Cardiac sarcoidosis

Cardiac sarcoidosis can have a variety of manifestations including conduction disease, congestive heart failure, valvular heart disease, pericardial effusions, tamponade, ventricular arrhythmias and sudden cardiac death. In patients with sarcoidosis, the reported incidence of cardiac involvement ranges from 20% in US autopsy studies to nearly 60% in Japan, where it accounts for the majority of deaths as a result of sarcoidosis. Despite this, the diagnosis of cardiac sarcoidosis remains difficult and no single diagnostic test has emerged that combines a high degree of sensitivity and specificity. Recent evidence suggests that gadolinium-enhanced magnetic resonance imaging can potentially offer high sensitivity and specificity for both disease presence and activity. It may also be possible to track response to treatment of cardiac sarcoidosis with cardiac magnetic resonance imaging. Corticosteroids are the mainstay of treatment for cardiac sarcoidosis as with systemic sarcoid but at present no prospective trial has shown a survival benefit. Pharmacological treatment of heart failure should follow standard heart failure guidelines, whereas anti-arrhythmic treatment is problematic. The role of implantable cardiac defibrillators in sarcoid has not been well defined, although the risk of ventricular arrhythmias and sudden cardiac death are high. Cardiac transplantation remains an option for younger patients, although overall cardiac involvement in sarcoidosis carries a relatively poor prognosis.     

Cardiac sarcoidosis detected with magnetic resonance imaging

A 47-year-old woman was admitted for multiple episodes of symptomaticsustained polymorphic ventricular tachycardia. Six weeks earlier,she had     

The utility of cardiac magnetic resonance imaging in post‐Fontan surveillance

Objective: Gated cardiac MRI offers the most detailed and accurate noninvasive method of assessing cardiac anatomy, particularly in patients with complex congenital heart disease. The proposed benefits of using cMRI as a routine screening tool in the Fontan population include early recognition of asymptomatic, postoperative anatomic and physiologic changes. In 2011, we therefore instituted at our center a recommended practice of cMRI screening in patients with Fontan physiology at 3 and 8 years postFontan operation. The purpose of this study was to determine the impact of this standardized practice of cMRI screening on the clinical management of a Fontan population.
Design: We retrospectively reviewed charts from our institutional Fontan database to determine which patients were eligible for cMRI under the current guidelines and who underwent imaging from November 2002 to June 2015. We reviewed the frequency of cMRI and number of changes in management based on the results. Statistical significance was determined using a chi-square test.
Results: There were 141 cMRIs performed on 121 patients who met inclusion criteria. The odds of a change in management were significantly greater after clinically indicated cMRI compared to screening cMRI (OR = 3.79, 95% CI: 1.48-9.66, P = .004). There were near significant odds of change in management if the cMRI occurred <8 years after Fontan regardless of whether it was for screening or clinically indicated purposes (OR = 2.43, 95% CI: 0.97-6.08, P = .052). The most frequent change in management was referral for catheterization with pulmonary artery angioplasty.
Conclusions: There is an important role for cMRI in routine surveillance of post-Fontan patients. Screening cMRI performed less than 8 years after Fontan palliation offers increased utility compared to studies performed later. The optimal timing of such imaging after Fontan palliation remains unclear.     

Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study

Background

It has been repeatedly reported that female patients with thalassemia major survive longer than males and that the difference is due to a lower rate of cardiac disease in females.

Design and Methods

We compared the cardiac iron load as measured by T2* magnetic resonance imaging in 776 patients (370 males) examined at the National Research Council as part of an Italian cooperative study. We also established normal left ventricular ejection fraction values for our population.

Results

The prevalence of cardiac disease was higher in males than in females (105 males versus 69 females; P<0.0001). Cardiac T2* was significantly lower in patients with heart dysfunction (P<0.0001), but no difference was observed according to sex. Twenty males and five females had a history of cardiac arrhythmias. Their cardiac T2* was not significantly lower than that of patients without arrhythmias (24 ms versus 26 ms; P=0.381), nor was there a difference between sexes. Liver T2* was significantly lower in males and females with heart dysfunction compared to those without. Ferritin levels were higher in patients of both sexes with heart dysfunction without significant differences between males and females.

Conclusions

Males and females are at the same risk of accumulating iron in their hearts, but females tolerate iron toxicity better, possibly as an effect of reduced sensitivity to chronic oxidative stress.     

Cardiac sarcoidosis detected by delayed-hyperenhancement magnetic resonance imaging

We report the case of a patient with sarcoidosis and ventricular tachycardia in whom cardiac magnetic resonance imaging provided supportive evidence of cardiac involvement by delineating regions of myocardial inflammation and fibrosis inconsistent with ischemic injury. The identification of cardiac involvement in patients with sarcoidosis is problematic, and the true incidence is unknown. Cardiac magnetic resonance imaging may help establish the actual incidence of cardiac involvement and allow further advances in monitoring and treatment options.     

Determination of cardiac involvement in sarcoidosis by magnetic resonance imaging and Doppler echocardiography

OBJECTIVES: To elucidate whether cardiac magnetic resonance imaging (MRI) could be useful in disclosing structural changes in the myocardium in sarcoidosis patients and to relate echo-Doppler derived indices of left ventricular function to electrocardiogram (ECG) findings. DESIGN: The MRI was performed in 18 consecutive patients with sarcoidosis. Left ventricular ejection fraction (LVEF), i.e. systolic function, was estimated echocardiographically by Simpson's two-dimensional method (n = 16). Diastolic function was estimated by age-corrected Doppler-derived indices: isovolumetric relaxation time (IVRT), deceleration time (DT) and early filling/atrial contraction ratio (E/A ratio). RESULTS: Eleven patients had conduction defects or dysrhythmias (ECG+) whilst seven patients had a normal ECG (ECG-). In two patients, high signalling, contrast-enhanced, isolated regions, suggestive of deposits, were seen in the left ventricular myocardium on MRI. Both these patients had abnormal ECGs and signs of systolic and/or diastolic dysfunction on echocardiography. LVEF was subnormal in seven of 10 of the ECG+ patients and in two of six of the ECG-. Signs of diastolic dysfunction were found in 59% and 56% of the measurements in the ECG+ and ECG- patients, respectively. CONCLUSION: We conclude (i) that myocardial deposits on MRI in sarcoidosis patients have a high specificity for cardiac involvement but a rather low sensitivity; (ii) that a substantial proportion of sarcoidosis patients with abnormal ECGs have echocardiographic signs of systolic and/or diastolic dysfunction.     

Cardiac pseudomass due to enlarged epicardial adipose tissue: Comparison of echocardiography and cardiac magnetic resonance imaging

A 75‐year‐old woman underwent preoperative echocardiography, which revealed a mass in the left atrium; however, cardiac magnetic resonance imaging showed no mass. Echocardiography revealed an enlargement of the epicardial adipose tissue in the left atrioventricular groove, therefore a pseudomass. Causes of cardiac pseudomass on echocardiography have been reported. Suspected pseudomasses should be reliably differentiated from a tumor or thrombus, and if new forms are found, the images must be shared for the benefit of clinicians.     

Early diagnosis of cardiac sarcoidosis using magnetic resonance imaging

Cardiac sarcoidosis can be a challenging diagnosis to make. The introduction of newer technologies, such as cardiac magnetic resonance imaging and positron emission tomography, have meant earlier diagnosis is possible. This is crucial as early treatment improves symptoms and prognosis.     

Electrocardiographic characteristics in patients with pulmonary sarcoidosis indicating cardiac involvement

ECG Characteristics of Cardiac Sarcoidosis. Introduction: Sarcoidosis is a multisystem granulomatous disease that can affect the heart. Early identification of cardiac sarcoidosis (CS) is critical because sudden death can be the initial presentation. We sought to evaluate the potential role of the ECG for identification of cardiac involvement in a cohort of patients with biopsy‐proven pulmonary sarcoidosis. Methods: Our cohort consisted of referred patients with biopsy‐proven pulmonary sarcoidosis who demonstrated symptoms consistent with cardiac involvement. The ECG characteristics collected were PR, QRS duration, QT interval, rate, bundle branch block (BBB), fragmented QRS (fQRS). QRS fragmentation was defined as 2 anatomically contiguous leads demonstrating RSR’ patterns in the absence of BBB. Results: There were 112 subjects included in the cohort. Of the 52 subjects eventually diagnosed with CS, 39 had an ECG demonstrating fQRS while 21 of the 60 of non‐CS patients had fQRS (75% vs 33.9%, P < 0.01). A RBBB or LBBB pattern were both more prevalent in the CS population (RBBB: 23.1% vs 6.7%, P = 0.016; LBBB: 3.8% vs 1.7%, P = 0.6). QRS duration remained significantly associated with CS after exclusion of those with BBB (93.5 +/− 10.6 vs 88 +/− 11 ms; P = 0.04). When fQRS and bundle branch block were combined, 90.4% of CS patient's ECGs contained at least one of the features, compared to 36.7% of noncardiac CS (P < 0.01). Conclusions: The presence of fQRS or BBB pattern in patients with pulmonary sarcoidosis is associated with cardiac involvement and therefore should prompt further evaluation. (J Cardiovasc Electrophysiol, Vol. pp. 1‐6)     

Implantable cardioverter defibrillator therapy in patients with cardiac sarcoidosis

ICD Shocks in Cardiac Sarcoidosis . Background: An implantable cardioverter defibrillator (ICD) is indicated for some patients with cardiac sarcoidosis (CS) for prevention of sudden death. However, there are little data regarding the event rates of ICD therapies in these patients. We sought to identify the incidence and characteristics of ICD therapies in this patient population. Methods: We performed a cohort study of patients with ICDs at 3 institutions. Cases were those patients with CS and an ICD implanted for primary or secondary prevention of sudden death. Additionally, we included a comparison with historical controls of ICD therapy rates reported in clinical trials evaluating the ICD for primary and secondary prevention of sudden death. Results: Of the 112 CS subjects identified, 36 (32.1%) received appropriate therapies for ventricular tachyarrhythmias (VT) over a mean follow‐up period of 29.2 months. VT storm (>3 episodes in 24 hours) occurred in 16 (14.2%) CS subjects. Inappropriate therapies occurred in 13 CS subjects (11.6%). Covariates associated with appropriate ICD therapies included left ventricular ejection fraction (LVEF) <55% (OR 6.52 [95% CI 2.43–17.5]), right ventricular dysfunction (OR 6.73 [95% CI 2.69–16.8]), and symptomatic heart failure (OR 4.33 [95% CI 1.86–10.1]). Conclusions: In our cohort of patients with CS and ICDs, almost one‐third receive appropriate therapies. This may be due to a myocardial inflammatory process leading to increased triggered activity and subsequent scarring leading to reentrant tachyarrhythmias. Adjusted predictors of ICD therapies in this population include left or right ventricular dysfunction. (J Cardiovasc Electrophysiol, Vol. 23, pp. 925‐929, September 2012)     

Cardiac lipoma diagnosed by cardiac magnetic resonance imaging

A 62-year-old man experienced a transient ischaemic attack.He underwent a transthoracic echocardiogram to rule out thepresence of embolic source. It showed a 10 mm mass with broadattachment to the left side of     

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。