Cardiac Sarcoidosis Presenting as Constrictive Pericarditis

In patients with cardiac sarcoidosis, the sarcoid granulomas usually involve the myocardium or endocardium. The disease typically presents as heart failure with ventricular arrhythmias, conduction disturbances, or both. Constrictive pericarditis has rarely been described in patients with sarcoidosis: we found only 2 reports of this association.We report the case of a 57-year-old man who presented with clinical and hemodynamic features of constrictive pericarditis, of unclear cause. He was admitted for treatment of recurrent pleural effusion. After a complicated hospital course, he underwent pericardiectomy. His clinical and hemodynamic conditions improved substantially, and he was discharged from the hospital in good condition. The pathologic findings, the patient''s clinical course, and his response to pericardiectomy led to our diagnosis of cardiac sarcoidosis presenting as constrictive pericarditis. In addition to the patient''s case, we discuss the nature and diagnostic challenges of cardiac sarcoidosis. Increased awareness of this disease is necessary for its early detection, appropriate management, and potential cure.     

Diagnosis and Management of Cardiac Sarcoidosis

Cardiac sarcoidosis is an underdiagnosed disease that may be present in as many as 25% of patients with systemic sarcoidosis. Although most commonly recognized in patients with other manifestations of sarcoidosis, it may occur in isolation and its presence is often not appreciated. Cardiac sarcoidosis may present as asymptomatic left ventricular dysfunction, congestive heart failure, atrioventricular block, atrial or ventricular arrhythmia and sudden death. Although untested in clinical trials, early use of high-dose steroid therapy may halt or reverse cardiac damage. This article reviews the clinical manifestations, diagnosis and treatment of sarcoidosis, with an emphasis on new imaging techniques and therapies.     

Ventricular tachycardia and cardiac sarcoidosis: correspondence between MRI and electrophysiology

A 44-year-old woman with a history of histologically provedpulmonary and mediastinal sarcoidosis and chronic atrial fibrillationpresented with palpitations followed by syncope. Clinical examinationwas normal except for arrhythmia and discrete swelling of theankles. ECG showed the     

Right Ventricular Sarcoidosis: Is It Time for Updated Diagnostic Criteria?

A 55-year-old woman with a history of complete heart block, atrial flutter, and progressive right ventricular failure was referred to our tertiary care center to be evaluated for cardiac transplantation. The patient''s clinical course included worsening right ventricular dysfunction for 3 years before the current evaluation. Our clinical findings raised concerns about arrhythmogenic right ventricular cardiomyopathy. Noninvasive imaging, including a positron emission tomographic scan, did not reveal obvious myocardial pathologic conditions. Given the end-stage nature of the patient''s right ventricular failure and her dependence on inotropic agents, she underwent urgent listing and subsequent heart transplantation. Pathologic examination of the explanted heart revealed isolated right ventricular sarcoidosis with replacement fibrosis. Biopsy samples of the cardiac allograft 6 months after transplantation showed no recurrence of sarcoidosis. This atypical presentation of isolated cardiac sarcoidosis posed a considerable diagnostic challenge. In addition to discussing the patient''s case, we review the relevant medical literature and discuss the need for updated differential diagnostic criteria for end-stage right ventricular failure that mimics arrhythmogenic right ventricular cardiomyopathy.     

Benign ventricular tachycardia in systemic sarcoidosis--a case of false tendon

A 22-year-old female patient was diagnosed as having systemic sarcoidosis with pulmonary, skin and ocular lesions, and ventricular tachycardia in resting ECG. Although cardiac sarcoidosis was strongly suspected at diagnosis, no clinical symptom such as palpitation or syncope developed during the three year observation period. Cardiac silhouette was unchanged in chest X-ray and 201thallium myocardial scintigraphy revealed no abnormality. Ventricular complex was suppressed by exercise or tachycardia. Two-dimensional echocardiogram showed abnormal fascicular bands attached from the mid-septum to the apex (false tendon). Therefore, it was concluded that this benign form of ventricular tachycardia might be due to the false tendon, rather than to the cardiac involvement of sarcoidosis. The cause of arrhythmia is important when evaluating the prognosis of a patient with a systemic disease.     

Cardiac tamponade with paroxysmal atrial flutter controlled by antituberculous therapy

We report a rare and unique case of possible extrapulmonary tuberculosis in an 83-year-old man who had cardiac tamponade and paroxysmal atrial flutter. The patient was admitted to our hospital because of syncope. The cardiac tamponade and paroxysmal atrial flutter were treated by pericardiocentesis and drainage of bloody pericardial fluid. Mycobacterium tuberculosis was not detected in diagnostic specimens, nor was any evidence of malignancy found. The remarkable elevation of adenosine deaminase and the predominance of lymphocytes in the pericardial fluid, considering the past history of tuberculosis, led to a diagnosis of extrapulmonary tuberculosis. After receiving standard antituberculous therapy by ethambutol, isoniazid, and rifampicin, the patient recovered and has remained well up to the present day. Thirty-six months have passed since his recovery without the recurrence of cardiac tamponade or any other cardiac events.     

Electrophysiology studies in patients with dilated cardiomyopathies

Dilated cardiomyopathy is a diverse group of heart diseases with variable arrhythmia substrates. The response to programmed stimulation is dependent on spontaneous arrhythmia presentation. In patients with dilated cardiomyopathy, the majority of sustained monomorphic VT is caused by a scar-related reentrant mechanism, similar to that of coronary artery disease. The arrhythmia is uniformly inducible and is often refractory to pharmacologic therapy. Sustained VT is associated with more extensive myocardial fibrosis and non-uniform anisotropy, involving both the endocardium and epicardium, compared to those without sustained reentry. The response to programmed stimulation is more variable in patients presenting with nonsustained arrhythmia, cardiac arrest or syncope. Inducibility of monomorphic VT is much lower compared to those with ischemic heart disease. Other non-reentrant mechanism, such as focal automaticity, can also be observed in patients with monomorphic VT, in the absence of myocardial scar or evidence of slow conduction. The utility of electrophysiology studies to determine prognosis and to guide therapy remains limited in this patient population. The clinical outcome does not correlate with arrhythmia inducibility, and suppression of induced arrhythmia does not predict a good prognosis. The diagnosis of sarcoidosis or Chagas' cardiomyopathy should be considered in patients with dilated cardiomyopathy of unknown etiology, particularly in those with marked regional wall motion abnormalities and inducible VT. Epicardial reentrant circuits may be more prevalent in these cardiomyopathies, especially in those with VT related to chronic Chagas' disease. Although bundle branch reentry VT is a common finding in patients with dilated cardiomyopathy, it can occur in cardiomyopathy of any type and may coexist with other myocardial reentrant VT. It often has a typical bundle branch block QRS pattern during VT and is associated with His-Purkinje conduction delay. Evidence of macroreentry involving the bundle branches can usually be demonstrated, and catheter ablation of the bundle branches provides an effective and specific treatment.     

Ventricular tachycardia in cardiac sarcoidosis controlled by radiofrequency catheter ablation

We report a case of a 78-year-old woman with cardiac sarcoidosis with a history of syncope and palpitation. Further assessment with echocardiography, gadolinium-enhanced cardiovascular magnetic resonance (CMR) and histology led to a diagnosis of cardiac sarcoidosis. As the patient suffered from ventricular tachycardia (VT) despite active corticosteroid therapy, an implantable cardioverter-defibrillator (ICD) was positioned. She was also administered a beta blocker, but an electrical storm appeared every several days requiring ICD therapy. The drug-refractory VT was finally controlled with a catheter ablation session, during which we could detect the VT focus in the right ventricular outflow tract next to the aneurysm by using an electroanatomic mapping system (CARTO). Referring to echocardiographic and CMR images proved very useful in detecting the aneurysm using the CARTO system.     

Narrow complex tachycardias. Differential diagnosis and management.

Narrow complex tachycardias are a common clinical problem and can be divided into those in which the arrhythmic circuit is located exclusively in the atrium (pharmacologic treatment is oriented toward altering atrial electrophysiologic properties) and those that involve the AV node or an accessory pathway (pharmacologic therapy is directed toward slowing conduction or increasing refractoriness in these structures). The electrocardiographic diagnosis of the mechanism responsible for SVT includes the regularity of the RR interval; the AV conduction ratio; the presence of P waves, P wave morphology, and the relationship of the P waves to the QRS complexes; and the response of the arrhythmia and atrial activity to vagal maneuvers. Acute therapy includes cardioversion in hemodynamically unstable patients and vagal maneuvers and specific pharmacologic therapy for SVT based on the electrocardiographic diagnosis. There have been recent exciting advances in the nonpharmacologic treatments of SVT, most notably surgery and radiofrequency percutaneous catheter ablation for AV nodal reentry, AV reciprocating tachycardia, atrial flutter, and atrial tachycardias.     

Metastatic Melanoma Presenting as Polymorphic Ventricular Tachycardia

A 41-year-old woman with metastatic melanoma was admitted to the hospital because of syncopal episodes, which had developed after the administration of an experimental chemotherapy agent that targeted Notch signaling, as part of a phase I clinical trial. Cardiac monitoring revealed recurrent episodes of polymorphic ventricular tachycardia correlating with the patient''s syncope. Investigations into the cause of the arrhythmia led to the discovery of metastatic lesions within the left ventricular myocardium. In presenting this case of polymorphic ventricular tachycardia as the antemortem clinical manifestation of metastatic melanoma involving the heart, we discuss the importance of recognizing that cardiac metastases can manifest themselves as arrhythmias in patients with malignant melanoma who are undergoing active anticancer treatment.Key words: Antineoplastic chemotherapy protocols/adverse effects, arrhythmias, cardiac/chemically induced/diagnosis/physiopathology/therapy, coronary disease/complications, heart neoplasms/pathology/secondary, melanoma/pathology, myocardium/pathology, receptors, notch/metabolism, recurrence/prevention & control, syncope/etiology, tachycardia, ventricular/chemically induced/etiologyA woman with metastatic melanoma was admitted to the hospital because of syncope and was diagnosed with episodes of polymorphic ventricular tachycardia (VT). The episodes developed after the administration of an experimental chemotherapy agent that targeted Notch signaling as part of a phase I clinical trial. The patient was found to have metastatic lesions in her myocardium. We present and discuss this case of polymorphic VT as a manifestation of cardiac metastasis in malignant melanoma in a patient who was undergoing active anticancer treatment.     

Sick sinus syndrome after chemotherapy for malignant lymphoma with right atrial tumor at initial presentation

A 56-year-old man was admitted complaining of throat discomfort and dyspnea. He was given a diagnosis of diffuse large B-cell lymphoma on the basis of findings from tumor biopsy specimens of his left pharynx. MRI tomograms and ultrasonic cardiograms revealed a right atrial tumor causing tricuspid stenosis. Although chemotherapy rendered the cardiac tumor indistinct on MRI and UCG images, gallium-67 scintigraphy still demonstrated abnormal cardiac uptake. After 6 courses of CHOP therapy, sick sinus syndrome with syncope suddenly developed in the patient. A cardiac pacemaker was immediately implanted, and radiotherapy was started. The patient's sinus rhythm returned to normal shortly afterward, and the gallium-67 uptake eventually disappeared. In this case gallium-67 scintigraphy was the only diagnostic procedure capable of detecting evidence of residual disease.     

Cardiac sarcoidosis with a rare initial manifestation: sustained monomorphic ventricular tachycardia

Sarcoidosis is a multisystem disease of unknown cause. Life-threatening complications or sudden death can occur when the disease involves the heart. Because cardiac sarcoidosis has diverse clinical presentations, its diagnosis can be a major challenge for clinicians. It is very rare for the initial manifestation of cardiac sarcoidosis to be sustained ventricular tachycardia, especially in a patient with no prior symptoms or history of the disease. Herein, we discuss the case of a 41-year-old black man who presented with nausea, vomiting, and palpitations on the day after he had consumed alcoholic beverages heavily. Electrocardiographic examination revealed sustained monomorphic ventricular tachycardia. An automatic implantable cardioverter-defibrillator corrected the patient's abnormal heart rhythm, and therapy with steroids and β-blockers resolved his symptoms. We describe the process that led to the diagnosis of cardiac sarcoidosis in this patient.     

Unusual Presentation of Listerial Myocarditis and the Diagnostic Value of Cardiac Magnetic Resonance

Listeria monocytogenes is an infrequent cause of bacterial myocarditis. Myocarditis without evidence of endocarditis is even rarer. Management in such cases involves early diagnosis, antibiotic therapy, and emergency treatment of arrhythmias.We report the case of a 47-year-old man who presented with features of acute ST-segment-elevation myocardial infarction complicated by ventricular tachycardia that necessitated urgent electrical cardioversion. Contrast-enhanced cardiac magnetic resonance images revealed hypertrophy, necrosis, and a mass that was determined to be an abscess caused by L. monocytogenes. Antibiotic treatment led to resolution of the listerial myocarditis.In addition to reporting our patient''s case, we discuss the comparative advantages of cardiac magnetic resonance versus transthoracic echocardiography in characterizing myocarditis, upon presentation and in follow-up evaluation.     

Arrhythmias and cardiac electrophysiology

This article contains a review of some of the most significant advances in cardiac arrhythmias that have taken place in the last year, particularly those concerning the most common clinical conditions associated with the risk of arrhythmia, such as sudden cardiac death, atrial fibrillation and syncope, and genetically determined and hereditary heart disease, as well as advances concerning the principal nonpharmacologic treatments, such as catheter ablation, and the use of implantable defibrillators and cardiac resynchronization therapy.     

Short QT interval syndrome: a case report

Twelve-lead electrocardiograms revealed fine atrial fibrillation and a short QT interval (SQTI) (<300 milliseconds) with an average ventricular rate of 54/min in a 20-year-old male presented with exertional dyspnea. His echocardiographic evaluation revealed interatrial septal aneurysm and slightly dilated pulmonary artery. An electrophysiologic study revealed atrial fibrillation with a very high frequency, short ventricular effective refractory period (130 milliseconds) and ventricular fibrillation inducible with 3 short coupled extrastimuli. Signs were consistent with the rare SQTI syndrome. Although SQTI syndrome is associated with increased risk for sudden cardiac death, the patient was free of arrhythmia symptoms and denied any syncope or presyncope. Family history was also negative for sudden cardiac death and for any symptom suggestive of arrhythmia. The patient refused implantable defibrillator and was treated with anticoagulation and quinidine therapy.     

Successful Management of a Young Athlete with Type 2 Long QT Syndrome by Genotype-specific Risk Stratification and Bridging Therapy with a Wearable Cardioverter Defibrillator

We herein report a 14-year-old boy with repetitive nocturnal syncope related to medication-refractory long QT syndrome (LQTS). Although the use of an implantable cardioverter-defibrillator (ICD) was inevitable to prevent sudden cardiac death, he refused immediate implantation in order to play in a baseball competition six weeks away. Given his genetic diagnosis of type 2 LQTS, which is associated with cardiac events unrelated to exercise, we prescribed a wearable cardioverter defibrillator (WCD) to be donned at night, without limiting his exercise participation. An ICD was implanted after the competition. We successfully performed the preplanned treatment while maximizing the patient''s quality-of-life with a WCD and genotype-specific risk stratification.     

Permanent atrial standstill: The clinical spectrum

Permanent atrial standstill appears to be an arrhythmia that develops because of extensive myocardial disruption. It has been noted in three clinical settings: in patients with long-standing cardiac disease, in patients with neuromuscular disease and in otherwise healthy people who present with vertigo, syncope or stroke. A patient with this arrhythmia and limbgirdle muscular dystrophy is presented.     

Syncope in patients with normal EKG and without cardiac disease: value of ambulatory esophageal electrophysiology in determining etiology

The aim of this study was to determine whether ambulatory oesophageal electrophysiological recordings are valuable in identifying the cause of syncope in patients with a normal ECG and without cardiac disease. One hundred and forty-five patients aged 16 to 88 years of age, without cardiac disease, and with a normal ECG without a documented arrhythmia, were examined for unexplained syncope: 55 patients complained of palpitations at the time of syncope. The electrophysiological study was carried out in the clinic with oesophageal recordings using a classical protocol: Wenckebach point and sinus node recovery time were determined; programmed atrial stimulation was used with delivery of 1 and 2 extra-stimuli on the basal rhythm and with 20/30 micrograms infusion of isoprenaline; blood pressure was monitored. The study was negative in 41 patients (28%). Sinus node dysfunction was observed in 9 patients (6%). A vaso-vagal reaction reproducing the symptoms was induced by isoprenaline in 21 patients (14.5%); a conduction defect was revealed in 2 cases (1%). Atrial fibrillation or tachycardia > 1 minute was induced in 22 patients (15%). Paroxysmal junctional tachycardia was induced in 50 patients (35%). Patients with a negative study were younger than those with sinus node dysfunction or atrial fibrillation (44 +/- 21, 71 +/- 9 and 63 +/- 14 years respectively). Treatment was guided by these results: cardiac pacing, antiarrhythmic therapy or radiofrequency ablation of the reentrant pathway were indicated and suppressed syncope in all but two patients. The authors conclude that electrophysiological studies in the out-patient clinic with oesophageal recordings is a safe, rapid and economic method of detecting arrhythmias (sinus node dysfunction or supraventricular tachycardia) in 60% of patients with syncope, especially if they have symptoms of palpitations.     

Serial observation of electrocardiographic responses to corticosteroid therapy in a patient with right ventricular-predominant cardiac sarcoidosis

Predominant or isolated right ventricular involvement in cardiac sarcoidosis is uncommon, but should always be considered in a case of right ventricular hypertrophy combined with ventricular arrhythmia and/or conduction disturbance. Although improvement in right ventricular hypertrophy and atrioventricular conduction disturbance following corticosteroid therapy has been reported, the detailed serial electrocardiographic responses during corticosteroid therapy, as well as temporal changes in the electrocardiographic, biochemical, and morphological responses, have not been reported. We describe the clinical course and supportive imaging findings of reversible right ventricular hypertrophy and cardiac conduction disturbance in a case of right ventricular-predominant cardiac sarcoidosis.     

Localization of isolated atrial involvement of cardiac sarcoidosis

The authors report the first case of cardiac sarcoidosis involving only the interatrial septum and the atrioventricular node in a 30 year old man with known lymph node sarcoidosis. The diagnosis was suspected after the finding of an apparently innocent 1st degree atrioventricular block and confirmed by transoesophageal echocardiography and by IMATRON computerised tomography. Isolated atrial involvement of sarcoidosis is rare in the absence of any left ventricular disease. This explains the negative findings at transthoracic echocardiography and radio-isotopic investigations usually recommended for diagnosing cardiac sarcoidosis.