Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients

El-Saiedi, Sonia A.; Seliem, Zeinab Salah; Esmail, Reem

doi:10.1017/s1047951113001030

Cited by 24 publications

(28 citation statements)

References 34 publications

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“…Six studies evaluated the predictive value of NSVT detected during ambulatory electrocardiographic (ECG) monitoring for SCD 12,15,19,20 or CVD 17,22 . Two found a significant relationship between NSVT and SCD 12,15 , with a further study reporting non-significance of NSVT but significance for inducible VT during an electrophysiology study (EPS) 19 .…”

Section: Non-sustained Ventricular Tachycardia (Nsvt)mentioning

confidence: 99%

“…Two found a significant relationship between NSVT and SCD 12,15 , with a further study reporting non-significance of NSVT but significance for inducible VT during an electrophysiology study (EPS) 19 . No studies reported a significant association of NSVT and CVD 17,22 .…”

Section: Non-sustained Ventricular Tachycardia (Nsvt)mentioning

confidence: 99%

“…A family history (FHx) of sudden cardiac death (SCD) was evaluated as a risk factor in seven studies 12,[17][18][19][20]22,27 . Only one study reported a statistically significant association with SCD (HR 10.6, 95%CI 1.2-90.2, p=0.03) 18 .…”

Section: Family History Of Sudden Cardiac Deathmentioning

confidence: 99%

“…Gender was investigated as a predictive factor for death in five studies 18,22,23,26,27 . The majority did not demonstrate a significant relationship, However, Lipshultz et al 27 reported an increased risk of CVD in female patients (HR 2.4, CI: 1.09-5.17, p=0.03).…”

Section: Gendermentioning

confidence: 99%

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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: Non-sustained Ventricular Tachycardia (Nsvt)mentioning

confidence: 99%

Section: Non-sustained Ventricular Tachycardia (Nsvt)mentioning

confidence: 99%

Section: Family History Of Sudden Cardiac Deathmentioning

confidence: 99%

Section: Gendermentioning

confidence: 99%

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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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“…Whereas the mean life expectancy of patients who survived into young adulthood does not markedly differ from that of the normal population 12 , sudden cardiac death is common in young adults with HCM, particularly athletes 13 , and some patients develop severe systolic dysfunction and heart failure. Less known is that neonatal forms of HCM rapidly evolve into systolic heart failure and death within the first year of life [14][15][16][17][18][19][20] . Some of these infants have homozygous or compound heterozygous frameshift MYBPC3 mutations 14,[16][17][18][19][20] , expected to result in low level or absence of mutant cMyBP-C.…”

mentioning

confidence: 99%

Mybpc3 gene therapy for neonatal cardiomyopathy enables long-term disease prevention in mice

et al. 2014

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Homozygous or compound heterozygous frameshift mutations in MYBPC3 encoding cardiac myosin-binding protein C (cMyBP-C) cause neonatal hypertrophic cardiomyopathy (HCM), which rapidly evolves into systolic heart failure and death within the first year of life. Here we show successful long-term Mybpc3 gene therapy in homozygous Mybpc3-targeted knock-in (KI) mice, which genetically mimic these human neonatal cardiomyopathies. A single systemic administration of adeno-associated virus (AAV9)-Mybpc3 in 1-day-old KI mice prevents the development of cardiac hypertrophy and dysfunction for the observation period of 34 weeks and increases Mybpc3 messenger RNA (mRNA) and cMyBP-C protein levels in a dose-dependent manner. Importantly, Mybpc3 gene therapy unexpectedly also suppresses accumulation of mutant mRNAs. This study reports the first successful long-term gene therapy of HCM with correction of both haploinsufficiency and production of poison peptides. In the absence of alternative treatment options except heart transplantation, gene therapy could become a realistic treatment option for severe neonatal HCM.

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Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters

et al. 2017

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Our aim was (1) to detect the presence of fibrosis by Cardiac magnetic resonance imaging (CMR) in the pediatric age group. (2) Correlate CMR findings with demographic data, LV function, and other echocardiographic parameters. We studied 40 pediatric patients diagnosed as HCM by echocardiography. All patients were subjected to clinical examination (in which the NYHA classification was determined for each patient), echocardiography, and CMR. CMR was done on a 1.5T Philips Achieva scanner in SSFP with delayed myocardial enhancement (DE-MRI). All demographic and functional parameters as well as pressure gradient across left ventricular outflow tract (LVOT) were correlated with the percentage of myocardial enhancement. We studied 13 female and 27 male patients from 45 days up to 18 years. The mean percentage of DE-MRI was 9.7 ± 9%. We found significant correlation between the NYHA classification and the pressure gradient across the LVOT (P = < 0.001) as well as the percentage of DE-MRI (P = 0.004). The percentage of DE-MRI showed positive correlation with LV myocardial mass index (P = 0.042). It didn't correlate with any other demographic or LV functional cardiac parameters. A good positive correlation was detected between the percentage of DE-MRI and the severity of pressure gradient across LVOT measured by echocardiography (r = 0.69 and P = <0.001). We found a significant correlation between the percentage of DE-MRI in children with HCM and the pressure gradient across LVOT, NYHA classification, and LV myocardial mass. This may help in the further management of those patients, planning for follow-up, and prognosis of the disease.

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Hypertrophic cardiomyopathy: prognostic factors and survival analysis in 128 Egyptian patients

Cited by 24 publications

References 34 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

Mybpc3 gene therapy for neonatal cardiomyopathy enables long-term disease prevention in mice

Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters

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