AL amyloidosis is the consequence of clonal production of amyloidogenic immunoglobulin light chain (LC) proteins, often resulting in a rapidly progressive and fatal amyloid cardiomyopathy. Recent work has found that amyloidogenic LC directly initiate a cardio-toxic response underlying the pathogenesis of the cardiomyopathy; however, the mechanisms that contribute to this proteotoxicity remain u...

Dandy Walker malformation (DWM) is a rare congenital brain anomaly characterized by cystic dilation of the fourth ventricle and hypoplasia of the cerebellar vermis. Other extracranial anomalies can be associated, including cardiac defects. We report a rare patient with DWM associated with progressive heart failure secondary to hypertrophic cardiomyopathy. He was diagnosed at 2 months of age and...

conclusions in the setting of acute left ventricular function depression in hocm, a comprehensive differential diagnosis should be established. treatment should be based on hemodynamic changes. after recovery, the prognosis is related to hocm. case presentation we present a unique case where tks occurred in a middle-aged male patient with hypertrophic obstructive cardiomyopathy (hocm) without a...

introduction dilated cardiomyopathy (dcm) is the leading cause of heart failure and arrhythmia. case presentation a 47-year-old male, diagnosed with dilated cardiomyopathy, died due to heart failure. during the screening of his family members, his 17-year-old daughter and 9-year-old son also had dilated cardiomyopathy. another daughter had died suddenly at the age of 12 years. conclusions we he...

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a condition characterized by fibrofatty replacement of the RV myocardium due to genetic abnormality. Structural changes may be absent or minor in early stages disease and localized specific region RV. Clinically it appears as electrical instability. To reduce risk arrhythmic events sudden cardiac death, device therapy pharmacotherapy rec...

Anaphylaxis is an acute, severe systemic allergic reaction that can be potentially fatal. It often transition to refractory hemodynamic instability (new onset myocardial dysfunction/ left ventricular dysfunction/LVD). Stress-related cardiomyopathy (SRC) now emerging as a significant contributor of dysfunction and has multifactorial etiologies, including the excessive catecholamine released in i...

Transthyretin amyloidosis is a fatal disorder that is characterized primarily by progressive neuropathy and cardiomyopathy. It occurs in both a mutant form (with autosomal dominant inheritance) and a wild-type form (with predominant cardiac involvement). This article guides clinicians as to when the disease should be suspected, describes the appropriate diagnostic evaluation for those with know...

Malignant disease and its treatment may produce a variety of complications. Many of these complications are relatively specific to the disease (e.g., leukostasis in acute myeloid leukemia) or to a class of chemotherapeutic agents (e.g., chronic cardiomyopathy with the anthracyclines). However, life-threatening complications associated with malignancy are common to a variety of tumor types; in a...

Isolated left ventricular noncompaction (LVNC) is a rare primary genetic cardiomyopathy characterized by prominent trabeculation of the left ventricular wall and intertrabecular recesses. Perioperative management of the patient with LVNC might be challenging due to the clinical symptoms of heart failure, systemic thromboembolic events, and fatal left ventricular arrhythmias. We conducted real t...