ABSTRACT
Marfan syndrome (MFS) is a heritable connective tissue disorder that is caused by a mutation of the FBN1 gene. It is characterized by cardiovascular, skeletal, and ocular manifestations, with thoracic aortic aneurysms being the main cardiovascular complication. Unconventionally, MFS can present with left ventricular noncompaction (LVNC), which introduces a supplementary aspect of cardiac dysfunction. We herein report the case of a 42-year-old male with MFS who presented with congestive heart failure and cardiogenic shock. His transthoracic echocardiography revealed a giant aortic root aneurysm, causing severe aortic regurgitation and dilated cardiomyopathy, along with LVNC. This case provides a brief overview of this rare medical condition, particularly the natural history of ascending thoracic aortic aneurysm, which is considered a silent complication and the most life-threatening one, combined with LVNC that correspondingly impairs the heart.
ABSTRACT
Electrical storm (ES) is a critical and potentially life-threatening cardiac rhythm disorder. It is characterized by the presence of three or more distinct episodes of sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) that necessitate appropriate termination. ES may occur in the setting of acute myocardial infarction or following myocardial reperfusion. An urgent treatment approach is necessary for better outcomes. We represent a case of a 64-year-old patient who presented with sudden chest pain and an episode of palpitations related to non-ST elevation myocardial infarction (NSTEMI), who has undergone percutaneous coronary intervention of the stenotic epicardial artery, but subsequently experienced an ES in the absence of stent thrombosis. ES presented in the form of sustained monomorphic VT that required synchronous direct current cardioversion, anti-arrhythmic drugs, deep sedation, and endotracheal intubation with a favorable course, with the patient being discharged after 14 days hospital stay. The practitioner should be mindful of the potential occurrence of ES following myocardial revascularization and should tailor the management approach.
