Treatment of hypertrophic cardiomyopathy in childhood

Colan, Steven D.

doi:10.1016/j.ppedcard.2010.11.004

Cited by 6 publications

(3 citation statements)

References 92 publications

(88 reference statements)

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“…The presence of outflow tract obstruction has not been found to be associated with an increased risk of sudden death; patients with outflow tract obstruction are at greater risk for symptoms and progression to death due to heart failure. Although the ability to define the etiology of HCM has improved overtime, this goal still remains elusive [6], [7]. Even in our study, we have not found any association between outflow obstruction and mortality rate.…”

Section: Discussioncontrasting

confidence: 66%

Diagnostic, Prognostic, and Therapeutic Implications and Outcomes of Children with Primary Hypertrophic Cardiomyopathy in Kosovo

Bejiqi¹,

Retkoceri²,

Bejiqi³

et al. 2020

Open Access Maced J Med Sci

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BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause, creating functional impairment of the cardiac muscle. It is a leading cause of sudden cardiac death in young athletes. The occurrence of HCM is a significant cause of sudden unexpected cardiac death in any age group and as a cause of disabling cardiac symptom. AIM: The aim of the study was identification of the manifestations, assessment, and follows-up of children with HCM by transthoracic echocardiography as an important tool for clinical management and better understanding of pathogenesis of HCM. MATERIALS AND METHODS: We present a comprehensive analysis of 43 patients seen in Kosovo, with clinical and echocardiographic signs of HCM. Retrospectively, we analyzed medical records, treatment, and outcomes of those children, who have continued follow-up at our institution. RESULTS: Twenty-three of them were male, aged between 4 months and 9 years at the first presentation (median of 2 years and 3 months). Cardiac failure, seen in almost half of them, was the most frequent presenting feature. At admission, the chest radiographs revealed an increased cardiothoracic ratio, to a mean of 72% in 5 infants and to 65% in 37 older children. Measured by transthoracic echocardiography, 28 patients had asymmetric hypertrophy of the left ventricle while 15 had concentric hypertrophy. The left ventricular ejection fraction was depressed in 21 patients. Patients with cardiac failure received various combinations of diuretics, B-blockers, angiotensin-converting enzyme inhibitors, and aspirin. Death occurred in 8 patients, in 4 of them shortly after admission; 4 patients left Kosovo and continued examination abroad, and the remaining 32 were followed up for a mean 42 months, with a range from 5 to 115 months. Surgical intervention was not performed on any of them despite the clinical and echocardiographic indications. Recovery was noted in 14 patients but requiring anti-failure medications. Slightly over two-fifths died. Of those with asymmetric form, 45% died, in half of those presenting in infancy, and 89% of those who presented at admission with signs of cardiac failure. CONCLUSION: With the exception of the studies of pacing, no conclusive evaluations of treatments for HCM have been conducted. Management strategy is, therefore, based largely on clinical experience and consensus of many specialists.

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Section: Discussioncontrasting

confidence: 66%

Diagnostic, Prognostic, and Therapeutic Implications and Outcomes of Children with Primary Hypertrophic Cardiomyopathy in Kosovo

Bejiqi¹,

Retkoceri²,

Bejiqi³

et al. 2020

Open Access Maced J Med Sci

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“…50 It will be important to test thin filament activators in other DCM models, especially those linked to mutations in the thick filaments and titin. Moreover, there are no specific early interventions in children from families with familial cardiomyopathies, 51 although early identification of HCM and DCM mutations are possible and more common. 52 Our data point to the importance of taking advantage of early childhood diagnosis and to start prevention therapy before the disease develops.…”

Section: Discussionmentioning

confidence: 99%

Early sensitization of myofilaments to Ca2+ prevents genetically linked dilated cardiomyopathy in mice

Alves

Warren

Simon

et al. 2017

Cardiovascular Research

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“…Tato léčba se však ukázala méně efektivní, než se předpokládalo, a působí v mnohých případech jako placebo [30]. Dnes se doporučuje u těch symptomatických nemocných s obstrukční formou, u nichž nelze provést myektomii nebo perkutánní septální ablaci (PTSMA) [31].…”

Section: Jak Léčit Hkmp?unclassified

Hypertrophic cardiomyopathy - what is new?

Gregor

Čurila

2012

Cor Vasa

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Práce shrnuje některé nové poznatky týkající se hypertrofických kardiomyopatií. Zabývá se jejich současnou definicí, genetickými nálezy a vztahem k fenotypu onemocnění, dále otázkami výskytu, prognózy onemocnění. Diskutovány jsou i současné názory na léčbu, především s ohledem na provádění perkutánních septálních ablací a myektomií u těžce symptomatických, obstrukčních forem.

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Treatment of hypertrophic cardiomyopathy in childhood

Cited by 6 publications

References 92 publications

Diagnostic, Prognostic, and Therapeutic Implications and Outcomes of Children with Primary Hypertrophic Cardiomyopathy in Kosovo

Diagnostic, Prognostic, and Therapeutic Implications and Outcomes of Children with Primary Hypertrophic Cardiomyopathy in Kosovo

Early sensitization of myofilaments to Ca2+ prevents genetically linked dilated cardiomyopathy in mice

Hypertrophic cardiomyopathy - what is new?

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