Congenital hypertrophic cardiomyopathy

    • [PDF File]Hypertrophic cardiomyopathy associated mitochondrial ...

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      hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, skeletal myopathy and mental retardation.9 The exact prevalence is unknown, although LAMP-2 mutations were noted in 1% of cases of HCM.9 Lysosomal storage disorders Fabry disease An X-linked disorder, caused by mutations of the alpha-galactosidase A gene.10 Fabry disease is a rare ...

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    • HYPERTROPHIC CARDIOMYOPATHY

      Congenital Anomalies in the Heart with Hypertrophic Cardiomyopathy JANE SOMERVILLEANDLAWSONMcDONALD Fromthe Institute ofCardiology, NationalHeartHospital, London W.1 The aetiology of hypertrophic cardiomyopathy remains unknown. Various theories have been suggested,andthereis evidencethatinsomepatients the condition may be inherited (Davies, 1952).

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    • A Case of Congenital Hypertrophic Cardiomyopathy

      HYPERTROPHIC CARDIOMYOPATHY Most often diagnosed during infancy or adolescence, hypertrophic cardiomyopathy (HCM) is the second most common form of heart muscle disease, is usually genetically transmitted, and comprises about 35–40% of cardiomyopathies in children. A diagram and echocardiogram comparing a normal heart and a heart with HCM are ...

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    • [PDF File]Noonan syndrome and cardiomyopathy: outcome predictors.

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      Cardiomyopathy: Definition “A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.”

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    • [PDF File]Hypertrophic Cardiomyopathy (HCM)

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      BrHeart 7 1985; 54: 543-7 Hypertrophic cardiomyopathy associated with a mitochondrial myopathyofvoluntary muscles and congenital cataract RCA SENGERS,* AMSTADHOUDERS,tEVANLAKWIJK-VONDROVICOVA,t KKUBAT,§ WRUITENBEEK* Fromthe Departments of*Paediatrics, tSubmicroscopic Morphology, ICardiology, and§Pathology, University …

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    • [PDF File]CAUSES OF HYPERTROPHIC CARDIOMYOPATHY

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      (1,2) Congenital heart disease (CHD) has been reported in as many as 90% of patients.(17) The two most common heart lesions seen are pulmonic stenosis (PS) and hypertrophic cardiomyopathy (HCM). Engle and Ehlers (1972) first described cardiomyopathy in patients with Noonan syndrome. In …

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    • [PDF File]Paediatric cardiomyopathy: new developments and insights

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      2017 Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death VA /SCD ICD (Class I) GDMT (Class I) WCD (Class IIb) ICD (Class I)* Primary prevention in pts with IHD, LVEF ≤40% EP study (especially in the presence of NSVT) Reassess LVEF >40 d after MI and/or >90 d after revascularization MI ...

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    • [PDF File]Congenital Anomalies in the Heart Hypertrophic …

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      Hypertrophic Cardiomyopathy Differential Diagnosis of Thickened LV Walls Cardiovascular Systemic Disease Acquired Congenital Hypertension Aortic stenosis Athlete’s heart Subaortic stenosis LV noncompaction Fabry disease Cardiac amyloidosis Hypereosinophilic syndrome

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    • [PDF File]Cardiomyopathy: An Overview

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      Paediatric cardiomyopathy: new developments and insights. Cardiomyopathies Definition! ... Hypertrophic cardiomyopathy! Primary cardiac disorder with a heterogeneous expression and ... Congenital coronary anomalies (19%) Mildly increased cardiac mass (10%) Ruptured aorta 5%

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    • [PDF File]ESC Guidelines on Hypertrophic Cardiomyopathy

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      Nov 15, 2017 · Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death.

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