Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder.

Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms. We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy.

Huge left atrial appendage aneurysm revealed by chronic hiccups.

Left atrial appendage (LAA) aneurysm is an extremely rare anomaly. So far, less than one hundred cases only have been reported worldwide. Revelation modes are dominated by complications such as arrhythmias and thromboembolic events. We herein report a pediatric case of huge congenital LAA aneurysm with an original revelation mode that has never been described before in medical literature.

Predictive factors of left atrial spontaneous echo contrast in patients with rheumatic mitral valve stenosis: a retrospective study of 159 patients.

BACKGROUND: Mitral 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. METHODOLOGY: This 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. RESULT: Left 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. CONCLUSION: Patients 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.

Giant left atrial myxoma mimicking severe mitral valve stenosis and severe pulmonary hypertension.

Myxoma is the most common primary tumor of the heart and can arise in any of the cardiac chambers. This paper reports A 50 -year-old woman without medical history and any cardiovascular risk factors was hospitalized for exertional dyspnea and palpitations from three months and signifiant weight loss. Transthoracic echocardiogram showed a giant left atrial myxoma mobile confined to the left atrium in systole, in diastole the tumor was seen prolapsing across the mitral valve into the left ventricle and partially obstructing it and causing severe functional mitral stenosis with a mean gradient of 21,3 mmHg. Severe pulmonary hypertension was confirmed by Doppler PAPs =137 mmHg. The patient was scheduled for cardiac surgery with good outcome.