Our analysis emphasizes increased in-hospital mortality and higher disease severity, over a short period of stay, in heart failure patients with left bundle branch block.
Title: Paroxysmal extreme pain disorder (PEPD) is an autosomal dominant painful neuropathy with many, but not all, cases linked to gain-of-function mutations in SCN9A which encodes voltage-gated sodium channel Na. 1.7. It is a very rare condition featured by flushing of the lower half of the body and excruciating burning pain caused by any stimulus below the waist or in the perianal region. PEPD may be associated with cardiovascular instability, especially prolonged sinus pauses, and thus has anesthetic implications. Pacemaker implantation is the alternative therapeutic option, but its indications have not been clarified yet.Background: This condition is well described in neurological literature, but to our knowledge, this is the first case report of a patient with paroxysmal extreme pain disorder with prolonged sinus pauses requiring anesthesia for an epicardial pacemaker even with the perioperative risk of the pathology. This clinical observation can help for a better management and understanding of the cardiac risk complications of PEPD especially for an infant whose diagnostic is frequently made at the stage of complication This clinical observation can put the item on the necessity of establishing recommendations for management of cardiac complications during PEPD. Case report:We extensively searched the literature on cardiac pacing in patients with PEPD and we described a new case of a 9 month old infant who was admitted in the emergency department for an episode of malaise apnea and hemifacial cyanosis relevant to PEPD. The neurologic exploration was normal. The diagnostic was confirmed by genetic study. The 24 hours recording demonstrated long pauses of 15 seconds during the crisis justifying the implantation of epicardial pacemaker without peri-operatory complications due to the high anesthetic risk of this pathology. IntroductionParoxysmal extreme pain disorder (PEPD) is an autosomal dominant painful neuropathy with many, but not all, cases linked to gain-of-function mutations in SCN9A which encodes voltage-gated sodium channel Na. 1.7. The paper should be of interest because this clinical observation can put the item on the severity of the cardiac risk complications of this disease especially for an infant whose diagnostic is frequently made at the stage of complication. This clinical observation can help for a better understanding of the management of anesthetic process Because of the potential for cardiovascular instability ObservationA 9-month-old infant was brought to the emergency department after an episode of malaise and hemifacial cyanosis. Her parent reported that the crisis was triggered by crying. The infant had vegetative manifestations like hemifacial redness, snorkeling and sweats with foam at the lips, motor manifestations like rubbing feet with reduced consciousness. So the infant turned blue around the lips and began gasping for air. During this time, her eyes "rolled back", and she did not interact with her parents. There were no clonic movements, and she did not respond to ...
BackgroundMitral valve stenosis is a common manifestation of chronic rheumatic heart disease. The presence of spontaneous echo contrast in the left atrium and left atrial appendage has been reported to be an independent predictor of thrombo-embolic risk in patients with mitral stenosis. The objective of this study was to retrospectively investigate various clinical and echocardiographic variables to predict the spontaneous echo contrast in these patients.MethodologyThis is a bicentric retrospective study which includes 159 cases of symptomatic mitral stenosis from January 2011 to June 2012. All of the patients had transthoracic and transesophageal echocardiography. Patients who had significant mitral regurgitation (> Grade I), significant aortic valve disease, previous mitral valvulotomy and anticoagulation or antiplatelet therapy were excluded from the study. Our study population was divided into two groups based on the presence (Group I) or absence (Group II) of spontaneous echo contrast.ResultLeft atrial spontaneous contrast was present in 34.6% of cases. Patients in this group have more frequent atrial fibrillation (P = 0.001), larger left atrial area (P = 0.027) and diameter (P=0.023), smaller mitral valve area (P = 0.025), and higher mean transmitral diastolic gradient (p = 0.003) as compared to patients without spontaneous echo contrast. There were no significant differences in the mean age (p = 0.38), duration of symptoms (p = 0.4) and left ventricular ejection fraction (p = 0.7) between patients with and without spontaneous echo contrast. On multivariate analysis, only mitral valve area and transmitral diastolic gradient (OR: 18.753, 1.21, CI [1,838-191,332], [1,064-1,376], p: 0.013, 0.004, respectively) were found to be independently associated to the presence of spontaneous echo contrast.ConclusionPatients with severe rheumatic mitral stenosis in atrial fibrillation or sinus rhythm have a higher risk of developing spontaneous echo contrast. These patients might benefit from prophylactic anticoagulation. The long-term outcomes can be ascertained in a study over a longer period and with periodic follow-up.
Tittle: Deep vein thrombosis in the lower limbs and pulmonary embolism are the most common thromboembolic phenomena in patients with inflammatory bowel disease. The inflammatory and thrombotic processes are connected, creating a vicious circle. Inflammation is at least implicated in maintaining the inflammatory process in ulcerative colitis and Crohn's disease. Many factors have been investigated as determinants of the pro-thrombotic tendency such as acquired risk factors or genetic and immune abnormalities, but a unique cause has not been found. Background: We felt that this case was of interest due to the rarity of the condition and the therapeutic challenge given the high risk of bleeding and the management of anti-inflammatory and immunological treatment. Case presentation: We describe the rare case of a patient with ulcerative colitis who suffered a massive pulmonary embolism that caused shock. Despite the therapeutic challenge and the high risk of hemorrhagic complications given the underlying disease, thrombolytic treatment was administered with a good result. Conclusion: Autoimmune disorders in general should be regarded not only as inflammatory disorders, but also as hyper-coagulable disorders. This clinical observation can put the item on the necessity of establishing recommendations for prevention of embolic events in patients with hemorrhagic colitis.
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