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Current management of patients with severe acute peripartum cardiomyopathy: practical guidance from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy

2016; Elsevier BV; Volume: 18; Issue: 9 Linguagem: Inglês

10.1002/ejhf.586

1879-0844

Johann Bauersachs, Mattia Arrigo, Denise Hilfiker‐Kleiner, Christian Veltmann, Andrew J.S. Coats, María G. Crespo‐Leiro, Rudolf A. de Boer, Peter van der Meer, Christoph Maack, Frédéric Mouquet, Mark C. Petrie, Massimo Piepoli, Vera Regitz‐Zagrosek, Maria Schaufelberger, Petar Seferović, Luigi Tavazzi, Frank Ruschitzka, Alexandre Mebazaa, Karen Sliwa,

Mechanical Circulatory Support Devices

European Journal of Heart FailureVolume 18, Issue 9 p. 1096-1105 HFA Practical GuidanceFree Access Current management of patients with severe acute peripartum cardiomyopathy: practical guidance from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy Johann Bauersachs, Corresponding Author Johann Bauersachs Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanyThese two authors contributed equally to this work.Corresponding author: Medizinische Hochschule Hannover, Carl-Neuberg-Strasse 1, D-30625 Hannover, Germany. Tel: +49 511 532 3841, Fax: +49 511 532 5412, Email: bauersachs.johann@mh-hannover.deSearch for more papers by this authorMattia Arrigo, Mattia Arrigo Department of Cardiology, AP-HP, Lariboisière University Hospital, Paris, France Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, SwitzerlandThese two authors contributed equally to this work.Search for more papers by this authorDenise Hilfiker-Kleiner, Denise Hilfiker-Kleiner Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanySearch for more papers by this authorChristian Veltmann, Christian Veltmann Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanySearch for more papers by this authorAndrew J.S. Coats, Andrew J.S. Coats Monash-Warwick Alliance, Monash University, Australia, and University of Warwick, UKSearch for more papers by this authorMaria G. Crespo-Leiro, Maria G. Crespo-Leiro Cardiology Service, Complexo Hospitalario Universitario A Coruña, La Coruña, SpainSearch for more papers by this authorRudolf A. De Boer, Rudolf A. De Boer Department of Cardiology, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorPeter van der Meer, Peter van der Meer Department of Cardiology, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorChristoph Maack, Christoph Maack Klinik für Innere Medizin III, Universitätsklinikum des Saarlandes, Homburg, GermanySearch for more papers by this authorFrederic Mouquet, Frederic Mouquet Department of Cardiology, Polyclinique du Bois, Lille, FranceSearch for more papers by this authorMark C. Petrie, Mark C. Petrie Department of Cardiology, Golden Jubilee National Hospital and Glasgow University, Glasgow, UKSearch for more papers by this authorMassimo F. Piepoli, Massimo F. Piepoli Department of Cardiology, Guglielmo da Saliceto Hospital, Piacenza, ItalySearch for more papers by this authorVera Regitz-Zagrosek, Vera Regitz-Zagrosek Institute of Gender in Medicine, Charité Universitaetsmedizin Berlin, and German Center for Cardiovascular Research, Berlin, GermanySearch for more papers by this authorMaria Schaufelberger, Maria Schaufelberger Section of Acute and Cardiovascular Medicine, Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Sahlgrenska University Hospital/Östra, Gothenburg, SwedenSearch for more papers by this authorPetar Seferovic, Petar Seferovic University Medical Center, Belgrade, SerbiaSearch for more papers by this authorLuigi Tavazzi, Luigi Tavazzi Maria Cecilia Hospital, Gruppo Villa Maria Care and Research, Ettore Sansavini Health Science Foundation, Cotignola, ItalySearch for more papers by this authorFrank Ruschitzka, Frank Ruschitzka Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, SwitzerlandSearch for more papers by this authorAlexandre Mebazaa, Alexandre Mebazaa Department of Anesthesiology and Critical Care Medicine, AP-HP, Saint Louis Lariboisière University Hospitals, Paris, FranceSearch for more papers by this authorKaren Sliwa, Karen Sliwa Hatter Institute for Cardiovascular Research in Africa & IDM, Inter-Cape Heart Group, Medical Research Council South Africa, Department of Medicine, University of Cape Town, Cape Town, South AfricaSearch for more papers by this author Johann Bauersachs, Corresponding Author Johann Bauersachs Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanyThese two authors contributed equally to this work.Corresponding author: Medizinische Hochschule Hannover, Carl-Neuberg-Strasse 1, D-30625 Hannover, Germany. Tel: +49 511 532 3841, Fax: +49 511 532 5412, Email: bauersachs.johann@mh-hannover.deSearch for more papers by this authorMattia Arrigo, Mattia Arrigo Department of Cardiology, AP-HP, Lariboisière University Hospital, Paris, France Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, SwitzerlandThese two authors contributed equally to this work.Search for more papers by this authorDenise Hilfiker-Kleiner, Denise Hilfiker-Kleiner Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanySearch for more papers by this authorChristian Veltmann, Christian Veltmann Department of Cardiology and Angiology, Medical School Hannover, Hannover, GermanySearch for more papers by this authorAndrew J.S. Coats, Andrew J.S. Coats Monash-Warwick Alliance, Monash University, Australia, and University of Warwick, UKSearch for more papers by this authorMaria G. Crespo-Leiro, Maria G. Crespo-Leiro Cardiology Service, Complexo Hospitalario Universitario A Coruña, La Coruña, SpainSearch for more papers by this authorRudolf A. De Boer, Rudolf A. De Boer Department of Cardiology, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorPeter van der Meer, Peter van der Meer Department of Cardiology, University Medical Center Groningen, Groningen, The NetherlandsSearch for more papers by this authorChristoph Maack, Christoph Maack Klinik für Innere Medizin III, Universitätsklinikum des Saarlandes, Homburg, GermanySearch for more papers by this authorFrederic Mouquet, Frederic Mouquet Department of Cardiology, Polyclinique du Bois, Lille, FranceSearch for more papers by this authorMark C. Petrie, Mark C. Petrie Department of Cardiology, Golden Jubilee National Hospital and Glasgow University, Glasgow, UKSearch for more papers by this authorMassimo F. Piepoli, Massimo F. Piepoli Department of Cardiology, Guglielmo da Saliceto Hospital, Piacenza, ItalySearch for more papers by this authorVera Regitz-Zagrosek, Vera Regitz-Zagrosek Institute of Gender in Medicine, Charité Universitaetsmedizin Berlin, and German Center for Cardiovascular Research, Berlin, GermanySearch for more papers by this authorMaria Schaufelberger, Maria Schaufelberger Section of Acute and Cardiovascular Medicine, Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Sahlgrenska University Hospital/Östra, Gothenburg, SwedenSearch for more papers by this authorPetar Seferovic, Petar Seferovic University Medical Center, Belgrade, SerbiaSearch for more papers by this authorLuigi Tavazzi, Luigi Tavazzi Maria Cecilia Hospital, Gruppo Villa Maria Care and Research, Ettore Sansavini Health Science Foundation, Cotignola, ItalySearch for more papers by this authorFrank Ruschitzka, Frank Ruschitzka Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, SwitzerlandSearch for more papers by this authorAlexandre Mebazaa, Alexandre Mebazaa Department of Anesthesiology and Critical Care Medicine, AP-HP, Saint Louis Lariboisière University Hospitals, Paris, FranceSearch for more papers by this authorKaren Sliwa, Karen Sliwa Hatter Institute for Cardiovascular Research in Africa & IDM, Inter-Cape Heart Group, Medical Research Council South Africa, Department of Medicine, University of Cape Town, Cape Town, South AfricaSearch for more papers by this author First published: 23 June 2016 https://doi.org/10.1002/ejhf.586Citations: 132 AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Introduction Acute heart failure (AHF) due to peripartum cardiomyopathy (PPCM) provides a challenge for treating physicians. Moreover, in patients still pregnant, therapeutic interventions need always to consider the health of both the mother and the foetus. Especially challenging are severe forms of PPCM, as the mortality of these women is quite high. The use of inotropic drugs and mechanical circulatory support devices may be necessary in the initial phase of severe forms of acute PPCM. Many patients, after initial stabilization, recover LV function.1-3 Unfortunately, some patients need further mechanical circulatory support or urgent heart transplantation despite maximal therapy. In addition, the time frame and extent of recovery are unpredictable, and patients may suffer from cardiac arrest due to ventricular fibrillation in the first months after diagnosis.4 The clinical course may be further aggravated by atrial and/or ventricular thrombus formation with subsequent cardio-embolic complications. As evidence-based data from randomized clinical trials are scarce, in this practical guidance we summarize recent data and clinical experience in the treatment of patients with severe acute PPCM to help physicians in the diagnosis, acute treatment, and long-term management of these young critically ill patients. Definition and pathophysiology The Working Group on PPCM of the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) recently proposed a new simplified definition of PPCM as an idiopathic cardiomyopathy frequently presenting with heart failure secondary to LV systolic dysfunction (LVEF <45%) towards the end of pregnancy or in the months following delivery, if no other cause of heart failure is found.1 Since no specific test to confirm PPCM exists, it remains a diagnosis of exclusion. In particular, aggravation of pre-existing heart disease by pregnancy-mediated haemodynamic changes should be differentiated from PPCM. The pathophysiology of PPCM remains poorly understood. The current status of knowledge of the pathophysiological mechanisms of PPCM has been published elsewhere.3 A ‘two-hit’ model of angiogenic imbalance in the heart during the peripartal period has recently been proposed, combining systemic antiangiogenic signals during late pregnancy and host susceptibility through insufficient local proangiogenic defences in the heart.1-3, 5 Angiogenic imbalance can further be triggered by oxidative stress activating cathepsin D, a protease responsible for the cleavage of the nursing hormone prolactin into the angiostatic and proapoptotic 16 kDa subfragment.4, 6 Clinical presentation of acute peripartum cardiomyopathy Most patients admitted with PPCM present typical symptoms of AHF associated with signs of congestion. Because early signs and symptoms of heart failure in PPCM patients may mimic physiological changes occurring during/after pregnancy, delayed diagnosis may occur. The differential diagnosis of acute PPCM includes myocarditis, pre-existing cardiomyopathy, valve disease, or congenital heart disease. In the case of cardiogenic shock, pregnancy-associated myocardial infarction, pulmonary embolism, and amniotic liquid embolism should be immediately ruled out to provide adequate care (Table 1). Table 1. Peripartal acute dyspnoea: differential diagnosis of acute peripartum cardiomyopathy PPCM Pre-existing CMP,valve disease orcongenital heartdisease Pregnancy-associatedmyocardial infarction Pulmonary embolism/amniotic liquid embolism Myocarditis History Most commonly post-partal onset of dyspnoea Earlier onset (during second trimester) Sometimes family history Retrosternal chest pain, abdominal discomfort, nausea Pleuritic chest pain Infection Biomarkers Elevated natriuretic peptides Elevated natriuretic peptides Elevated troponin Elevated D-dimer, troponin, natriuretic peptides Elevated troponin Possibly. elevated natriuretic peptides Echocardiography Left and/or right ventricular dysfunction Evidence of pre-existing valve disease or congenital defect Regional hypokinesis/akinesis RV dysfunction, elevated RV pressure, McConnell's sign Regional or general hypokinesis Additional tests Consider MRI Consider MRI Consider genetic test Coronary angiography CT-scan or. V/Q scintigraphy; consider angiography MRI Consider myocardial biopsy CMP, cardiomyopathy; MRI, magnetic resonance imaging; PPCM, peripartum cardiomyopathy; RV, right ventricular. Evaluation of acute peripartum cardiomyopathy As for any AHF, initial evaluation of patients with suspected acute PPCM includes two parts, which should be performed simultaneously to allow timely diagnosis and treatment delivery: evaluation of cardiopulmonary distress; and confirmation of the diagnosis with additional tests. Evaluation of cardiopulmonary distress Evaluation of cardiopulmonary distress is crucial because it will influence subsequent treatment and patients' allocation. The presence of criteria defining cardiopulmonary distress should lead to intensive cardiac care unit admission: haemodynamic instability (systolic blood pressure 130 b.p.m. or 25/min; peripheral oxygen saturation 2.0 mmol/L; low central–venous oxygen saturation <60%, if available; altered mental state; cold, clammy, mottled skin; oliguria 110 mmHg, intravenous vasodilators (e.g. nitrates) should be started. At the same time, oxygenation should be optimized (target peripheral oxygen saturation, SpO2 > 95%). Non-invasive ventilation (NIV) reduces respiratory distress and may decrease intubation and mortality rates.17 Intubation with mechanical ventilation should be considered in the case of altered mental state or persistent hypoxaemia. In the presence of signs of cardiogenic shock, haemodynamics should be rapidly restored to avoid irreversible organ damage. Inotropes and vasopressors may be considered, although the use of catecholamines is associated with adverse effects in patients with advanced heart failure or cardiogenic shock.18, 19 Experimental evidence and clinical experience suggest that catecholamines such as dobutamine are less favourable in PPCM patients due to metabolic compromise.20 Therefore, catecholamines should be avoided whenever possible or used only with extreme caution. Levosimendan, in contrast to dobutamine and adrenaline, does not increase myocardial oxygen demand and may be considered as the preferred inotropic agent as continuous infusion of 0.1 µg/kg/h for 24 h without an initial loading dose (bolus) for patients with severe PPCM.21 A recent small study including 28 patients showed that the use of levosimendan in patients with PPCM induced rapid haemodynamic recovery and profound decongestive effects.22 In case levosimendan is unavailable, dobutamine is the other option, while adrenaline should be avoided. As for other causes of shock, noradrenaline should be the first-line vasopressor. Patients with haemodynamic instability despite treatment should undergo urgent delivery irrespective of gestation duration. Caesarean section with combined spinal and epidural analgesia and involvement of an experienced interdisciplinary team are recommended. The administration of adjunctive therapies with the prolactin blocker bromocriptine has shown promising results in several case series and in a small proof-of-concept study,23 and should be considered for patients with cardiopulmonary distress. The starting dose of bromocriptine is usually 2.5 mg twice daily, but an increased dose may be necessary to lower prolactin levels in selected cases (see below). As thrombo-embolic events have been reported during the use of bromocriptine (albeit mostly at higher dosages), bromocriptine treatment should always be accompanied by at least prophylactic anticoagulation with heparin.24 Anticoagulation with heparin should also be started in all patients with acute PPCM and severely reduced LV systolic function (LVEF ≤35%). Indeed, the combination of reduced EF and the procoagulant activity during the peripartal phase exposes patients to a clinically important risk of cardio-embolic events. In general, patients with severe distress should be transferred early to an experienced centre whenever possible. For patients with persistent haemodynamic instability despite medical treatment, mechanical circulatory support should be considered (see below). Advanced management of severe acute peripartum cardiomyopathy Implantation of a mechanical circulatory support should be considered early as a rescue therapy in patients who cannot be stabilized with medical therapy alone. If necessary, a device for temporary support should be implanted in the acute phase, either as ‘bridge-to-recovery’, if ventricular function improves during the subsequent days and weaning can be achieved, or as ‘bridge-to-bridge’, if haemodynamic impairment persists and circulatory support has to be ensured by switching to a more durable (and usually more invasive) device. Because of the higher proportion of patients with at least partial recovery of ventricular function compared with other cardiomyopathies, an initial ‘bridge-to-transplantation’ strategy is seldom necessary. Since several devices exist, and there is little evidence about which device should be preferred;25 we provide here an overview of some devices based on experts' opinion. For the choice of the initial device, several factors should be taken into account (needed haemodynamic support, periprocedural risks, costs), but the oxygenation status of the patient plays a central role. If the patient is adequately oxygenated, percutaneous [e.g. intra-aortic balloon pump (IABP), Impella®] or surgical (e.g. CentriMag®, AbiomedBVS 5000®) devices can be used to restore circulation. In contrast, in the presence of impaired oxygenation, other devices with integrated oxygenation should be used [e.g. TandemHeart®, veno-arterial extracorporeal membrane oxygenation (ECMO)]. Most importantly, as the treatment of patients on mechanical circulatory support is very challenging, the choice of the device should also consider the local availability and the experience of the involved care team (physicians, nurses, and perfusionists). Percutaneous devices offer the advantage of fast and easier placement and removal without the need for open surgery, but complications related to the access site (bleeding, infection, ischaemic limbs) are not uncommon. An IABP provides less haemodynamic support compared with other devices, but on the other hand is easily placed and needs less strict anticoagulation. Given the negative results of the IABP-SHOCK II trial and the lack of data in PPCM, the value of this device in patients with severe PPCM is uncertain, although it is used in selected cases by some centres.26 The Impella® rotary pump is an alternative percutaneous device for temporary support. It is inserted percutaneously from the femoral artery and is placed in the left ventricle through the aortic valve. Depending on the model, it provides a higher degree of haemodynamic support compared with IABP (up to 5 L/min) but is associated with haemolysis and, especially in the context of PPCM, where a procoagulant state is frequent, a stricter anticoagulation regime than for IABP is needed.27 In a small trial in patients with cardiogenic shock complicating myocardial infarction, there was no difference in terms of survival between IABP and Impella®.28 Clinical experience in several PPCM patients with the Impella 3.5 device suggests effective LV support over up to 7–10 days when used as bridge-to-recovery in most patients. The marked decrease in the need for catecholamines may importantly contribute to the beneficial outcome observed in several patients (Figure 3). The TandemHeart® device offers similar haemodynamic support to Impella® (up to 5 L/min) with additional improvement in oxygenation. The placement of this device is performed percutaneously in the catheterization laboratory but it requires a more complex placement with atrial trans-septal puncture. No evidence of improved outcomes in patients with cardiogenic shock receiving TandemHeart® compared with IABP exists.29, 30 Figure 3Open in figure viewerPowerPoint Example of the management of a peripartum cardiomyopathy (PPCM) patient with cardiogenic shock in the intensive care unit (ICU). The figure depicts the management of a patient with newly diagnosed PPCM in cardiogenic shock with severely reduced EF requiring mechanical ventilation and high dose vasopressor (norepinephrine), and inotropic support (dobutamine) at the acute presentation. Upon referral to the Acute and Advanced Heart Failure Unit of Medical School Hannover, temporary circulatory support with the Impella CP® rotary pump was initiated with concurrent invasive haemodynamic monitoring including pulmonary artery wedge pressure (PAWP) by means of a Swan–Ganz catheter. Note the decline of catecholamine dosage and PAWP after insertion of the Impella CP® rotary pump. Additionally, two cycles of 24-h infusion of levosimendan were administered with an interval of 1 week, and diuretic therapy was given throughout the intensive care. After 10 days, when haemodynamic stabilization was achieved, the patient could be extubated and standard heart failure therapy including an ACE inhibitor (later replaced by an ARB due to cough), mineralocorticoid receptor antagonist, and beta-blocker was established. Heart rate reduction with ivabradine was initiated early during ICU course. The patient was treated with a wearable cardioverter-defibrillator for 6 months and had an uneventful course; EF showed a partial recovery during the following 6 months. Extracorporeal membrane oxygenation with veno-arterial cannulation offers the maximal available haemodynamic effect with biventricular support and additional improvement in oxygenation.31 As increased prolactin levels during ECMO treatment have been reported, which may be specifically detrimental in patients with PPCM,32 effective suppression of prolactin under sequential measurements of prolactin levels could be considered in this particular situation with bromocriptine doses up to 10 mg twice daily. After the initial phase, if no weaning from mechanical circulatory support can be achieved after a maximum of 7–10 days, a switch to a durable device should be planned. As for temporary support, several devices exist and little evidence is available to guide the choice of the optimal device. Special attention should be paid to right ventricular function. In the presence of impaired right ventricular function, a biventricular assist device (BiVAD or total artificial heart) may be chosen (e.g. Berlin Heart EXCOR®). Alternatively, several strategies of transient right ventricular support in patients after left ventricular assist device (LVAD) implantation have been adopted in different centres (e.g. veno-arterial ECMO, Impella® RP, or similar). In patients with preserved right ventricular function, LVADs should be preferred. The most commonly used devices are the continuous-flow axial (HeartMate II®) and centrifugal (HeartWare®) LVADs which have shown promising results in patients with end-stage heart failure.33-35 Given the high likelihood of at least partial recovery of ventricular function in PPCM, temporary devices should always be the preferred initial strategy. Cardiac transplantation is reserved for patients where mechanical circulatory support is not possible or satisfactory ventricular recovery after 6–12 months is not achieved. Post-transplant outcomes in women with PPCM appear to be worse than in other recipients: in particular, women with PPCM show higher mortality, a higher incidence of rejection with shorter graft survival, and higher rates of re-