RESUMO
Behçet's disease is a chronic multisystemic vasculitis of unknown etiology that evolves in relapses and remissions with a predominance of cutaneous and ocular lesions. It is more frequent in young adults, with a predominantly male population from the Mediterranean and Middle East. Cardiac involvement is rare (1 to 6%) but represents one of the most serious complications that can affect the three tunics of the heart. Cardiac thrombus is uncommon, its diagnosis relies mainly on echocardiography, and the development of the empirical treatment is based on immunosuppressive therapy and anticoagulation depending on the case. We report the case of a 21-year-old man admitted for a 2-month history of fever, loss of weight, and body condition in association with an intracardiac thrombus in the context of Behçet's disease. Intracardiac thrombus is a rare manifestation of Behçet's disease; the diagnosis must be made quickly in order to allow early management.
RESUMO
Cardiac tamponade is a life-threatening condition that requires emergency pericardiocentesis. In rare cases, pericardial drainage may be followed by "pericardial decompression syndrome" (PDS), a poorly understood but potentially fatal syndrome characterized by acute ventricular dysfunction. It may present in different clinical forms of varying severity and be managed differently depending on the clinical context. In this article, we report an atypical presentation of this syndrome, with the development of laminar tricuspid regurgitation after pericardial drainage. To our knowledge, this complication has never been reported in the medical literature. Our understanding of the pathophysiology of this condition is based entirely on case reports. And because clinical studies are difficult to perform, the best defense against PDS is early detection so that it can be recognized and treated quickly.
RESUMO
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.
RESUMO
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.
RESUMO
The term "aortopulmonary window" (APW), often referred to as "aortopulmonary septal defect," refers to a rare congenital medical disorder where there is an improper direct link between the main pulmonary artery and the ascending aorta. It can be combined with other cardiac congenital conditions or be an isolated lesion. Herein, we report the incidental discovery of a minor, restrictive aortopulmonary septal defect in a 60-year-old male who denied having any clinical symptoms. Incidentally detected APW in adulthood is uncommon and, hence, can be readily overlooked, a fortiori, in asymptomatic patients.
RESUMO
Coronary artery ectasia (CAE) is an entity causing inappropriate dilatation of the coronary tree, that is angiographically defined, albeit arbitrarily, by the diameter of the ectatic segment being more than 1.5 times larger in comparison with an adjacent healthy reference segment. Although the causative mechanisms are poorly understood, atherosclerosis is greatly implicated in the causation of CAE. Clinical, angiographic, and therapeutic features have been puzzling clinicians. We illustrate three different angiographic subsets, co-existing with myocardial bridge/coronary slow flow and diversely presenting as asymptomatic, pauci, and frankly symptomatic with stable and acute coronary syndrome. These cases illuminate the diversity of CAE's clinical and angiographic presentations and pathologic progression, shedding light on this medical condition and its implications.
RESUMO
Right heart thrombi can form in situ or lodge in the right cardiac chambers, originating from deep venous thrombosis. The latter carries a poor prognosis, taking into account the very high mortality rate. We herein report a case of an 83-year-old man who developed thrombus in the inferior vena cava that extended up to the right atrium, along with two distinct masses attached to the right ventricle wall.
