Project description:Accessory mitral valve (AMV) is a rare congenital abnormality with a usually early-age clinical onset, being potentially a cause of subvalvular obstruction of the left ventricular outflow tract. This report describes the case of a 60-year-old patient presented with palpitations and chest pain. Primary evaluation revealed a ventricular tachycardia episode while transthoracic echocardiography showed an intracardiac additional structure at the level of the left ventricular outflow tract. After transoesophageal echocardiography and paraclinical investigations this structure was proven to be an AMV tissue which did not provoke left ventricular outflow obstruction. This case presents an unusual late-age clinical onset of AMV without a clinically significant LVOT (left ventricular outflow tract) obstruction and highlights the importance of transthoracic and transoesophageal echocardiography in the diagnosis of this rare cardiological entity.
Project description:BackgroundCardiac involvement in Fabry disease is usually characterized by left ventricular hypertrophy (LVH) without obstruction at rest.Case summaryA 59-year-old female patient with progressive chest tightness misdiagnosed as having hypertrophic cardiomyopathy due to LVH with obstruction was finally diagnosed with Fabry disease. Echocardiography showed LVH with severe obstruction, "binary sign," papillary muscle hypertrophy, and depressed longitudinal strain in the basal inferolateral region. The patient felt chest tightness worsened 1 year after receiving enzyme replacement therapy. Percutaneous endocardial septal radiofrequency ablation was performed to relieve obstruction.DiscussionIt is rare for women with Fabry disease to present with severe symptoms, but it is possible. LVH with obstruction should not be a potential point of view to relax the vigilance of Fabry disease. Percutaneous endocardial septal radiofrequency ablation may help to relieve left ventricular outflow tract obstruction in Fabry disease.Take-home messagePaying attention to echocardiographic characteristics is helpful for the identification of Fabry disease.
Project description:Left ventricular outflow tract obstruction (LVOTO) has been reported with bio-prosthetic and mechanical mitral valves (MV), though it is more common with the former. The obstruction can be dynamic or fixed. We hereby report a case of fixed LVOTO following bio-prosthetic MV replacement (MVR).
Project description:Left ventricular outflow tract obstruction (LVOTO) complicated with unstable angina (uAP) has not been described widely, but patients with these two conditions have several problems. Differentiation of the two conditions is also often difficult because the chest symptoms are similar. Moreover, nitrates are commonly used for ischemic heart disease, but have the effect of worsening LVOTO. We experienced three cases of dynamic LVOTO with a sigmoid-shaped septum, and without typical hypertrophic obstructive cardiomyopathy, that were complicated with uAP. In all cases, LVOTO was improved after initial percutaneous coronary intervention (PCI) for the left anterior descending artery lesion. Next, a dobutamine stress test was performed and LVOTO was provoked again in two cases, but not in a case with small acute myocardial infarction of the basal septum during PCI. All cases remained asymptomatic with beta-blocker therapy. Therefore, PCI and beta-blocker administration for LVOTO with uAP resulted in favorable clinical courses in all three cases. These outcomes suggest that revascularization including PCI should have priority in the therapeutic strategy for a case of acute coronary syndrome with LVOTO.
Project description:We present a 74-year-old woman with kyphosis and symptoms of pre/syncope. Heart catheterization revealed dynamic left ventricular outflow tract obstruction (DLVOTO) with Brockenbrough Braunwald response only when kyphotic posture was assumed. She had a positive response to metoprolol. DLVOTO is a challenging diagnosis in the absence of resting LVOTO. (Level of Difficulty: Beginner.).
Project description:A 57-year-old man with high-grade synovial sarcoma was found to have cardiac involvement of his malignancy. Intracardiac tumor resulted in dynamic left ventricular outflow tract obstruction and severe mitral regurgitation. He underwent mitral valve replacement and had no cardiovascular symptoms at 1-year follow-up. (Level of Difficulty: Intermediate.).
Project description:We describe the first case of successful management of left ventricular outflow tract obstruction developing late after transcatheter aortic valve replacement with right ventricular apical pacing. The possible mechanisms of obstruction resolution are described. (Level of Difficulty: Advanced.).