Severe Idiopathic Dilated Cardiomyopathy Research Articles

The prognostic value of the left atrial strain rate determined using cardiovascular magnetic resonance feature tracking imaging in patients with severe idiopathic dilated cardiomyopathy.

Left atrial strain rate (LASR) is rarely used as an indicator of cardiovascular disorder prognosis in dilated cardiomyopathy (DCM). In the present study, we aimed to explore the prognostic value of the LASR in patients with severe idiopathic DCM [left ventricle ejection fraction (LVEF) <35%] using cardiac magnetic resonance feature tracking (CMR-FT). A retrospective cohort study was performed. At the time of enrollment, patients who underwent cardiovascular magnetic resonance (CMR) imaging were selected for the study. Left atrium (LA) strain rate was measured in patients with severe idiopathic DCM from four-chamber and two-chamber views using a 3.0-T CMR scanner. The study endpoint was defined as a combination of all-cause death, implantable cardioverter-defibrillator (ICD), hospitalization due to cardiac failure, and heart transplantation. Cox model analyses were used to assess the statistical significance of the risk factors. Fifty-eight patients with a confirmed diagnosis of severe idiopathic DCM were ultimately included in the analysis [mean age of 46 years; 41 (73%) men]. The median follow-up was 43 months (13-72 months). Adverse cardiovascular events occurred in 29 patients: all-cause death occurred in 15 participants (25.8%), ICD was performed in 5 participants (8.6%), heart failure hospitalization occurred in 8 participants (13.8%), and 1 participant (1.7%) underwent heart transplantation. From univariate analysis, the left atrium reservoir strain rate (LASRs) [HR 0.12; 95% CI (0.02, 0.55), P=0.007] and the left atrium booster pump strain rate (LASRa) [HR 3.21; 95% CI (1.08, 9.58), P=0.036] significantly predicted the association with cardiovascular events. In model 1 after adjusting for basic cardiovascular risk factors, including age, hypertension, diabetes mellitus, and N-terminal pro-brain natriuretic peptide (NT-proBNP), the LASRs [HR 0.12, 95% CI (0.02, 0.58), P=0.009] was assessed. The mitral valve regurgitation, LA maximum volume index, and LA diastatic volume index were added in model 2 based on model 1. LASRs [HR 0.13, 95% CI (0.02. 0.82) P=0.030] was the independent prediction factor after adjustment for model 1 and model 2. The LASRs had a good prognostic value with an area under the curve of receiver operating characteristic of 0.697 (0.617, 0.777) at 1 year, 0.716 (0.643, 0.788) at 3 years, 0.716 (0.651, 0.798) at 5 years and 0.703 (0.597, 0.809) at 7 years. LA reservoir strain rate and active strain rate were powerful prognostic markers. The LASRs by MRI-FT provided independent prognostic value in patients with severe idiopathic DCM.

The prognostic significance of the echocardiographic evaluation of left ventricular myocardial deformation indices at peak cardiopulmonary exercise in patients with severe dilated cardiomyopathy

Abstract Background Cardiopulmonary exercise testing (CPET) integrally estimates exercise capacity (peak VO2) and cardiovascular (CV) prognosis (VE/VCO2 slope) in patients with dilated cardiomyopathy (DCM). Myocardial deformation indices (MDI), measured by speckle tracking imaging (STI), allow reliable measurements of left ventricular (LV) function. Little is known about the exercise-induced changes of the multidimensional mechanical properties of the heart and their impact in patient's survival. We aimed to investigate the predictive role regarding 3-year survival of LV MDI (at rest and peak exercise) as well as the main CPET parameters (peak VO2, VE/VCO2 slope) in DCM patients with reduced ejection fraction (EF) using as primary combined end-point the heart transplantation and all cause death. Patients and methods We evaluated LV function using STI at rest and at peak exercise during the same CPET session in 53 DCM patients (54±12 years, 76% males, ejection fraction 33±9%).We measured global longitudinal strain (GLS), longitudinal strain rate at systole (LSRS) and diastole (LSRD) at baseline and at peak CPET along with CPET parameters. After a period of 38±15 months, all DCM patients were evaluated by a telephone interview. Results From the whole population, totally 7 patients (13%) reached the primary end-point (Group A) as 2 patients were submitted to heart transplantation and 5 patients died (60±13 years, 86% males, EF = 28±9%) while 46 patients remained alive at 3-year follow-up (Group B, 53±12 years, 74% males, EF = 33±9%). We found that Group A patients had similar age, sex and EF but reduced peak VO2 (p=0.04) and increased VE/VCO2 slope (p=0.004), impaired GLS at baseline and peak exercise (p=0.02 and p=0.04, respectively) as well as LSRS at peak exercise (p=0.05) compared to Group B patients. Conclusions The evaluation of GLS (rest and peak exercise) and LSRS (peak exercise) as systolic indices of LV myocardial deformation adds valuable information regarding CV prognosis in patients with severe idiopathic DCM. A combined baseline work-up protocol consisted of MDI evaluation at rest and exercise plus the CPET indices may characterize the true severity of heart failure in a DCM population. Funding Acknowledgement Type of funding sources: None.

First evidence of the presence of adenovirus type 8 in myocardium of patients with severe idiopathic dilated cardiomyopathy.

Previous studies have detected adenovirus and cytomegalovirus (CMV) in cardiac tissue of patients with myocarditis. Therefore, in this study, we investigated the frequency of these viruses, which may be involved in the development of severe dilated cardiomyopathy (DCM). Myocardial tissue from of 23 cardiac transplant candidates with acute idiopathic DCM below the age of 40 years were analyzed by amplification of adenovirus and CMV DNA and subsequent sequencing. Adenovirus was detected in four (17.4%) and CMV in one (4.3%) of the patients. All controls were negative for the presence of both viruses. Our study shows that myocardial infection with adenovirus may play an important role in the pathogenesis of severe DCM and suggests that vaccination against adenovirus might be helpful in decreasing the prevalence of severe idiopathic DCM. This is the first study in which adenovirus type 8 has been detected in the hearts of patients with DCM.

ST-Elevation Myocardial Infarction in the Presence of Biventricular Paced Rhythm

BackgroundIn the diagnosis of acute myocardial infarction (AMI), the presence of baseline left bundle branch block or a permanent pacemaker rhythm poses a challenge. ObjectiveWe present a case report highlighting this challenge, along with a review of pertinent literature. Case ReportA 70-year-old female with known severe idiopathic dilated cardiomyopathy and moderate coronary artery disease who was status post−biventricular pacemaker/implantable cardioverter defibrillator insertion was brought to our institution via Emergency Medical Services with recurrent firing of her implantable cardioverter defibrillator and syncope. After stabilization in the Emergency Department and treatment with intravenous amiodarone, the patient admitted to having ongoing chest pains. The electrocardiogram revealed evidence of biventricular pacing with superimposed ST-segment elevations in the anterolateral leads indicative of myocardial injury. She underwent prompt angiography, thrombectomy, and bare-metal stent insertion to a totally occluded proximal left anterior descending coronary artery, with resolution of her chest pain and improvement in the ST-segment changes. ConclusionsDespite proposed criteria that aid in the recognition of AMI with underlying left bundle branch block and paced rhythm; the advent of new pacing modalities and the potential variability of pacing sites impose additional diagnostic challenges requiring higher level of suspicion and better physician awareness.

First-in-Man Transfemoral Transcatheter Aortic Valve Implantation for Severe Aortic Regurgitation in a Patient with Left Ventricular Assist Device

Purpose Patients with left ventricular assist device (LVAD) may develop significant aortic regurgitation (AR) and require high-risk surgical intervention. Transcatheter aortic valve implantation (TAVI) has emerged as a therapeutic alternative for patients with severe aortic stenosis not amenable for surgery but has also been used in high risk patients for pure AR with good results. We report the first-in-man case of transfemoral (TF) TAVI in an LVAD patient with severe AR. Methods and Materials A 55 year old female with severe idiopathic dilated cardiomyopathy underwent HeartMate II LVAD implantation on November 2010 as bridge to transplant. Following a hemodynamic uneventful one year at home she gradually developed symptoms of severe left heart failure and echo showed severe non calcified AR. Our local Heart Team recommended TF TAVI as the preferred option and after discussing the options the patient gave her consent for that approach. The procedure was performed on August 8, 2012, under mild sedation without reduction or interruption of the LVAD rotation speed (9400 rpm). The right femoral artery was used for access under local anesthesia and an 18Fr guiding sheath was introduced percutaneously. No pre-dilation of the aortic valve was performed. A stiff guide-wire was placed in the left ventricle, carefully avoiding its entrapment in the LVAD apical inflow cannula, and a 29mm CoreValve (Medtronic, Minneapolis, MN) was implanted without need for rapid pacing. Aortography showed minimal residual AR and no blockage of the LVAD outflow graft in the ascending aorta. The patient was stable hemodynamicaly throughout the procedure and recovered with no complications. Echo on the second post-TAVI day showed no residual AR and she was discharged home. On 3 months follow up her clinical status has improved significantly and she remains with no AR on repeat echo studies. Results TF TAVI should be considered as a safe and viable solution for severe AR in LVAD patients.

Usefulness of scintigraphy to predict electrical storms in severe idiopathic dilated cardiomyopathy

Although several predictors of an electrical storm (ES) are indicated in patients with idiopathic dilated cardiomyopathy (IDCM), whether the severity of the myocardial tissue damage (SMTD) evaluated by myocardial perfusion SPECT (MPS) has an association with an ES remains unclear. The purpose of this study was to elucidate the clinical significance of SMTD for the prediction of ES in IDCM patients with an ICD. Thirty-seven (27 men, mean age 58 ± 15 years) IDCM patients receiving ICD implantations for secondary prevention with preoperative MPS were enrolled in this study. The medical history, physical and laboratory findings, electrocardiograms, echocardiograms and MPS findings were evaluated. The SMTD was assessed by the summed scores of 17 segments using a 4-point system (0, normal ~3, severe defect). During a mean follow-up of 43.9 ± 30.7 months, an ES developed in 12/37 (32.4 %) patients. The SMTD score predicted an ES with a 92 % sensitivity and 56 % specificity, at a cut-off score of 10. In addition, a multivariate analysis showed that the SMTD score remained an independent predictor of an ES (HR 1.09/score 1 increase, 95 % CI 1.01-1.19, p = 0.02). The SMTD score was significantly associated with three indices of late potentials on the signal-averaged electrocardiograms, and was significantly higher in patients with positive late potentials (p = 0.0006). SMTD score assessed by MPS has a strong correlation to the late potentials and higher SMTD score may increase the risk of ES among patients with IDCM and an ICD.

Effect of Implantable Cardioverter-Defibrillator on Left Ventricular Ejection Fraction in Patients With Idiopathic Dilated Cardiomyopathy

Current guidelines have indicated an implantable cardioverter-defibrillator (ICD) for patients with severe idiopathic dilated cardiomyopathy, for both primary and secondary prevention. Compared to coronary artery disease, the overall benefit has been smaller. A more refined risk assessment, using the left ventricular ejection fraction (LVEF) and prevention mode (primary/secondary), is still needed to guide ICD implantation. Patients included in 2 large ICD registers were analyzed regarding the appropriate therapies and improvement of LVEF, overall and in subgroups of prevention mode and LVEF < 20% versus > 20%. Overall, 349 patients were included; 70% were men, the mean age was 54 years, and the mean follow-up was 33 months. Cardiac resynchronization therapy (CRT) was used in 57%, and secondary prevention was present in 30%. ICD therapies were delivered to 33% of the patients, in most for ventricular tachycardia. Patients receiving an ICD for secondary prevention and non-CRT were more likely to have arrhythmic events (both p < 0.05). The cumulative event rates at 5 years were 53% for secondary and 33% for primary prevention (p < 0.001). Depending on the prevention mode and LVEF status (< 20% vs > 20%), the event rates ranged from 30% to 76%. The mean LVEF improved by 10%, independently of the stimulation mode (CRT 22% to 31%, non-CRT 26% to 35%; p < 0.0001). A persistent improvement to > 35% was seen in only 25% of CRT patients but in 45% of non-CRT patients (p = 0.004). In conclusion, the results from the present study have demonstrated that in patients with idiopathic dilated cardiomyopathy, the potential for LVEF improvement is considerable and that the rate of ICD interventions strongly depends on the prevention mode and LVEF. These findings could be the basis for additional risk stratification tools.

A safety and feasibility study of cell therapy in dilated cardiomyopathy

The aim of this study was to determine if bone marrow mononuclear cell (BMMC) transplantation is safe for moderate to severe idiopathic dilated cardiomyopathy (IDC). Clinical trials have shown that this procedure is safe and effective for ischemic patients, but little information is available regarding non-ischemic patients. Twenty-four patients with IDC, optimized therapy, age 46 ± 11.6 years, 17 males, NYHA classes II-IV, and left ventricular ejection fraction <35% were enrolled in the study. Clinical evaluation at baseline and 6 months after stem cell therapy to assess heart function included echocardiogram, magnetic resonance imaging, cardiopulmonary test, Minnesota Quality of Life Questionnaire, and NYHA classification. After cell transplantation 1 patient showed a transient increase in enzyme levels and 2 patients presented arrhythmias that were reversed within 72 h. Four patients died during follow-up, between 6 and 12 weeks after therapy. Clinical evaluation showed improvement in most patients as reflected by statistically significant decreases in Minnesota Quality of Life Questionnaire (63 ± 17.9 baseline vs 28.8 ± 16.75 at 6 months) and in class III-IV NYHA patients (18/24 baseline vs 2/20 at 6 months). Cardiopulmonary exercise tests demonstrated increased peak oxygen consumption (12.2 ± 2.4 at baseline vs 15.8 ± 7.1 mL·kg⁻¹·min⁻¹ at 6 months) and walked distance (377.2 ± 85.4 vs 444.1 ± 77.9 m at 6 months) in the 6-min walk test, which was not accompanied by increased left ventricular ejection fraction. Our findings indicate that BMMC therapy in IDC patients with severe ventricular dysfunction is feasible and that larger, randomized and placebo-controlled trials are warranted.

A diagnosis not to be missed: Behcet’s disease as a cause of dilated cardiomyopathy in a young Arab male patient

We report a 33-year-old Arab male patient who was thought to have severe idiopathic dilated cardiomyopathy (DCM) associated with complete atrioventricular block for more than 6 years, then was found to possess features suggestive of underlying Behcet's disease in the form of recurrent oral and genital ulcers, cutaneous folliculitis, superficial thrombophlebitis, pathergism, partially thrombosed portal vein and a positive human leukocyte antigen -B51.

Critical Level of Cerebral Oxygenation During Exercise in Patients With Left Ventricular Dysfunction

In a recent study the indexes of cerebral oxygenation decreased during maximal exercise in nearly half of all patients with left ventricular dysfunction. Whether these levels decrease severely enough to influence mental status or level of consciousness was evaluated in the present study. Forty-two patients with idiopathic dilated cardiomyopathy (IDC) and 29 healthy subjects underwent a symptom-limited maximal exercise test. The cerebral oxyhemoglobin (O(2)Hb) and tissue oxygenation index (TOI) were continuously monitored using near-infrared spectroscopy. The changes in O(2)Hb and TOI were also measured in 7 subjects: 2 who experienced episodes of reduced consciousness caused by sudden decreases in blood pressure during exercise recovery and 5 who exhibited sustained ventricular tachycardia during an electrophysiological study. The change in cerebral O(2)Hb during exercise in patients with IDC averaged 0.38+/-3.39 micromol/L, significantly lower than in the normal subjects (4.30+/-4.47 micromol/L, p<0.0001). The cerebral O(2)Hb decreased during exercise in 18 of 42 patients with IDC. The change in cerebral TOI in the IDC patients during exercise was significantly less than that in the normal subjects (-2.0+/-4.7 vs 2.1+/-5.8%, p=0.002). The mean decreases in cerebral O(2)Hb and TOI were -5.34 micromol/L and -9.7%, respectively, in the patients with reduced consciousness during exercise recovery, and -2.52 micromol/L and -16.5%, respectively, in those with ventricular tachycardia. The indexes of cerebral oxygenation may drop severely enough during maximal exercise in some patients with severe IDC that consciousness is affected.

Immunoadsorption in idiopathic dilated cardiomyopathy, a 3-year follow-up

Objective: Recent studies have shown that immunoadsorption (IA) leads to improvements in left ventricular function and in functional status in selected patients with idiopathic dilated cardiomyopathy (DCM) and circulating autoantibodiesautoantibod. Most of the few studies dealing with this topic describe only short-term results. The aim of this study was to ascertain the prolonged effects of IA over a period of 3 years. Materials and methods: Our study included nine patients with circulating β 1-adrenoreceptor antibodies who suffered from idiopathic DCM (left ventricular ejection fraction <30%). IA was performed using an adsorber against immunoglobulins (Ig Therasorb, Baxter). During therapy and after 3 months, hemodynamic parameters were monitored using a Swan–Ganz thermodilution catheter. All patients were monitored under clinical and echographical examinations over a period of at least 3 years. Results: During IA hemodynamic measurements show increases in both cardiac and stroke volume index (from 2.0 (S.E.M. 0.16) to 3.0 (S.E.M. 0.32) l min −1 m −2, and from 26.1 (S.E.M. 2.63) to 38.9 (S.E.M. 3.56) ml/m 2, respectively). Hemodynamic stabilization was observed after the following 3 months (CI 2.6 (S.E.M. 0.14) l min −1 m −2, SVI 37.9 (S.E.M. 2.14) ml/m 2). After 36 months five patients were still alive. Antibody titers increased in patients who deteriorated. The five patients who are still alive show an increase in left ventricular ejection fraction and no significant increase in antibody titers. Conclusion: Immunoadsorption may improve short-term hemodynamics as well as long-term follow-up of patients with severe idiopathic dilated cardiomyopathy. Increase in antibody titers is accompanied by deterioration of cardiovascular function.

Altered myocardial and endothelial expression of cyclooxygenase-2 after prolonged mechanical support in patients with severe idiopathic dilated cardiomyopathy: S. E. Belland, R. Sangrigoli, V. Jeevanandam, H. J. Eisen, Temple University School of Medicine, Philadelphia, PA

Altered myocardial and endothelial expression of cyclooxygenase-2 after prolonged mechanical support in patients with severe idiopathic dilated cardiomyopathy: S. E. Belland, R. Sangrigoli, V. Jeevanandam, H. J. Eisen, Temple University School of Medicine, Philadelphia, PA

Sustained paradoxical vasodilation during orthostasis in heart failure: a factor in the edema pathogenesis?

Baroreceptor-induced peripheral reflex vasoconstriction during upright posture is an important edema-prevention mechanism in humans. Congestive heart failure (CHF) has been associated with blunted baroreceptor control of regional blood flow during short-term head-up tilt. The effect of prolonged unloading of baroreceptors on subcutaneous blood flow of the calf was investigated in 12 healthy subjects and in 13 patients with severe idiopathic dilated cardiomyopathy (New York Heart Association functional class III or IV). The subjects were studied both supine and sitting for 3-h periods. When sitting, subcutaneous vascular resistance decreased -26 +/- 19% in CHF patients and increased 90 +/- 69% in control subjects (P < 0.0001). The corresponding subcutaneous blood flow increased 43 +/- 29% in patients with CHF compared with the decrease of -42 +/- 17% in control subjects (P < 0.0001). I conclude that patients with CHF secondary to idiopathic dilated cardiomyopathy have an abnormal baroreceptor-mediated peripheral vasodilation during orthostatic stress that is sustained for hours. This extended paradoxical vasodilation may participate as an additional pathophysiological mechanism contributing to lower extremity edema in patients with CHF.

Myocardial energetics and efficiency in patients with idiopathic cardiomyopathy: Response to dobutamine and amrinone

Nine consecutive patients having severe idiopathic dilated cardiomyopathy were studied for their response in ventricular function, coronary sinus blood flow and myocardial oxygen consumption, lactate extraction and efficiency following incremental doses of dobutamine, followed by the combination of dobutamine and the phosphodiesterase inhibitor amrinone. Results, presented as baseline and the response to the peak dose (15 micrograms/kg/min) of dobutamine and to the combination of dobutamine and amrinone (each at 15 micrograms/kg/min) (differences compared with baseline) were: wedge pressure decreased from 28 +/- 7 to 26 +/- 8 mm Hg (p = NS) and to 20 +/- 6 mm Hg (p less than 0.01); cardiac index rose from 1.47 +/- 0.44 L/min/m2 to 2.89 +/- 1.1 L/min/m2 (p less than 0.01) and to 3.64 +/- 1.05 L/min/m2 (p less than 0.001); myocardial oxygen consumption remained invariant (18 +/- 8, 17 +/- 5, and 19 +/- 5 ml/min) despite progressive increments in minute work from 2.96 +/- 1.1 to 6.98 +/- 3.9 kg - m/min (p less than 0.01) and to 9.38 +/- 4.3 kg - m/min (p less than 0.001); myocardial lactate extraction rose from 21 +/- 10% to 30 +/- 15% (p = NS) and to 35 +/- 10% with the addition of amrinone (p less than 0.01). No patient had net lactate efflux into the coronary sinus, and myocardial efficiency improved from 9.5 +/- 5% to 21.7 +/- 13.0% (p less than 0.01) and to 28.0 +/- 18.0% (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Effects of hydralazine on pressure-volume and stress-volume relations in congestive heart failure secondary to idiopathic dilated cardiomyopathy

The mechanism by which hydralazine improves cardiac function in patients with heart failure is not well characterized. Hydralazine may improve left ventricular (LV) function by decreasing afterloading wall stress or by increasing myocardial contractility. The effect of intravenous hydralazine was assessed in 8 patients with severe idiopathic dilated cardiomyopathy. Hydralazine increased stroke volume index (from 24 ± 8 to 40 ± 9 ml/m 2, p < 0.01) and decreased systemic vascular resistance (from 1,603 ± 619 to 810 ± 317 dynes s cm −5, p < 0.01) and peak LV wall stress (from 476 ± 118 to 410 ± 68 kdynes/cm 2, p = 0.02). Two groups were defined by normal or high LV wall stress. Patients with high LV stress had higher LV end-diastolic pressure (38 ± 12 vs 17 ± 8 mm Hg, p < 0.01), LV end-diastolic volume index (184 ± 24 vs 149 ± 7 ml/m 2, p < 0.01) and systemic vascular resistance (1,423 ± 686 vs 846 ± 293 dynes s cm −5, p = 0.01). Hydralazine decreased stress more in these patients (−101 ± 57 vs −6 ± 9 kdynes/cm 2, p = 0.02), LV end-diastolic pressure (−12 ± 7 vs 2 ± 2 mm Hg, p = 0.02), systolic pressure (−15 ± 13 vs 3 ± 4 mm Hg, p = 0.03) and systemic vascular resistance (−1,053 ± 247 vs −363 ± 83 dynes s cm −5, p < 0.01) than in patients with normal LV stress. Decreased LV stress was caused by decreased systolic and diastolic pressures and/or volumes. Late systolic pressure-volume relations in patients with normal LV stress suggested increased myocardial contractility, but this was not confirmed by LV dP/dt. Hydralazine improves LV function in patients with dilated cardiomyopathy by reducing elevated LV wall stress, with little inotropic effect.