Acute coronary syndrome in Behcet's syndrome: A systematic review.

Behcet syndrome is a rare vasculitis that affects both arteries and veins. Vasculo-Bechet Syndrome (VBS) is seen predominantly in men. Genetic predisposition and immune dysregulation leading to inflammation, endothelial damage, and impaired fibrinolysis contribute to its pathogenesis. Isolated case reports of Behcet syndrome (BS) with associated acute coronary syndrome (ACS) have been reported in the past. In this study, we present the first systematic review of such cases. A systematic search was conducted using Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases from 1980-2018 to identify case reports of myocardial infarction associated with BS. Cases that fulfilled the criteria for BS were selected for analysis. Demographic data, electrocardiography, echocardiography, angiography findings, and management were analyzed when available. We identified 62 case reports. Most subjects were men with a mean age of 37 years. Twenty-one percent were smokers, but other traditional cardiovascular risk factors were less common. Myocardial infarction was confirmed in half of the cases with findings on electrocardiogram (ECG). Echocardiogram revealed wall motion abnormality in 76% of patients, and angiography showed double-vessel disease in more than half of the cases. Mortality was reported in 1.6% of the cases. This systematic review shows that ACS in BS affects young males with low prevalence of coronary artery disease risk factors. Chest pain is the most common presenting feature and ST-segment elevation myocardial infarction (STEMI) was the most common ECG finding. Immunotherapy may be helpful to prevent future ACS in these patients.

Steal and strain: A case of coronary artery fistula presenting with coronary steal syndrome and underlying bronchiectasis.

BACKGROUND: Coronary artery fistula is a relatively rare disorder with an incidence rate of 0.05-0.9%, and the majority of fistulae are detected incidentally. Most coronary artery fistulae are congenital, and the acquired variant is very rare. Herein, we present a possible acquired coronary artery to pulmonary artery fistula, most likely secondary to bronchiectasis in the adjacent lung. We will analyze the hemodynamic significance of the fistula in this case and also seek to understand the outcomes of various treatment modalities. CASE PRESENTATION: A 56-year-old male patient presented with hypoxemia secondary to acute pulmonary edema during a hypertensive emergency. He developed myocardial ischemia after treatment with diuretics and nitroglycerin, due to shunting of blood from the right coronary artery to the right lower lobe branch of the right pulmonary artery, via the fistula. This resulted in coronary steal syndrome. Coronary angiogram confirmed the fistula connecting the right coronary artery to the right lower lobe branch of the right pulmonary artery. An attempt at coil embolization was unsuccessful due to the inability to advance the microcatheter beyond the fistula. DISCUSSION: The majority of coronary artery fistulae are asymptomatic as they are hemodynamically not significant and are incidentally detected by coronary angiography, CT angiogram, echocardiogram or multi-detector row computed tomography (MDCT) with 3D reconstruction. The development of congenital fistula can be explained by the Hackensellner involution-persistence hypothesis, but the anatomy in this case and the bronchiectasis in the part of the lung adjacent to the fistula makes an acquired cause very likely due to local inflammation and the age of patient at initial diagnosis. An initial diagnosis of bronchiectasis was made at age 51, which was 5 years prior to the detection of the coronary artery fistula in this patient. Symptoms have been described mostly in the elderly and include chest pain, dyspnea, fatigue, syncope, and palpitations. Such symptomatic fistula should be treated either by percutaneous transluminal embolization or surgical ligation. CONCLUSION: This is a unique case of acquired coronary to pulmonary artery fistula in the setting of bronchiectasis in a patient in which PTE was attempted and failed. More research is required to understand the pathophysiology of acquired fistula. The decision regarding the method of closure should be individualized and decided on a case by case basis.

COVID-19-Associated Coagulopathy: An Exacerbated Immunothrombosis Response.

Since the onset of the global pandemic in early 2020, coronavirus disease 2019 (COVID-19) has posed a multitude of challenges to health care systems worldwide. In order to combat these challenges and devise appropriate therapeutic strategies, it becomes of paramount importance to elucidate the pathophysiology of this illness. Coronavirus disease 2019, caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV2), is characterized by a dysregulated immune system and hypercoagulability. COVID-associated coagulopathy (CAC) was recognized based on profound d-dimer elevations and evidence of microthrombi and macrothrombi, both in venous and arterial systems. The underlying mechanisms associated with CAC have been suggested, but not clearly defined. The model of immunothrombosis illustrates the elaborate crosstalk between the innate immune system and coagulation. The rendering of a procoagulant state in COVID-19 involves the interplay of many innate immune pathways. The SARS-CoV2 virus can directly infect immune and endothelial cells, leading to endothelial injury and dysregulation of the immune system. Activated leukocytes potentiate a procoagulant state via release of intravascular tissue factor, platelet activation, NETosis, and inhibition of anticoagulant mechanisms. Additional pathways of specific relevance in CAC include cytokine release and complement activation. All these mechanisms have recently been reported in COVID-19. Immunothrombosis provides a comprehensive perspective of the several synergistic pathways pertinent to the pathogenesis of CAC.

Brugada Pattern in Diabetic Ketoacidosis: A Case Report and Scoping Study.

Brugada syndrome is a rare cardiac arrhythmia which is associated with right bundle branch block pattern (RBBB) and ST-segment elevation in right precordial leads. SCNA5 mutation is the most common genetic abnormality associated with Brugada syndrome. Brugada pattern not related to genetic mutations has been previously reported in the setting of fever, metabolic conditions, lithium use, marijuana and cocaine abuse, ischemia and pulmonary embolism, myocardial and pericardial diseases. Multiple isolated cases of Brugada pattern associated with diabetic ketoacidosis (DKA) have been previously reported. We here present a case of type 1 Brugada pattern in a 23 year-old-male who presented with DKA. Brugada pattern in DKA is attributed to acidosis and multiple electrolyte abnormalities including hyperkalemia which alter ion channel expression in the heart thus leading to Brugada pattern which subsequently resolved with treatment of DKA. In such patients, Brugada pattern is not reproducible on procainamide induction cardiac electrophysiology study (EPS). Our scoping study demonstrates male predominance 20/22 cases of (DELETE this highlighted area) Brugada pattern in DKA, a finding that is consistent with prevalence of this disease among males.

Doppler echocardiography imaging in detecting multi-valvular lesions: a clinical evaluation in children with acute rheumatic fever.

RATIONALE: Doppler echocardiography has been demonstrated to be accurate in diagnosing valvular lesions in rheumatic heart disease (RHD) when compared to clinical evaluation alone. OBJECTIVE: To perform Doppler echocardiography in children clinically diagnosed by the Jones criteria to have acute rheumatic fever (ARF), and to then compare the effectiveness of echo in detecting single/multi-valvular lesions with that of the initial clinical evaluation. METHODS AND RESULTS: We enrolled 93 children who were previously diagnosed with ARF by clinical examination. Presence of valvular lesions were enlisted, first by clinical auscultation, and then by performing Doppler echocardiography. We found that Doppler echocardiography was a sensitive technique, capable of detecting valvular lesions that were missed by clinical auscultation alone. Echocardiography of patients with carditis revealed mitral regurgitation to be the most common lesion present (53 patients, 56.98%), followed by aortic regurgitation in 21 patients (22.6%). The difference between clinical and echocardiographic diagnosis in ARF children with carditis was statistically significant for mitral regurgitation, aortic regurgitation and tricuspid regurgitation. Clinical auscultation alone revealed 4 cases of mitral stenosis, 39 mitral regurgitation, 14 aortic regurgitation, 9 tricuspid regurgitation; in contrast, echo revealed 5 cases of mitral stenosis, 53 mitral regurgitation, 21 aortic regurgitation, 18 tricuspid regurgitation. CONCLUSION: Doppler echocardiography is a more sensitive technique for detecting valvular lesions. In the setting of ARF, echo enables a 46.9% higher detection level of carditis, as compared to the clinical examination alone. Echo was very significant in detecting regurgitation lesions, especially for cases of tricuspid regurgitation in the setting of multivalvular involvement. The results of our study are in accordance with previous clinical studies, all of which clearly demonstrate the advantages of Doppler echocardiography, paving the way for its probable inclusion as one of the Jones major criteria for diagnosing ARF.