Long-term prognosis in patients with echocardiographic left ventricular dysfunction masquerading as dilated cardiomyopathy]

The long-term prognosis and prognostic factors were assessed in patients with left ventricular dilatation with impaired function secondary to idiopathic dilated cardiomyopathy (DCM) or to heart diseases of various pathogeneses masquerading as DCM (DCM-like). The echocardiographic criteria for DCM-like disease were 1) left ventricular end-diastolic dimension exceeding 60 mm and 2) fractional shortening less than 15%. Those who showed improvement in either of these 2 parameters within 3 months were excluded from this study. One hundred and fifty-eight of 35,250 serially examined patients fulfilled the definitions. The pathogeneses of diseases were valvular heart disease (VHD) in 30 patients, ischemic heart disease (IHD) in 37, alcoholic cardiomyopathy (AC) in 12, hypertensive heart disease (HHD) in 31 and DCM in 48. All of the survivors were followed for more than 24 months; an average of 40 months. During this period, there were a total of 75 deaths, 18 (27%) of whom were judged as sudden death. Five-year survival rates calculated using the Kaplan-Meier method in AC and HHD were 86% and 77%, respectively, which were significantly higher than those in DCM (48%; p < 0.05); whereas, patients with VHD and IHD had lower values (60% and 46%, respectively) which were nearly equal to those in DCM. Factors that contributed to the increase in total cardiac mortality were advanced age, higher NYHA functional class, larger cardiac size on chest radiograph and history of heavy alcohol intake. Each of these factors were closely related to the specific pathogeneses as follows: age to IHD, cardiac size to VHD, and NYHA functional class to DCM. Long-term prognosis of DCM-like heart diseases, especially those of IHD and VHD, seemed to be as bad as those of DCM.     

Left ventricular aneurysm in a patient with mildly dilated cardiomyopathy

We describe the case of a patient with mildly dilated idiopathic cardiomyopathy and left ventricular aneurysm, diagnosed in absence of a prior clinical history and anatomo-pathological features of myocardial infarction. To ascertain the diagnosis of idiopathic cardiomyopathy, the patient underwent cardiac catheterization with coronary angiography, that showed the lack of epicardial artery stenosis and a slow run-off of the contrast. An endomyocardial biopsy showed the presence of hypertrophic myocytes and interstitial fibrosis. Moreover, a thoracic high resolution computed tomography showed the features of pulmonary bilateral basal emphysema, interstitial thickening and bronchiectasis. Alfa1-anti-trypsin plasma levels were reduced. The patient, because of worsening of clinical and hemodynamic conditions, underwent at age of 36 a combined heart-lung transplantation. The pathological examination of the native organs confirmed the previous diagnosis. At the moment, this is the second report in the literature concerning the presence of left ventricular aneurysm in a patient with idiopathic cardiomyopathy without an underlying coronary artery disease or prior history of myocardial infarction.     

Causes and prognosis of syncope in patients with primary dilated cardiomyopathy

The causes of adverse prognosis of patients with primary dilated cardiomyopathy remain controversial. Classically, it is thought that syncope is associated with an increased risk of mortality. The aim of this study was to try and identify the causes and prognostic significance of syncope in patients with primary dilated cardiomyopathy. Sixty-five patients aged 31 to 80 with primary dilated cardiomyopathy were admitted for investigation of syncope. The average ejection fraction was 27 +/- 10%. Invasive and non-invasive investigations including complete electrophysiological investigations, were performed. Sustained monomorphic ventricular tachycardia was induced in 14 patients (21.5%), ventricular flutter or fibrillation was induced in 9 patients (14%), a supraventricular arrhythmia in 17 patients (26%), and a conduction defect alone or associated with another arrhythmia in 7 patients (11%). A pathological result of tilt testing was observed in 5 patients (8%). No cause of syncope could be demonstrated in 15 patients (23%). During follow-up (4 +/- 2 years) there was a mortality of 15% which was only correlated with the reduction in left ventricular ejection fraction. The authors conclude that there are many causes of syncope in primary dilated cardiomyopathy: ventricular arrhythmias represent only 35% of cases and do not impact on the prognosis; above all, left ventricular ejection fraction is the most important prognostic factor.     

扩张性心肌病伴高血压患者心功能及预后的变化

目的观察扩张性心肌病患者伴有高血压与不伴高血压者临床特征的差异。方法对我院1989年至2006年155例诊断扩张性心肌病的住院病例的临床资料进行回顾性分析。结果(1)扩张性心肌病伴高血压的患者出现心力衰竭后血压高于140/90 mm Hg者占30%。(2)伴高血压的扩张性心肌病组左心室后壁厚度(LVPW)、室间隔厚度(IVS)、左心室射血分数(LVEF)、左心室心肌重量(LVMW)等各指标高于无高血压组。(3)治疗后伴有高血压组心功能改善更明显。结论(1)利用血压水平作为排除高血压所引起的心脏损害的标准有一定的局限性。(2)少部分伴高血压的扩张性心肌病可能由于高血压病史不详而属于高血压引起的心脏损害的范畴。     

63例扩张型心肌病的长期随访分析

目的:评价扩张型心肌病的远期预后。方法:扩张型心肌病患者63例,给予强心、利尿、β受体阻滞剂、血管紧张素转化酶抑制剂及中药生脉(参麦)复方制剂治疗,定期随访,随访时间3～13年。随访项目包括:心功能改善情况、体力活动适应程度、院外服药治疗、再次入院次数,死亡,了解并登记死亡原因。结果:63例中随访3～5年16例,5～10年35例,10年以上12例,平均随访时间6.1年。随访期间死亡18例(病死率28.6%);存活45例,存活者患病时间2～15年,其中28例能从事一般正常劳动及工作(62.2%),生活可自理者10例(22.2%)。结论:由于治疗方案的改进,特别是中西医结合药物的应用,使扩张型心肌病患者的预后改善,平均存活时间延长,多数患者的生存质量提高。     

Exercise-induced changes of functional mitral regurgitation in asymptomatic or mildly symptomatic patients with idiopathic dilated cardiomyopathy

It has remained unclear why functional mitral regurgitation (MR), even if it is of a mild degree, has prognostic importance in patients with idiopathic dilated cardiomyopathy (IDC). Exercise-induced changes in functional MR, which might be a clue to this question, have not been fully clarified. Thus, in this study, semisupine exercise echocardiography was performed on 32 asymptomatic or mildly symptomatic patients with IDC (29 men, mean age 45 +/- 14 years). The mean ejection fraction was 28 +/- 10% (range 13% to 45%). The effective regurgitant orifice (ERO) area of MR was measured, as well as echocardiographic parameters including mitral valve geometry. ERO at rest was associated best with systolic mitral tenting area (r(S) = 0.85, p <0.001). Functional MR did not newly appear during exercise in 9 subjects without MR at rest. In the remaining 23 subjects with functional MR at rest, all showed exacerbations of MR, with a median ERO of 10.5 mm(2) (interquartile range 6.3 to 16.5) to 18.7 mm(2) (interquartile range 9.5 to 29.3) (p <0.001). An increase in ERO was correlated best with the enlargement of tenting area (r(S) = 0.90, p <0.001) and was the strongest independent determinant of exercise duration (beta = -0.55, p = 0.002, multiple R(2) = 0.46). In conclusion, functional MR complicated with IDC was significantly exacerbated during exercise, with mitral valve deformation, which was strongly related to exercise intolerance; thus, the clinical impact of functional MR in patients with IDC could be more serious than can be expected by its degree at rest.     

扩张型心肌病患者肠道病毒感染及其对预后的影响

目的：探讨肠道病毒感染在扩张型心肌病（ DCM ）发生发展中的作用。方法采用RT-PCR法检测109例DCM患者肠道病毒RNA表达,其中肠道柯萨奇B组病毒RNA阳性49例（阳性组）,阴性60例（阴性组）。采用ELISA法测定两组血清病毒RNA表达水平,比较两组近期预后（心衰再发率、再入院率、病死率等）。结果阳性组血清肠道病毒RNA水平明显高于阴性组,P＜0．05;再入院率、急性心衰再发率、病死率均明显高于阴性组（P均＜0．05）。结论肠道病毒感染在扩张型心肌病发生发展过程中起重要作用;肠道病毒RNA高表达者近期预后较差。     

Long-term treatment of ischemic dilated cardiomyopathy with continuous positive airway pressure.

An 81-year-old man with ischemic dilated cardiomyopathy complained of frequently awakening from sleep due to choking; subsequent polysomnography revealed Cheyne-Stokes respiration (CSR) with sleep apnea. With continuous positive airway pressure (CPAP) through a nasal mask, both the CSR and symptoms disappeared. After 6-12 months, chest X-ray and echocardiographic findings continued to improve without any change in pharmacological treatment. For three years, CPAP had been effective to eliminate CSR during sleep. Long-term CPAP treatment, which is rarely applied for congestive heart failure in Japan, is useful in alleviating the adverse effects of CSR and, thereby, maintaining a good quality of life in these patients.     

Long-term prognosis of dilated cardiomyopathy revisited: an improvement in survival over the past 20 years.

BACKGROUND: Because of their favorable prognostic effects, angiotensin converting enzyme inhibitors (ACEI), angiotensin II receptor blockers (ARB) and beta blockers have become background therapy in dilated cardiomyopathy (DCM). However, there are few reports concerning the long-term prognosis of Japanese patients with DCM in relation to these treatments. METHODS AND RESULTS: One hundred and fifty patients with DCM were divided into 2 groups: group A (n=46) (diagnosis: 1982-1989) and group B (n=104) (diagnosis: 1990-2002). During follow-up period of 6.9+/-4.8 years, 62 patients died and 1 patient had a heart transplant. The survival rate at 5 and 10 years was 60.9% and 34.8%, respectively, in group A patients, and 80.9% and 65.3%, respectively, in group B patients (p=0.0079). In group A patients, ACEI/ARB or beta blockers were less frequently used (p<0.0001), whereas antiarrhythmics (class Ia or Ib) were more often used (p<0.0001). The patients treated with ACEI/ARB and beta blockers showed a better survival rate than those without (p<0.0001). The patients with antiarrhythmics showed a worse survival rate than those without (p<0.0001). CONCLUSION: The prognosis of Japanese patients with DCM has significantly improved over the past 20 years. This improvement may be explained partly through the increased use of ACEI/ARB and beta blockers and a declining use of antiarrhythmics.     

扩张型心肌病患者心脏再同步化治疗的疗效观察

目的:观察在组织多普勒指导下对药物难治性扩张型心肌病(DCM)充血性心力衰竭的心脏再同步化治疗(CRT)的疗效。方法:选择18例经药物治疗后无效的DCM充血性心力衰竭患者行CRT。术前行二维超声心动图和组织多普勒检查,术后1周在组织多普勒指导下行房室和室间延迟优化。术后1个月、3个月、6个月及1年随访患者的临床症状,并比较各项参数的变化。结果:CRT后①心功能从Ⅲ～Ⅳ级改善为Ⅰ～Ⅲ级,左室射血分数由(26.42±6.7)%增加到(33.44±6.0)%,P<0.01;②左室舒张末内径由(75.31±8.52)mm减少至(67.24±10.57)mm,P<0.05;③左右室间收缩时间差、左室内不同步指数有显著改善,但随着术后时间的延长无明显变化。结论:CRT可以改善DCM患者的左、右室间和左室内收缩活动不同步,逆转心室扩大,提高心功能,其近期疗效显著。     

Familial dilated cardiomyopathy: a worse prognosis compared with sporadic forms.

OBJECTIVE--To establish the time of onset of dilated cardiomyopathy (DCM) by review of annual chest x rays, which are obligatory in Hungary. DESIGN--A retrospective survey of chest x rays of a cohort of confirmed cases of DCM, to assess time of onset of cardiomegaly. Clinical course was compared by follow up over a mean of six years from the time of diagnosis. SUBJECTS--240 patients with DCM (31 familial, 209 non-familial). Diagnosis was made by echocardiography in all cases and confirmed by coronary angiography and heart biopsy in some cases. MAIN RESULTS--At diagnosis, the mean age of the patients was 31.8 years in the familial group and 39.6 years in the non-familial group (P < 0.05). The time between the onset of cardiomegaly (cardiothoracic ratio > 0.45) and clinical diagnosis was 8.0 and 10.1 years respectively (P < 0.05). The six year survival was 6% in the familial group and 23% in the non-familial group (P < 0.05). CONCLUSIONS--The familial form of DCM is the more malignant form: it occurs at an earlier age and progresses more rapidly than non-familial DCM.     

Familial dilated cardiomyopathy in patients transplanted for idiopathic dilated cardiomyopathy

OBJECTIVE: To evaluate the prevalence, clinical features, and pattern of inheritance of familial dilated cardiomyopathy (DCM) in heart transplant patients. PATIENTS AND METHOD: Patients with idiopathic DCM who had undergone heart transplantation were invited to participate. Patients with alcohol abuse were excluded. A clinical evaluation, 12-lead ECG, echocardiogram, blood tests, and DNA extraction were performed in patients and relatives. Familial DCM was defined as the presence of at least one relative with idiopathic DCM. Possible familial DCM was considered when at least one relative had left ventricular enlargement (LVE) (> 112% predicted LVEDD). RESULTS: One hundred and ninety-nine relatives of 43 families were studied. DCM was familial in 11 probands (25.6%) and possibly familial in 11 (25.6%). Fifteen relatives had DCM (7.5%), 26 (13.1%) LVE, and 5 (2.5%) hypertrophic cardiomyopathy. The pattern of inheritance was autosomal dominant in most families. Five probands (3 with familial DCM) had antecedents of consanguinity and possible recessive inheritance. Six probands (14%, 1 with familial DCM) had relatives with conduction system defects. Creatine kinase was moderately increased in 9 relatives (4.5%), 3 of them with LVE. Fifteen patients had at least moderate alcohol intake. Three of them had familial DCM (relatives without alcohol abuse) and 6 had possible familial DCM. CONCLUSIONS: The prevalence of familial DCM is high in patients who undergo heart transplant. Left ventricular enlargement, conduction system abnormalities, and elevated creatine kinase may be early markers of familial disease. Hypertrophic cardiomyopathy is present in some relatives of patients with idiopathic DCM. Familial DCM is present in patients with a previous diagnosis of alcoholic DCM.     

扩张型心肌病的自然病史及预后(附50例死亡病例分析)

目的:了解近年来扩张型心肌病（DCM）的预后情况。方法：回顾性分析住院1990年以来死亡的50例DCM患者的临床资料及预后。结果：50例患者男性42例（84％），女性8例（16％），平均发病年龄40±14．6岁。生前均接受过地高辛及利尿剂治疗；37例（74％）接受过血管紧张素转换酶抑制剂治疗。发病后存活期为2个月至22年，平均4±4．6年。49例（98％）死于心功能恶化和（或）心律失常，1例（2％）死于肺栓塞。发病后1、2、3、5和10年病死率分别为24％、38％、60％、80％和92％。结论：DCM患者尽管接受目前常规药物治疗，其病死率仍很高。