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Hypertrophic cardio...
Hypertrophic cardiomyopathy in childhood and adolescence - strategies to prevent sudden death.
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- Östman-Smith, Ingegerd, 1947 (författare)
- Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics
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(creator_code:org_t)
- 2010-08-19
- 2010
- Engelska.
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Ingår i: Fundamental & clinical pharmacology. - : Wiley. - 1472-8206 .- 0767-3981. ; 24:5, s. 637-52
- Relaterad länk:
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https://gup.ub.gu.se...
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https://doi.org/10.1...
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Abstract
Ämnesord
Stäng
- 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.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Fysiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Physiology (hsv//eng)
Nyckelord
- 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
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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