Multi-Modality Imaging for the Identification of Arrhythmogenic Substrates Prior to Electrophysiology Studies

Noninvasive cardiac imaging is crucial for the characterization of patients who are candidates for cardiac ablations, for both procedure planning and long-term management. Multimodality cardiac imaging can provide not only anatomical parameters but even more importantly functional information that may allow a better risk stratification of cardiac patients. Moreover, fusion of anatomical and functional data derived from noninvasive cardiac imaging with the results of endocavitary mapping may possibly allow a better identification of the ablation substrate and also avoid peri-procedural complications. As a result, imaging-guided electrophysiological procedures are associated with an improved outcome than traditional ablation procedures, with a consistently lower recurrence rate.

From pathogenesis to treatment, a systemic review of cardiac lipoma

Cardiac lipoma is an uncommon primary cardiac tumor. With the advancement of diagnostic methods and treatment techniques, more cases of cardiac lipomas have been reported and suggest that the entity previously widely thought to display classic features may also show atypical findings. A systemic review of the rare cardiac tumor was done by searching the literature of cardiac lipoma. We endeavor to summarize the clinical features of the rare disease from pathogenesis to treatment. Literature of cardiac lipoma was retrospectively searched through PubMed and 255 cases of cardiac lipoma were included into this analysis. Cardiac lipomas can occur anywhere within the heart, 53.1% were located within the cardiac chambers, 32.5% in the pericardium, 10,7% within the myocardium and 3.7% involved multiple structures. More than half of the reported cardiac lipomas (66%) may be clinically symptomatic, presenting with symptoms ranging from chest discomfort to syncope depending on their size and location as well as extent of myocardial involvement. Noninvasive cardiac imaging has replaced the role of autopsy and cardiothoracic surgery in detection and diagnosis of cardiac lipomas. Most symptomatic patients (83.7%) were treated by resection of cardiac lipomas and 68.3% of asymptomatic patients also underwentprophylactic resection. Overgrowth and myocardial infiltration of lipomas may result in unsuccessful resection. Recurrence of cardiac lipomas was rare but reported in a few cases. The early detection and accurate diagnosis of cardiac lipoma is of great significance in clinical management, to avoid an unfavourable outcome due to overgrowth.

Amyloidosis: diagnosis and new therapies for a misunderstood and misdiagnosed disease

Amyloidosis is a group of diseases characterized by extracellular deposition of amyloid fibril complexes. Fibril deposition results in organ dysfunction and possible failure. Amyloidosis is regarded as a rare disease, but in general is underdiagnosed. The two main types of systemic amyloidosis are immunoglobulin light chain and transthyretin amyloidosis. The increased availability of noninvasive cardiac imaging, genetic testing and improved laboratory assays and protein identification methods have led to increased diagnosis. However, in many cases, the diagnosis is not made until the patient develops organ impairment. Earlier diagnosis is required to prevent irreversible organ failure. Novel treatments for immunoglobulin light chain and transthyretin amyloidosis that halt disease progression, prolong and increase quality of life have recently become available.

Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment—A Systematic Review

Restrictive cardiomyopathy (RCM) is the least common among cardiomyopathies. It can be idiopathic, familial, or secondary to systematic disorders. Marked increase in left and/or right ventricular filling pressures causes symptoms and signs of congestive heart failure. Electrocardiographic findings are nonspecific and include atrioventricular conduction and QRS complex abnormalities and supraventricular and ventricular arrhythmias. Echocardiography and cardiac magnetic resonance (CMR) play a major role in diagnosis. Echocardiography reveals normal or hypertrophied ventricles, preserved systolic function, marked biatrial enlargement, and impaired diastolic function, often with restrictive filling pattern. CMR offering a higher spatial resolution than echocardiography can provide detailed information about anatomic structures, perfusion, ventricular function, and tissue characterization. CMR with late gadolinium enhancement (LGE) and novel approaches (myocardial mapping) can direct the diagnosis to specific subtypes of RCM, depending on the pattern of scar formation. When noninvasive studies have failed, endomyocardial biopsy is required. Differentiation between RCM and constrictive pericarditis (CP), nowadays by echocardiography, is important since both present as heart failure with normal-sized ventricles and preserved ejection fraction but CP can be treated by means of anti-inflammatory and surgical treatment, while the treatment options of RCM are dictated by the underlying condition. Prognosis is generally poor despite optimal medical treatment.