Peripartal Cardiomyopathy Research Articles

Peripartal cardiomyopathy: literature review and clinical case description

The cardiovascular system during pregnancy undergoes a number of compensatory and adaptive changes, such as an increase in heart rate, cardiac output and total peripheral resistance, which causes an increase in the load on the myocardium. Peripartum cardiomyopathy is a serious polyetiological complication of pregnancy and the postpartum period, as well as an important cause of disability and mortality for both mother and fetus. The non-specificity of complaints and the paucity of clinical symptoms in the early stages of the disease lead to delayed diagnosis and significantly increase the frequency of unfavorable outcomes. In the article we present our own clinical observation of the acute development of severe peripartum cardiomyopathy in a multipregnant woman at 36 weeks of gestation, complicated by cardiogenic shock and antenatal fetal death.

Peripаrtal Cardiomyopathy

The purpose of the study is to describe a clinical case of peripartal cardiomyopathy (CM) and conduct literature analysis. Peripartal CM is defined as reduced ejection fraction of the left ventricle <45%, reduced fractional shortening <30%, as well as increased end-diastolic size of the left ventricle > 2.7 cm/m2. At the same time, there should be no detectable causes of heart failure, as well as development of heart failure in the last month of pregnancy or within 5 months after childbirth. The review presents data on possible causes of peripartal CM. Risk factors for peripartal CM are: age younger than 20 years and older than 30 years, preeclampsia, hypertension, multiple pregnancy, prolonged duration of viral infection, macro- and micronutrient insufficiency, etc. Prolactin and its 16 kDa fragment, as well as excessive activity of cathepsin D, play a leading role in pathogenesis of peripartal CM. A step-by-step algorithm for diagnosis of peripartal CM in real clinical conditions is described. Material and methods. The patient is K., 18 years old, in anamnesis: no prior history of cardiovascular diseases, according to the patient, she had pneumonia a year ago. The patient has not registered increased blood pressure. Physiological pregnancy proceeded satisfactorily; natural delivery was on time. On the 56th day after childbirth, the patient was admitted to the intensive care unit with complaints: shortness of breath, muscle weakness, palpitations, lower leg edema. From anamnesis: 1 month after childbirth, the next day after psychoemotional overstrain, the patient began to notice shortness of breath, lack of sleep, muscle weakness, decreased lactation. The examination revealed an increase in the sedimentation rate of erythrocytes, C-reactive blood protein, proteinuria, hematuria, as well as a decrease in the ejection fraction of the left ventricle. Results. Together with standard therapy, addition of prolactin secretion inhibitors and dopamine receptor stimulators led to significant improvement of the patient’s condition: proteinuria significantly decreased, edematous syndrome was gone, and LV contractile function significantly recovered. Conclusion. The clinical case demonstrates the development of a rare variant of peripartal CM, debuting with proteinuria and preserved kidney function, as well as normal serum prolactin levels. Apparently, there are different phenotypes of peripartal CM.

Current understanding of pathophysiological mechanisms of peripartal cardiomyopathy

The aim of the study. To analyze literature data in order to study the pathophysiological mechanisms of development of peripartum cardiomyopathy. Research methods and materials. A review of modern Russian and foreign, mainly English-speaking, literature on the pathogenetic mechanisms of IPAH development was carried out. We took into account publications not older than 6 years, published in specialized medical journals and guidelines. Results. According to the data of modern studies devoted to the peculiarities of the development of peripartum cardiomyopathy, much attention is paid to the risk factors of the disease, in particular the age-related obstetric history, pregnancy with twins or more, and arterial hypertension. In addition, the significance of genetic predisposition, inflammatory syndrome has been proven, and the vasculo-hormonal theory is being studied. Difficult issues of the etiopathogenesis of the disease, including myocarditis, the role of the prolactin-cathepsin-prolactin 16 kDa system, and malnutrition are discussed.Conclusion. Pathophysiological mechanism of development of peripartum cardiomyopathy. appears as a complex combination of inflammatory changes in the myocardium with the participation of a cardiotoxic subfragment – prolactin 16 kDa, which causes damage and dysfunction of the endothelium under hemodynamic stress against the background of possible genetic changes, which leads to damage to cardiomyocytes and a decrease in myocardial contractility.

Amniotic Fluid Embolism: An Update of the Evidence

This article reviews the current evidence on amniotic fluid embolism (AFE), focusing on epidemiology, pathogenesis, clinical presentation, diagnosis and treatment. AFE is a rare and life-threatening obstetric condition, occurring in 2 to 8 per 100 000 births. With a mortality of 0.5 to 1.7 deaths per 100,000 deliveries in the developed world and 1.9 to 5.9 deaths per 100,000 deliveries in the developing world, it is among the leading direct causes of maternal death. Furthermore, persistent neurological impairment has been reported in 6-61% of survivors. Even though there is no shared pathogenetic theory for AFE, the transfer of amniotic fluid components in the maternal circulation may lead either to an inflammatory-mediated anaphylactoid response or to complement activation. There is great variability in the presentation of AFE from classical cardiopulmonary collapse with coagulopathy to minor and subclinical presentations. In severe cases, AFE should be distinguished from other acute and life-threatening emergencies, such as pulmonary embolism, myocardial infarction and peripartal cardiomyopathy. Thus, the diagnosis is one of exclusion, very often on post-mortem report. In fact, several diagnostic biomarkers have been proposed, but they have not been established in routine clinical diagnosis. Prompt recognition of the symptoms and immediate interdisciplinary management are pivotal for prognosis. The traditional treatment is supportive and aimed at treating cardiovascular, pulmonary, and coagulation derangements. Recently, novel treatment strategies have been proposed in the field of invasive hemodynamic support and C1 esterase inhibitors represent a potential therapeutic option. However, due to the lack of specific diagnostic tests, the difficulty of establishing the diagnosis and excluding competing diagnoses, and the complex treatment required, AFE still remains a major clinical challenge.

Epidemiological and clinical features, ultrasound findings and prognosis of right-sided infective endocarditis in a teaching hospital in Ouagadougou : cardiovascular topics

Right-sided infective endocarditis is rare. It accounts about 5 to 10% of all infective endocarditis cases and is prevalent in patients with congenital heart disease, intravascular devices and drug addiction. Our study aimed to describe the epidemiological, clinical and echocardiographic characteristics of right-sided endocarditis and evaluate the prognosis after treatment. From January 2010 to December 2011 we recruited all patients admitted to Yalgado Ouedraogo Teaching Hospital for infective endocarditis, and selected those who had a right-sided location. The Duke criteria were used for diagnosis. We analysed entry points and underlying heart disease. The causative organisms were tracked using blood sample cultures. Ultrasound characteristics were described, and treatment and prognosis were evaluated. Patients' follow up was conducted from recruitment to 30 June 2012. In the two-year period, 14 cases of right-sided infective endocarditis were recorded, including seven cases in children. They accounted for 29.1% of all infective endocarditis cases. The mean age was 25.5 ± 12.5 years (range 9-80 years). The venous route was implicated in 12 cases (85.7%). Blood cultures were positive in 11 patients. The bacteria isolated were Streptococcus pneumonia in six cases, Staphylococcus aureus in three and Hemophilus influenza in two cases. HIV status was positive in three patients. Underlying heart diseases were dominated by congenital heart disease in six cases and peripartal cardiomyopathy in four others. Vegetations were located in the right heart in only 11 cases. With antibiotic treatment, a lowering of temperature was shown within an average of 10 days of follow up. Two fatalities were reported. This study showed that right-sided endocarditis is common in our clinical practice. This infection was prevalent in patients with congenital heart disease or peripartal cardiomyopathy in our context, and the venous route seemed to be the main entry point.

A new approach to treat peripartal cardiomyopathy complicated by cardiogenic shock: inhibition of prolactin-secretion in combination with mechanical circulatory-support to achieve sustained myocardial-recovery

Introduction: Peripartum Cardiomyopathy (PPCM) is defined by an acute onset of heart-failure in the late-stage pregnancy up to several months postpartum. As symptomatic treatment, a left-ventricular assist-device (LVAD) may be initiated to support myocardial-recovery. Furthermore, the prolactine-inhibitor bromocriptine has been suggested as specific therapy, since recent findings showed that the postpartum up-regulated hormone prolactin mediates PPCM. We report the first case of LVAD-therapy combined with inhibition of prolactin-secretion to achieve myocardial-recovery in PPCM complicated by cardiogenic-shock.

Hypertrophic and Hypertensive Hypertrophic Cardiomyopathy—A True Association?

The cardiomyopathies were previously defined as ''heart muscle diseases of unknown cause'' and were differentiated from specific heart muscle disease with known cause. With increasing understanding of etiology and pathogenesis, the difference between cardiomyopathy and specific heart muscle disease has become indistinct. The term specific cardiomyopathies are used to describe heart diseases that are associated with specific cardiac or systemic disorders. These were previously defined as specific heart muscle diseases. They included ischemic cardiomyopathy, valvular cardiomyopathy, hypertensive cardiomyopathy, inflammatory cardiomyopathy, metabolic cardiomyopathy, general system disease, muscular dystrophies, sensitivity and toxic reactions, and peripartal cardiomyopathy. The cardiomyopathies are therefore classified by the dominant pathophysiology or, if possible, by etiological/pathogenetic factors. Topol in 1985 described a syndrome called hypertensive hypertrophic cardiomyopathy that included severe concentric cardiac hypertrophy, a small left ventricular cavity, and supernormal indexes of systolic function without concurrent medical illness or ischemic heart disease. The aim of this review was to highlighted this syndrome from pathophysiological, clinical, diagnostical view and clear all the possible correlations with genetic, inflammatory, and other markers.

Herzerkrankungen in der Schwangerschaft

During pregnancy, the changes of cardiovascular physiology can impose additional load and risk on the cardiovascular system of women with heart disease. Care of women with heart disease and childbearing potential should include preconception risk stratification and counselling. Risk stratification rests on a carefully obtained history and examination, electrocardiography and echocardiography. Exercise capacity is an important predictor of maternal cardiovascular events. High-risk conditions include severe pulmonary hypertension, cyanotic lesions, severe left ventricular obstruction and aortic disease in Marfan-Syndrome. High-risk patients should be referred to and cared for by tertiary centres. A multidisciplinary team approach with cardiologists, obstetricians and anaesthetists during pregnancy, delivery and the postpartum period is recommended. Meticulous attention should be paid to effective anticoagulation for prosthetic heart valves. Risks and benefits of the anticoagulation strategy should be fully discussed with the patient. Peripartal cardiomyopathy is a disease that occurs during he late stages of pregnancy and the peripartum period and is associated with congestive heart failure, thrombembolism, cardiac death and recurrence in subsequent pregnancies. Dilated cardiomyopathy with impaired functional reserve or markedly impaired left ventricular function constitutes a high risk for the pregnant women. In addition to sodium restriction, treatment of heart failure consists of loop diuretics, vasodilators, digoxin and beta-blockers, if appropriate. Coronary heart disease and myocardial infarction are rare during pregnancy but should be considered in women with chest pain. Pharmacological therapy of rhythm disorders should be reserved for arrhythmias resulting in maternal or fetal hemodynamic compromise and for arrhythmias with intolerable symptoms.

Cardiomyopathy: Magnetic Resonance Imaging Evaluation

The term “cardiomyopathy” encompasses a broad and multi-etiologic spectrum of different entities, all of which are characterized by myocardial disease associated with cardiac dysfunction. The most commonly used classification system has been published in the 1995 World Health Organization/International Society and Federation of Cardiology task force report and classifies the cardiomyopathies according to the dominant functional impairment as follows 1 : ● Dilated cardiomyopathy ● Hypertrophic cardiomyopathy ● Restrictive cardiomyopathy ● Arrhythmogenic right ventricular cardiomyopathy ● Unclassified cardiomyopathies It is important to keep in mind that overlapping forms exist and that patients may present with features of more than one category. Cardiomyopathies may be idiopathic or associated with an underlying cardiac or systemic disorder, so-called “specific cardiomyopathies.” The term “specific cardiomyopathy” includes ischemic, valvular, hypertensive, inflammatory and metabolic cardiomyopathies, myocardial involvement by general systemic diseases, muscular dystrophies, neuromuscular disorders, sensitivity and toxic reactions, and peripartal cardiomyopathy. 1 The objective of this article was to provide a comprehensive overview of the role of cardiac magnetic resonance (MR) imaging in the evaluation of the major nonischemic cardiomyopathies.

Long QT Syndrome - A case report

Long QT syndrome (LQTS) is an uncommon disease due to genetic defect and responsible for polymorphic VT (torsade-de pointes-TdP) and sudden cardiac death. A case of 25 year-old woman with palpitation, severe headache and recurrent syncopal episode since 16 year-old is reported. The ECG showed bigeminy ventricular premaure contraction (VPC) , prolonged QTc interval and abnormal T wave. Peripartal cardiomyopathy was diagnosed recently after the first delivery. In July 2002, she was hospitalized due to recurrent syncope, seizure proceeded by TdP and VF. On admission she need several times DC shock and temporary pacemaker with relatively high rate. Beta-blocker and implantation of dual chamber permanent pacemaker finally could control the malignant arrhythmias. During follow-up for 4 months, she was doing well and no syncopal episode occurred. (Med J Indones 2003; 12: 109-13) Keywords: LQTS, arrhythmia, pacemaker, beta-blocker

Peripartum cardiomyopathy. Perioperative anaesthesiological management of a rare complication of pregnancy

Peripartum cardiomyopathy is a rare disorder with an incidence from 1:3,000 to 1:15,000 live births and thus not often described in the anaesthesiology literature. The etiology of this disease is still not known but the symptoms are similar to idiopathic dilated cardiomyopathy. Echocardiographic findings show a dilatation of the left ventricle in addition to abnormal wall motion with a severe reduction of the cardiac function. Despite the rarity of this disorder, the anaesthesiologist or ICU physician should consider peripartal cardiomyopathy as a differential diagnosis to ensure an adequate perioperative management. There seems to be an increased incidence in pregnant women who are elderly (age >30 years),who have a history of gestosis/hypertension,have a gemini pregnancy or are of black origin. The prognosis depends on the recovery of the left ventricular contractility within the first 6 months after onset of the disease. The mortality rate is reported to vary between 25% and 50%. Heart transplantation is regarded as the last resort which has successfully been performed with several patients. This case describes the perioperative management of a 32-year-old women with peripartum cardiomyopathy.

Epidemiologie – Ätiologie der dilatativen Kardiomyopathie

Among the cardiomyopathies,--dilated cardiomyopathy (dcm), hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy--, dcm is the most frequent entity. Its prevalence in the United States amounts to 36 cases per 100,000 inhabitants, men being almost 3-fold more involved than women. The etiology of dcm is very heterogenous; 50% of the cases are due to idiopathic dcm whereas the other half comprises a broad spectrum of various etiologies such as myocarditis, ischemic heart disease, peripartal cardiomyopathy, hypertension, HIV infection, toxic cardiomyopathy and others. In 20 to 30% of the cases of idiopathic dcm a genetic transmission of the disease has been found. Another 20 to 30% of idiopathic dcm are associated with inflammatory and immunological phenomena. Infectious myocarditis with enteroviruses, especially with coxsackie-virus type B has been suggested to be an important trigger for an immune-mediated dcm. In both, familiar dcm and infection with coxsackie-virus B, an impairment of constituents of the myocardial cytoskeleton has been shown. This is regarded as a possible pathogenetic mechanism in the development of dcm.

Postpartum cardiomyopathy and acute myocarditis

Postpartum cardiomyopathy may be defined as congestive cardiac failure due to primary myocardial disease occurring in the first 5 months after delivery. Various terms such as postpartum heart disease,’ peripartum cardiomyopathy,2 idiopathic myocardiopathy of the puerperium,3 pregnancy cardiomyopathy,4 and peripartal cardiomyopathy> have been applied in the literature to delimit this entity. Clinical presentation is characterized by insidious left-sided cardiac failure, which is invariably followed by varying intervals by symptoms of right ventricular failure. We report a case of postpartum cardiomyopathy whose initial clinical presentation consisted of repeated pulmonary thromboembolism and histologic findings of acute inflammatory myocarditis in myocardial biopsy. A 24-year-old woman presented 3 months after the birth of her third child with cough, bilateral pleuritic pain, and dyspnea. There was no history of previous illness, alcoholic abuse, or anovulatory drug self-administration. Routine ECG (Fig. 1) and chest x-ray examination performed 1 year prior to admission were normal. Physi&l examination revealed cyanosis, tachycardia, elevated jugular venous pressure, and hepatomegaly. Present were hypoxemia, hypocapnia, and ECG findings of right heart strain (Fig. 2). A pulmonary perfusion-ventilation scan showed multiple and bilateral filling defects. Gynecologic examination, upper abdomen ultrasound record, lower limbs, and pelvic venography were normal. M-mode and

Maternal death associated with sickle cell trait

A case report is presented of a patient with sickle cell trait who died an abrupt intrapartum death precipitated by probable peripartal cardiomyopathy and apparently aggravated by intravascular sickling. Notable autopsy findings included massive sicklemia in all organ systems.

Peripartal cardiomyopathy

A case of peripartal cardiomyopathy diagnosed post partum by preoperative routine examination for a sterilization operation is discussed. Data from the literature show that 50% of the patients suffering from peripartal cardiomyopathy recover nearly completely; the other patients with distinct symptoms of cardial insufficiency die almost without exception from the disease. Current concepts of diagnosis and treatment are reviewed.

Variable Prognosis in Congestive Cardiomyopathy

Prognosis of 36 patients with congestive cardiomyopathy was studied in relation to various clinical factors. Half life of the survival curve after overt heart failure was about 7 years. Although left ventricular function was a major determinant of clinical course in congestive cardiomyopathy in general, its relation to prognosis was variable according to the type of cardiac involvement. In peripartal cardiomyopathy and in a type of cardiomyopathy named subacute cardiomyopathy with pulmonary thrombosis in this paper, factor(s) other than left ventricular function, possibly including pulmonary thrombosis, may be operative as more important determinant of extremely poor prognosis in these subtypes. Alcoholic cardiomyopathy was also unique in its favorable prognosis in association with reversible cardiomegaly following abstention from alcohol.

Peripartal cardiomyopathy in Japan. A critical reappraisal of the concept.

Clinical and autopsy records of our 5 cases of peripartal cardiomyopathy, as well as those of 18 reported Japanese cases, were analyzed and were compared to our findings in 8 non-peripartal female and 34 male patients of primary cardiomyopathy.The incidence of cardiomyopathy was significantly higher in peripartal period than in the rest of the adult period in 10 married female patients (P<0.002).The clinical picture of peripartal cardiomyopathy was characterized by acute onset of severe dyspnea and subsequent subacute course leading either to early death or to complete recovery. Short history simulating upper respiratory infection preceded severe symptoms in some cases, and suggestions for viral infection were obtained in a few patients.Autopsy revealed intracardiac thrombi and pulmonary and systemic thromboembolism, but only minimal histological change of the myocardium in many cases.The nature of this disease as a primary cardiomyopathy of usual sense was doubted seriously and essential contribution of intravascular blood coagulation under possible participation of viral infection was speculated.