Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a case study of a 58-year-old patient with hypertrophic cardiomyopathy, mid-ventricular obstruction, and apical aneurysm who had an episode of syncope due to ventricular tachycardia. Cardiovascular magnetic resonance imaging revealed asymmetrical left ventricular hypertrophy with mid-ventricular obstruction and an apical aneurysm. His coronary angiography was normal, and his ventricular tachycardia was induced by hypertrophic cardiomyopathy without ischemia. Apical aneurysmectomy, left ventricular reconstruction, and cryoablation at the rim of the aneurysm were performed. Fifteen days after the operation, an automatic implantable cardioverter-defibrillator was implanted. The postoperative course was uneventful, and ventricular tachyarrhythmia did not recur during 18 months of follow-up.
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PMID:Hypertrophic cardiomyopathy with apical aneurysm: left ventricular reconstruction and cryoablation for ventricular tachycardia. 2154 32

The aetiology of left ventricular hypertrophy (LVH) in an athlete is often difficult to identify. We describe a 29-year-old fitness instructor who was referred for investigation of syncope. He gave a history of intensive weight lifting and anabolic steroid use at supra-therapeutic doses for the preceding 6 years. Electrocardiography showed inferolateral repolarisation abnormalities and a transthoracic echocardiogram demonstrated asymmetrical LVH with reduced left ventricular cavity dimensions. There was no left ventricular outflow tract obstruction or systolic motion of the anterior mitral valve leaflet. These findings were confirmed on cardiac magnetic resonance imaging (CMR). The differential diagnosis included athlete's heart, steroid-induced cardiomyopathy and non-obstructive hypertrophic cardiomyopathy. The patient was advised to discontinue both steroid use and intensive training. After 3 years of steroid abstinence but continued training, the syncopal episodes and the ECG abnormalities completely resolved, associated with regression of LVH on echocardiography and CMR.
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PMID:Identifying the aetiology of left ventricular hypertrophy in an athlete: importance of lifestyle modification. 2213 27

Severe asymmetrical hypertrophic cardiomyopathy without heart block accompanied by neuromuscular hypotonia and feeding difficulties was evident shortly after birth in the second child of a mother with systemic lupus erythematosus who had no indication of gestational diabetes. High-level anti-ribonucleoprotein (RNP) and Smoth (Sm) antibodies arising from transplacental transfer of maternal antibodies were detected in the child's serum. The cardiac abnormalities improved with a commensurate decline in antibody titers. Previously reported cases of neonatal cardiomyopathy with endocardial fibroelastosis have been ascribed to the transplacental transfer of maternal Sjogrens Syndrome (SS) A (Ro) and Sjogrens Syndrome (SS) B (La) antibodies and have been more severe and persistent compared with our patient. We advocate close monitoring of all babies of mothers with systemic autoimmunity for changes in heart rate during pregnancy and signs of heart failure and neuromuscular weakness after delivery.
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PMID:Resolution of neonatal hypertrophic cardiomyopathy presumed secondary to acquired maternal ribonucleoprotein and smith autoantibodies. 2414 43

Sarcoidosis is a multisystem, granulomatous disease of unknown etiology often seen in young adults, with cardiac involvement in more than one-quarter of sarcoid patients. The clinical presentation of cardiac sarcoid depends upon the location and extent of myocardium involved. Although cardiac sarcoid may produce asymmetrical septal hypertrophy, it is most commonly considered in the differential diagnosis of dilated cardiomyopathy. The hypertrophic stage of cardiac sarcoid is rarely seen. We describe a case of cardiac sarcoid in a young patient wherein a distinctive appearance of the cardiac sarcoid spectrum from "hypertrophic" stage to thinned/scarred stage, masquerading as hypertrophic cardiomyopathy followed by dilated cardiomyopathy, is demonstrated.
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PMID:Cardiac sarcoid: a chameleon masquerading as hypertrophic cardiomyopathy and dilated cardiomyopathy in the same patient. 2450 39

Systolic anterior motion of the mitral leaflet causing left ventricular outflow tract obstruction is commonly seen in hypertrophic cardiomyopathy and also in patients with advanced mitral valve disease with excessive anterior leaflet tissue or a reduced aortic-mitral angle. We describe 2 octogenarians who presented with systolic mitral leaflet anterior motion in advanced mitral valve disease with severe mitral annular calcification and associated asymmetrical septal hypertrophy.
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PMID:Systolic anterior motion of mitral valve with calcified annulus in octogenarians. 2457 May 31

Costello syndrome is a rare syndrome associated with de novo mutations in the HRAS gene. It is mostly revealed during in the first months of life by growth retardation, facial dysmorphic features, skin and cardiac abnormalities and subsequent cognitive deficit of varying severity. We report a case of Costello syndrome in a 3-month-old infant. The initial cardiac investigations were normal except frequent premature atrial complexes. After few months, worsening arrhythmia with bursts of ventricular tachycardia were noted as well as the secondary progressive obstructive left ventricular hypertrophic cardiomyopathy (HCM). Cardiac involvement is determinant for the prognosis of Costello syndrome. It frequently consists of hypertrophic cardiomyopathy (one third of patients), with involvement of the left ventricle in half of the cases. It is often asymmetrical and associated with obstruction of the outflow recalling family hypertrophic cardiomyopathy. The natural history of HCM in Costello syndrome and its management remains poorly known because of paucity of reported cases. Progression of the HCM can be very rapid like the reported case. On the other hand, the spontaneous regression of the HCM in some patients has been reported. In addition, cardiac threatening arrhythmias may be noted. So that, cardiac assessment and monitoring with regular echocardiography and electrocardiogram follow up is mandatory.
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PMID:Cardiac events in Costello syndrome: One case and a review of the literature. 2471 41

The mechanisms responsible for the development of apical aneurysms in cases of hypertrophic cardiomyopathy (HCM) are currently unclear but likely involve multiple factors. Here, we present a case of HCM with marked subendocardial fibrosis involving the apical and proximal portions of the left ventricle. A 71-year-old man with left ventricular hypertrophy presented with signs and symptoms of heart failure. The presence of asymmetrical left ventricular hypertrophy and bilateral, thickened ventricular walls with an apical aneurysm on transthoracic echocardiography suggested a diagnosis of HCM with ventricular dysfunction. No intraventricular pressure gradients with obstruction were identified. Late gadolinium enhancement (LGE) with cardiac magnetic resonance imaging and endomyocardial biopsies showed subendocardial fibrosis involving the apical aneurysm and proximal portion. Whereas LGE in a transmural pattern is commonly observed in HCM apical aneurysms, subendocardial LGE, as noted in the present case, is a relatively rare occurrence. Thus, the present case may provide unique insights into the adverse remodeling process and formation of apical aneurysms in cases of HCM.
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PMID:A rare case of hypertrophic cardiomyopathy with subendocardial late gadolinium enhancement in an apical aneurysm with thrombus. 2508 9

Hypertrophic cardiomyopathy is one of the most common causes of sudden cardiac death among young adults and adolescents. Unfortunately, the first manifestation of the condition may be sudden death during exertion, such as sporting activities. Other clinical symptoms include exertional dyspnea, angina, and syncope. Postmortem examination often reveals asymmetrical septal thickening and mural plaque formation in the left ventricular outflow tract. Histologic analysis shows cardiac myocyte hypertrophy, myofiber disarray, and interstitial and replacement fibrosis. Molecular analysis for known genetic abnormalities is essential to genetic counseling of living relatives of decedents to assess and reduce the risk of sudden cardiac death from hypertrophic cardiomyopathy.
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PMID:Sudden cardiac death: a modern pathology approach to hypertrophic cardiomyopathy. 2572 39

A 75-year-old female presented for left total hip reimplantation and suffered pulseless electrical activity arrest upon lateral positioning and administering vancomycin. Resuscitation was achieved according to Advanced Cardiac Life Support protocol. Post-event echocardiography showed hypertrophic cardiomyopathy with asymmetrical septal thickening, an under-filled left ventricle, dynamic left ventricular outflow obstruction, and severe mitral regurgitation related to systolic anterior motion of the mitral valve. Laboratory analysis showed a tryptase level of 209 ng/mL. After multispecialty evaluation, it was concluded that the patient's arrest was due to vancomycin anaphylaxis in the setting of previously undiagnosed hypertrophic cardiomyopathy leading to acute left ventricular outflow tract obstruction. After medical optimization of the patient's cardiomyopathy and an evaluation of potential intraoperative allergic triggers, the patient underwent a successful hip reimplantation without incident. This case presents a novel combination of events leading to intraoperative cardiac arrest. Rapid identification and an understanding of the cause(s) of cardiac arrest in this setting are critical for effective perioperative care.
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PMID:A rare combination of undiagnosed hypertrophic cardiomyopathy revealed by intraoperative anaphylaxis resulting in acute left ventricular outflow obstruction and cardiac arrest. 2837 94

We present a case of Fabry disease with an uncommon pattern of asymmetrical hypertrophy with septal prominence resulting in an erroneous diagnosis of hypertrophic cardilmyopathy clinically. The deceased presented for a medicolegal autopsy following his sudden death after an AV node ablation. Fabry disease continues to be an important misdiagnosis of hypertrophic cardiomyopathy in a clinical setting. Early diagnosis of Fabry disease is essential so that early treatment can be instituted.
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PMID:Sudden death following AV node ablation in a man with Fabry disease mimicking hypertrophic cardiomyopathy. 2721 40


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