Dilated Cardiomyopathy Presenting Research Articles

54-Year-Old Man With Acute Dyspnea on Exertion

54-Year-Old Man With Acute Dyspnea on Exertion

IRON OVERLOAD CARDIOMYOPATHY IN THALASSEMIA MAJOR :A CASE REPORT

BACKGROUND: Cardiomyopathy is a major cause of morbidity and mortality in patients with thalassemia .Repeated blood transfusions leads to iron overload culminating in heart failure & death CASE PRESENTATION:A 16 year old boy known case of β-thalassemia major on multiple blood transfusions and iron chelation therapy presented to emergency with features of congestive cardiac failure, predominantly of right heart failure with pedal edema and ascites .on evaluation Transthoracic echocardiography showed dilated cardiomyopathy and raised cardiac enzymes .patient was started on guideline directed management for heart failure along with iron chelation therapy CONCLUSION: This is a case of β- thalassemia major with severe iron overload leading to dilated cardiomyopathy presenting with congestive cardiac failure.

Efficacy of ivabradine in HIV-associated dilated cardiomyopathy.

Ivabradine is a pure heart rate-lowering drug that acts directly on the sinus node and does not affect atrioventricular or intraventricular conduction times, myocardial contractility or ventricular repolarization. Ivabradine is used in the treatment of adults with New York Heart Association (NYHA) class II-IV chronic heart failure (CHF) with systolic dysfunction, in sinus rhythm, with a heart rate of ≥75 beats per minute (bpm). This report describes the beneficial effect of ivabradine on clinical parameters in a HIV-infected patient with dilated cardiomyopathy presenting with NYHA class III CHF symptoms. Ivabradine improved functional capacity and left ventricular (LV) systolic function in our patient with HIV-related dilated cardiomyopathy and CHF. This report also lists the summaries of potential clinically significant interaction, likely to require additional monitoring, alteration of drug dosage or timing of administration, between ivabradine and antiretroviral drugs.

Clinical presentation of dilated cardiomyopathy in patients above age of 45 years and its correlation with electrocardiography and echocardiography

Clinical presentation of dilated cardiomyopathy in patients above age of 45 years and its correlation with electrocardiography and echocardiography

Machine Learning Outcome Prediction in Dilated Cardiomyopathy Using Regional Left Ventricular Multiparametric Strain.

The clinical presentation of idiopathic dilated cardiomyopathy (IDCM) heart failure (HF) patients who will respond to medical therapy (responders) and those who will not (non-responders) is often similar. A machine learning (ML)-based clinical tool to identify responders would prevent unnecessary surgery, while targeting non-responders for early intervention. We used regional left ventricular (LV) contractile injury patterns in ML models to identify IDCM HF non-responders. MRI-based multiparametric strain analysis was performed in 178 test subjects (140 normal subjects and 38 IDCM patients), calculating longitudinal, circumferential, and radial strain over 18 LV sub-regions for inclusion in ML analyses. Patients were identified as responders based upon symptomatic and contractile improvement on medical therapy. We tested the predictive accuracy of support vector machines (SVM), logistic regression (LR), random forest (RF), and deep neural networks (DNN). The DNN model outperformed other models, predicting response to medical therapy with an area under the receiver operating characteristic curve (AUC) of 0.94. The top features were longitudinal strain in (1) basal: anterior, posterolateral and (2) mid: posterior, anterolateral, and anteroseptal sub-regions. Regional contractile injury patterns predict response to medical therapy in IDCM HF patients, and have potential application in ML-based HF patient care.

Vitamin D Deficiency Induced Infantile Heart Failure in the Twenty-First Century: A Fault in Public Health Primary Prevention

Introduction Vitamin D deficiency and its consequence continues to be reported even in the current era of established guidelines for vitamin D supplementation in all infants. We present a case of infantile dilated cardiomyopathy presenting with cardiogenic shock caused by persistent hypocalcemia secondary to vitamin D deficiency. We hope to bring attention to this easily treatable and preventable public health issue. Case Report A seven month old, exclusively breastfed, African American male presented in March to the emergency department with initial …

ELECTROCARDIOGRAPHIC PROFILE OF DILATED CARDIOMYOPATHY IN PATIENTS ATTENDING A TERTIARY CARE HOSPITAL OF WESTERN NEPAL

INTRODUCTION: Cardiomyopathy is a disease of the heart muscle that leads to deterioration of myocardial functioning. It has different morphological variation with dilated cardiomyopathy being the most common form comprising over 90% of all cases. Heart failure (HF) is the most common clinical presentation of dilated cardiomyopathy. Various electrocardiographic changes are common in these patients. The most common cause of sudden death in these patients is arrhythmia. This study aimed at characterizing different electrocardiographic changes in patients of dilated cardiomyopathy admitted for heart failure.MATERIAL AND METHODS: A retrospective analysis of records of admitted patients from l" January 2015 to 30th April 2018 was conducted. Study was conducted after obtaining ethical clearance from institutional review board. Data was collected in a preformed proforma and analyzed using SPSS 20.0 version.RESULTS: Consecutive 400 cases of dilated cardiomyopathy with systolic dysfunction (LVEF<40%) were taken for the study. Two hundred and fifty two (63 %) were male. The mean age of patients was 59.33 (± 15.76) years. The most common risk factors in our patients were smoking (66%) followed by systemic hypertension (49%), IHD (32%), and diabetes mellitus (33%) respectively. Out of 400 patients, abnormal ECG were found in 82.5%. The most common abnormality was LVH (30.7%) followed by LBBB (22.5%) and AF (20.5%).CONCLUSION: The percentage of abnormal electrocardiographic findings was high in our study. All patients of dilated cardiomyopathy should undergo electrocardiography screening for risk stratification and to prevent serious consequences with early necessary intervention.Journal of Universal College of Medical SciencesVol. 6, No. 1, 2018, Page: 40-45

X-Linked Dilated Cardiomyopathy Presenting as Acute Rhabdomyolysis and Presumed Epstein-Barr Virus-Induced Viral Myocarditis: A Case Report

Patient: Male, 23Final Diagnosis: Primary X-linked dilated cardiomyopathySymptoms: RhabdomyolysisMedication: —Clinical Procedure: —Specialty: General and Intrenal MedicineObjective:Rare co-existance of disease or pathologyBackground:Rhabdomyolysis and primary dilated cardiomyopathies without skeletal muscle weakness are rare features of X-linked dystrophinopathies. We report a rare case of an X-linked dilated cardiomyopathy (XLDCM) presenting with acute rhabdomyolysis and myocarditis. We illustrate the confounding diagnostic influence of a reactivated, persistent EBV myocarditis as the presumed cause for this patient’s XLDCM.Case Report:A 23-year-old Australian man presented with acute rhabdomyolysis and elevated creatine kinase (CK) levels. He was managed conservatively with intravenous hydration and developed acute pulmonary edema. Cardiac MRI and transthoracic echocardiogram revealed a dilated cardiomyopathy and viral myocarditis. Extensive serological investigations identified reactivation of EBV, which was presumed to account for his viral myocarditis. The patient recovered and was discharged with down-trending CK levels. Follow-up transthoracic echocardiograms and cardiac MRI showed a persisting dilated cardiomyopathy. His CK continued to remain elevated and his EBV IgM serology remained positive. An inflammatory polymyositis with either a primary autoimmune pathophysiology or secondary to a chronic EBV infection was considered. Oral corticosteroids were trialed and reduced his CK significantly until therapy was ceased. Massively parallel sequencing eventually identified a two-exon deletion targeting Xp21 consistent with the diagnosis of a rare XLDCM.Conclusions:Rhabdomyolysis and co-existing primary dilated cardiomyopathies are rare diagnostic manifestations in a minority of X-linked dystrophinopathies. Chronic viral infections and their reactivation may complicate the diagnostic process and incorrectly attribute an inherited cardiomyopathy to an acquired infective etiology. EBV reactivation rarely induces myocarditis. Therefore, primary and unresolving dilated cardiomyopathy with persistently elevated CK must prompt consideration of an underlying dystrophinopathy.

Electrical storm in dilated cardiomyopathy treated using epicardial radiofrequency ablation as a first line therapy

We report a patient with non-ischemic dilated cardiomyopathy and low left ventricular systolic function (28%) presenting with an electrical storm originated in epicardial scar and ablated by radiofrequency. This case report suggests that a strategy of epicardial catheter ablation is reasonable for the patient presenting with electrical storm related to structural disease with a low left ventricular ejection fraction.

Effect of hypertension at presentation on prognosis in patients with dilated cardiomyopathy presenting with normal renal angiogram.

Background & objectives:Dilated cardiomyopathy (DCM) is a progressive disease of heart with systolic and diastolic dysfunction carrying a poor long-term prognosis. The prognostic index and predictors of mortality are considered to be useful in guiding the treatment. This study was undertaken to evaluate the effects of hypertension at presentation on prognosis in patients with DCM presenting with normal renal and coronary angiogram.Methods:An observational, analytical, non-interventional and a combination of retrospective and prospective study was conducted in patients between 15 and 75 yr of age with DCM having on and off symptoms while receiving treatment in a cardiology outpatient department for more than a year. Sixty patients who fulfilled the inclusion criteria were enrolled in the study. Left ventricular systolic and diastolic functions were assessed by echocardiography along with New York Heart Association (NYHA) functional class prospectively and at baseline retrospectively. Patients were grouped into two categories: DCM with hypertension at presentation (HTNAP, Category 1) and DCM without hypertension at presentation (NHTNAP, Category 2). The primary end-points were the number and dose of parenteral drugs at hospitalization, duration of hospital stay and change in the left ventricular (LV) systolic function expressed as LV ejection fraction, and the secondary end-points included overall mortality, change in LV and right ventricular systolic and diastolic functions and change in the NYHA functional class between baseline and three month follow up in patients.Results:Thirty five and 25 patients presented with HTNAP and NHTNAP, respectively (total 60). The overall mortality was 10 per cent (6/60). The number of hospitalizations was less in HTNAP category and of days of hospital stay was 6.3 in HTNAP and 9.8 in NHTNAP, the difference being significant (P < 0.001). The HTNAP category required less parenteral diuretics and inotropes compared with the NHTNAP category. The echocardiographic parameters showed better improvements in the HTNAP group as compared to the NHTNAP group. Overall, the patients in the <35 yr of age showed the best prognosis (P < 0.001).Interpretation & conclusions:Normal or high blood pressure response at acute presentation of DCM leads to better prognosis which may be due to an intact renovascular and an active sympathetic system and can depict the stage of DCM.

Psoriasis induced toxic shock-like syndrome mimicking hemolytic uremic syndrome

Introduction: Over the past decade the incidence of invasive group A streptococcal infections causing streptococcal toxic shock syndrome (stss) or toxic shock like syndrome has been increasing. Group A streptococcus (Streptococcus pyogenes) can cause several different conditions including simple cases such as pharyngitis to severe cases involving bacteremia. case report: A 37-year-old female with history of dilated cardiomyopathy and psoriasis presenting with multiple episodes of watery diarrhea, multi- organ dysfunction, septic shock with an unclear underlying etiology later found to be secondary to toxic shock-like syndrome. conclusion: the mortality rate associated with toxic shock-like syndrome has been found to be high and a patient normally presents with signs of high fever, hypotension, liver injury, acute renal failure, coagulopathy, and at times soft tissue infection. It can be a difficult diagnosis to make as it can involve multi-organ dysfunction and as cultures are not readily available at time of presentation; its awareness needs to be differentiated to that

Peripartum cardiomyopathy and familial dilated cardiomyopathy : a tale of two cases : online article - case report

Peripartum cardiomyopathy (PPCM) is a form of pregnancyrelated heart failure that is associated with considerable morbidity and mortality. Most patients present with acute postpartal heart failure that otherwise resembles the clinical presentation of dilated cardiomyopathy (DCM). There is increasing recognition that PPCM may be due to genetic factors in a significant proportion of cases. There is evidence that at least 7% of cases of PPCM may be part of the spectrum of familial DCM. We report on two cases of PPCM, with relatives demonstrating familial DCM, both patients displaying autosomal dominant patterns of inheritance, and showing severe cardiomyopathy among proband and affected relatives. Family screening for familial DCM should be indicated in all cases of unexplained PPCM.

How can optimization of medical treatment avoid unnecessary implantable cardioverter-defibrillator implantations in patients with idiopathic dilated cardiomyopathy presenting with “SCD-HeFT Criteria?”

Background: To assess the proportion and long-term outcomes of patients with idiopathic dilated cardiomyopathy and potential indications for implantable cardioverter-defibrillator before and after optimization of medical treatment, 503 consecutive patients with idiopathic dilated cardiomyopathy were evaluated from 1988 to 2006. Results: A total of 245 patients (49%) satisfied the “Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) criteria,” defined as a left ventricular ejection fraction of 0.35 or NYHA class I), 125 were evaluated after 5.5 ± 2 months. Of these 227 patients, 13 (10%) developed “SCD-HeFT criteria” (group B1), 111 (89%) remained without “SCD-HeFT criteria” (group B2), and 1 (1%) had worsened to NYHA class IV. The 10-year mortality/heart transplantation and sudden death/sustained ventricular arrhythmia rate was 57% and 37% in group A1, 23% and 20% in group A2 (p < 0.001 for mortality/heart transplantation and p – 0.014 for sudden death/sustained ventricular arrhythmia vs. group A1), 45% and 41% in group B1 (p – NS vs. group A1), 16% and 14% in group B2 (p – NS vs. group A2), respectively. Conclusion: Two thirds of patients with idiopathic dilated cardiomyopathy and “SCD-HeFT criteria” at presentation did not maintain implantable cardioverter-defibrillator indications 3–9 months later with optimal medical therapy. Their long-term outcome was excellent, similar to that observed for patients who had never met the “SCD-HeFT criteria.”

Autosomal Recessive Dilated Cardiomyopathy due to DOLK Mutations Results from Abnormal Dystroglycan O-Mannosylation

Genetic causes for autosomal recessive forms of dilated cardiomyopathy (DCM) are only rarely identified, although they are thought to contribute considerably to sudden cardiac death and heart failure, especially in young children. Here, we describe 11 young patients (5–13 years) with a predominant presentation of dilated cardiomyopathy (DCM). Metabolic investigations showed deficient protein N-glycosylation, leading to a diagnosis of Congenital Disorders of Glycosylation (CDG). Homozygosity mapping in the consanguineous families showed a locus with two known genes in the N-glycosylation pathway. In all individuals, pathogenic mutations were identified in DOLK, encoding the dolichol kinase responsible for formation of dolichol-phosphate. Enzyme analysis in patients' fibroblasts confirmed a dolichol kinase deficiency in all families. In comparison with the generally multisystem presentation in CDG, the nonsyndromic DCM in several individuals was remarkable. Investigation of other dolichol-phosphate dependent glycosylation pathways in biopsied heart tissue indicated reduced O-mannosylation of alpha-dystroglycan with concomitant functional loss of its laminin-binding capacity, which has been linked to DCM. We thus identified a combined deficiency of protein N-glycosylation and alpha-dystroglycan O-mannosylation in patients with nonsyndromic DCM due to autosomal recessive DOLK mutations.

How Can Optimization of Medical Treatment Avoid Unnecessary Implantable Cardioverter-Defibrillator Implantations in Patients With Idiopathic Dilated Cardiomyopathy Presenting With “SCD-HeFT Criteria?”

To assess the proportion and long-term outcomes of patients with idiopathic dilated cardiomyopathy and potential indications for implantable cardioverter-defibrillator before and after optimization of medical treatment, 503 consecutive patients with idiopathic dilated cardiomyopathy were evaluated from 1988 to 2006. A total of 245 patients (49%) satisfied the "Sudden Cardiac Death in Heart Failure Trial (SCD-HeFT) criteria," defined as a left ventricular ejection fraction of ≤0.35 and New York Heart Association (NYHA) class II-III on registration. Among these, 162 (group A) were re-evaluated 5.4 ± 2 months later with concurrent β-blockers and angiotensin-converting enzyme inhibitor use. Of the 162 patients, 50 (31%) still had "SCD-HeFT criteria" (group A1), 109 (67%) had an improved left ventricular ejection fraction and/or New York Heart Association class (group A2), and 3 (2%) were in NYHA class IV. Of the 227 patients without baseline "SCD-HeFT criteria" (left ventricular ejection fraction >0.35 or NYHA class I), 125 were evaluated after 5.5 ± 2 months. Of these 227 patients, 13 (10%) developed "SCD-HeFT criteria" (group B1), 111 (89%) remained without "SCD-HeFT criteria" (group B2), and 1 (1%) had worsened to NYHA class IV. The 10-year mortality/heart transplantation and sudden death/sustained ventricular arrhythmia rate was 57% and 37% in group A1, 23% and 20% in group A2 (p <0.001 for mortality/heart transplantation and p = 0.014 for sudden death/sustained ventricular arrhythmia vs group A1), 45% and 41% in group B1 (p = NS vs group A1), 16% and 14% in group B2 (p = NS vs group A2), respectively. In conclusion, two thirds of patients with idiopathic dilated cardiomyopathy and "SCD-HeFT criteria" at presentation did not maintain implantable cardioverter-defibrillator indications 3 to 9 months later with optimal medical therapy. Their long-term outcome was excellent, similar to that observed for patients who had never met the "SCD-HeFT criteria."

Efficacy of ivabradine in idiopathic dilated cardiomyopathy patients with chronic heart failure

Abstract Background The aim of this study was to investigate the effect of ivabradine on symptoms, quality of life, effort tolerance, and echocardiographic parameters in patients with idiopathic dilated cardiomyopathy presenting with New York Heart Association (NYHA) class III or IV heart failure (HF) symptoms. Methods We screened 167 patients hospitalized for NYHA class III or IV chronic HF symptoms and left ventricular (LV) ejection fraction Results After 3 months’ treatment, adding ivabradine significantly reduced the heart rate from 96 to 72 bpm ( p p = 0.045), NYHA class symptoms ( p = 0.004), exercise tolerance ( p = 0.03), and quality of life ( p = 0.02). The average number of hospitalizations for HF over a mean longer-term follow-up of 13.5 months was 1.0 ± 1.4 in the ivabradine group versus 2.1 ± 1.1 in the control group ( p = 0.003). Heart rate reduction was significantly correlated with better exercise tolerance, quality of life, LV ejection fraction, and NYHA class, together with fewer HF hospitalizations. Multivariate analysis showed heart rate reduction to be a stronger predictor for better LV ejection fraction ( p = 0.024) and decreased hospitalizations than ivabradine use. Conclusion Adding ivabradine to optimal medical treatment in HF patients improved symptoms, quality of life, effort tolerance, and echocardiographic parameters, and reduced hospitalization. This beneficial ivabradine effect is probably due to its heart rate–reducing properties.

Electrical storm originating from a left ventricular epicardial scar in a patient with completely normal endocardial voltage

We report a patient with non-ischemic dilated cardiomyopathy presenting with an electrical storm because of a poorly tolerated monomorphic ventricular tachycardia. Electroanatomical mapping revealed a scar restricted to the epicardium, whereas the endocardial voltage map was completely normal. Epicardial catheter ablation based on substrate mapping and limited pace and entrainment mapping eliminated the tachycardia.

A Wolf in Sheep's Clothing: A Case of Dilated Cardiomyopathy Presenting with Nonspecific Digestive Symptoms: Insights into Nonocclusive Mesenteric Ischemia

We report on a 32-year-old male patient who presented to the emergency room for abdominal pain associated with nausea and vomiting. The patient experienced these symptoms for the last 3 months and was taken in charge on an outpatient basis. Assessment in the emergency room showed hemodynamic collapse, there were no signs of acute surgical abdomen. Emergent cardiac echogram showed severely dilated hypokinetic cardiomyopathy. The diagnosis of acute heart failure associated with nonocclusive mesenteric ischemia was retained. A review of the pertinent literature is presented.

Dilated cardiomyopathy presenting in childhood: aetiology, diagnostic approach, and clinical course

To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification. Retrospective study of 41 consecutive children affected by dilated cardiomyopathy - aged 0-14 years; median 33.4 plus or minus 49.25 - between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan-Meier method. Follow-up ranged from 10 days to 162 months - median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%. In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

Peripartum Cardiomyopathy in Africa: Challenges in Diagnosis, Prognosis, and Therapy

Peripartum cardiomyopathy (PPCM) is a form of heart failure affecting women of childbearing age, which can be associated with considerable mortality and chronic debilitating disease. Most patients present with acute postpartal heart failure that resembles the clinical presentation of idiopathic dilated cardiomyopathy. Historically, patients with PPCM have shown high rates of rapid recovery, with 6-month recovery rates averaging at 50%. However, recent prospective long-term follow-up of patients with PPCM in developing societies suggest recovery occurring only well into the second year after diagnosis, and recovery is poorly predicted by baseline left ventricular function. Beyond any potentially inherent factors contributing to poorer outcomes of patients with PPCM in developing societies, prognosis in these settings will continue to lag behind as the challenges faced to optimizing diagnosis remain immense. New insights into the role of inflammatory, apoptotic, and other genetic pathways may improve prognosis through the early detection and more targeted treatment of PPCM.