Myocardial biopsy findings and gadolinium enhanced cardiovascular magnetic resonance in dilated cardiomyopathy

BACKGROUND: In some patients suffering from dilated cardiomyopathy (DCM) magnetic resonance imaging (MRI) shows late gadolinium enhancement with variable distribution. Myocardial biopsies in DCM reveal a chronic myocardial inflammatory process in almost 50% and myocardial persistence of adenoviral or enteroviral genome in about 15% of the patients. AIMS: We prospectively investigated whether the pattern of late gadolinium enhancement correlates with myocardial biopsy findings. METHODS AND RESULTS: 42 patients with DCM and 42 control subjects underwent contrast MRI. In the DCM group, endomyocardial biopsies were performed and evaluated for inflammation and viral genome. None of the control subjects showed late gadolinium enhancement whereas in 29 DCM patients (69%) gadolinium enhancement was detectable (p<0.001). 21 of the DCM patients (50%) showed midwall septal enhancement, 7 patients (17%) showed a patchy distribution of hyperenhancement and 1 patient (2%) showed enhancement typical for ischemic heart disease. In myocardial biopsy analysis, 2 patients (5%) showed persistence of viral genome, 18 patients (43%) showed inflammation and in 22 patients (52%) neither virus nor inflammation was detected. The pattern of late gadolinium enhancement and myocardial biopsy findings were not significantly correlated (p = 0.854). CONCLUSION: MRI as a non-invasive technique cannot replace myocardial biopsy for the differential diagnosis of DCM.     

扩张型心肌病患者磁共振下心肌特征与室性心律失常的关系

目的:探究非缺血性扩张型心肌病(NIDCM)患者磁共振下心肌强化特征与发生室性心律失常(VA)事件的关系,找出相关危险因素.方法:将2019年11月-2021年7月于郑州大学第一附属医院就诊的154例NIDCM患者纳入研究.根据发生VA事件的情况将其分为对照组及失常组,比较两组患者的一般资料及心脏磁共振延迟强化(LGE...     

Assessing left atrial function in patients with atrial fibrillation and valvular heart disease using cardiovascular magnetic resonance imaging

BackgroundAtrial fibrillation (AF) is common arrhythmia in valvular heart disease (VHD) and is associated with adverse outcomes.HypothesisTo evaluate the left atrial (LA) function in patients with AF‐VHD by cardiovascular magnetic resonance imaging feature tracking (CMR‐FT) using LA strain (ε _s/ε _e/ε _a) and their corresponding strain rate (SRs/SRe/SRa).MethodsThis was a retrospective cross‐sectional inter‐reader and intra‐reader reproducibility conducted from July 1, 2020, to January 31, 2021. A total of 39 patients with AF‐VHD (rheumatic heart valvular disease [RHVD] [n = 22], degenerative heart valvular disease [DHVD] [n = 17]) underwent MRI scans performed with drug‐controlled heart rate before correcting the rhythm and valves through maze procedure. Fifteen participants with normal cardiac MRI were included as healthy control. ε _s/SRs, ε _e/SRe, and ε _a/SRa, corresponding to LA reservoir, conduit, and booster‐pump function, were assessed using Feature Tracking software (CVI42 v5.12.1).ResultsCompared with healthy controls, LA global strain parameters (ε _s/ε _e/ε _a/SRs/SRe/SRa) were significantly decreased (all p < 0.001), while LA size and volume were increased in AF‐VHD group (all p < 0.001). In the subgroup, RHVD group showed lower LA total ejection fraction (LATEF) and strain data than DHVD group (12.6% ± 3.3% vs. 19.4 ± 8.6, p = 0.001). Decreased LATEF was significantly related to altered LA strain and strain rate, especially in ε _s, ε _e, and SRs (Pearson/Spearman r/ρ = 0.856/0.837/0.562, respectively; all p < 0.001). Interstudy and intrastudy reproducibility were consistent for LA volumetry and strain parameters (intraclass correlation coefficient: 0.88–0.99).ConclusionsCMR‐FT can be used to assess the LA strain parameters, and identify LA dysfunction and deformation noninvasively, which could be a helpful functional imaging biomarker in the clinical treatment of AF‐VHD.     

Pathophysiologic significance of left ventricular hypertrophy in dilated cardiomyopathy

Background and hypothesis: Patients with dilated cardiomyopathy (DCM) with left ventricular hypertrophy (LVH) have been found to have a better prognosis than patients without LVH. However, the pathophysiologic mechanism for that has not been investigated. We sought to clarify the pathophysiologic significance of LVH in DCM. Methods: We performed isoproterenol infusion echocar-diography (0.02 m?g/kg/min) in 17 patients with DCM, and measured plasma epinephrine and norepinephrine levels at rest and at the end of ergometer exercise in 14 of the 17 patients. Patients were classified into groups according to the presence (9 patients) (LVH+) or absence (8 patients) (LVH-) of LVH. Left ventricular hypertrophy was defined as an inter-ventricular thickness or posterior wall thickness ≥13 mm. Results: Although there was no significant difference between groups in fractional shortening at rest during isoproterenol infusion, fractional shortening was significantly higher in the LVH (+) group than in the LVH (-) group (29 ± 9 vs. 17 ± 8%;p<0.025). Although there was no significant difference in plasma norepinephrine level, it was significantly lower in the LVH (+) group than in the LVH (-) group (233 ± 169 vs. 519 ± 258 pg/ml;p<0.05) at the end point of the exercise. Conclusion: Systolic reserve, represented by the response to isoproterenol, is greater in patients with DCM with LVH than in those without LVH, and a lower plasma level of norepinephrine is needed to activate the myocardium during ex ercise in patients with DCM with LVH. This pathophysiologic characteristic could be one of the mechanisms which explain a better prognosis in patients with DCM with LVH.     

扩张性心肌病患者心房Cx43蛋白的表达及其意义

目的:观察扩张性心肌病（dilated cardiomyopathy,DCM）患者心房连接蛋白43（Cx43）的表达、磷酸化的Cx43（pCx43）及其分布特征。探讨Cx43与房颤（atrial fibrillation,AF）之间的关系。方法: 18例接受心脏移植术的DCM患者中,8例无AF史且手术时为窦性心律（SR,SR组）,6例有AF史但手术时为SR（AF+SR组）,4例有AF史且手术时有AF（AF+AF组）。用超声心动图(ECG)测定左心房大小与左心室收缩功能;用免疫印迹法及免疫荧光染色法检测Cx43、pCx43及其分布。结果: DCM患者的左心房直径(LAD)、左室射血分数(LVEF)在SR、AF+SR、AF+AF组间均没有显著差异。与SR组相比,AF+SR组心房组织中Cx43和pCx43无显著差异;AF+AF组总Cx43的表达量及pCx43显著升高(P<0.01及P<0.05)。病理观察提示,AF+AF组Cx43和pCx43升高,Cx43向细胞两侧分布,且pCx43升高。结论: DCM患者的房颤节律伴随Cx43的表达、磷酸化及其分布异常。     

Atenolol in dilated cardiomyopathy: A clinical instrumental study

Summary The usefulness of beta blockers in the treatment of congestive heart failure has been questioned. We selected 11 patients, mean age 47.1±13.8, affected by dilated cardiomyopathy in NYHA class III, who had been taking digoxin and diuretics for a long time. Atenolol 50 mg was added to conventional therapy. Both before and 3 months after treatment a clinical evaluation, chest x-ray, an exercise test, and an echocardiogram were performed. We observed an improvement of NYHA class in five patients. However, the exercise test showed no improvement: 2310±1299 vs. 2902±983 total kgm (ns). The echocardiogram showed improvements of the end-systolic diameter (from 6.3±1 cm to 5.9±0.8 cm; p<0.02), the fractional shortening (from 13.6±6.3% to 15.2±5.6%; p<0.05) the radius/thickness ratio (from 4.14±0.5 to 3.5±0.5; p<0.05), and the wall stress (from 208±49 g/cm² to 163.5±41 g/cm²; p<0.02). The inotropic state index did not show any changes. We conclude that in some patients with dilated cardiomyopathy beta blockers may improve the clinical status and left ventricular performance.     

Myocardial histological changes in dilated cardiomyopathy during a long-term left ventricular assist device support

Summary As the myocardium in patients with dilated cardiomyopathy (DCM) is deteriorating progressively, resulting in a decrease in left ventricular function, patients with end-stage DCM may require implantation of a left ventricular assist device (LVAD) unless they undergo heart transplantation. Although LVAD has been reported to provide excellent hemodynamic support, no data are currently available about the effects of long-term LVAD support on the myocardium in patients with DCM. We describe two patients with end-stage DCM who underwent LVAD implantation and were supported with LVAD for 524 and 245 days, respectively. Serial myocardial biopsies showed increases in myocardial cell diameter and intercellular fibrosis, despite excellent hemodynamic support by LVAD. These data suggest that the myocardium in patients with end-stage DCM deteriorates progressively, even if the preload of the left ventricle is reduced by LVAD.     

Impaired Systolic torsion in dilated cardiomyopathy: Reversal of apical rotation at mid-systole characterized with magnetic resonance tagging method

Abstract Left ventricular (LV) torsion plays an important role in squeezing the blood out of the heart. To characterize the systolic torsion in LV dysfunction, we studied using magnetic resonance imaging myocardial tagging method in 26 subjects: 17 patients with dilated cardiomyopathy (DCM, LV ejection fraction [EF], 27 ± 8%) and 9 healthy control subjects. Grid-tagged LV short-axis cine images were acquired at base, mid and apex levels. Tag-intersections were tracked during the systole, thereby determining rotation angle (positive indicated clockwise from the apex). Peak torsion was defined as the maximum difference in rotation angle between the base and apex. Time to peak torsion was expressed as % systole by dividing the time by a total systolic time. Amplitude of the rotation at peak was less in DCM than in controls at both the base (0.1 ± 2.9 vs. 2.6 ± 1.6°, P < 0.05) and apex (−5.9 ± 5.3 vs. −11.2 ± 2.5°, P < 0.01). Amplitude of peak torsion was then less in DCM than in controls (6.1 ± 3.4 vs. 13.6 ± 2.5°, P < 0.001), and the timing of peak was earlier (66 ± 22 vs. 104 ± 16% systole, P < 0.001). The amplitude of peak torsion was correlated with LVEF (r=0.74, P < 0.001). In conclusion, amplitude of systolic torsion was impaired in proportion to LV function. Systolic torsion in LV dysfunction was characterized by the discontinuing counter-rotation of the apex to the base before end-systole. Presented in part at the Annual Scientific Session of ACC 2004 in New Orleans, USA There are no financial obligations that could lead to conflict of interest regarding this study.     

心脏再同步化治疗晚期扩张型心肌病13例的效果

目的: 观察心脏再同步化治疗（CRT）晚期充血性心力衰竭的临床疗效。方法: 晚期扩张型心肌病患者13例接受CRT,NYHA心功能分级为Ⅲ～Ⅳ级,左室射血分数（LVEF）为（27.4±9.7）%,左室舒张末期内径（LVEDD）为(72.8±9.6)mm,QRS时限为( 137.8＋30.4)ms。术后观察QRS时限的变化,随访左室电极起博阈值、心功能分级、LVEF及LVEDD。结果: 术后QRS时限减少为（123.8±17.1）ms。所有患者随访3～38月,左室电极慢性阈值为（1.1±0.6）V/0.4 ms。与术前相比,NYHA心功能分级从(3.4±0.5)降低为(1.5±0.9); LVEF从(27.4±9.7)%上升至(43.5±18.5)%(P<0.05);LVEDD从(72.8±9.6)mm缩小为(65.5±11.6)mm（P<0.05）。结论: CRT可改善心功能,提高LVEF,并可逆转左心室重构。     

A rare cause of reversible dilated cardiomyopathy: hypocalcemia

Hypocalcemia is a rare cause of reversible heart failure. We reported a 40-year-old woman who had severe heart failure resistant to the usual antifailure therapy. She had severe hypocalcemia due to hypoparathyroidism after strumectomy. Echocardiography showed a large left ventricle with very low ejection fraction of 25% and moderate mitral regurgitation. After supplementation of calcium and vitamin D, her clinical situation and hemodynamics improved rapidly. At 15 months, myocardial impairment resolved fully. In conclusion, hypocalcemia should be considered in the differential diagnosis of resistant severe heart failure.     

扩张型心肌病患者窦性心率震荡的研究

目的 探讨扩张型心肌病患者窦性心律震荡（HRT）的特征及其对预后的影响。方法 选择扩张型心肌病并室早的患者187例，选择同期有室早而无器质性心脏病100例作对照组，分别检测窦性心率震荡的初始（TO）和窦性心率震荡的斜率（TS）：将窦性心率震荡分类为HRT1（TO〈0且TS〉2．5ms／RR间期）、HRT2（TO≥0或TS≤2．5ms／RR间期）、HRT3（TO≥0且TS≤2．5ms／RR间期），分别对不同心功能分级，存活组及死亡组进行分析。结果 扩张型心肌病与对照组比较TO、TS均有显著性差异（P〈0．001和P〈0．005）。在心功能Ⅲ级与Ⅳ级时HRT2,在两组间无显著性差异（p〉0．05），其他不同分级心功能的窦性心率震荡分类均有显著差异（p〈0．05或p〈0．01）。存活组与死亡组比较窦性心率震荡分类亦有显著性差异（HRT1p〈0．01，HRT2p〈0．05，HRT3p〈0．01）。结论 扩张型心肌病患者存在室早后窦性心率震荡减弱或消失现象，窦性心率震荡对其预后有一定的预测价值。     

中青年扩张型心肌病伴心力衰竭患者心功能的临床研究

目的探讨青年人扩张型心肌病伴或不伴心力衰竭与心脏功能关系及临床意义。方法对我院扩张型心肌病伴有心力衰竭患者65例（心力衰竭组）和不伴有心力衰竭患者58例（非心力衰竭组）给予超声心动图、心电图检查。比较两组心脏的大小、功能以及发生心律失常的情况。结果①心力衰竭组的左心室收缩末内径（LVESd）、左心室舒张末内径（LVEDd）、左心房内径（LAD）、左心室射血分数（LVEF）,左心室短轴缩短分数（LVFS）与非心力衰竭组比较,差异有统计学意义（P〈0.01）。②心力衰竭组在心电图上的QRS时限、肢导低电压、异常Q波、ST-T改变的比例略高于非心力衰竭组,差异无统计学意义（P〉0.05）。心力衰竭组的房性心律失常、室性心律失常和传导阻滞发生率与非心力衰竭组比较,差异有统计学意义（P〈0.01）。③心力衰竭组患者入院时心脏功能Ⅲ级、Ⅳ级分别占58.4%、21.5%。两组经住院治疗后,心脏功能较入院时明显改善（P〈0.05）。结论扩张型心肌病伴心力衰竭、心律失常时,LVEDd明显增大,LVEF显著降低,并与心脏功能有一定的相关性。早期诊断和治疗,除去心力衰竭诱发因素,能够改善心脏功能,提高患者的生活质量,改善预后。     

Influence of the force--frequency relationship on haemodynamics and left ventricular function in patients with non-failing hearts and in patients with dilated cardiomyopathy

In isolated human myocardium it was shown that a positive force-frequencyrelationship occurs in non-failing myocardium; however, theforce-frequency relationship was found to be inverse in myocardiumfrom failing human hearts. In order to investigate the clinicalrelevance of these experimental findings, the influence of heartrate changes on haemodynamics and left ventricular functionwas studied in eight patients without heart failure and in ninewith failing dilated cardiomyopathy (NYHA II–III). Rightventricular pacing was performed at a rate slightly above sinusrate and at 100, 120 and 140 beats. min^–1 Haemodynamicparameters were obtained by right heart catheterization andby high-fidelity left ventricular pressure measurements. Leftventricular angiography was performed at basal pacing rate andat 100 and 140 beats. min^–1 With increasing heart rate,cardiac index increased in patients with normal left ventricularfunction from 2·9 ± 0·2 to 3·5 ±0·21. min^–1. m^–2 (P<0·01) and decreasedcontinuously in patients with dilated cardiornyopathy from 2·6± 0·1 to 2·2 ± 0·11. min^–1. m^–2 (P<0·05). With increasing heart rate,the maximum rate of left ventricular pressure rise increasedin non-failing hearts from 1388 ± 86 to 1671 ±88 mmHg. s^–1 (P<0·01) and did not change infailing hearts. Ejection fraction decreased from 27 ± 3% to 19 ±2% in patients with dilated cardiomyopathy (P<0·05)when the pacing rate was changed from 84 ± 2 beats. min^–1to 140 beats. min^–1, which was associated with a significantlyincrease in end-systolic volume without significantly changesin end-diastolic volume. In patients with normal left ventricularfunction, when the pacing rate was changed from 85 ±3 beats. min^–1 to 140 beats. min^–1, end-diastolicvolume decreased significantly by 13%, whereas left ventricularend-systolic volume and ejection fraction did not significantlychange. Left ventricular systolic and end-diastolic pressuresdid not significantly change with pacing tachycardia in eithergroup. The frequency-related changes in left ventricular volumesand pressures indicate that the differrent haemodynamic effectsof pacing tachycardia in both groups of patients result predominantlyfrom frequency effects on myocardial function and not from frequencyeffects on preload or afterload. These data indicate that recentexperimental findings of positive force-frequency effects innon-failing and negative force-frequency effects in failinghuman myocardium are relevant for the intact heart.     

超声声学定量技术评价扩张型心肌病患者左房功能的临床研究

目的　探讨超声声学定量 (AQ)技术评价扩张型心肌病 (DCM)患者左房功能的临床价值。方法　使用 AQ技术对 6 0例 DCM患者 (DCM组 )和 5 5例查体健康者 (对照组 )左房功能进行测量。结果　与对照组比较 ,DCM组左室收缩末期左房容量 (ESV)、快速排空末期左房容量 (EREV)、左室舒张末期左房容量 (EDV)、左房收缩期左房排空容量 (AE)、左房排空分数 (AEF)、峰值左房排空率 (PAER)均显著增加 (P<0 .0 5 ,<0 .0 1) ;快速排空期左房排空容量 (RE)、左房射血分数 (L AEF)、左房总排空容量 (L AV )、峰值充盈率 (PFR)均明显降低 (P <0 .0 5 ,<0 .0 1) ;DCM心功能 级组左房快速排空分数 (REF)、RE/ AE、峰值快速排空率 (PRER)、PRER/PAER升高 (P <0 .0 5 ) ,心功能 、 、 级组减低 (P <0 .0 5 )。结论　 DCM患者左房收缩功能减低 ;整个心动周期左房容量扩大 ,左房储血功能增加 ;左房的通道功能在心功能 级组升高 ,心功能 、 、 级组减低     

Cardioprotective effects of trimetazidine on patients with dilated cardiomyopathy and left bundle branch block

Background The effects of trimetazidine on patients with dilated cardiomyopathy(DCM) and left bundle branch block(LBBB) are not clear. Methods Sixty consecutive patients with DCM, LBBB and heart dysfunction(35% ≤ LVEF ≤ 45%) were randomly allocated to heart failure therapy plus trimetazidine group(20 mg three times a day; 30 patients) or heart failure therapy alone group(30 patients). During follow-up of 6months, QRS morphology, plasma NT-proBNP, 6-minute walk test(6MWT) and echocardiographic indexes were measured. Results At the 6th month, a significant functional improvement was noted in patients receiving trimetazidine added to heart failure treatment. In patients from the trimetazidine group, an increase of left ventricular ejection fraction(LVEF) was noted(from 38 ± 5.5% to 47 ± 7.1%, P = 0.020). The increase of LVEF in the trimetazidine group was associated with a more significant reduction of the left ventricular endsystolic diameter(LVESD) compared with the control group at 6-month follow-up. The NT-pro BNP levels decreased significantly in the trimetazidine group(3.11 ± 0.47 at baseline and 2.77 ± 0.45 at 6-month followup, P 0.01) and the 6MWT increased significantly in the trimetazidine group(325 ± 44 m at baseline and419 ± 56 m at 6-month follow-up, P 0.01). The differences in NT-pro BNP levels(2.77 ± 0.45 vs 2.96 ±0.46, P = 0.036) and 6MWT(419 ± 56 m vs 366 ± 54 m, P = 0.032) between the two groups were significant at 6-month follow-up. Conclusions Trimetazidine can improve LV function caused by DCM and LBBB. The positive effects of trimetazidine on LV function are especially evident in patients with optimization of drug therapy for heart failure, which strongly suggests an additive effect of these therapy modalities.     

Partial left ventriculectomy improves left ventricular end systolic elastance in patients with idiopathic dilated cardiomyopathy

OBJECTIVE: To assess the effect of partial left ventriculectomy (PLV) on estimate of left ventricular end systolic elastance (Ees), arterial elastance, and ventriculoarterial coupling. PATIENTS: 11 patients with idiopathic dilated cardiomyopathy before and two weeks after PLV, and 11 controls. INTERVENTIONS: Single plane left ventricular angiography with simultaneous measurements of femoral artery pressure was performed during right heart pacing before and after load reduction. RESULTS: PLV increased mean (SD) Ees from 0.52 (0.27) to 1.47 (0.62) mm Hg/ml (p = 0.0004). The increase in Ees remained significant after correction for the change in left ventricular mass (p = 0.004) and end diastolic volume (p = 0.048). As PLV had no effect on arterial elastance, ventriculoarterial coupling improved from 3.25 (2.17) to 1.01 (0.93) (p = 0.017), thereby maximising left ventricular stroke work. CONCLUSION: It appears that PLV improves both Ees and ventriculoarterial coupling, thus increasing left ventricular work efficiency.     

心脏磁共振对肥厚型心肌病应用价值的研究

肥厚型心肌病(HCM)是最常见的遗传性心肌病,在普通人群中其发病率为1/500,HCM临床表现多样,主要发病机制是心肌细胞肥厚、排列紊乱、心肌纤维化及胶原沉积以及不稳定的心电活动容易导致恶性心律失常所致。近年来心脏磁共振(CMR)被广泛应用于HCM的诊断以及与其他疾病的鉴别诊断,CMR不仅能够准确评估心室功能、心室壁厚度,并且钆延迟强化(LGE)能够无创检测心肌纤维化及瘢痕。重要的是越来越多的研究证明LGE与HCM等多种心血管病的发生相关,并可以预测其发生。本文将回顾相关文献,总结CMR对HCM的应用价值,使CMR更好的应用于临床。