Diagnosis and screening of hypertrophic cardiomyopathy in children

Dadlani, Gul H.; Harmon, William G.; Perez-Colon, Elimarys; Sokoloski, Mary C.; Wilmot, Ivan; Lipshultz, Steven E.

doi:10.1016/j.ppedcard.2010.11.005

Cited by 5 publications

(2 citation statements)

References 11 publications

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“…Nonetheless, while the modality and frequency of screening remain uncertain, it is common to review the medical histories of first-degree relatives and to assess their cardiac structure and function when the patient receives the diagnosis [19,50]. In addition, although the general population is not screened for HCM in the USA, ECG has a negative predictive value greater than 99% [51].…”

Section: Hcm In Childhoodmentioning

confidence: 99%

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

et al. 2013

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Pediatric cardiomyopathies, which are rare but serious disorders of the muscles of the heart, affect at least one in every 100,000 children in the USA. Approximately 40% of children with symptomatic cardiomyopathy undergo heart transplantation or die from cardiac complications within 2 years. However, a significant number of children suffering from cardiomyopathy are surviving into adulthood, making it an important chronic illness for both pediatric and adult clinicians to understand. The natural history, risk factors, prevalence and incidence of this pediatric condition were not fully understood before the 1990s. Questions regarding optimal diagnostic, prognostic and treatment methods remain. Children require long-term follow-up into adulthood in order to identify the factors associated with best clinical practice including diagnostic approaches, as well as optimal treatment approaches. In this article, we comprehensively review current research on various presentations of this disease, along with current knowledge about their causes, treatments and clinical outcomes.

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Section: Hcm In Childhoodmentioning

confidence: 99%

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

et al. 2013

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“…Contrary to recent progress in treatment, children with HCM therapy based on the use of betablockers, calcium channel blockers, antiarrhythmic drugs (disopyramide and amiodarone), pacemaker therapy (asynchronous ventricular pacing), implantation of the intracardiac defibrillator, and in extremely severe forms surgical myectomy and percutaneous radiofrequency septal reduction [8], [9]. Our account of HCM based on experience in a national referral center in Prishtina corroborates the dismal accounts of the disease which have been published previously from other centers.…”

Section: Discussionmentioning

confidence: 99%

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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The Value of National Institutes of Health (NIH) Registry-Based Research in Identifying Childhood Cardiac Disease Outcomes: The Pediatric Cardiomyopathy Registry Experience

Wilkinson

Westphal

Ross

et al. 2014

Pediatric and Congenital Cardiac Care

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Diagnosis and screening of hypertrophic cardiomyopathy in children

Cited by 5 publications

References 11 publications

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

Pediatric Cardiomyopathies: causes, epidemiology, Clinical course, Preventive Strategies and Therapies

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

The Value of National Institutes of Health (NIH) Registry-Based Research in Identifying Childhood Cardiac Disease Outcomes: The Pediatric Cardiomyopathy Registry Experience

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