Failure to Raise Blood Pressure During Exercise is a Poor Prognostic Sign in Patients With Hypertrophic Non-Obstructive Cardiomyopathy.

Isobe, Naoki; Toyama, Takuji; Taniguchi, Koichi; Kubota, Sachio; Suzuki, Tadashi; Nagaoka, Hideki; Adachi, Hitoshi; Naito, Shigeto; Hara, Hiroshi

doi:10.1253/circj.67.191

Cited by 8 publications

(4 citation statements)

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“…Hypertrophic cardiomyopathy has been known to be maledominant, and incidence differences by sex increase with age. 18,[23][24][25][26] Colan et al 18 used the Pediatric Cardiomyopathy Registry data in the United States, and compared infant (<1 y/o) with non-infant (⩾1 y/o) hypertrophic cardiomyopathy. They reported that there was a male predilection in non-infant hypertrophic cardiomyopathy compared to infant hypertrophic cardiomyopathy.…”

Section: Discussionmentioning

confidence: 99%

Patient characteristics and incidence of childhood hospitalisation due to hypertrophic cardiomyopathy in the United States of America 2001–2014

et al. 2019

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This study investigated patient characteristics in paediatric hospitalisations for hypertrophic cardiomyopathy. We used Nationwide Inpatient Sample, which is the largest all-payer inpatient database in the United States, yielding nationally representative estimates, from 2001 to 2014. ICD-9-CM diagnostic codes identified hospitalisations for patients with hypertrophic cardiomyopathy and <18 years. Outcomes included yearly rate of hospitalisation, death, admission via emergency department, and need for surgery. Predictors of interest were age groups (<1, 1–9, and ⩾10 y/o), sex, and race/ethnicity. Logistic regression modelled associations, adjusted by patient- and hospital-level variables. With 2302 weighted hospitalisations, hospitalisation rates were 0.22 per 100,000 children/year, with higher rates for <1 y/o (0.42) and ⩾10 y/o (0.31). Male-to-female ratios were more prominent in the oldest age group; 2.7:1 in ⩾10 y/o versus less than 1.7:1 for <10 y/o. In-hospital mortality was 1.5%, with highest mortality rates among the <1 y/o (6.3%). Children ⩾10 y/o had 5.59 times higher risk of admission from the emergency department than 1–9 y/o age group. Both ⩾10 and <1 y/o age groups had lower risk of surgical intervention compared to the 1–9 y/o group with odds ratio 0.56 and 0.26, respectively. Black children had higher risk of admission from the emergency department than White children with odds ratio 2.78. A relation between age group and sex was observed, with sex-based differences in prevalence and treatment of hypertrophic cardiomyopathy becoming more pronounced with age. Further studies are needed to clarify mechanisms behind age and racial disparity in hospitalisation, especially admission source.

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Section: Discussionmentioning

confidence: 99%

Patient characteristics and incidence of childhood hospitalisation due to hypertrophic cardiomyopathy in the United States of America 2001–2014

et al. 2019

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“…Exercise-induced hypotension is a well-recognized feature of HCM. [20][21][22] A prospective study has revealed that an abnormal BP response to treadmill-exercise testing is associated with an increased risk of sudden cardiac death in HCM, especially in young patients; 23 however, because treadmill-exercise testing has a risk of cardiac event in patients with HCM, we examined the BP response to daily life activities among HCM patients with and without symptoms. In the present study, the systolic BP in daily life was significantly lower in HCM, especially in the HCM patients with symptoms, than in the control group.…”

Section: Discussionmentioning

confidence: 99%

“…An abnormal BP response during exercise has been reported as one of the predictive factors of sudden cardiac death in HCM, [20][21][22] especially in young patients, 23 but sudden death also occurs during sedentary periods or mild activity. 5 Recently, ambulatory BP monitoring has been used to manage childhood hypertension, [24][25][26][27][28] with results showing that BPs in childhood are quite different for the various time periods (resting, at school, at home, and sleeping).…”

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confidence: 99%

Heart Rate Variability and Ambulatory Blood Pressure Monitoring in Young Patients With Hypertrophic Cardiomyopathy

Yanagi

Yoshinaga

Horigome

et al. 2004

Circ J

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he major causes of death of patients with hypertrophic cardiomyopathy (HCM) include sudden death, progressive heart failure, and HCM-related stroke associated with atrial fibrillation. Of these, sudden cardiac death is the most common in young patients. [1][2][3][4] Sudden death in HCM is associated with many factors: a family history of sudden death, the presence of an obstructive region, abnormal blood pressure (BP) response during exercise, increased left ventricular wall thickness, and the presence of an increased QT interval, ventricular arrhythmia, or myocardial bridging of the left anterior descending artery. 1,[3][4][5] A study on sudden death in patients with HCM and a mean age of 28 years demonstrated a circadian pattern over a 24-h period, with a prominent midmorning peak and a less striking early-evening peak. 5 The peak frequency of sudden death after awakening in the morning is similar to adult subjects with or without coronary artery disease. [6][7][8] Circulation Journal Vol.68, August 2004 The data regarding circadian variability suggest that the autonomic nervous system plays an important role in the genesis of sudden cardiac death, not only in coronary artery disease, 6,9,10 but also in HCM. [11][12][13][14][15][16] Many indices using 24-h Holter monitoring have been evaluated in HCM and those reports have described altered sympathetic and/or vagal activity; 2,5,[10][11][12][13][14][16][17][18][19] the results, however, are controversial, and the data for children are scant, despite the fact that sudden death primarily occurs in children and adolescents.An abnormal BP response during exercise has been reported as one of the predictive factors of sudden cardiac death in HCM, 20-22 especially in young patients, 23 but sudden death also occurs during sedentary periods or mild activity. 5 Recently, ambulatory BP monitoring has been used to manage childhood hypertension, [24][25][26][27][28] with results showing that BPs in childhood are quite different for the various time periods (resting, at school, at home, and sleeping). 26 These results suggest that the BP response to daily life activities should be investigated in children with HCM. Therefore, the purpose of this study was to determine the presence or absence of altered autonomic activity and abnormal BP response to daily life activities, and to predict sudden cardiac death in young patients with HCM. Methods SubjectsAll patients with a diagnosis of HCM made before 19 years of age, and who were being treated at the Depart- Background Sudden cardiac death commonly occurs in young patients with hypertrophic cardiomyopathy (HCM); however, their heart rate variability (HRV) and blood pressure (BP) response to daily life activities is not well known. Methods and Results HRV and ambulatory BP monitoring were performed in 20 patients (age range: 7-21 years) and 57 age-matched healthy volunteers (age range: 10-22 years). Time domain variables and spectral data were obtained at hourly intervals throughout the day. To determine the BP response to d...

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“…Studies have demonstrated that an abnormal blood pressure response has a very low positive predictive value of 15 %, but a high negative predictive value of 95 % in determining risk of SCD. 45,46 If a patient has a dynamic obstruction causing an abnormal response to exercise, the guidelines suggest re-assessing any abnormal blood pressure response after treatment to relieve the obstruction.…”

Section: Abnormal Blood Pressure Response To Exercisementioning

confidence: 99%

ICD Therapy for Primary Prevention in Hypertrophic Cardiomyopathy

Trivedi

Knight

2016

Arrhythmia &Amp; Electrophysiology Review

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HCM is a heterogeneous disease that is classically transmitted in an autosomal dominant fashion. HCM often arises from mutations in genes responsible for the formation of cardiac sarcomeres.Sarcomeric mutations, initially discovered in the 1980s, are found in up to 70 % of patients with a family history of HCM.11 The most common mutations are in the myosin heavy chain (MYH7 gene) and myosin binding protein C (MYBPC3 gene). 12 There is marked heterogeneity in penetrance, however, and a poor understanding of how the different genotypes manifest as HCM phenotypes. Currently, there is minimal evidence to support the use of genetic testing to identify patients who are at high risk of SCD. Small studies have found that patients with a more malignant inheritance of their mutations (i.e. homozygous mutations) as well as the presence of a MYH7 gene mutation have AbstractHypertrophic cardiomyopathy (HCM) is a common and heterogeneous disorder that increases an individual's risk of sudden cardiac death (SCD). This review article discusses the relevant factors that are involved in the challenge of preventing SCD in patients with HCM.The epidemiology of SCD in patients is reviewed as well as the structural and genetic basis behind ventricular arrhythmias in HCM. The primary prevention of SCD with implantable cardioverter-defibrillator (ICD) therapy is the cornerstone of modern treatment for individuals at high risk of SCD. The focus here is on the current and emerging predictors of SCD as well as risk stratification recommendations from both North American and European guidelines. Issues related to ICD implantation, such as programming, complications and inappropriate therapies, are discussed. The emerging role of the fully subcutaneous ICD and the data regarding its implantation are reviewed. KeywordsHypertrophic cardiomyopathy, sudden cardiac death, implantable cardioverter defibrillator, subcutaneous implantable cardioverter-defibrillator Disclosure: Dr Trivedi has no conflicts of interest to declare. Dr Knight has been a consultant to manufacturers of implantable defibrillators and participated in clinical trials sponsored

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Failure to Raise Blood Pressure During Exercise is a Poor Prognostic Sign in Patients With Hypertrophic Non-Obstructive Cardiomyopathy.

Cited by 8 publications

References 18 publications

Patient characteristics and incidence of childhood hospitalisation due to hypertrophic cardiomyopathy in the United States of America 2001–2014

Patient characteristics and incidence of childhood hospitalisation due to hypertrophic cardiomyopathy in the United States of America 2001–2014

Heart Rate Variability and Ambulatory Blood Pressure Monitoring in Young Patients With Hypertrophic Cardiomyopathy

ICD Therapy for Primary Prevention in Hypertrophic Cardiomyopathy

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