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- Östman-Smith, Ingegerd,1947Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics (author)
Hypertrophic cardiomyopathy in childhood and adolescence - strategies to prevent sudden death.
- Article/chapterEnglish2010
Publisher, publication year, extent ...
- 2010-08-19
- Wiley,2010
Numbers
- LIBRIS-ID:oai:gup.ub.gu.se/144114
- https://gup.ub.gu.se/publication/144114URI
- https://doi.org/10.1111/j.1472-8206.2010.00869.xDOI
Supplementary language notes
- Language:English
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- SwePubSwePub
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Notes
- Clinically overt hypertrophic cardiomyopathy is the most common cause of sudden unexpected death in childhood and has significantly higher sudden death mortality in the 8- to 16-year age range than in the 17- to 30-year age range. A combination of electrocardiographic risk factors (a limb-lead ECG voltage sum >10 mV) and/or a septal wall thickness >190% of upper limit of normal for age (z-score > 3.72) defines a paediatric high-risk patient with great sensitivity. Syncope, blunted blood pressure response to exercise, non-sustained ventricular tachycardia and a malignant family history are additional risk factors. Of the medical treatments used, only beta-blocker therapy with lipophilic beta-blockers (i.e. propranolol, metoprolol or bisoprolol) have been shown to significantly reduce risk of sudden death, with doses ≥ 6 mg/kg BW in propranolol equivalents giving around a tenfold reduction in risk. Disopyramide therapy is a very useful adjunct to beta-blockers to improve prognosis in those patients that have dynamic outflow obstruction in spite of large doses of beta-blocker, and its use in patients with hypertrophic cardiomyopathy is not associated with significant pro-arrhythmia mortality. Calcium-channel blockers increase the risk of heart failure-associated death in hypertrophic cardiomyopathy (HCM) patients with severe generalized hypertrophy and should be avoided in such patients. Amiodarone does not protect against sudden death, and long-term use in children usually has to be terminated because of side effects. Therapy with internal cardioverter defibrillator implantation has high paediatric morbidity, 27% incidence of inappropriate shocks, and does not absolutely protect against mortality but is indicated as secondary prevention or in very high-risk patients.
Subject headings and genre
- MEDICIN OCH HÄLSOVETENSKAP Medicinska och farmaceutiska grundvetenskaper Fysiologi hsv//swe
- MEDICAL AND HEALTH SCIENCES Basic Medicine Physiology hsv//eng
- Adolescent
- Adrenergic beta-Antagonists
- adverse effects
- therapeutic use
- Adult
- Age Factors
- Anti-Arrhythmia Agents
- adverse effects
- therapeutic use
- Cardiomyopathy
- Hypertrophic
- complications
- therapy
- Child
- Death
- Sudden
- Cardiac
- etiology
- prevention & control
- Defibrillators
- Implantable
- Electrocardiography
- Humans
- Risk Factors
- Young Adult
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- Göteborgs universitetInstitutionen för kliniska vetenskaper, Avdelningen för pediatrik (creator_code:org_t)
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- In:Fundamental & clinical pharmacology: Wiley24:5, s. 637-521472-82060767-3981
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