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Septal Hypertrophy (septal + hypertrophy)
Selected AbstractsMiliary Neonatal Hemangiomatosis with Fulminant Heart Failure and Cardiac Septal Hypertrophy in Two InfantsPEDIATRIC DERMATOLOGY, Issue 4 2004A. H. O'Hagan M.B., M.R.C.P. We report two infants with this condition who had fulminant cardiac failure and cardiac septal hypertrophy. The first was a 5-day-old boy who presented with increasing numbers of cutaneous hemangiomata associated with worsening cardiac failure. Magnetic resonance imaging (MRI) showed extensive hepatic hemangioma. Despite treatment with systemic corticosteroids and subcutaneous interferon alfa-2b his disease progressed. Hepatic artery embolization was unsuccessful. The infant died of congestive cardiac failure at 6 weeks of age. Postmortem examination showed a massively enlarged cardiac interventricular septum and biventricular hypertrophy. The second patient was a 1-week-old girl who also had cutaneous hemangioma and cardiac decompensation. MRI showed extensive intrahepatic involvement. She was treated early with corticosteroids and interferon ,, which resulted in involution of the cutaneous and hepatic lesions. Cardiac septal hypertrophy did not persist at late follow-up, and the association of miliary neonatal hemangiomatosis and cardiac septal hypertrophy has not yet been established. [source] Transcoronary Ablation of Septal Hypertrophy Does Not Alter ICD Intervention Rates in High Risk Patients with Hypertrophic Obstructive CardiomyopathyPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 4 2005THORSTEN LAWRENZ Introduction: Transcoronary ablation of septal hypertrophy (TASH) is safe and effectively reduces the intraventricular gradient in patients with hypertrophic obstructive cardiomyopathy (HOCM). To analyze the potential of anti- and proarrhythmic effects of TASH, we studied the discharge rates of implanted cardioverter defibrillators (ICD) in patients with HOCM who are at a high risk for sudden cardiac death. Methods: ICD and TASH were performed in 15 patients. Indications for ICD-implantation were secondary prevention in nine patients after resuscitation from cardiac arrest with documented ventricular fibrillation (n = 7) or sustained ventricular tachycardia (n = 2) and primary prevention in 6 patients with a family history of sudden deaths, nonsustained ventricular tachycardia, and/or syncope. All the patients had severe symptoms due to HOCM (NYHA functional class = 2.9). Results: During a mean follow-up time of 41 ± 22.7 months following the TASH procedure, 4 patients had episodes of appropriate discharges (8% per year). The discharge rate in the secondary prevention group was 10% per year and 5% in the group with primary prophylactic implants. Three patients died during follow-up (one each of pulmonary embolism, stroke, and sudden death). Conclusion: In conclusion, on the basis of ICD-discharge rates in HOCM-patients at high risk for sudden death, there is no evidence for an unfavorable arrhythmogenic effect of TASH. The efficacy of ICD treatment for the prevention of sudden cardiac death in HOCM could be confirmed, however, mortality is high in this cohort of hypertrophic cardiomyopathy patients. [source] The Importance of Ventricular Septal Morphology in the Effectiveness of Dual Chamber Pacing in Hypertrophic Obstructive CardiomyopathyPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 9 2000PETKOW DIMITROW It has been reported that older patients with hypertrophic obstructive Cardiomyopathy (HOCM) benefited the most from dual chamber (DDD) pacing. Since in older patients the distribution of septal hypertrophy and left ventricular (LV) cavity shape differs from that in younger patients, we decided to study the efficacy of DDD pacing on the reduction of LV outflow tract (LVOT) gradient in different patterns of septal hypertrophy. We compared HOCM patients with nonreversed septal curvature, thus preserving the elliptical LV cavity contour (common in the elderly), (group I) versus patients with reversed septal curvature, deforming the LV cavity to a crescent shape (common in the young), (group II). Eighteen HOCM patients were studied (11 patients in group I and 7 patients in group II). After implantation of a DDD pacemaker, the LVOT gradient was measured using Doppler echocardiography at various programmed AV delay intervals to determine the maximal percentage decrease of LVOT gradient from baseline. The measurement was repeated after at least a 6-month follow-up (chronic DDD pacing). The baseline LVOT gradient was comparable between groups (79 ± 28 vs 81 ± 25 mmHg, P = 0.92). The LVOT gradient reduction at acute DDD pacing was significantly greater in group I than group II (61 ± 18% vs 23 ± 10%, P = 0.0001). This difference in favor of the patients from group I was maintained at midterm follow-up (69 ± 17% vs 40 ± 17% P = 0.0076). In conclusion, patients with normal septal curvature and preserved elliptical LV cavity shape had a greater reduction of LVOT gradient after DDD pacing than patients with reversed septal curvature deforming LV cavity. The proposed criterion assessing the septal curvature may be useful to predict the efficacy of DDD pacing in the reduction of LVOT gradient. [source] Miliary Neonatal Hemangiomatosis with Fulminant Heart Failure and Cardiac Septal Hypertrophy in Two InfantsPEDIATRIC DERMATOLOGY, Issue 4 2004A. H. O'Hagan M.B., M.R.C.P. We report two infants with this condition who had fulminant cardiac failure and cardiac septal hypertrophy. The first was a 5-day-old boy who presented with increasing numbers of cutaneous hemangiomata associated with worsening cardiac failure. Magnetic resonance imaging (MRI) showed extensive hepatic hemangioma. Despite treatment with systemic corticosteroids and subcutaneous interferon alfa-2b his disease progressed. Hepatic artery embolization was unsuccessful. The infant died of congestive cardiac failure at 6 weeks of age. Postmortem examination showed a massively enlarged cardiac interventricular septum and biventricular hypertrophy. The second patient was a 1-week-old girl who also had cutaneous hemangioma and cardiac decompensation. MRI showed extensive intrahepatic involvement. She was treated early with corticosteroids and interferon ,, which resulted in involution of the cutaneous and hepatic lesions. Cardiac septal hypertrophy did not persist at late follow-up, and the association of miliary neonatal hemangiomatosis and cardiac septal hypertrophy has not yet been established. [source] Treatment of severe valvular aortic stenosis and subvalvular discrete subaortic stenosis and septal hypertrophy with Percutaneous CoreValve Aortic Valve Implantation,CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS, Issue 5 2010Ariel Finkelstein MD Abstract Background: Percutaneous Aortic Valve Implantation (PAVI) is a procedure gaining popularity and becoming more widely used for the treatment of patients with severe aortic stenosis who are at high risk for surgery. Here we show, for the first time, that a successful and complete elimination of both valvular and subvalvular pressure gradients can be achieved with a slight modification of the valve implantation technique. Methods and Results: A 91-year-old woman presented with shortness of breath at rest, effort angina, and pulmonary congestion. Echocardiography revealed calcified aortic stenosis with a peak gradient of 75 mm Hg accros the valve, and discrete subaortic stenosis (DSS) and marked hypertrophy of the basal septum with systolic anterior motion of the mitral valve (SAM). The intra ventricular gradient had a dynamic pattern across the DSS and the septal hypertrophy and measured 75 mm Hg. The total gradient across the left ventricular outflow (valvular and subvalvular) was 125 mmHg. PAVI with a 23 mm CoreValve was performed with an intentional lower positioning of the valve towards the LV outflow tract; so that the valve struts cover the subaortic membrane and part of the thickened basal septum. At the end of the procedure, the SAM disappeared, and the left ventricular ouflow was widely open. At 1 month follow up the patient was asymptomatic, no pressure gradient was measured between the LV apex and the aorta. Conclusions: This is the first report of successful treatment of severe valvular aortic stenosis and combined subvalvular aortic stenosis due to DSS and septal hypertrophy with SAM with percutaneous aortic valve implantation. © 2010 Wiley-Liss, Inc. [source] Persistent Troponin Elevation in a Patient with Cardiac AmyloidosisCLINICAL CARDIOLOGY, Issue 11 2009Bjoern F. Kraemer MD Abstract A 79-year-old patient repeatedly presented with chest discomfort and dyspnea on exertion. With echocardiography a prominent left ventricular and septal hypertrophy was detected with reduced left ventricular function. Despite successful revascularization and excellent results after stenting, the patient showed persistently elevated troponin levels. To investigate the abnormal findings of persistent troponin elevation, septal hypertrophy, and heart failure we performed endomyocardial biopsies which showed widespread myocardial amyloidosis. Amyloid subtyping revealed transthyretin amyloidosis. This is the first case showing persistent troponin elevation in a patient with tranthyretin amyloidosis. Very few other cases have been published on the topic of cardiac amyloidosis and troponin elevation so far. Our case serves as an illustrating example in the differential diagnosis of nonischemic causes of persistent troponin elevation. It is important to consider cardiac amyloidosis in patients with troponin elevation and heart failure since the clinical management differs significantly from other causes of heart failure. Copyright © 2009 Wiley Periodicals, Inc. [source] Cardiac troponin T Arg92Trp mutation and progression from hypertrophic to dilated cardiomyopathyCLINICAL CARDIOLOGY, Issue 5 2001Noboru Fujino M.D. Abstract Background: Mutations in the cardiac troponin T gene causing familial hypertrophic cardiomyopathy (HCM) are associated with a very poor prognosis but only mild hypertrophy. To date, the serial morphologic changes in patients with HCM linked to cardiac troponin T gene mutations have not been reported. Hypothesis: The aim of this study was to determine the long-term course of patients with familial HCM caused by the cardiac troponin T gene mutation, Arg92Trp. Results: The Arg92Trp missense mutation was present in 10 individuals from two unrelated pedigrees. They exhibited different cardiac morphologies: three had dilated cardiomyopathy-like features, five had asymmetric septal hypertrophy with normal left ventricular systolic function, one had electrocardiographic abnormalities without hypertrophy, and one had the disease-causing mutation but did not fulfill the clinical criteria for the disease. The mean maximum wall thickness was 14.1 ± 6.0 mm. The three patients with dilated cardiomyopathy-like features had progressive left ventricular dilation. Three individuals underwent right ventricular endomyocardial biopsy. There was a modest degree of myocardial hypertrophy (myocyte diameter: 18.9 ± 5.2 m,m), and minimal myocardial disarray and mild fibrosis were noted. Conclusion: The Arg92Trp substitution in the cardiac troponin T gene shows a high degree of penetrance, moderate hypertrophy, and early progression to dilated cardiomyopathy in Japanese patients. Early identification of individuals with this mutation may provide the opportunity to evaluate the efficacy of early therapeutic interventions. [source] | ||||||||||