Risk Factors and Mode of Death in Isolated Hypertrophic Cardiomyopathy in Children

Decker, Jamie A.; Rossano, Joseph W.; Smith, E. O’Brian; Cannon, Bryan C.; Clunie, Sarah K.; Gates, C.; Jefferies, John L.; Kim, Jeffrey; Price, Jack F.; Dreyer, William J.; Towbin, Jeffrey A.; Denfield, Susan W.

doi:10.1016/j.jacc.2009.03.051

Cited by 124 publications

(142 citation statements)

References 11 publications

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“…HCM is the most common monogenic cardiac disorder and is a common cause of sudden death in the young population [35]. Varying degrees of myocardial fibrosis are present in HCM [36] and the occurrence of myocardial fibrosis correlates with wall thickness, wall motion abnormalities [37] and ventricular tachycardia [38].…”

Section: Hypertrophic Cardiomyopathy (Hcm)mentioning

confidence: 99%

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Roller¹,

Harth²,

Schneider³

et al. 2015

Fortschr Röntgenstr

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!Cardiac magnetic resonance imaging (CMRI) is a versatile diagnostic tool. One of its main advantages is the possibility of tissue characterization. T1-weighted images for scar and T2-weighted images for edema visualization are key methods for tissue characterization. Otherwise these sequences are strongly limited for the detection of diffuse myocardial pathologies. Recently, rapid technical innovations have generated new techniques. T1, T2 mapping and evaluation of the extracellular volume fraction (ECV) allow quantification of diffuse myocardial pathologies and showed great potential in the visualization of fibrosis, edema, amyloid, iron overload and lipid. In the future these techniques might enable the detection of early cardiac involvement, even act as a prognosticator. Moreover, therapy monitoring and follow-up might be possible due to versatile parameter quantification with these new techniques. Key points:▶ CMR allows for tissue characterization via T1-and T2-weighted sequences.▶ In cases of diffuse, global myocardial pathologies, correct image interpretation with traditional CMR sequences might be difficult.▶ T1, T2 mapping and ECV can quantify diffuse, global myocardial pathologies.

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Section: Hypertrophic Cardiomyopathy (Hcm)mentioning

confidence: 99%

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Roller¹,

Harth²,

Schneider³

et al. 2015

Fortschr Röntgenstr

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show abstract

“…Diagnosis at < 12 months of age, malformation syndromes, certain inborn errors of metabolism, low LV FS [6]; exercise-related abnormal blood pressure [30], mixed functional phenotypes, higher LVED posterior wall thickness or end-diastolic ventricular septal thickness, or two or more risk factors [31].…”

Section: Hypertrophicmentioning

confidence: 99%

“…Unlike in adults, where the assessment of ventricular dysfunction is the absolute LV wall thickness or hypertrophy, in children, absolute values are not useful because of their constantly changing body surface area. Instead, diagnosis depends on the association of LV dysfunction indexed to body surface area, defined as fractional shortening Z-score [30].…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Sixteen (16) prospective or retrospective studies meeting the inclusion criteria were included in this meta-analysis [12,24,26,30,31,[54][55][56][57][58][59][60][61][62][63][64]. Altogether, the studies investigated a total sample of 6,665 patients aged younger than 18 years with pediatric cardiomyopathies having mean high mortality rate of 12% within five years of diagnosis.…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

“…The study period ranged from 1958 [12] to 2012 [61]. Echocardiographic defined left ventricular dysfunction (also used in the recruitment of pediatric patients constituting the PCMR data), was the dominant method of diagnosis adopted by 14 studies (87.5%) [12,24,26,30,31,[54][55][56][59][60][61][62][63][64]. The remaining two studies adopted genetic testing and biopsy [57] and angiography [58].…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

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Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

Albakri¹

2018

Pediatr Dimensions

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Cardiomyopathies define a heterogeneous group of heart muscle disorders. They are an important cause of heart failure and a leading indication for heart transplantation in pediatric patients. Research on cardiomyopathy has however focused on the adult populations with fewer dedicated clinical trials investigating pediatric cardiomyopathy. Diagnosis and clinical management of pediatric cardiomyopathy are also challenging because of the lack of specific methods for diagnosis and treatment. Assessment of ventricular structure using absolute values in adults are not useful in children because of their constantly changing body surface area. Therapies developed for adults applied to pediatric patients also have had varied clinical outcomes. This paper therefore reviews existing research evidence on pediatric cardiomyopathy, with the objective of broadening its clinical understanding and management.

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Long-term Outcomes of Pediatric-Onset Hypertrophic Cardiomyopathy and Age-Specific Risk Factors for Lethal Arrhythmic Events

et al. 2018

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Pediatric-onset HCM is rare and associated with adverse outcomes driven mainly by arrhythmic events. Risk extends well beyond adolescence, which calls for unchanged clinical surveillance into adulthood. In this study, predictors of adverse outcomes differ from those of adult populations with HCM. In secondary prevention, the implantable cardioverter defibrillator did not confer absolute protection in the presence of limiting symptoms of heart failure.

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Risk Factors and Mode of Death in Isolated Hypertrophic Cardiomyopathy in Children

Cited by 124 publications

References 11 publications

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

Long-term Outcomes of Pediatric-Onset Hypertrophic Cardiomyopathy and Age-Specific Risk Factors for Lethal Arrhythmic Events

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