Is cardiac MRI an effective test for arrhythmogenic right ventricular cardiomyopathy diagnosis?

AIM:To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy(ARVC)and compare cardiac MR(cMR)findings against clinical diagnosis.METHODS:A retrospective analysis of 114(age range16 to 83,males 55%and females 45%)patients referred for cMR with a suspected diagnosis of ARVC between May 2006 and February 2010 was performed after obtaining institutional approval for service evaluation.Reasons for referral including clinical symptoms and family history of sudden death,electrocardiogram and echo abnormalities,cMR findings,final clinical diagnosis and information about clinical management were obtained.The results of cMR were classified as major,minor,non-specific or negative depending on both functional and tissue characterisation and the cMR results were compared against the final clinical diagnosis.RESULTS:The most common reasons for referral included arrhythmias(30%)and a family history of sudden death(20%).Of the total cohort of 114 patients:4 patients(4%)had major cMR findings for ARVC,13patients(11%)had minor cMR findings,2 patients had non-specific cMR findings relating to the right ventricle and 95 patients had a negative cMR.Of the 4 patients who had major cMR findings,3(75%)had a positive clinical diagnosis.In contrast,of the 13 patients who had minor cMR findings,only 2(15%)had a positive clinical diagnosis.Out of the 95 negative patients,clinical details were available for 81 patients and none of them had ARVC.Excluding the 14 patients with no clinical data and final diagnosis,the sensitivity of the test was 100%,specificity 87%,positive predictive value29%and the negative predictive value 100%.CONCLUSION:CMR is a useful tool for ARVC evaluation because of the high negative predictive value as the outcome has a significant impact on the clinical decision-making.

WJC 6~th Anniversary Special Issues(3):Cardiomyopathy Hypertrophic cardiomyopathy in 2013:Current speculations and future perspectives

Hypertrophic cardiomyopathy(HCM),the most variable cardiac disease in terms of phenotypic presentation and clinical outcome,represents the most common inherited cardiomyopathic process with an autosomal dominant trait of inheritance.To date,more than 1400 mutations of myofilament proteins associated with the disease have been identified,most of them "private" ones.This striking allelic and locus heterogeneity of the disease certainly complicates the establishment of phenotype-genotype correlations.Additionally,topics pertaining to patients' everyday lives,such as sudden cardiac death(SCD)risk stratification and prevention,along with disease prognosis,are grossly related to the genetic variation of HCM.This review incorporates contemporary research findings and addresses major aspects of HCM,including preclinical diagnosis,genetic analysis,left ventricular outflow tract obstruction and SCD.More specifically,the spectrum of genetic analysis,the selection of the best method for obstruction alleviation and the need for a unique and accuratefactor for SCD risk stratification are only some of the controversial HCM issues discussed.Additionally,future perspectives concerning HCM and myocardial ischemia,as well as atrial fibrillation,are discussed.Rather than enumerating clinical studies and guidelines,challenging problems concerning the disease are critically appraised by this review,highlighting current speculations and recommending future directions.

Value of red blood cell distribution width in prediction of diastolic dysfunction in cirrhotic cardiomyopathy

BACKGROUND Clinical diagnosis of cirrhotic cardiomyopathy(CCM) often encounters challenges of lack of timeliness and disease severity, with the commonly positive indicator usually associated with advanced heart failure.AIM To explore suitable biomarkers for early CCM prediction.METHODS A total of 505 eligible patients were enrolled in this study and divided into four groups according to Child-Pugh classification: Group Ⅰ, Class A without CCM(105 cases);Group Ⅱ, Class A with CCM(175 cases);Group Ⅲ, Class B with CCM(139 cases);and Group Ⅳ, Class C with CCM(86 cases). Logistic regression and receiver operating characteristic(ROC) curve analyses were performed to determine whether red blood cell distribution width(RDW) was an independent risk factor for CCM risk. The relationships between RDW and Child-Pugh scores, Model for End-Stage Liver Disease(MELD) scores, and N-terminal pro-brain natriuretic peptide(NT-proBNP) were analyzed by Pearson correlation analysis.RESULTS A constant RDW increase was evident from Group Ⅰ to Group Ⅳ(12.54 ± 0.85, 13.29 ± 1.19, 14.30 ± 1.96, and 16.25 ± 2.13, respectively). Pearson correlation analysis showed that RDW was positively correlated with Child-Pugh scores(r = 0.642, P < 0.001), MELD scores(r = 0.592, P < 0.001), and NT-proBNP(r = 0.715, P < 0.001). Furthermore, between Group Ⅰ and Group Ⅱ, RDW was the only significant index(odds ratio: 2.175, 95% confidence interval [CI]: 1.549-3.054, P < 0.001), and it reached statistical significance when examined by ROC curve analysis(area under the curve: 0.686, 95%CI: 0.624-0.748, P < 0.001).CONCLUSION RDW can serve as an effective and accessible clinical indicator for the prediction of diastolic dysfunction in CCM, in which a numerical value of more than 13.05% may indicate an increasing CCM risk.

Phenotype Analysis of Mononuclear Cells in Endomyocardial Biopsy Specimens from Patients with Viral Myocarditis and Dilated Cardiomyopathy

This study investigated the distribution and phenotype of mononuclear cells in endomyocardial biopsy(EMB)specimens from patients with viral myocarditis (VMC,25 cases) and dilated cardiomyopathy (DCM, 10 cases).T lymphocytes,including Th/i cells in VMC and Tc/s cells in DCM, predominated in the infiltrating mononuclear cells in these specimens. The morphological relationship between infiltration of T lymphocytes and pathological changes of myocardia was also observed and neither macrophages nor B lymphocytes were found.

Hypertensive cardiomyopathy:A clinical approach and literature review

Hypertensive cardiomyopathy(HTN-CM) is a structural cardiac disorder generally accompanied by concentric left ventricular hypertrophy(LVH) associated with diastolic or systolic dysfunction in patients with persistent systemic hypertension.It occurs in the absence of other cardiac diseases capable of causing myocardial hypertrophy or cardiac dysfunction.Persistent systemic hypertension leads to structural and functional myocardial abnormalities resulting in myocardial ischemia,fibrosis,and hypertrophy.HTN-CM is predominantly a disease of impaired relaxation rather than impaired contractility,so patients are usually asymptomatic during resting conditions.However,their stiff left ventricles become incapable of handling increased blood volume and cannot produce appropriate cardiac output with the slight change of circulating volume that may occur during exercise.Importantly,the accompanying LVH is itself a risk factor for mortality and morbidity.Therefore,early detection of LVH development in patients with hypertension(referred to as HTN-CM) is critical for optimal treatment.In addition to pathological findings,echocardiography and cardiac magnetic resonance imaging are ideal tools for the diagnosis of HTN-CM.Timely diagnosis of this condition and utilization of appropriate treatment are required to improve morbidity and mortality in hypertensive patients.This review article presents an overview of the multidimensional impact of myocardial disorder in patients with hypertension.Relevant literature is highlighted and the effects of hypertension on cardiac hypertrophy and heart failure development are discussed,including possible therapeutic options.

A new diagnostic feasibility for cardiomyopathy utilizing acoustic microscopy

Aims: Dilated cardiomyopathy often shows left ventricular systolic dysfunction, although histologically it always exhibits non-specific abnormality. We hypothesized that myocyte sound speed might be altered due to incomplete protein accumulation in cells. Methods and Results: Ninety eight biopsied samples were obtained from 49 patients comprising 43 with clinical dilated cardiomyopathy and 6 with hypertrophic cardiomyopathy. Sound speed was evaluated in deparaffinized 10 μm thick sections using an acoustic microscope (frequency range: 50 - 105 MHz). Conventional histology revealed 7 cases of persistent myocarditis derived from clinical dilated cardio- myopathy samples. Histology of the remaining dilated cardiomyopathy patients indicated non-specific abnormality. All hypertrophic cardiomyopathy cases exhibited myocardial disarray. Ten normal autopsied hearts were compared as controls. The sound speed of controls was 1627 ± 30m/sec. The sound speed in dilated cardiomyopathy samples (1700 ±51m/sec) was 1.045-fold faster compared to controls. The sound speed in hypertrophic cardiomyopathy samples (1734 ±51m/sec, 1.066-fold compared to controls) was faster than that of the myocarditis group (1672 ±30m/sec, 1.028-fold) (P = 0.0218). Furtheremore, desmin expression was evaluated as extent of emergence (grading 0 - 4). The desmin expression score in hypertrophic cardiomyopathy samples (2.7 ± 0.8) was significantly higher than in other groups (dilated 2.0 ± 1.4, myocarditis 1.6 ± 1.5 vs., controls 0, P ≤ 0.0001, 0.0001, 0.0129, respectively). Conclusion: Cardio-myopathy enhanced the sound speed, which correlated with the elasticity of myocytes, following the impaired compliance of left ventricle, despite the absence of histological changes. The elevation of sound speed of myocytes may be linked to cytoskeletal changes. Myocyte sound speed may be a new diagnostic tool for diagnosis of idiopathic cardiomyopathy independently of conventional histological diagnosis.

Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy:A case report

BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.

Do Antineoplastic Drugs Play an Additional Role in the Progression of Non-Compaction Cardiomyopathy? A Case Report

Non-compaction cardiomyopathy is a rare form of cardiomyopathy;its most common clinical manifestations are heart failure (HF), ventricular arrhythmia, thromboembolism, and sudden cardiac death. We report a rare case of a 63-year-old man with chest tightness, worsening lower leg edema, dyspnea, and decreased exercise tolerance. He had a medical history of gastric cancer treated with subtotal gastrectomy and post-operative chemotherapy with paclitaxel and fluorouracil three years ago. At that time, he was diagnosed with non-compaction cardiomyopathy, and the thickened and reticulated trabecular muscle was exclusively confined to left ventricular apex. Five months ago, he was admitted to our hospital with heart failure and treated for dilated cardiomyopathy, echocardiography revealed severe trabecular noncompact myocardium in both ventricles, which was confirmed by cardiac magnetic resonance imaging (CMR). It is generally accepted that non-compacted myocardium forms in the early embryonic stage, which raises a question in our case whether acquired factors, such as antineoplastic drugs, potentially accelerate the pathological progression of non-compaction cardiomyopathy. Considering there are disparities between current screening tools such as echocardiography and CMR regarding diagnostic criteria, multi-detector CT may be an alternative examination method that could provide a new perspective for diagnosis.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative,and patients with cardiac sarcoidosis(CS) who have impaired left ventricular(LV) systolic function are sometimes diagnosed with dilated cardiomyopathy(DCM).Late gadolinium enhancement(LE) in magnetic resonance imaging is now a critical finding in diagnosing CS,and the novel Japanese guideline considers myocardial LE to be a major criterion of CS.This article describes the value of LE in patients with CS who have impaired LV systolic function,particularly the diagnostic and clinical significance of LE distribution in comparison with DCM.LE existed at all LV segments and myocardial layers in patients with CS,whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM.Transmural(nodular),circumferential,and subepicardial and subendocardial LE distribution were highly specific in patients with CS,whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM.Since sarcoidosis patients with LE have higher incidences of heart failure symptoms,ventricular tachyarrhythmia and sudden cardiac death,the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.

Investigation of cardiomyopathy using cardiac magnetic resonance imaging part 2:Rare phenotypes

Cardiac magnetic resonance imaging(CMRI) has emerged as a useful tertiary imaging tool in the investigation of patients suspected of many different types of cardiomyopathies.CMRI images are now of a sufficiently robust quality to enable high spatial and temporal resolution image acquisition.This has led to CMRI becoming an effective non-invasive imaging tool for many cardiomyopathies.In this two-part review we outline the typical sequences used to image cardiomyopathy and present the imaging spectrum of cardiomyopathy.Part Ⅰ focused on the current classification of cardiomyopathy,the basic CMRI sequences used in evaluating cardiomyopathy and the imaging spectrum of common phenotypes.Part Ⅱ illustrates the imaging spectrum of the more rare phenotypes.

Noninvasive Diagnosis of Cardiac Amyloidosis by MRI and Echochardiography

This study described the radiological features on echocardiography and MRI specific to cardiac amyloidosis confirmed on biopsy. Eleven cases of biopsy-proven cardiac amyloidosis were retrospectively reviewed in this study. All patients underwent biopsy, cardiac MRI and echocardiography. The main echocardiography and MRI findings were as follows: diffuse ventricular and septum wall thickening, atrial enlargement, pericardial effusion, restricted left ventricular (LV) systolic and diastolic function, characteristic granular sparkling of myocardium. MRI revealed a characteristic pattern of global subendocardial late enhancement, extending in varying degrees into the neighboring myocardium. The findings agreed with the infiltration distribution of amyloid protein. Typical abnormalities seen on echocardiography and MRI should have important diagnostic and prognostic value of cardiac amyloidosis. MRI should be considered in the diagnosis of cardiac amyloidosis if echocardiographic features are suspicious.

Reversible congestive heart failure associated with hypocalcemia:A case report

BACKGROUND Hypoparathyroidism is a rare disease that may occur due to primary or secondary etiologies.The estimated incidence in the United States is 24–37/100000 personyears.Congestive heart failure associated with hypocalcemia due to hypoparathyroidism is an even rarer presentation.CASE SUMMARY Here,we present a 64-year-old woman with congestive heart failure following hypocalcemia.The patient was transferred to our emergency department with complaints of rapidly progressive dyspnea,shortness of breath and heaviness of the chest for 4 d.She had a history of undergoing thyroidectomy and partial tracheotomy 2 years prior due to a malignant thyroid tumor.Muscle spasms had been present 1 year ago,and cataracts were treated with intraocular lens replacement in both eyes.Most tests were within normal ranges,except serum calcium at 1.33 mmol/L(2.20–2.65 mmol/L),ionized calcium at 0.69 mmol/L(1.15–1.29 mmol/L),and parathyroid hormone at<1.0 pg/mL(12–88 pg/mL).Echocardiography revealed an ejection fraction of 28.48%.Cardiac function was quickly reversed by restoring the serum calcium concentration.Significant improvements were noted with an ejection fraction of up to 48.50%at follow-up.CONCLUSION For patients with potential hypocalcemia,monitoring calcium levels and dealing with hypocalcemia in time to avoid serious complications are important.

EFFECTS OF ENALAPRIL AND SPIRONOLACTONE REFRACTORY CONGESTIVE HEART FAILURE

The 5-year-survival rate for patients with severe congestive beart failure(CHF)isapproximately 50% for those judsed to be in New Yock Heart Associstion(NYHA)class Ⅲ;for patients in classⅣ;the 5-year-survial rate decilimes to about 20%,fatal of136-impstients(89 men and 47 women)were enrolled,mean age was 68 years(range39-84 year)and the mean duration of heart faihure was 26 months(range 3-32months).the primary etiologic factor was ischemic heart disease in 92patients,hypertension in 26.dilated cardiomyopathy in 14,and mitral insufficiency in4.short and long-term clinical effects of the anglotensin-converting enzyme(ACE)inhibtor enalapril in severe congesttive heat failure(CHF)were evaluated during a 2-year open study of 136 inpatients with New York Heart Association(NRHA)funtional class Ⅲ or Ⅳ CHF refractory to treatment with cardial glycosides andhigh doses of loop dluretics,Enalapr was added to each petients reglmen,whichcomprised combinations of urosomide(136patients),digitalis(102patients),andspironolactone(89pstients),By the end of the first month of Enalapriladrninistration,improvement in NRHA functional class was seen in 89 patients(72%).During the first year of Enalapril treatment,the number of hospital admissionsand hospital days delined signiflcantly(p【0.001)and functional class improvedsigniflcantly(p【0.001).Although most patients to lerated Enalapric well..44%experiencde hypotension,which in 10% of patients necessited termination ofEnalapril therapy.Although mean serum potassium levels traded to increase,serioushyperkalemia did not occur,After 1 year,a subset of 30 patients who had not initiallyrecelved spironolactone detersted clinically and manfested inreasing urinaryaldosterone leveis.Hypotension precluded increasing the Enalapril dose,butintroduction of spiroonolsctone improve dclinical status in this cohort.The resuitssaggest that rational therapy for severe CHF.In cludss,addition of the aldosteroneantagonist spironactone to low doses of Enalapril(or abother ACE inhibitor)andhigh doses of loop dluretlcs provided rebal function is adequate.

The Thinking on TCM Differential Treatment of Congestive Heart Failure

Congestive heart failure(CHF),the manifestationat the late stage of cardiovascular diseases,is a commonand critical illness.Traditional Chinese medicine showsa good therapeutic effectiveness in treatment of CHF,especially for latent heart failure,refractory heart failureand digitalis poisoning.The thinking on treatment ofCHF by traditional Chinese medicine on the basis ofclinical practice is described below.

心脏磁共振在肥厚型心肌病诊治中的应用进展

肥厚型心肌病是最常见的遗传性心脏病,可导致患者发生心源性猝死、心力衰竭等严重心血管事件。因此,准确的诊断与危险分层对于临床诊疗至关重要。心脏磁共振可以无创评估患者的心脏结构、功能以及组织特征,在肥厚型心肌病的鉴别诊断以及预后判断中均可发挥重要价值。包括特征追踪、弥散张量成像以及4D flow在内的磁共振新技术也在临床中得到初步应用,有望在肥厚型心肌病发生发展机制研究以及早期诊断中发挥重要作用。

左心室肥厚诊断和治疗临床路径中国专家共识2023

左心室肥厚是一类常见的心脏结构性改变,经影像学检查确诊。起病隐匿,早期症状不典型,易被忽视,延误诊治。多种疾病和情形可致左心室肥厚,近年来针对部分特定病因的治疗显示可显著改善患者预后,故需重视对左心室肥厚相关疾病的病因诊断。本专家共识由中国医师协会心血管内科医师分会心力衰竭学组和心衰中心心肌病专家委员会组织编写,全面介绍了左心室肥厚的早期筛查线索、规范诊治及随访路径。旨在深化广大临床一线医务工作者(尤其是基层医院)加强对左心室肥厚的认识,建立早期识别、早期转诊的意识,提高对常见及罕见左心室肥厚相关疾病的诊治能力,减少漏诊和误诊。

心脏磁共振影像组学在非缺血性心肌病中的应用研究进展

非缺血性心肌病的病因复杂,主要由遗传、心肌代谢、心肌结构改变等所致,而非冠状动脉病变引起。心脏磁共振(CMR)能够高分辨显示心肌组织特征,并能够提供客观的心功能参数。传统CMR图像观察及分析手段对图像的利用十分有限,而CMR影像组学技术的出现为非缺血性心肌病的研究提供了新的方法。本文将综述CMR影像组学在非缺血性心肌病中的应用研究进展,主要包括其在肥厚型心肌病、扩张型心肌病、心肌淀粉样变性诊断及预后预测中的应用价值。

可逆性特发性甲状旁腺功能减退性心肌病1例并文献复习

报道1例特发性甲状旁腺功能减退性心肌病患者的临床资料,并进行文献复习。

复发型马德龙综合征合并扩张型心肌病1例

报道1例复发型马德龙综合征合并扩张型心肌病患者的临床资料,并进行文献复习。