding heart transplantation in Spain and the results obtained using the technique, incorporating data for the year 2004. In 2004, 294 heart transplants were carried out, which brings the total number of procedures performed since 1984 to 4680. Clinically, the typical heart transplant recipient in Spain is male, aged around 50 years, has blood group A, has non-revascularizable coronary artery disease, and is in NYHA functional class IV/IV. The percentage of emergency heart transplantations was 35%, which is higher than in the previous year (29%), and higher than the mean for the preceding 5 years (22%). The early mortality rate was 10%, which is lower that the mean for the preceding 5 years (13%). After combining the results for 2004 with those of previous years, the probability of survival at 1, 5 and 10 years was 80%, 70% and 60%, respectively. When the survival rates for different time periods were analyzed, a significant improvement could be seen in the last 5 years, with recent survival rates being 85% and 72% at 1 and 5 years, respectively. The most frequent cause of death in the first month was acute graft failure; in the first year, infection and rejection; and, over the long term, tumors and a combination of graft vasculopathy and sudden death. A comparative analysis of survival rates showed that long-term results in Spain are slightly better than those published in the world literature. Moreover, survival has tended to improve gradually in recent years.