Clinical Features and Outcomes of Childhood Hypertrophic Cardiomyopathy

Nugent, Alan; Daubeney, Piers E.F.; Chondros, Patty; Carlin, John B.; Colan, Steven D.; Cheung, Michael; Davis, Andrew M.; Chow, C. W.; Weintraub, Robert G.

doi:10.1161/circulationaha.104.530303

Cited by 180 publications

(183 citation statements)

References 36 publications

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“…Presentation in infancy (<1 year) was consistently associated with an increased risk of CVD, likely secondary to congestive cardiac failure 7,22,28 ; however, outside infancy, the majority of studies found no association between age and the risk of death 12,20,[22][23][24]27 .…”

Section: Agementioning

confidence: 99%

Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

Norrish

Cantarutti

Pissaridou

et al. 2017

Eur J Prev Cardiolog

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Results: Twenty five studies (3394 patients) met inclusion criteria. We identified four conventional major risk factors which were evaluated in at least 4 studies and found to be statistically associated with an increased risk of death in at least 2 studies: previous adverse cardiac event (pooled hazard ratio 5.4 (95% CI 3.67-7.95), p <0.001 ); non-sustained ventricular tachycardia (pooled hazard ratio 2.13 (95% CI 1.21-3.74), p=0.009); unexplained syncope (pooled hazard ratio 1.89 (95% CI 0.69-5.16), p=0.22);and extreme left ventricular hypertrophy (pooled hazard ratio 1.80 (95% CI 0.75-4.32), p=0.19). Left atrial diameter did not meet the major risk factor criteria, however is likely to be an additional significant risk factor. 'Minor' risk factors included a family history of sudden cardiac death, gender, age, symptoms, ECG changes, abnormal blood pressure response to exercise and left ventricular outflow tract obstruction. Conclusions:A lack of well-designed, large population based studies in childhood hypertrophic cardiomyopathy means the evidence-base for individual risk factors is not robust. We have identified four clinical parameters which are likely to be associated with increased risk of SCD, SCD-equivalent event or CVD. Multi-centre prospective studies are needed to further determine their relevance in predicting SCD in childhood HCM and to identify novel risk markers. Condensed abstract:3 A systematic review and meta-analysis of clinical risk factors predicting sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy (HCM) was performed identifying four 'major' factors: previous adverse cardiac event; non-sustained ventricular tachycardia; syncope and extreme left ventricular hypertrophy. Well-designed multi-centre studies are required in the future to confirm these findings.4

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Section: Agementioning

confidence: 99%

Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

Norrish

Cantarutti

Pissaridou

et al. 2017

Eur J Prev Cardiolog

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“…A recent population-based study of outcome in children diagnosed at Ͻ10 years of age from Australia 26 found an annual mortality of 1.5% in children diagnosed at Ͼ1 year of age, including patients with MFS. The survival rates in these 2 studies are much better than previously reported in children with HCM (3% to 8%) thought to be idiopathic.…”

Section: Outcome In Ihcmmentioning

confidence: 99%

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

et al. 2007

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Background-Current information on the epidemiology and outcomes of hypertrophic cardiomyopathy (HCM) in children is limited by disease diversity and small case series. Methods and Results-The Pediatric Cardiomyopathy Registry has collected prospective and retrospective data on children diagnosed with HCM since 1990. We identified the various causes of HCM in childhood and determined the relationship between outcomes, cause, and age at presentation. Of 855 patients Ͻ18 years of age with HCM, 8.7% (nϭ74) had inborn errors of metabolism, 9.0% (nϭ77) had malformation syndromes, 7.5% (nϭ64) had neuromuscular disorders, and 74.2% (nϭ634) had idiopathic HCM. Children with HCM associated with inborn errors of metabolism and malformation syndromes have significantly worse survival than the other 2 groups. Patients with idiopathic HCM diagnosed before 1 year of age (nϭ227) had worse survival from the time of diagnosis than those diagnosed after 1 year of age (nϭ407). Patients with idiopathic HCM who survived to at least 1 year of age, however, had an annual mortality rate of 1% that was similar regardless of whether they were diagnosed before or after 1 year of age. Conclusions-In children, HCM is a diverse disorder with outcomes that depend largely on cause and age. Patients presenting before 1 year of age have the broadest spectrum of causes and the poorest outcome. In those children with idiopathic HCM who survive beyond age 1, however, survival is independent of age at diagnosis, with an annual mortality rate (1%) that is much lower than previously reported in children and is not different from has been found in population-based studies in adults.

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“…26,27 In the latter, this primary neurologic event may cause a cardiac death, mediated through abnormalities of cardiovascular autonomic function. 28 Chest pain is almost never present in patients with primary electrical disorders but is more likely in patients with cardiomyopathies, 29,30 congenital coronary artery abnormalities, 31 or aortic disease (eg, dissection or rupture associated with Marfan syndrome 32 ). Other nontypical cardiac presentations also may misdirect patients to other consulting medical subspecialties.…”

Section: Warning Signs and Symptomsmentioning

confidence: 99%

Pediatric Sudden Cardiac Arrest

Campell¹,

Berger²,

Ackerman³

et al. 2012

Pediatrics

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Pediatric sudden cardiac arrest (SCA), which can cause sudden cardiac death if not treated within minutes, has a profound effect on everyone: children, parents, family members, communities, and health care providers. Preventing the tragedy of pediatric SCA, defined as the abrupt and unexpected loss of heart function, remains a concern to all. The goal of this statement is to increase the knowledge of pediatricians (including primary care providers and specialists) of the incidence of pediatric SCA, the spectrum of causes of pediatric SCA, disease-specific presentations, the role of patient and family screening, the rapidly evolving role of genetic testing, and finally, important aspects of secondary SCA prevention. This statement is not intended to address sudden infant death syndrome or sudden unexplained death syndrome, nor will specific treatment of individual cardiac conditions be discussed. This statement has been endorsed by the American College of Cardiology, the American Heart Association, and the Heart Rhythm Society.

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Clinical Features and Outcomes of Childhood Hypertrophic Cardiomyopathy

Cited by 180 publications

References 36 publications

Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children

Pediatric Sudden Cardiac Arrest

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