scholarly journals A case report of a 40-year-old woman with endomyocardial fibrosis in a non-tropical area: from initial presentation to high urgent heart transplantation

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Gernot Wagner ◽  
Markus Haumer ◽  
Gerhard Poelzl ◽  
Dominik Wiedemann ◽  
Andreas Kliegel ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Chest Pain ◽  
Heart Transplantation ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular ◽  
Diagnostic Workup ◽  
Lv Function ◽  
Endomyocardial Fibrosis ◽  
Tropical Regions

Abstract Background Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Diagnosis is based on patient history, heart failure symptoms, and echocardiographic signs of restrictive ventricular filling, atrioventricular valve regurgitation and frequently apical thrombus. Following is a case report of an Austrian patient with EMF who eventually had to undergo a heart transplant. This case report strives to promote awareness for this in non-tropical areas uncommon but nevertheless detrimental disease. Case presentation A 40-year-old woman was presented at our emergency department with chest pain and fever up to 38.1° Celsius. Plasma troponin-T levels and inflammatory markers were slightly elevated, but the echocardiogram was without pathological findings. The patient was hospitalized on the suspicion of acute myocarditis and discharged soon after improvement. Eight months later, she was presented again with chest pain and symptoms of heart failure. The echocardiogram showed normal systolic left ventricular (LV) function with LV wall thickening and severe restrictive mitral regurgitation as well as aortic and tricuspid regurgitation. Coronary angiogram was normal but right heart catheterization showed pulmonary hypertension due to left heart disease. Further diagnostic workup with cardiac magnetic resonance imaging revealed subendocardial late enhancement and apical thrombus formation in the left ventricle compatible with the diagnosis of EMF. A comprehensive diagnostic workup showed no evidence of infection, systemic immunologic or hematological disease, in particular hypereosinophilic syndrome. After a multidisciplinary consideration of several therapeutic options, the patient was listed for heart transplantation. On the waiting list, she deteriorated rapidly due to progressive heart failure and finally underwent a heart transplantation. Histological examination confirmed the diagnosis of EMF. Six years after her heart transplantation, the patient was presented in an excellent clinical condition. Conclusions Even in non-tropical regions, the diagnosis of EMF should always be considered in restrictive cardiomyopathy. Knowledge of the distinct phenotype of EMF facilitates diagnosis, but comprehensive workup and therapeutic management remain challenging and require a multidisciplinary approach.

scholarly journals Endomyocardial Fibrosis in non-tropical patient with no obvious eosinophilia presented with chest pain mimicking ACS and left ventricular thrombus

2021 ◽  
Author(s):  
ahmad matarneh ◽  
Yousef Hailan ◽  
Sabir Abdulkarim ◽  
Maryam Alkuwari ◽  
Wafer dabdoob
Keyword(s):  
Heart Failure ◽  
Chest Pain ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Thrombus ◽  
Endomyocardial Fibrosis ◽  
Ventricular Thrombus

Endomyocardial fibrosis is a disease that causes restrictive cardiomyopathy, it varies in presentation ranging from asymptomatic to full-blown heart failure. its pathogenesis is poorly understood and it poses a diagnostic and treatment challenge due to its infrequent occurrence.

scholarly journals Idiopathic endomyocardial fibrosis in a Western European: a case report

2020 ◽  
Vol 4 (3) ◽  
pp. 1-5
Author(s):  
Guillaume Théry ◽  
Laurent Faroux ◽  
Philippe Deleuze ◽  
Damien Metz
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Restrictive Cardiomyopathy ◽  
Functional Class ◽  
First Episode ◽  
Endomyocardial Fibrosis ◽  
Optimal Management ◽  
Poor Outcomes ◽  
End Stage

Abstract Background Endomyocardial fibrosis (EMF) is a rare cause of restrictive cardiomyopathy, mainly found in tropical/subtropical country. Endomyocardial fibrosis causes severe congestive symptoms and may lead to end-stage heart failure. Case summary A French Caucasian 44-year-old man without noticeable medical history and who had never travelled outside of France was hospitalized for a first episode of acute heart failure revealing an atypical appearance of the left ventricle. Cardiac magnetic resonance (CMR) identified EMF, but investigations did not identify any aetiology (no eosinophilia). Despite optimal management of chronic heart failure, functional class declined rapidly resulting in several hospitalizations for heart failure. The patient finally underwent an elective heart transplantation with good results at 6-month follow-up. Discussion Endomyocardial fibrosis exact physiopathology remains unclear, although association with eosinophilia has been reported. Diagnosis is challenging and is based on multi-modal imagery with a central role of CMR. There is no consensus on optimal management, medical therapy having poor outcomes and rate of peri-operative complications being high. Heart transplantation should be considered for eligible patients.

scholarly journals Left Ventricular Thrombus as a Complication of Clozapine-Induced Cardiomyopathy: A Case Report and Brief Literature Review

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Shahbaz A. Malik ◽  
Sarah Malik ◽  
Taylor F. Dowsley ◽  
Balwinder Singh
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Chest Pain ◽  
New Left ◽  
Left Ventricular ◽  
Heart Failure Therapy ◽  
Bundle Branch Block ◽  
Ventricular Thrombus ◽  
First Case ◽  
History Of

A 48-year-old male with history of schizoaffective disorder on clozapine presented with chest pain, dyspnea, and new left bundle branch block. He underwent coronary angiography, which revealed no atherosclerosis. The patient’s workup was unrevealing for a cause for the cardiomyopathy and thus it was thought that clozapine was the offending agent. The patient was taken off clozapine and started on guideline directed heart failure therapy. During the course of hospitalization, he was also discovered to have a left ventricular (LV) thrombus for which he received anticoagulation. To our knowledge, this is the first case report of clozapine-induced cardiomyopathy complicated by a LV thrombus.

scholarly journals 125 Sex differences in myocarditis natural history

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Matteo Castrichini ◽  
Marco Merlo ◽  
Chiara Baggio ◽  
Giulia Gagno ◽  
Davide Maione ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chest Pain ◽  
Natural History ◽  
Heart Transplantation ◽  
Ventricular Arrhythmias ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Definite Diagnosis ◽  
The Impact

Abstract Aims The role of sex in determining the profile and the outcomes of patients with myocarditis is widely unexplored. Our study seeks to evaluate the impact of sex as a modifier factor in the clinical characterization and natural history of patients with definite diagnosis of myocarditis. Methods and results We retrospectively analysed a single-centre cohort of consecutive patients with definite diagnosis (i.e., endomyocardial biopsy or cardiac magnetic resonance proven) of myocarditis. A sub-analysis was performed after division of population according to the main symptom of presentation (i.e., chest pain, ventricular arrhythmias, and heart failure). Clinical and echocardiographic data were evaluated at diagnosis and at last available evaluation (i.e., median of 30 months). The study outcome measure was a composite of all-cause mortality or heart transplantation. We enrolled 312 patients (187; 60% presenting with chest pain; 19; 6% with ventricular arrhythmias; 106; 34% with heart failure). Most of patients (211, 68% of the whole population) were males, consistently in the three modes of presentation. Despite no clinically relevant differences were found at baseline presentation, males presented a larger indexed left ventricular end-diastolic volume (LVEDVi) (62 ± 23 vs. 52 ± 20, P = 0.011 in males vs. females respectively) at follow-up evaluation. At a median follow-up of 62 months, 36 (17%) males vs. females experienced death or heart transplantation (Log-rank P = 0.037). At multivariable Cox analysis, male sex emerged as a predictor of mortality (HR: 2.358; 1.044–5.322; P = 0.039 and left ventricular ejection fraction (LVEF) < 50% (HR: 8.169; 1.226–54.425; P = 0.030)]. Results were consistent in patients presenting with heart failure and chest pain, while arrhythmic group was too small to be reliably interpreted. Conclusions In a large cohort of patients with definite diagnosis of myocarditis, females experienced a more favorable long-term prognosis than male, despite a similar clinical profile at baseline.

scholarly journals Normalization of left ventricular filling pressure after cardiac surgery for the Loeffler’s endocarditis: a case report

2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Andrea Carcaterra ◽  
Stéphane Mock ◽  
Hajo Müller ◽  
Ariane Testuz
Keyword(s):  
Heart Failure ◽  
Cardiac Involvement ◽  
Hypereosinophilic Syndrome ◽  
Rapid Evolution ◽  
Balance Sheet ◽  
Heart Association ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular ◽  
Filling Pressure ◽  
Endomyocardial Fibrosis

Abstract Background Loeffler endocarditis is a rare restrictive cardiomyopathy, characterized by hypereosinophilia and fibrous thickening of the endocardium causing progressive onset of heart failure and appearance of thrombi on the walls of the heart chambers. Case summary A 72-year-old man known for hypertension and dyslipidaemia consults for progressive dyspnoea up to New York Heart Association (NYHA) Classes 2–3 over 3 weeks. The biological balance sheet shows a high eosinophil level and an echocardiography shows a mild echodensity fixed to the left apex. After exclusion of a secondary cause of hypereosinophilia, diagnosis of endomyocardial fibrosis in the context of a hypereosinophilic syndrome (HES) is therefore retained. The patient’s clinical presentation with cardiac involvement leads us to start a treatment with corticosteroids. The patient is then regularly followed every 6 months with an initially stable course without complications. Two years later, he develops progressive signs of heart failure. Transthoracic echocardiography shows a left ventricular (LV) dilatation with a normal ejection fraction, but decreased volume due to a large echodense mass in the apex, and moderate aortic regurgitation caused by myocardial infiltration. In view of this rapid evolution, resection of the LV mass with concomitant aortic valve replacement is performed. Pathology confirms eosinophilic infiltration. The clinical course is very good with a patient who remains stable with dyspnoea NYHA Classes 1–2, and echocardiography at 1 year shows a normalization of LV filling pressure. Discussion HES represents a heterogeneous group of disorders characterized by overproduction of eosinophils. One of the major causes of mortality is associated cardiac involvement. Endocardial fibrosis and mural thrombosis are frequent cardiac findings. Echocardiography plays a crucial role in initial diagnosis of endomyocardial fibrosis, and for regular follow-up in order to adapt medical treatment and monitor haemodynamic evolution of the restrictive physiology and of valvular damage caused by the disease’s evolution. This case also shows that surgery can normalize filling pressure and allow a clear improvement on the clinical condition even at the terminal fibrotic state.

scholarly journals Challenges in the diagnosis of peripartum cardiomyopathy: a case series

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Fabio Chirillo ◽  
Anna Baritussio ◽  
Umberto Cucchini ◽  
Ermanno Toniolli ◽  
Angela Polo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Multimodality Imaging ◽  
Systolic Dysfunction ◽  
Case Series ◽  
Left Ventricular ◽  
Peripartum Cardiomyopathy ◽  
Lv Function ◽  
Prognostic Information ◽  
Clinical Scenarios ◽  
Anterolateral Wall

Abstract Background Peripartum cardiomyopathy (PPCM) is usually characterized by overt heart failure, but other clinical scenarios are possible, sometimes making the diagnosis challenging. Case summary We report a case series of four patients with PPCM. The first patient presented with acute heart failure due to left ventricular (LV) systolic dysfunction. Following medical treatment, LV function recovered completely at 1 month. The second patient had systemic and pulmonary thromboembolism, secondary to severe biventricular dysfunction with biventricular thrombi. The third patient presented with myocardial infarction with non-obstructed coronary arteries and evidence of an aneurysm of the mid-anterolateral LV wall. The fourth patient, diagnosed with PPCM 11 years earlier, presented with sustained ventricular tachycardia. A repeat cardiac magnetic resonance, compared to the previous one performed 11 years earlier, showed an enlarged LV aneurysm in the mid-LV anterolateral wall with worsened global LV function. Discussion Peripartum cardiomyopathy may have different clinical presentations. Attentive clinical evaluation and multimodality imaging can provide precise diagnostic and prognostic information.

scholarly journals Left ventricular systolic function decreases in lamin a/c cardiomyopathy wihout concomitant ventricular dilatation

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
ES Eystein Skjolsvik ◽  
OL Oyvind Haugen Lie ◽  
MC Monica Chivulescu ◽  
MR Margareth Ribe ◽  
AIC Anna Isotta Castrini ◽  
...  
Keyword(s):  
Heart Failure ◽  
Left Ventricular ◽  
University Hospital ◽  
Lv Function ◽  
Lamin A ◽  
Age Related ◽  
End Stage Heart Failure ◽  
Lv Volumes ◽  
End Stage

Abstract Funding Acknowledgements Type of funding sources: Foundation. Main funding source(s): This work was supported by the Norwegian Research Council [203489/030] onbehalf Department of Cardiology, Research group for genetic cardiac diseases and sudden cardiac death, Oslo University Hospital, Rikshospitalet, Oslo, Norwa Background Lamin A/C disease is an inheritable cardiomyopathy characterized by conduction abnormalities, ventricular arrhythmias and end stage heart failure with complete age-related penetrance. Purpose To assess left ventricular structural and functional progression in patients with lamin A/C cardiomyopathy. Methods We included and followed consecutive lamin A/C genotype positive patients with clinical examination and echocardiography at every visit. We evaluated progression of left- ventricular size and function by mixed model statistics. Results We included 101 consecutive lamin A/C genotype positive patients (age 44 [29-54] years, 39% probands, 51%female) with 576 echocardiographic exams during 4.9 (IQR 2.5-8.1) years of follow-up. LV ejection fraction (LVEF) declined from 50 ± 12% to 47 ± 13%, p < 0.001 (rate -0.5%/year). LV end diastolic volumes (LVEDV) remained stationary with no significant dilatation in the total population (136 ± 45ml to 138 ± 43ml, p = 0.60), (Figure). In the subgroup of patients >58 years, we observed a decline in LV volumes 148, SE 9 ml to 140, SE 9 ml p < 0.001 (rate -2.7 ml/year) towards end stage heart failure. Conclusions LVEF deteriorated, while LV size remained unchanged during 4.9 years of follow-up in patients with lamin A/C cardiomyopathy. In patients <58 years, we observed a reduction in LV volumes. These findings represent loss of LV function without the necessary compensatory dilation to preserve stroke volume indicating high risk of decompensated end stage heart failure in lamin A/C. Abstract Figure.

scholarly journals Uric Acid, Oxidative Stress and Inflammation in Chronic Heart Failure with Reduced Ejection Fraction

2015 ◽  
Vol 23 (4) ◽  
pp. 397-406 ◽  
Author(s):  
Adriana Iliesiu ◽  
Alexandru Campeanu ◽  
Daciana Marta ◽  
Irina Parvu ◽  
Gabriela Gheorghe
Keyword(s):  
Oxidative Stress ◽  
Heart Failure ◽  
Uric Acid ◽  
Chronic Heart Failure ◽  
Ejection Fraction ◽  
Xanthine Oxidase ◽  
Left Ventricular ◽  
Paraoxonase 1 ◽  
Lv Function ◽  
The Relationship

Abstract Background. Oxidative stress (OS) and inflammation are major mechanisms involved in the progression of chronic heart failure (CHF). Serum uric acid (sUA) is related to CHF severity and could represent a marker of xanthine-oxidase activation. The relationship between sUA, oxidative stress (OS) and inflammation markers was assessed in patients with moderate-severe CHF and reduced left ventricular (LV) ejection fraction (EF). Methods. In 57 patients with stable CHF, functional NYHA class III, with EF<40%, the LV function was assessed by N-terminal of the prohormone brain natriuretic peptide (NT-proBNP) levels and echocardiographically through the EF and E/e’ ratio, a marker of LV filling pressures. The relationship between LV function, sUA, malondialdehyde (MDA), myeloperoxidase (MPO), paraoxonase 1 (PON-1) as OS markers and high sensitivity C-reactive protein (hsCRP) and interleukin 6 (IL-6) as markers of systemic inflammation was evaluated. Results. The mean sUA level was 7.9 ± 2.2 mg/dl, and 61% of the CHF patients had hyperuricemia. CHF patients with elevated LV filling pressures (E/e’ ≥ 13) had higher sUA (8.6 ± 2.3 vs. 7.3 ± 1.4, p=0.08) and NT-proBNP levels (643±430 vs. 2531±709, p=0.003) and lower EF (29.8 ± 3.9 % vs. 36.3 ± 4.4 %, p=0.001). There was a significant correlation between sUA and IL-6 (r = 0.56, p<0.001), MDA (r= 0.49, p= 0.001), MPO (r=0.34, p=0.001) and PON-1 levels (r= −0.39, p= 0.003). Conclusion. In CHF, hyperuricemia is associated with disease severity. High sUA levels in CHF with normal renal function may reflect increased xanthine-oxidase activity linked with chronic inflammatory response.

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