Pulsed tissue Doppler and strain imaging discloses early signs of infiltrative cardiac disease: a study on patients with familial amyloidotic polyneuropathy.

BACKGROUND: Familial amyloidotic polyneuropathy (FAP) is a hereditary systemic amyloidosis with cardiac involvement. As early identification of the cardiac involvement is of major clinical interest we performed this study to test the hypothesis that tissue Doppler imaging (TDI) and strain imaging (SI) might disclose cardiac involvement in patients with early stages of FAP. METHODS: Twenty-two patients with FAP and 36 healthy controls were studied. Standard M-mode and Doppler echocardiography were performed. TDI and SI were used to assess the regional longitudinal left ventricular (LV) lateral and septal and right ventricular (RV) wall functions. All time intervals were corrected for heart rate by dividing with R-R interval and presented as percentage. RESULTS: We found that patients in comparison with controls had increased LV and RV wall thickness and by using TDI a prolonged isovolumic relaxation time (IVRt) at the septal segment (15.0+/-7.0 vs 10.7+/-4.1%, p<0.05) and prolonged isovolumic contraction time (IVCt) at LV lateral (12.8+/-4.3 vs 10.1+/-3.3%, p<0.05), septal (12.5+/-3.5 vs 8.9+/-1.9%, p<0.001) and RV free wall segments (12.0+/-3.6 vs 8.3+/-2.1%, p<0.001). Strain was reduced at LV lateral basal segment (-4.6+/-14.0 vs -20.2+9.1, p<0.001), RV free wall mid segment (-16.2+/-12.8 vs -29.4+/-15.2) as well as both septal segments (-4.1+/-11.7 vs -16.2+/-9.0%, p<0.001, -8.8+/-11.5 vs -19.4+/-8.4%, p<0.001 for septal basal and mid-segment). Even in the absence of septal hypertrophy the septal strain was reduced and the regional IVCt was prolonged. CONCLUSIONS: This is the first clinical study using TDI and strain in patients with FAP showing functional abnormalities before any morphological echocardiographic abnormalities were present. Both the left and right heart functions are involved and the disease should therefore be regarded as biventricular.     

Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study.

AIMS: Non-compaction of the left ventricular myocardium (NCVM) is reportedly exceedingly rare and associated with a high morbidity and mortality. A different genetic background has been suggested for NCVM with [non-isolated NCVM (ni-NCVM)] and without [isolated NCVM (i-NCVM)] other congenital heart defects. We prospectively evaluated both the NCVM subgroups regarding frequency of occurrence and cardiovascular complications in a paediatric population. Results In a prospective, single-centre study, 66/5220 consecutive patients (1.26%) were diagnosed (25 i-NCVM, 41 ni-NCVM). The median age was 4 years (range 0-21), the median follow-up 12 months (range 0-51). The occurrence of congestive heart failure (CHF) at follow-up was 68.0%. CHF was as frequently seen in i-NCVM and ni-NCVM patients (77.5 vs. 62.1%, P = 0.322). The occurrence of arrhythmias (20.0%) and thrombo-embolic events (13.9%) was not different between subgroups. The cardiomyopathy related mortality was 7.1%, with three and one deaths in the i-NCVM and ni-NCVM groups, respectively (P = 0.126). CONCLUSION: When prospectively evaluated, NCVM appears to have been previously under-diagnosed. Whereas arrhythmias and thrombo-embolic events were rare, CHF was frequently found. An equally aggressive anticongestive treatment regimen would seem indicated for both the NCVM subgroups.     

Temporal variation in optimal atrioventricular and interventricular delay during cardiac resynchronization therapy

BackgroundTailored atrioventricular delay (AVd) and interventricular delay (VVd) combination improves hemodynamics in patients treated with cardiac resynchronization therapy (CRT). Whether tailored AVd-VVd combination changes over time is not known.Methods and ResultsTwenty-two patients (18 M, aged 69.9 ± 12.5 years, New York Heart Association class III, QRS ≥ 130 ms, ejection fraction 29.6 ± 8.8%) were implanted with a biventricular device with programmable VVd. Myocardial performance index (MPI) was evaluated during pacing at different VVds and AVds at baseline and after 6 and 12 months. The optimal AVd-VVd combination was identified by the minimum MPI. After optimization, the appropriate AVd-VVd combination was programmed in each patient. MPI at 6-month follow-up after optimization was significantly higher compared with baseline (.79 ± .21 vs. .59 ± .15, P < .05). Re-optimization of AVd-VVd combination was required after 6 months in 21 of 22 (95%) patients. Re-optimization significantly reduced MPI compared with the value prior to re-optimization (.56 ± .15 vs. .79 ± .21, P < .05). The MPI remained unchanged at 12-month compared with 6-month follow-up (.59 ± .19 vs. .56 ±.15, P = NS). Clinical symptoms and reverse left ventricular remodeling were sustained at 6-month and 12-month follow-up.ConclusionOptimal AVd and VVd combination changes over time in patients with heart failure. Sustained improvement in clinical symptoms and reverse left ventricular remodeling after CRT are not temporally associated with improvement in MPI.     

缺血性与扩张性心肌病终末期心肌白细胞介素-6 mRNA表达与心功能参数间的关系

目的在检测晚期心力衰竭患者心肌组织中白细胞介素-6(IL-6)mRNA表达的基础上,探讨IL-6mRNA与心功能参数间的关系,为揭示IL-6参与心力衰竭发生发展提供依据.方法以Northern-blot法检测27例接受心脏移植患者(缺血性心肌病12例,扩张性心肌病15例)不同部位心肌组织中IL-6mRNA表达水平,并以线性回归比较IL-6mRNA与移植前血流动力学和超声心动参数间的关系.结果所有患者心肌组织中均有Ⅱ-6mRNA表达,扩张性心肌病左心室IL-6mRNA表达水平高于缺血性心肌病(P=0.006).IL-6mRNA在左心室的表达水平与左心室射血分数负相关(r=-O.62,P＜O.05),在左、右心房的表达水平与左心室舒张末内径正相关(r=0.70,P＜0.05;r=0.93,P＜O.001),在右心室的表达水平与心排指数负相关(r=-O.49,P＜0.05),在右心房的表达水平不但与肺血管阻力正相关(r=0.63,P＜O.05),也与左心室收缩末内径正相关(r=0.82,P＜0.001).结论晚期心力衰竭患者心肌组织中有IL-6mRNA表达,Ⅱ-6mmRNA水平与部分心功能参数相关,提示IL-6可能参与心力衰竭的发生发展.     

Cardiac and neuromuscular implications of left bundle branch block in left ventricular hypertrabeculation/noncompaction

BACKGROUND:

Left bundle branch block (LBB) is frequently found in left ventricular hypertrabeculation/noncompaction (LVHT).

OBJECTIVES:

To compare LVHT patients with and without LBB regarding LVHT location and extension, left ventricular function, symptoms, electrocardiographic findings, prevalence of neuromuscular disorders (NMDs) and mortality during follow-up.

METHODS:

The charts of patients who underwent transthoracic echocardiographic examination at the Krankenanstalt Rudolfstiftung (Wien, Austria) between June 1995 and November 2006 were examined.

RESULTS:

LVHT was diagnosed in 102 patients (30 women) with a mean (± SD) age of 53±16 years (range 14 to 94 years). A specific NMD was diagnosed in 21 patients and an NMD of unknown etiology was diagnosed in 47. The neurological investigation was normal in 14 patients and 20 patients refused the investigation. The 24 patients with LBB were older (61 versus 51 years of age; P<0.01), and suffered from exertional dyspnea (96% versus 59%; P<0.01) and heart failure (79% versus 46%; P<0.01) more often than patients without LBB. LBB patients had less frequent tall QRS complexes (8% versus 47%; P<0.01) and ST-T wave abnormalities (4% versus 50%; P<0.01) than patients without LBB. Patients with LBB had a larger left ventricular end-diastolic diameter (73 mm versus 61 mm; P<0.01), worse left ventricular fractional shortening (15% versus 26%; P<0.01) and more extensive LVHT (1.8 versus 1.5 ventricular segments; P<0.05). The prevalence of NMDs did not differ between patients with and without LBB. Survival did not differ between patients with and without LBB during follow-up.

CONCLUSIONS:

LBB is associated with increased age, decreased systolic function and increased extension of LVHT. Whether LBB is a prognostic factor in LVHT remains speculative.     

Significant improvement of myocardial function following cardiac support device implantation: illustration by two-dimensional strain.

Cardiac support devices (CSD) offer a new therapeutic alternative to patients with end-stage refractory heart failure and severe left ventricle (LV) dilatation. Passive mechanical constraint has been shown to reduce LV dilatation as well as to improve LV function and patient symptoms. Using a new echocardiographic technique to evaluate myocardial strain from bidimensional acquisitions, we describe the case of a patient with significant improvement in global and regional LV functions, as well as LV dyssynchrony following CSD implantation.     

Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure

BackgroundIsolated ventricular non-compaction (IVNC) is a rare disorder characterized by prominent trabecular meshwork and deep recesses. We retrospectively assessed the clinical characteristics and natural course of IVNC in adults diagnosed at our hospital.Methods and ResultsSixty-seven adult patients (44 male, mean age 41 ± 18 years) with the diagnosis of IVNC were evaluated in this retrospective cohort. Its prevalence was found to be .14%. Forty-seven patients (70%) had class I/II functional capacity. Fifty-seven patients (85%) had electrocardiographic abnormalities, and the most common one was left ventricular (LV) hypertrophy (25%). LV systolic function was depressed in 44 patients (66%), with a median ejection fraction (EF) of 35% (range: 20%–48%) at diagnosis. Multiple regression analysis revealed that age at initial presentation, the total number of affected segments, and the ratio of non-compaction/compaction (NC/C) were the independent predictors of LV systolic dysfunction. Familial occurrence of IVNC was 33%. During a mean follow-up of 30 months (range: 9–50 months), major complications including ventricular tachycardia, heart failure requiring hospitalization, and cerebrovascular events were observed in 36%, 34%, and 9% of the patients, respectively. Ten patients (15%) with IVNC died in this study. LVEF at initial presentation and functional capacity at last visit were found to be independent predictors of mortality.ConclusionThis study suggests that IVNC is a form of cardiomyopathy with higher prevalence and relatively better prognosis than previously reported. Age at initial presentation, ratio of NC/C, and number of affected segments seem to be major determinants of LV systolic dysfunction, while initial LVEF and last functional capacity predict mortality in this cohort.     

Heart failure in general and cardiac transplant patients with COVID-19

Coronavirus disease 2019 (COVID-19) is primarily an infection of the respiratory tract, but it can have multisystem manifestations. Cardiac complications of COVID-19 can range from acute myocardial injury, cardiac arrhythmias, or heart failure, amongst others. Heart failure (HF) in COVID-19 can be a de novo process or due to worsening of pre-existing cardiovascular ailment. HF in a patient with COVID-19 not only poses challenges in clinical presentation and management of COVID-19 but also affect prognosis of the patient. This article aims to succinctly revisit the implications of this pandemic regarding pre-existing HF or new-onset HF based on prevailing data. It also focuses on the management and special recommendations from prior studies and guidelines.     

肥厚型心肌病扩张期2例报告及文献复习

目的 :探讨肥厚型心肌病 (HCM)的命名伴心室扩张和心力衰竭的可能原因。方法 :报告本病 2例并结合文献复习进行分析。结果与结论 :1以 HCM扩张期命名较为恰当。 2其发生的可能原因是心肌缺血、酒精性心肌损害和病毒持续感染。 3预后较差。     

Surgical treatment of functional mitral regurgitation in dilated cardiomyopathy

Functional mitral regurgitation is a significant complication of end-stage cardiomyopathy. Dysfunction of one or more components of the mitral valve apparatus occurs in 39–74% and affects almost all heart failure patients. Survival is decreased in subjects with more than mild mitral regurgitation irrespective of the aetiology of heart failure. The goal of treating functional mitral regurgitation is to slow or reverse ventricular remodelling, improve symptoms and functional class, decrease the frequency of hospitalization for congestive heart failure, slow progression to advanced heart failure (time to transplant) and improve survival. This article reviews the role of mitral valve surgery in patients with heart failure and dilated cardiomyopathy.     

慢性心力衰竭校正QT间期与QT离散度及心功能相关性

目的 :探讨慢性心力衰竭患者校正QT间期 (QTc)与QT离散度 (QTd)及心功能超声参数相关性。方法 :研究了 12 6例左室射血分数 (LVEF) <4 5 %的慢性心力衰竭患者QTc与QTd、QRS间期、临床指标、心脏超声心动图参数的相关性。结果 :QTc与年龄 (r =0 .2 1,P <0 .0 1)、左室舒张末期直径 (r =0 .2 6 ,P <0 .0 1)、左房直径 (r =0 .2 4 ,P <0 .0 1)、QRS间期 (r =0 .37,P <0 .0 1)、QTd(r =0 .4 1,P <0 .0 1)呈正相关 ;与短轴缩短率 (r=- 0 .2 8,P <0 .0 1)、LVEF(r =- 0 .33,P <0 .0 1)呈负相关 ;与二尖瓣E波和A波峰值速度比值、等容舒张时间无相关性 (P >0 .0 5 )。多元回归分析显示 ,QTc和QTd、QRS间期、LVEF存在独立相关性。结论 :QTc与心力衰竭患者预后影响因素有关 ;QTc与QTd存在独立相关性 ,其生物相关性值得进一步研究     

Tissue Doppler Imaging in the assessment of selection and response from cardiac resynchronization therapy.

Mechanistic studies, observational evaluations, and randomized trials have consistently demonstrated the beneficial effects of cardiac resynchronization therapy (CRT) in patients with moderate-to-severe chronic systolic heart failure and ventricular dyssynchrony who have failed optimal medical treatment. However, despite the promising results, in some patients undergoing CRT, the symptoms of heart failure do not improve or even worse. One of the most important reasons for this failure is probably the lack of distinct mechanical dyssynchrony before implantation. This review discusses the actual and potential role of Tissue Doppler Imaging in selection of patients and optimisation of CRT.     

Evidence for a role of immunoproteasomes in regulating cardiac muscle mass in diabetic mice

The ubiquitin-proteasome system plays an important role in regulating muscle mass. Inducible immunoproteasome subunits LMP-2 and LMP-7 are constitutively expressed in the heart; however, their regulation and functions are poorly understood. We here investigated the hypothesis that immunoproteasomes regulate cardiac muscle mass in diabetic mice. Type 1 diabetes was induced in wildtype mice by streptozotocin. After hyperglycemia developed, insulin and the proteasome inhibitor epoxomicin were used to treat diabetic mice for 6 weeks. Isolated mouse hearts were perfused with control or high glucose solution. Catalytic proteasome β-subunits and proteolytic activities were analyzed in the heart by immunoblotting and fluorogenic peptide degradation assays, respectively. Insulin and epoxomicin blocked loss of heart weight and improved cardiac function in diabetic mice. LMP-7 and its corresponding chymotryptic-like proteasome activity were increased in diabetic hearts and high glucose-treated hearts. Myosin heavy chain protein was decreased in diabetic hearts, which was largely reversed by epoxomicin. High glucose decreased LMP-2 protein levels in perfused hearts. In diabetic hearts, LMP-2 expression was downregulated whereas expression of the phosphatase and tensin homologue deleted on chromosome ten (PTEN) and the muscle atrophy F-box were upregulated. Moreover, mice with muscle-specific knockout of PTEN gene demonstrated increased cardiac muscle mass, while mice with LMP-2 deficiency demonstrated PTEN accumulation, muscle mass loss, and contractile impairment in the heart. Therefore, we concluded that high glucose regulates immunoproteasome subunits and modifies proteasome activities in the heart, and that dysregulated immunoproteasome subunits may mediate loss of cardiac muscle mass in experimental diabetic mice.     

The effect of ventricular pacing on measurements of left ventricular function: a comparison between echocardiographic methods and with radionuclide ventriculography.

AIMS: Different methods exist for measuring left ventricular function echocardiographically; each may be error prone due to the abnormal pattern of ventricular activation during pacing. METHODS AND RESULTS: Echocardiography was undertaken on 307 patients with permanent pacemakers; a subset of 57 underwent radionuclide ventriculography. Intrinsic and paced beats were analysed for left ventricular function by: Simpson's bi-plane, Teicholz M-mode, wall-motion scoring and 'eyeball' assessment. Agreement between techniques and with radionuclide ventriculography were compared according to intrinsic or paced beats. Echocardiographic measures of ejection fraction give mean values 5% higher than radionuclide ventriculography (Simpson's 30+/-9%, vs. Teicholz 30+/-13% vs. radionuclide ventriculography 25+/-9%, p=0.03). Agreement between Simpson's, Teicholz and radionuclide ventriculography by Bland-Altman analysis showed poor agreement (Simpson's vs. Teicholz range (4xSD)=57%, Simpson's vs. radionuclide ventriculography=36%, Teicholz vs. radionuclide ventriculography=46%, p=0.02), the level of agreement deteriorates with ventricular pacing (Simpson's vs. Teicholz range=61%, Simpson's vs. radionuclide ventriculography=34%, Teicholz vs. radionuclide ventriculography=47%, p=0.02). The correlation between wall motion analysis and radionuclide ventriculography is moderately poor (all subjects r=0.58, ventricular pacing r=0.52, not pacing r=0.66). CONCLUSION: Echocardiography and radionuclide ventriculography are the only non-invasive techniques to assess left ventricular function in the paced population. Results are poorly interchangeable and the accuracy of any comparison dependent on the underlying rhythm.     

Propranolol prevents enhanced stress signaling in Gs alpha cardiomyopathy: potential mechanism for beta-blockade in heart failure

Beta-adrenergic receptor (beta-AR) blockade is now widely utilized therapeutically for heart failure, but its cellular mechanism of action is not clear. Mice with cardiac-specific overexpressed Gs alpha develop cardiomyopathy with age, which can be prevented by beta-AR blockade, making this model potentially useful for addressing this question. Our hypothesis was that distal mechanisms in beta-AR signaling, i.e. mitogen-activated protein kinases, were a potential mechanism. At 6-9 months, when cardiomyopathy began to develop in Gs alpha mice, there were significant increases in phospho-kinase levels of p38 MAP kinase (p38 MAPK), and p70(S6K) compared to wild type. In contrast, phospho-kinase levels of ERK and Akt were increased at 9-10 months, but phospho-kinase levels of c-Jun N-terminal kinase (JNK) increased only at 15-20 months (when cardiomyopathy was fully manifest). Treatment of 9-10 months old Gs alpha mice with propranolol for 5 weeks reverted the phospho-kinase levels of these kinases known to be involved in the growth and death of cardiac myocytes. Another novel observation of this study was that there were also decreases in total protein levels of p38 MAPK, p70(S6K), JNK, and Akt following beta-AR blockade. Thus, chronically enhanced beta-AR signaling elicits a differential pattern of altered mitogen-activated protein kinases, which was reversed with beta-AR blockade, raising the possibility that the beneficial effects of beta-AR blockade therapy in heart failure may be due in part to the inhibition of these pathways.