Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.
Amyloidosis ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; Biopsy ; Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic ; Cicatrix ; Death, Sudden, Cardiac ; Diagnosis ; Diagnosis, Differential ; Early Diagnosis ; Electrocardiography ; Electrons ; Gadolinium ; Granuloma ; Heart Defects, Congenital ; Humans ; Incidence ; Magnetic Resonance Imaging ; Myocarditis ; Positron-Emission Tomography ; Prognosis ; Sarcoidosis ; Sensitivity and Specificity

Differentiating arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) from other cardiomyopathies is clinically important but challenging. Although the modified Task Force Criteria can facilitate diagnosis of ARVD/C according to clinical manifestations, histopathological examination plays a pivotal role in excluding other diseases that can mimic ARVD/C. Here, we report a patient with amyloidosis that initially presented similarly to ARVD/C. The diagnosis was confirmed by endomyocardial biopsy, and catheter ablation eliminated the ventricular tachyarrhythmias through an epicardial approach.
Advisory Committees ; Amyloidosis* ; Arrhythmogenic Right Ventricular Dysplasia ; Biopsy ; Cardiomyopathies ; Catheter Ablation* ; Catheters* ; Diagnosis ; Humans ; Tachycardia ; Tachycardia, Ventricular

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.
Arrhythmogenic Right Ventricular Dysplasia/*diagnosis ; Female ; Heart Ventricles/pathology ; Humans ; Lung/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Myocardium/pathology ; Sarcoidosis/*diagnosis ; Thyroid Gland/pathology ; Ventricular Fibrillation/diagnosis/*etiology

The clinical diagnosis of right ventricular (RV) cardiomyopathies is often challenging. It is difficult to differentiate the isolated left ventricular (LV) noncompaction cardiomyopathy (NC) from biventricular NC or from coexisting arrhythmogenic ventricular cardiomyopathy (AC). There are currently few established morphologic criteria for the diagnosis other than RV dilation and presence of excessive regional trabeculation. The gross and microscopic changes suggest pathological similarities between, or coexistence of, RV-NC and AC. Therefore, the term arrhythmogenic right ventricular cardiomyopathy is somewhat misleading as isolated LV or biventricular involvement may be present and thus a broader term such as AC should be preferred. We describe an unusual case of AC associated with a NC in a 27-year-old man who had a history of permanent pacemaker 7 years ago due to second-degree atrioventricular block.
Adult ; Arrhythmogenic Right Ventricular Dysplasia ; Atrioventricular Block ; Cardiomyopathies* ; Diagnosis ; Echocardiography* ; Humans

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Electrocardiography ; Humans ; Male ; Middle Aged

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Humans ; Male

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Death, Sudden ; Humans ; Male ; Middle Aged

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; therapy ; Child, Preschool ; Diagnostic Errors ; Fatal Outcome ; Female ; Humans

OBJECTIVETo summarize the experience in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC) in children.

METHODSA total of 14 children (7 females and 7 males) with ARVC were involved. The cases underwent electrocardiography, echocardiography, cardiac CT or MRI examinations. All cases were treated with sotalol or amiodarone in combination with propranolol. In 2 cases with drug-refractory ventricular extrasystoles, catheter ablation treatment was performed. In 6 cases with obvious impaired ventricular function, additional pharmacological therapy including vasodilators, diuretics, and digitalis were given.

RESULTSVentricular extrasystoles occurred in all 14 cases and ventricular tachycardia in 8 cases. Ten cases showed Epsilon wave on electrocardiography. All 14 cases had enlarged right ventricle and reduced right ventricular ejection fraction. CT or MRI examination showed right ventricular dilatation and a thinned wall of right ventricle in 10 cases. Ventricular extrasystoles or tachycardia disappeared in 7 cases and was reduced in 4 cases after treatment. The two children receiving catheter ablation treatment did not present ventricular extrasystoles or tachycardia in a 3-month follow-up. The heart function was improved in the 6 children with obvious impaired ventricular function after pharmacological therapy.

CONCLUSIONSThe clinical manifestations are diverse in children with ARVC. A definite diagnosis of ARVC should be based on a combination of electrocardiography and echocardiography examinations. Pharmacological therapy is effective partially. Catheter ablation treatment appears to be a promising option in patients with drug-refractory ventricular extrasystoles.

Adolescent ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; drug therapy ; Child ; Child, Preschool ; Echocardiography ; Electrocardiography ; Female ; Humans ; Male ; Tomography, X-Ray Computed

BACKGROUNDThere are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes.

METHODSThirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records.

RESULTSOf these patients 33 were male and 6 female (age at the first presentation was (34.9 +/- 9.8) years). The most common symptoms were palpitation (62%) and syncope (44%). Right precordial QRSd >or= 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V(1) + V(2) + V(3)/V(4) + V(5) + V(6) >or= 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V(1)-V(3) >or= 55 ms (96% and 90% of 28 patients, respectively). Radiofrequency catheter ablation (RFCA) for ventricular tachycardia (VT) was successful in 15 (68%) of 22 patients. The recurrence rate of VT was 46% (7/15) during the follow-up of (16.7 +/- 11.2) months. Seven patients had cardioverter/defibrillator (ICD) implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of (35.6 +/- 19.0) months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone.

CONCLUSIONSThis study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.

Adolescent ; Adult ; Anti-Arrhythmia Agents ; therapeutic use ; Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; drug therapy ; physiopathology ; therapy ; Catheter Ablation ; Defibrillators, Implantable ; Electrocardiography ; Female ; Heart Transplantation ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Young Adult