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

Lillie Frank Abercrombie Section of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030, USA.
Journal of the American College of Cardiology (Impact Factor: 16.5). 07/2009; 54(3):250-4. DOI: 10.1016/j.jacc.2009.03.051
Source: PubMed

ABSTRACT This study was designed to review outcomes of pediatric isolated hypertrophic cardiomyopathy (HCM) managed uniformly at a single institution and assess whether reported adult risk factors for sudden death are predictive in pediatric HCM.
Cardiac death in HCM occurs suddenly (SCD) or may be nonsudden (non-SCD). Little data exists on non-SCD in children. Risk factors for SCD in adult HCM are characterized and consensus management strategies detailed. Their application to children is uncertain and treatment strategies vary.
A retrospective cohort study of children with HCM was performed. Primary end points were cardiac death and transplantation. Frequency and outcomes of known adult risk factors were assessed. Outcomes analysis was performed using Kaplan-Meier curves and Cox regression analysis.
Ninety-six patients were included. The average age at diagnosis was 10.6 +/- 5.4 years, and mean follow-up was 6.4 +/- 5.2 years. Primary end points occurred in 11 patients over the 20-year follow-up (11%), 4 underwent cardiac transplant and 7 died (3 suddenly). Extreme left ventricular hypertrophy (z-score: >6) and an abnormal blood pressure response to exercise were predictive of non-SCD (p < 0.02 and p < 0.03, respectively). Kaplan-Meier survival analysis predicts an 82% survival over a 20-year period.
In children with isolated HCM managed primarily with exercise restriction and medication, cardiac death occurred infrequently. Non-SCD or transplant was at least as common as SCD. Extreme left ventricular hypertrophy and blunted blood pressure response to exercise were associated with an increased risk of non-SCD.

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    • "Echocardiography has important implications for prognosis. A high degree of LVH could indicate a worse prognosis44,45). "
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    ABSTRACT: Cardiomyopathy (CMP) is a heterogeneous disease caused by a functional abnormality of the cardiac muscle. CMP is of 2 major types, dilated and hypertrophic, and is further classified as either primary or secondary. Secondary CMP is caused by extrinsic factors, including infection, ischemia, hypertension, and metabolic disorders. Primary CMP is diagnosed when the extrinsic factors of secondary CMP are absent. Furthermore, the World Health Organization, American Heart Association, and European Cardiology Association have different systems for clinically classifying primary CMP. Primary CMP is rare and associated with a family history of the disease, implying that genetic factors might affect its incidence. In addition, the incidence of CMP varies widely according to patient ethnicity. Genetic testing plays an important role in the care of patients with CMP and their families because it confirms diagnosis, determines the appropriate care for the patient, and possibly affects patient prognosis. The diagnosis and genetic identification of CMP in patients' families allow the possibility to identify novel genes that may lead to new treatments. This review focuses on the epidemiology, pathophysiology, diagnosis, and treatment of CMP, with the aim of providing pediatricians with insights that may be helpful in the early identification and management of idiopathic CMP in children.
    Korean Journal of Pediatrics 02/2013; 56(2):52-59. DOI:10.3345/kjp.2013.56.2.52
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    • "It was reported that the occurrence of myocardial fibrosis was related to ventricular wall thickness, regional wall motion abnormalities [7] and ventricular tachycardia [8]. Several studies have found that sudden cardiac death [4] and heart failure [9] are relevant to myocardial fibrosis in patients with HCM. Up-to-date, the widely used late gadolinium enhancement (LGE) magnetic resonance imaging (MRI) in the clinic is the method of choice to detect fibrosis in ischemic and non-ischemic myocardial disease [10]. "
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    ABSTRACT: Purpose: To investigate the diagnostic value of T1 mapping imaging of evaluating fibrosis in patients with hypertrophic cardiomyopathy (HCM). Materials and methods: 21 subjects with HCM and 18 healthy volunteers underwent conventional late gadolinium enhancement (LGE) imaging and T1 mapping imaging. The region of myocardium in HCM is divided into remote area of LGE, peri-LGE, LGE (halo-like LGE and typical patchy LGE). These regions combined with normal volunteers' myocardium were calculated by the reduced percent of T1 value (RPTV). Results: The RPTV in healthy volunteers was no significant comparing with that in the remote area of LGE in HCM subjects (3.98 ± 3.19 vs. 3.34 ± 2.75, P>0.05). There were significant statistical differences in pairwise among the remote area of LGE, peri-LGE, halo-like LGE and typical patchy LGE in the RPTV (P<0.0001). ROC curves indicated that the T1 mapping imaging has a greater area under the curve comparing with that of traditional LGE imaging (0.975 ± 0.07 vs. 0.753 ± 0.26, P<0.0001). Conclusions: HCM has a high prevalence of fibrosis and with varying severity. T1 mapping imaging can be a useful method to evaluate the severity of the fibrosis in HCM.
    European journal of radiology 01/2013; 82(5). DOI:10.1016/j.ejrad.2012.12.014 · 2.37 Impact Factor
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    • "In children, the risk of non-sudden cardiac death is as high as sudden cardiac death. Extreme left ventricular hypertrophy and a blunted blood pressure response to exercise are risk factors (Decker et al., 2009). Risk factors for SCD are extreme left ventricular hypertrophy on the electrocardiogram and a septal thickness over 190% of normal, with a sensitivity of 91% and a specificity of 78% (Ostman-Smith et al., 2005). "
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