老年人扩张型心肌病的临床特征和预后

目的探讨老年人扩张型心肌病（ＤＣＭ）的临床特征和预后。方法采用临床观察和应用ＣＯＸ回归模型多因素分析的方法对２９例老年人和４６例非老年人ＤＣＭ的临床资料进行对比分析。结果（１）老年人ＤＣＭ室性心律失常发生率较非老年人低（χ２＝５．４９，Ｐ值＜０．０５）；（２）老年人ＤＣＭ平均生存期较非老年人长（ｔ值＝２．７８５，Ｐ值＜０．０５）；（３）老年人ＤＣＭ对洋地黄敏感性增加，易发生洋地黄毒性反应；（４）老年人ＤＣＭ的主要死因为充血性心力衰竭，而非老年人为室性心律失常（心动过速或心室颤动）。结论（１）心功能恶化是ＤＣＭ室性心律失常的一个主要原因；（２）老年人扩张型心肌病较非老年人恶性心律失常事件发生率高，发病年龄越早，预后越差     

MRI characteristics and clinical value of hypertrophic cardiomyopathy with scar-like late enhancement

<正>Objective To explore the prevalence,MRI characteristics and clinical evaluation of hypertrophic cardiomyopathy (HCM) patients with infarct-like late enhancement (LGE). Methods HCM patients were diagnosed via cardiac magnetic resonance (CMR) from October,2009 to December,2013. 1 411 HCM patients were diagnosed via CMR. LGE was found in 465 patients,of     

Primary restrictive cardiomyopathy: clinical and pathologic characteristics

Twenty-four patients with restrictive cardiomyopathy were identified at St. Thomas' Hospital during a 17-year period. All had endomyocardial biopsy, but in two patients the biopsy specimens were small and nondiagnostic. Seven patients had amyloidosis and five had other specific heart muscle diseases. The remaining 10 patients with primary restrictive cardiomyopathy had myocyte hypertrophy or interstitial fibrosis, or both. Patients with primary restrictive cardiomyopathy presented earlier but survived longer after presentation than did those with amyloidosis. In each group, survival after cardiac catheterization was related to cardiac index but not to filling pressures. Primary restrictive cardiomyopathy was associated with complete heart block in four patients, two of whom had skeletal myopathy. One had a family history of dominantly inherited skeletal myopathy. Primary restrictive cardiomyopathy was present in a mother and daughter. Two other patients had a family history of heart failure, sudden death or complete heart block, alone or in combination, at a young age. Restrictive hemodynamics and complete heart block were present in patients even in the absence of significant fibrosis. The data suggest that primary restrictive cardiomyopathy may be a distinct myopathy with dominant inheritance and incomplete penetrance that is expressed morphologically as myocyte hypertrophy and interstitial fibrosis. Skeletal myopathy may be associated with the cardiomyopathy.     

Liver recipients undergoing hepatic transplantation and subsequent retransplantation: a comparison between clinical characteristics

BACKGROUND/AIMS: In order to establish a rational strategy for organ distribution and optimal patient management, we postulate it is mandatory not only to understand the pathophysiology of failing grafts but also to better recognize the baseline clinical characteristics of the recipients shortly before receiving a second liver allograft. METHODOLOGY: Between March 1986 and December 1997, 1061 patients underwent 1087 orthotopic liver transplantation at three Hospitals in Madrid (122 retransplants). RESULTS: Mean follow-up was 36 months (range, 1-90), 40.6% of the recipients were alive and survival at 1, 3 and 5 years was 62%, 53%, and 46%. Almost 50% of the recipients were UNOS 1 before retransplantation. In comparison to p-OLT (15% UNOS 1), it is clearly shown that the retransplants have been performed in the sickest patients with more adverse prognostic indicators (higher AST, bilirubin, creatinine serum levels, higher Child-Pugh score, higher rate of ascites and lower serum levels of albumin and prothrombin activity). CONCLUSIONS: Liver retransplantation is acceptable and significant differences in recipients' baseline characteristics suggest an impact on lower survival.     

Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management

Arrhythmogenic right ventricular cardiomyopathy, also known as right ventricular dysplasia, is a genetically determined heart muscle disease associated with arrhythmia, heart failure, and sudden death. Autosomal dominant inheritance is typical. The identification of causative mutations in cell adhesion proteins has shed new light on its pathogenesis. Fibrofatty replacement of the myocardium, the hallmark pathologic feature, may be a response to injury caused by myocyte detachment. Sudden death is often the first manifestation in probands, emphasizing the importance of evaluating asymptomatic relatives for the disease. Standardized guidelines facilitate the clinical diagnosis of right ventricular dysplasia. However, familial studies have highlighted the need to broaden the diagnostic criteria, which are highly specific but lack sensitivity for early disease. Modifications have been proposed for the diagnosis of right ventricular dysplasia in relatives. Early right ventricular dysplasia is characterized by a "concealed phase" in which electrocardiographic and imaging abnormalities are often absent, but patients may nonetheless be at risk for arrhythmic events. Detection at this stage remains a clinical challenge, underscoring the potential value of mutation analysis in identifying affected persons. Serial evaluation of patients with suspected right ventricular dysplasia is recommended as clinical features may develop during the follow-up period. The onset of symptoms such as palpitation or syncope may herald an active phase of a previously quiescent disease, during which patients are at increased risk for sudden death. Greater awareness of right ventricular dysplasia among physicians and judicious use of implantable cardioverter-defibrillators may help to prevent unnecessary deaths.     

Genetically determined myocarditis: clinical presentation and immunological characteristics

PURPOSE OF REVIEW: Myocarditis is a clinically heterogeneous myocardial inflammatory disease, diagnosed by endomyocardial biopsy; it may be idiopathic, infectious, or autoimmune and may lead to dilated cardiomyopathy. Myocarditis and dilated cardiomyopathy represent different stages of an organ-specific autoimmune disease in genetically predisposed individuals. RECENT FINDINGS: In animal models, cell-mediated or antibody-mediated autoimmune myocarditis/dilated cardiomyopathy can be induced by viral infection or immunization with heart-specific autoantigens, or can develop spontaneously in genetically predisposed strains. Susceptibility is based on multiple major histocompatibility complex and nonmajor histocompatibility complex genes. In patients the diagnosis of autoimmune myocarditis/dilated cardiomyopathy requires exclusion of viral genome on endomyocardial biopsy and detection of serum heart-reactive autoantibodies. They are directed against multiple antigens that are found in patients and relatives from about 60% of familial and nonfamilial pedigrees. They predict dilated cardiomyopathy development among relatives, years before disease. Some antibodies have functional effects on cardiac myocytes in vitro, in animals and possibly in a dilated cardiomyopathy subset, responsive to extracorporeal immunoadsorption. SUMMARY: In myocarditis/dilated cardiomyopathy, cardiac-specific and disease-specific antibodies of IgG class are potential biomarkers for identifying 'at risk' relatives as well as those patients in whom, in the absence of active infection of the myocardium, immunosuppression, and/or immunomodulation may be beneficial. Future studies should better define the genetic basis of human autoimmune myocarditis/dilated cardiomyopathy.     

Incidence and clinical presentation of IBD in children: comparison between prospective and retrospective data in a selected Norwegian population

OBJECTIVE: The results of recent research suggest that there is an increasing incidence of inflammatory bowel disease (IBD) among children. Newly diagnosed IBD was compared between two consecutive 6-year periods in the same catchment area of southeastern Norway. MATERIAL AND METHODS: Children subjected to endoscopy from 1993 to 2004 were recorded retrospectively in the first 6-year period and prospectively for the subsequent period. The mean size of the child population under 16 years in the area was 70,500. The study reports on incidence numbers, age at diagnosis, disease distribution and clinical presentation at diagnosis. RESULTS: There were 23 incident cases of IBD in the first period and 25 in the subsequent period. The rates of Crohn's disease (CD) for the two periods were, respectively, 1.95 and 3.64, and for ulcerative colitis (UC) 3.67 and 2.05/100,000 children/year. Total incidence rates of IBD for the two periods were 5.6 and 5.7, respectively, similar to the findings of the IBSEN study of 1990-94. The change in CD and UC rates from the first to the second period can be explained by better methods of diagnosis. CONCLUSIONS: The total incidence of IBD was not changed between time periods, whereas a trend towards an increase in CD and a reduction in UC was recorded. The incidence rates are in accordance with previously reported national and international data from the past decade. The extent of disease in CD and UC may indicate a serious prognosis of IBD among children.     

Absence of viral nucleic acids in early and late dilated cardiomyopathy

OBJECTIVE—To investigate whether viral infection acts as a trigger factor for the development of dilated cardiomyopathy in genetically predisposed individuals with a family history of disease.
SETTING—Patients attending the cardiomyopathy unit in a cardiac tertiary referral centre.
DESIGN—Nested polymerase chain reaction (nPCR) was used to determine whether enteroviral, adenoviral, or cytomegaloviral nucleic acids were detectable in the myocardium of 19 asymptomatic relatives of patients with dilated cardiomyopathy; all these relatives had echocardiographic abnormalities thought to represent early disease. Explanted hearts from patients with end stage dilated cardiomyopathy were also studied and were compared with 25 controls (ischaemic heart disease (21), valvar heart disease (2), hypertrophic cardiomyopathy (1), restrictive cardiomyopathy (1)). Myocardial tissue from two fatal cases of culture positive coxsackie myocarditis was used as a positive control.
RESULTS—No viral nucleic acid was detected in any group other than in those with myocarditis. Spiking of random wells with purified recombinant viral nucleic acids confirmed the sensitivity and reproducibility of the assays.
CONCLUSIONS—Myocardial viral infection is not detectable in relatives of patients with dilated cardiomyopathy who are suspected of having early disease. There is no evidence that viruses act as a trigger factor for initiating the dilated cardiomyopathy in these patients.


Keywords: viral infection; dilated cardiomyopathy     

Alcoholic cardiomyopathy: incidence,clinical characteristics,and pathophysiology

In the United States, in both sexes and all races, long-term heavy alcohol consumption (of any beverage type) is the leading cause of a nonischemic, dilated cardiomyopathy, herein referred to as alcoholic cardiomyopathy (ACM). ACM is a specific heart muscle disease of a known cause that occurs in two stages: an asymptomatic stage and a symptomatic stage. In general, alcoholic patients consuming > 90 g of alcohol a day (approximately seven to eight standard drinks per day) for > 5 years are at risk for the development of asymptomatic ACM. Those who continue to drink may become symptomatic and develop signs and symptoms of heart failure. ACM is characterized by an increase in myocardial mass, dilation of the ventricles, and wall thinning. Changes in ventricular function may depend on the stage, in that asymptomatic ACM is associated with diastolic dysfunction, whereas systolic dysfunction is a common finding in symptomatic ACM patients. The pathophysiology of ACM is complex and may involve cell death (possibly due to apoptosis) and changes in many aspects of myocyte function. ACM remains an important cause of a dilated cardiomyopathy, and in latter stages can lead to heart failure. Alcohol abstinence, as well as the use of specific heart failure pharmacotherapies, is critical in improving ventricular function and outcomes in these patients.     

Rare clinical presentation of nonobstructive hypertrophic cardiomyopathy: apical aneurysm with thrombus

Hypertrophic cardiomyopathy patients rarely have left ventricular apical aneurysms without coronary artery disease and the pathophysiological processes responsible for apical wall thinning and aneurysm formation are not known exactly today. Here we present a rare nonobstructive hypertrophic cardiomyopathy case with an apical thrombotic aneurysm.     

缺血性心肌病的临床特征

目的 :探讨缺血性心肌病 (ICM)的临床特点。方法 :对 2 1例 ICM的临床资料进行分析。结果 :所有病例发生在老年人 ,均并发血脂异常 ,19例并发原发性高血压 ,15例并发老年退行性主动脉瓣病 ,9例并发糖尿病。所有病例均并发各种心律失常 ,以传导阻滞及室性心律失常多见。均伴随不同程度的心功能不全 ,按 NYHA心功能分级多在 级以上 ,且呈多支血管病变。结论 :该病多发生在老年人 ,冠状动脉病变广泛。病史、超声心动图及放射性核素心肌显像对诊断具有意义 ,而冠状动脉造影对该病诊断具决定性价值。治疗上主要是改善心功能及心肌缺血、控制心律失常、抗凝及抗血小板治疗 ,必要时可考虑行冠状动脉搭桥术及心脏移植术。     

Gender-related differences in the clinical presentation and outcome of hypertrophic cardiomyopathy

OBJECTIVES: The goal of this study was to assess gender-related differences in a multicenter population with hypertrophic cardiomyopathy (HCM). BACKGROUND: Little is known regarding the impact of gender on the heterogeneous clinical profile and clinical course of HCM. METHODS: We studied 969 consecutive HCM patients from Italy and the U.S. followed over 6.2 +/- 6.1 years. RESULTS: Male patients had a 3:2 predominance (59%), similar in Italy and the U.S. (p = 0.24). At initial evaluation, female patients were older and more symptomatic than male patients (47 +/- 23 years vs. 38 +/- 18 years; p < 0.001; mean New York Heart Association [NYHA] functional class 1.8 +/- 0.8 vs. 1.4 +/- 0.6; p < 0.001), and more frequently showed left ventricular outflow obstruction (37% vs. 23%; p < 0.001). Moreover, female patients were less often diagnosed fortuitously by routine medical examination (23% vs. 41% in male patients, p < 0.001). Female gender was independently associated with the risk of symptom progression to NYHA functional classes III/IV or death from heart failure or stroke compared with male gender (independent relative hazard 1.5; p < 0.001), particularly patients > or =50 years of age and with resting outflow obstruction (p < 0.005). Hypertrophic cardiomyopathy-related mortality and risk of sudden death were similar in men and women. CONCLUSIONS: Women with HCM were under-represented, older, and more symptomatic than men, and showed higher risk of progression to advanced heart failure or death, often associated with outflow obstruction. These gender-specific differences suggest that social, endocrine, or genetic factors may affect the diagnosis and clinical course of HCM. A heightened suspicion for HCM in women may allow for timely implementation of treatment strategies, including relief of obstruction and prevention of sudden death or stroke.     

Multi-vessel coronary stenting--procedural results and late clinical outcomes: a comparison with single-vessel stenting

The purpose of this study was to assess the 1-year clinical outcome of patients with multi-vessel coronary artery disease (CAD) who underwent coronary stenting, and to compare the results with single-vessel coronary stenting carried out during the same period. We evaluated the in-hospital and 12-month clinical outcomes [death, Q-wave myocardial infarction (MI) and repeat revascularization rates at one year] in 384 consecutive patients treated with coronary stents in 2 (92% of patients) or 3 of the native coronary arteries and compared the outcome to 624 consecutive patients undergoing stenting in a single coronary artery between January 1, 1997 and January 31, 1999. The overall procedural success was obtained in 99% of patients with 2- or 3-vessel stenting and 98% of patients with single-vessel stenting. Procedural complications were similar (2.9% vs 2.6%; p = 0.12). During follow-up, target lesion revascularization was 16% in multi-vessel and 14% in single-vessel stenting (p = 0.38) and repeat revascularization was also similar for both groups (19% vs. 20%; p = 0.73). There was no difference in death (0.8% vs. 1.3%; p = 0.31) and Q-wave MI (0.7% vs. 1.4%; p = 0. 16) in the 2 groups. Overall cardiac event-free survival was similar for both groups (76% vs. 78%; p = 0.54). Multi-vessel stenting in carefully selected patients in our experience had a high procedural success with very low complication rates. The one-year clinical outcomes were acceptable and were similar to the results of single-vessel stenting.     

Collagenous colitis and lymphocytic colitis: Patient characteristics and clinical presentation

Objective. Collagenous colitis and lymphocytic colitis (collectively known as microscopic colitis) are characterized by chronic diarrhea, normal endoscopic and radiologic findings, and typical findings on histologic examination of colonic tissue. The purpose of this study was to define the background characteristics of patients with microscopic colitis, as well as to present symptoms, coexistent autoimmune diseases, and a possible association with the use of non-steroidal anti-inflammatory drugs (NSAIDs) and ticlopidine. Material and methods. A retrospective chart review was carried out on all cases of collagenous colitis and lymphocytic colitis diagnosed at a single center from July 1992 to July 2002. Results. Of the 104 patients identified, 66 had collagenous colitis, 35 had lymphocytic colitis, and 3 were diagnosed with both disorders at different times. The mean age of patients was 64 years (26–88 years), with a female:male ratio of 4.8:1. The most common presenting symptoms were diarrhea (95%), weight loss (41%), abdominal pain (40%), fecal urgency (29%), and nocturnal stools (22%). Autoimmune disease was diagnosed in 29% of patients, 35% were using an NSAID, and 2% were using ticlopidine. Conclusions. Collagenous colitis and lymphocytic colitis occur more often in females than in males, at a wide age range, with a mean in the seventh decade. Certain symptoms are characteristic, but are not specific to these disorders. There may be an association with the presence of a coexistent autoimmune disorder and the use of drugs such as NSAIDs.     

Collagenous colitis and lymphocytic colitis: patient characteristics and clinical presentation

OBJECTIVE: Collagenous colitis and lymphocytic colitis (collectively known as microscopic colitis) are characterized by chronic diarrhea, normal endoscopic and radiologic findings, and typical findings on histologic examination of colonic tissue. The purpose of this study was to define the background characteristics of patients with microscopic colitis, as well as to present symptoms, coexistent autoimmune diseases, and a possible association with the use of non-steroidal anti-inflammatory drugs (NSAIDs) and ticlopidine. MATERIAL AND METHODS: A retrospective chart review was carried out on all cases of collagenous colitis and lymphocytic colitis diagnosed at a single center from July 1992 to July 2002. RESULTS: Of the 104 patients identified, 66 had collagenous colitis, 35 had lymphocytic colitis, and 3 were diagnosed with both disorders at different times. The mean age of patients was 64 years (26-88 years), with a female:male ratio of 4.8:1. The most common presenting symptoms were diarrhea (95%), weight loss (41%), abdominal pain (40%), fecal urgency (29%), and nocturnal stools (22%). Autoimmune disease was diagnosed in 29% of patients, 35% were using an NSAID, and 2% were using ticlopidine. CONCLUSIONS: Collagenous colitis and lymphocytic colitis occur more often in females than in males, at a wide age range, with a mean in the seventh decade. Certain symptoms are characteristic, but are not specific to these disorders. There may be an association with the presence of a coexistent autoimmune disorder and the use of drugs such as NSAIDs.