Dilated Cardiomyopathy Presenting Research Articles

Systemic inflammation in nonischemic dilated cardiomyopathy

We investigated links between inflammatory systemic activation and clinical presentation of nonischemic dilated cardiomyopathy (NIDC). Thirty-one consecutive patients with NIDC (age 57 +/- 10 years, left ventricular ejection fraction 32% +/- 7%) were enrolled in the study: subjects with ischemic heart disease, valvular heart disease, congenital malformations, pulmonary, renal, inflammatory, or metabolic diseases were excluded. All patients underwent physical examination, electrocardiography, chest radiology, echocardiography, and coronary angiography. Plasma levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and fibrinogen were ascertained. New York Heart Association (NYHA) functional class was significantly correlated with concentrations of fibrinogen (r = 0.42, P < 0.05) and CRP (r = 0.52, P < 0.01), and with ESR (r = 0.46, P < 0.05). Left ventricular ejection fraction was inversely related to fibrinogen (r = -0.41, P < 0.05) and ln CRP (r = -0.46, P < 0.05). Correlations between NYHA class and markers of inflammation remained significant also after correction for age, sex, and cardiovascular risk factors. Ongoing treatment with statins was associated with reduced CRP levels. Inflammatory markers are increased in patients with NIDC proportionally with severity of symptoms and systolic impairment. Systemic inflammation might be related to deterioration of NYHA class.

Using multiple buddy wires to facilitate left ventricular lead advancement in cardiac resynchronization therapy

Left ventricular lead positioning is always the critical step in cardiac resynchronization therapy because of the complex anatomy of coronary sinus branches. We describe the case of a 46-year-old man with dilated cardiomyopathy and complete left bundle branch block presenting with heart failure. The placing of the left ventricular lead into the posterolateral branch was hampered by an angulated portion at the proximal branch even with the assistance of anchor wire technique and one to two additional parallel wires' support. The use of three buddy wires facilitated the advancement of the left ventricular lead.

High Risk of Ventricular Arrhythmias in Patients With Nonischemic Dilated Cardiomyopathy Presenting With Syncope

It is not entirely clear whether the presentation of syncope in patients with nonischemic dilated cardiomyopathy (NIDC) is an ominous prognostic indicator, because randomized controlled implantable cardioverter-defibrillator (ICD) trials generally exclude such patients. This study compared 108 consecutive patients with NIDC presenting with syncope with 71 consecutive patients with NIDC who presented with sustained ventricular arrhythmias, with regard to freedom from any ventricular arrhythmias or life-threatening arrhythmias and all-cause mortality. There was no significant difference between the groups in the 3 outcomes during the follow-up of 43.5 +/- 32.1 months. Male gender and ICD therapy predicted increased risk for any ventricular arrhythmias. A reduced left ventricular ejection fraction and increased age were predictive of increased mortality. In conclusion, patients with NIDC presenting with syncope are a high-risk group, with event rates similar to patients with NIDC presenting with sustained arrhythmias, and should be considered for ICD therapy.

Kimura's disease in a patient with idiopathic dilated cardiomyopathy on ambulatory peritoneal dialysis

Sir, Kimura’s disease, endaemic in Asia, manifests as solitary or multiple subcutaneous nodules in the head and neck, marked peripheral eosinophilia and elevated serum IgE [1]. Many cases of this are associated with nephrotic syndrome of unknown cause; and there is no evidence in these patients for a relationship between the end-stage renal disease and Kimura’s disease. We describe a patient on chronic ambulatory peritoneal dialysis (CAPD) with a unique concomitant presentation of idiopathic dilated cardiomyopathy and Kimura’s disease. A 17-year-old Taiwanese boy was diagnosed as having nephrotic syndrome when he was 12 years old, and he was treated with oral prednisolone. Then he was lost to follow-up, having turned to traditional Chinese herbal drugs. At the age of 16, he presented at our hospital again with dyspnoea and oliguria. Haemodialysis was performed initially because of acute pulmonary oedema; then he received CAPD. Idiopathic eosinophilia was noted at this admission [white blood cells (WBCs), 6050/ml; segmented neutrophils, 59.4%; lymphocytes, 10.9%; monocytes, 4.1%; eosinophils, 25.3%; basophils, 0.3%; absolute eosinophil count, 1530/ml (normal 50–450/ml)]. His IgE was 2098 IU/ml (normal <200 IU/ml) and his haemoglobin was 9.9 mg/dl. About 6 months later, two subcutaneous nodules were found in his neck. Excisional biopsy was done which showed florid germinal centres, eosinophilic infiltration, eosinophilic abscesses and an increase of post-capillary venules—findings compatible with Kimura’s disease [2]. The patient’s cardiothoracic ratio (CTR) was 57.8%. Cardio-echography revealed dilatation of all four chambers with poor left ventricular performance [left ventricular ejection fraction (LVEF) 46%] and moderate pericardial effusion. Idiopathic dilated cardiomyopathy was diagnosed. The patient, however, refused pericardiocentesis or myocardial biopsy. We tried methylprednisolone 500 mg intravenously for 3 days then oral prednisolone 40 mg/day tapered to 10 mg/day for 6 months. His eosinophil count decreased from 3429 to 391/ml (from 34.5 to 6.1 as a percentage of WBCs). His CTR remained essentially unchanged (58.2%), and his left ventricular function did not improve (LVEF 45%) after 6 months of steroid treatment. Since then, the patient has not received steroids, because he has had no appreciable improvement of cardiac function. A systemic component is usually absent in Kimura’s disease, but the disease is often associated with proteinuria and nephrotic syndrome [3]. In our case, we could not identify the aetiology of idiopathic dilated cardiomyopathy because myocardial biopsy was not performed. We can, however, exclude dialysis-related cardiomyopathy, because the patient was well nourished and free from oedema, and he had the following laboratory parameters: creatinine clearance (Ccr), 73.82 l/week; weekly Kt/V, 2.46; and normalized protein catabolic rate (nPCR), 1.59 g/day. Based on a report by Sekiguchi et al., that steroid therapy was effective in 16 of 25 cases of eosinophilic heart disease [4], we tried steroids on this patient for 6 months, at the end of which his eosinophil count was decreased but without significant improvement of his left ventricular function. In conclusion, this is a unique case in which Kimura’s disease and idiopathic dilated cardiomyopathy co-existed. Their as yet undefined relationship may be evaluated further.

Dilated cardiomyopathy presenting during fetal life

To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy. A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology. Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation. A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses. Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.

P01.30: Dilated cardiomyopathy presenting during fetal life

P01.30: Dilated cardiomyopathy presenting during fetal life

What do implantable cardioverter/defibrillators teach us about the mechanisms of sudden cardiac death?

Almost two decades of experience with the ICD have resulted in a better understanding of the nature of life-threatening ventricular tachyarrhythmias. The most pertinent achievements in this respect stem from clinical follow-up observations in patients with important clinical entities such as dilated cardiomyopathy or the Brugada syndrome. For instance, patients with dilated cardiomyopathy presenting with syncope and a negative electrophysiological study have been shown to have a high incidence of appropriate usage of their ICD. On the other hand, relative little has been gained from the extensive electrogram storage capabilities of third and fourth generation ICDs. Basically, careful evaluation of these electrograms has confirmed previous anecdotal data obtained from analysis of Holter recordings of patients who died suddenly while wearing the ECG recorder. In most instances, VT/VF episodes are triggered by relatively late-coupled premature beats whereas short--long--short sequences have been observed only in few patients. One future research avenue concerns the more detailed analysis of changes in cardiac autonomic tone preceding the occurrence of sustained ventricular tachyarrhythmias which can be assessed from the electrogram storage of the ICD.

The natural history of dilated cardiomyopathy presenting during childhood Piers Daubeney, Alan Nugent, Steven Colan, Michael Cheung, Andrew Davis, John Carlin, Robert Weintraub. National Australian Childhood Cardiomyopathy Study, Melbourne, Australia

The natural history of dilated cardiomyopathy presenting during childhood Piers Daubeney, Alan Nugent, Steven Colan, Michael Cheung, Andrew Davis, John Carlin, Robert Weintraub. National Australian Childhood Cardiomyopathy Study, Melbourne, Australia

▪ Idiopathic Dilated Cardiomyopathy Presenting in Pregnancy

▪ Idiopathic Dilated Cardiomyopathy Presenting in Pregnancy

Idiopathic dilated cardiomyopathy presenting in pregnancy

To describe the clinical course and management of a patient who presented with idiopathic dilated cardiomyopathy in early pregnancy. A 27 yr old, previously well, Chinese primigravida presented at 18 wk gestation with a history of irregular heart beat and decreased exercise tolerance. Echocardiography showed moderate left ventricular dysfunction with left ventricular ejection fraction of 35-40%. Idiopathic dilated cardiomyopathy was diagnosed. She declined termination of pregnancy and was managed medically with furosemide, digoxin and potassium supplements. Low molecular weight heparin was prescribed. Emergency Cesarean delivery was performed at 31 wk gestation because of deteriorating liver function and a non-reassuring fetal heart rate pattern. General anesthesia was given because of relative urgency, the patient's wish, and concerns about potential risk of spinal hematoma. Invasive monitoring with pulmonary and radial artery catheters was used and low dose inotropic support was given. Postoperatively, she was managed in the intensive care and coronary care units where she was treated with dobutamine, furosemide, digoxin, potassium, captopril, losartin and warfarin. Her postoperative course was complicated by a severe embolic stroke five weeks after delivery and she died five months later. Idiopathic dilated cardiomyopathy may rarely present in pregnancy. A multidisciplinary approach and close peripartum monitoring are important in management and termination of pregnancy should be considered. Thromboembolic complications are a major risk.

PEDIATRIC MYOCARDIAL DISEASE

Cardiomyopathies are diseases of the heart muscles. This article reviews the causes, clinical presentation, diagnosis, management, and long-term outcomes of dilated and hypertrophic cardiomyopathy.

Shock occurrence and survival in 49 patients with idiopathic dilated cardiomyopathy and an implantable cardioverter-defibrillator.

To determine shock occurrence and survival, 49 patients with idiopathic dilated cardiomyopathy presenting with cardiac arrest (82%), syncope (12%) or ventricular tachycardia without syncope (6%) were followed for 28 +/- 28 months after cardioverter-defibrillator (ICD) implant according to the intention to treat principle. Using the Kaplan-Meier method, the actuarial incidence for any spontaneous shocks was 20%, 58%, and 77%, and the incidence of appropriate shocks was 16%, 49%, and 72% at 1, 3, and 5 years of follow-up, respectively. Only two of 49 study patients (4%) with an active ICD died suddenly during follow-up. Another two patients, however, with an inactive device died suddenly, resulting in a sudden death rate of 2% per year with an active ICD, and 5% per year, according to the intention to treat principle. The incidence of cardiac death from any cause was 8%, 25%, and 35%, and the incidence of total mortality was 14%, 39%, and 49% during 1, 3, and 5 years of follow-up, respectively. There was no difference in the Kaplan-Meier survival curves for shocked vs non-shocked patients. Thus, in this selected patient population with idiopathic dilated cardiomyopathy the majority of patients received 'appropriate' shocks during follow-up, and the sudden death rate with active ICD is low.

Dilated cardiomyopathy in children: determinants of outcome.

To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification. Supraregional paediatric cardiology unit. Retrospective analysis. The natural history of dilated cardiomyopathy in children is not well characterised. Previous studies have shown a variable relation between age at presentation and outcome, and sudden death has been infrequent. Retrospective study of 63 consecutive patients with idiopathic dilated cardiomyopathy presenting between 1979 and 1992. Survival curves were constructed by the Kaplan-Meier method. Age at diagnosis ranged from 1 day to 15 years (median 12 months) and follow up ranged from 1 day to 13 years (median 19 months). Actuarial survival from presentation was 79% at one year (95% confidence interval (95% CI) 66%-88%) and 61% (44%-74%) at five years. Univariate analysis showed that mural thrombus, left ventricular end diastolic pressure > 20 mm Hg, and age at presentation > 2 years were predictors of adverse outcome, but on multivariate analysis only age at presentation was significant. Left ventricular echocardiographic indices either did not improve or deteriorated in 36 children (17 of whom died, four suddenly, and three were transplanted), partially improved in 16 (three of whom died, all suddenly), and returned to normal in 11 (all of whom have survived). Older age at presentation and lack of improvement in systolic function are associated with an adverse outcome, and early transplantation should be considered in these patients. There is a persistent risk of late sudden death in those children in whom echocardiographic dimensions remain abnormal.

The incidence of myocarditis in endomyocardial biopsy samples from patients with congestive heart failure

We present the combined experience of three Yugosiavian cardiovascular centers in the application of endomyocardial biopsy for the diagnosis of myocarditis in patients who present clinically with congestive heart failure. The study group comprised 107 patients (mean age, 40.8 years; range, 19 to 61 years). On the basis of patient history and diagnostic tests, the following clinical diagnoses were established: dilated cardiomyopathy (85), myocarditis (16), and alcohol-induced heart disease (6). EMB samples were taken from the left ventricle (95) or both ventricles (12) by use of a King's College bioptome, with a mean of 3.2 samples per patient. Histologic evidence of myocarditis was noted in 10 of 85 patients (12%) with a clinical diagnosis of dilated cardiomyopathy, in 2 of 6 patients (33%) with alcohol-induced heart disease, and in 12 of 16 patients (75%) with a clinical diagnosis of myocarditis. There was confirmation of the clinically suspected diagnosis in 63% of cases, a change of diagnosis based on histology in 15% of cases, and nonspecific findings in 22%. However, useful information was obtained in 78% of the cases, and there was a 22% incidence of histologically proven myocarditis for the entire group. Our results indicate that endomyocardial biopsy is beneficial in determining the true incidence of myocarditis in patients with a clinical presentation of dilated cardiomyopathy.

Superior vena caval thrombosis in a child: An uncommon presentation of idiopathic dilated cardiomyopathy

A nine year old girl who presented with a thrombosis of the right internal jugular vein and superior vena cava was found to be in cardiac failure and to have a dilated cardiomyopathy. After transient improvement she deteriorated and was accepted for cardiac transplantation. Following transplantation her condition has been much improved.

Arterial microembolisation: an unusual presentation of dilated cardiomyopathy.

Systemic embolisation is common in patients with dilated cardiomyopathy. Microembolisation as a presenting sign of dilated cardiomyopathy, however, has not been reported before. A 37 year old woman in whom dilated cardiomyopathy presented as arterial microembolisation to the toes is described.

Histopathologic and electrophysiologic correlations in idiopathic dilated cardiomyopathy and sustained ventricular tachyarrhythmia

Idiopathic dilated cardiomyopathy is associated with a high incidence of serious ventricular arrhythmias, which carry a significant risk of sudden death. 1 In contrast to patients with coronary artery disease, in whom ventricular tachycardia arises as a result of reentrant circuits that form during the healing of an infarct, 2–4 the histologic substrate for ventricular arrhythmias in dilated cardiomyopathy has not been identified. Using various semiquantitative scoring methods, several groups have attempted to correlate histopathology with hemodynamic data as well as prognosis in patients with idiopathic dilated cardiomyopathy. 5–8 Myocarditis has been reported in patients with dilated cardiomyopathy and unexplained ventricular arrhythmias. 9 However, a possible correlation between the inducibility of sustained ventricular tachycardia during electrophysiologic study and histopathology has not been systematically examined. A preliminary report from Stanford University suggested that a semiquantitative histologic score may be useful in predicting response to β blockers in patients with dilated cardiomyopathy. 10 To determine if an identifiable histologic correlate for inducible sustained ventricular tachycardia exists in patients with idiopathic dilated cardiomyopathy presenting with sustained ventricular tachyarrhythmia, we analyzed histologic and electrophysiologic data from 25 such patients using the same scoring system.

Echocardiographic evaluation of dilated cardiomyopathy in the human fetus

The diagnosis of dilated cardiomyopathy was established and subsequently confirmed in 6 of 625 fetuses studied by echocardiography. All 6 had structurally normal hearts. Abnormal findings included reduced fractional shortening index in 5, atrioventricular valve regurgitation in 3, abnormal chamber dimensions in 3 and nonimmune hydrops in 4. In 2 fetuses referred because of a family history of dilated cardiomyopathy in previous siblings, echocardiographic abnormalities were absent on a first examination performed at 20 weeks of gestation. This suggested that a normal fetal echocardiogram in a midtrimester fetus does not always rule out the subsequent development of dilated cardiomyopathy. However, all fetuses followed serially developed some abnormality later in pregnancy. Only 2 neonates survived, 1 of whom required a heart transplant during infancy. Death from cardiac failure occurred in 1 fetus and 3 neonates. This study demonstrates that dilated cardiomyopathy may develop during fetal life and might be diagnosed by echocardiography if serial studies are performed. Dilated cardiomyopathy presenting prenatally appears to have a poor prognosis.

Dilated cardiomyopathy in infants and children

The outcome of medical treatment of dilated cardiomyopathy in infants and children was reviewed to develop a predictive index for selection of patients likely to benefit from cardiac transplantation. The clinical findings, laboratory investigations, treatment and outcome of 20 patients (Group 1) <2 years of age at presentation and 12 patients (Group 2) .2 years of age at onset were compared. Of 20 Group 1 patients, 5 (25%) died. Available autopsies (four patients) showed endocardial fibroelastosis. Of 15 survivors, 10 showed improvement in cardiac status and 5 remained unchanged. Ninety-three percent of survivors had dilated cardiomyopathy consistent with endocardial fibroelastosis by angiocardiography. All 12 Group 2 patients died. In addition to age at presentation and poor outcome, Group 2 differed from Group 1 in having a higher incidence of other family members with cardiomyopathy, more significant rhythm disturbances at presentation and a more rapid course to death. Risk factors of poor outcome in both groups included persistent cardiomegaly and the development of significant arrhythmias by Holter electrocardiographic monitoring.Cardiac transplantation is recommended for children with dilated cardiomyopathy presenting after age 2 years who survive 1 month. Those patients <2 years old at presentation whose condition has not improved after 1 year and who have persistent cardiomegaly or complex ventricular arrhythmias may also benefit from transplantation.

Idiopathic Dilated Cardiomyopathy: Clinical Profile of 100 Patients

Clinicians continue to face the challenges of identifying and treating the idiopathic dilated cardiomyopathy to improve symptoms and survival. A study on idiopathic dilated cardiomyopathy was done in the Department of Cardiology, University Cardiac Center, Bangabandhu Sheikh Mujib Medical University, Dhaka, from January 2004 to December 2009. The aim of this study was to examine clinical profile of patients with idiopathic dilated cardiomyopathy. The age range was 18 to 65 years and 70% subjects were male. Most common symptom was dyspnea (86%) and cough (75%). 75% subjects had sinus tachycardia, 42% had ventricular ectopics and 40% had left bundle branch block. Mean diastolic dimension was 60±9 mm, ejection fraction was 28±8%, left atrial dimension was 40±6 mm and 36% were having mitral regurgitation. Left ventricular failure (75%) and various type of arrhythmias (62%) were the main complications. 8% subjects were died during hospital stay. Hence the clinical presentation of idiopathic dilated cardiomyopathy varies from patient to patient, but most patients present later, i.e. at some point in the spectrum of heart failure. Key words: Idiopathic dilated cardiomyopathy; left ventricular failure; ventricular ectopics. DOI: 10.3329/uhj.v6i1.7182University Heart Journal Vol.6(1) 2010 pp.9-12