A cohort study of childhood hypertrophic cardiomyopathy

Östman‐Smith, Ingegerd; Wettrell, Göran; Riesenfeld, Tomas

doi:10.1016/s0735-1097(99)00421-0

Cited by 151 publications

(45 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some may improve with high-dose b blockers. 45 Surgical relief of the symptomatic person with obstructive cardiomyopathy may be successful and an occasional patient has undergone cardiac transplantation.…”

Section: The Heartmentioning

confidence: 99%

The cardiofaciocutaneous syndrome

Roberts

Allanson

Jadico

et al. 2006

Journal of Medical Genetics

229

228

View full text Add to dashboard Cite

Section: The Heartmentioning

confidence: 99%

The cardiofaciocutaneous syndrome

Roberts

Allanson

Jadico

et al. 2006

Journal of Medical Genetics

229

228

View full text Add to dashboard Cite

“…[1,8,9,12,13] Symptomatic improvement persists in about 70% needed, as treatment with a high dose of a β-blocker is often of patients for at least 5 years after the operation. [8,9,12,13] Reported required for the alleviation of symptoms, but may also have complications include perioperative ventricular septal defect, third prognostic merit in children; [6] thus β-blockers should be preferred degree AV block requiring pacemaker implantation, cerebral emas the first choice of treatment. The propranolol dose can start at bolism, and left bundle branch block apparent in almost 40% of 1.5-3 mg/kg/day, and be increased gradually to a minimum of 6 patients treated postoperatively.…”

Section: Medical Treatment In Obstructive Hcmmentioning

confidence: 99%

“…[14,15] Intraoperative transesophageal echocardiography given to children with bronchospasm or hypoglycemia. [6] provides exact measurement of the septal thickness after the Verapamil should be used with caution in children with LVOT resection, as well as the residual gradient, so as to guarantee obstruction, as it can possibly lead to peripheral vasodilatation and adequate excision and prevention of ventricular septal defect. [14] serious hemodynamic complications.…”

Section: Medical Treatment In Obstructive Hcmmentioning

confidence: 99%

Management of Hypertrophic Cardiomyopathy in Children

Seggewiß

Rigopoulos

2003

Pediatric Drugs

View full text Add to dashboard Cite

Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disease characterized by unexplained left ventricular hypertrophy, typically involving the interventricular septum. Hypertrophy may be present in infants, but commonly develops during childhood and adolescence. Management of children with HCM aims to provide symptomatic relief and prevention of sudden death, which is the primary cause of death. Unfortunately, no randomized comparative trials to date have assessed different treatment options in HCM. Medical treatment with negative inotropic agents (beta-adrenoceptor antagonists [beta-blockers], verapamil) is the first therapeutic choice in all symptomatic patients. Beta-blockers also appear to have prognostic merit in children. Surgical myectomy is effective in reducing symptoms in children with left ventricular (LV) obstruction who are unresponsive to medical treatment, although a repeat operation may be needed in a substantial proportion of patients due to relapse of LV obstruction. The recently introduced percutaneous septal ablation can also be regarded as a feasible alternative in this cohort. Technical limitations of both invasive therapeutic options should be carefully considered, preferably in experienced centers. Results of recent randomized trials indicate that dual chamber pacing, once considered a therapeutic option for patients with HCM, should only be used as treatment for conduction abnormalities. Regular clinical risk stratification for sudden death is of vital importance for the prevention of sudden death in young patients. Familial history of sudden death at a young age, LV hypertrophy >3 cm, unexplained syncope, nonsustained ventricular tachycardia in Holter monitoring, and abnormal blood pressure response during exercise are currently considered clinical risk factors for sudden death. Each factor has a low positive predictive accuracy, but patients having two or more of these risk factors are deemed as high risk. Secondary prevention of sudden death in patients successfully resuscitated from cardiac arrest and/or sustained ventricular tachycardia warrants treatment with an implantable cardioverter defibrillator (ICD). Primary prevention of sudden death in patients considered to be at high risk should aim at the management of obvious arrhythmogenic mechanisms (paroxysmal atrial fibrillation, sustained monomorphic ventricular tachycardia, conduction system disease, accessory pathway, myocardial ischemia), and the prevention and/or management of ventricular tachyarrhythmias with amiodarone and/or ICD implantation, respectively. The choice of treatment in children is greatly influenced by technical aspects, such as adverse effects of amiodarone, and ICD implantation difficulties or complications. Amiodarone could also be used as a bridge in children at high risk, until they reach adulthood, possibly achieving a lower risk status, or until their physical growth permits ICD implantation as long-term therapy.

show abstract

“…Reduced coronary vasodilator reserve (CVR) is one of the causes of these associations, even among patients with angiographically normal coronary arteries (2). Reduction in CVR leads to precipitation of myocardial ischemia and remortality rate has been reported in patients with these conditions (6,7,10). However, another study has reported that the mortality rate in HCM is not particularly high (11).…”

Section: Introductionmentioning

confidence: 99%

Different Coronary Blood Flow Increase in Left Ventricular Hypertrophy Due to Hypertension Compared to Hypertrophic Cardiomyopathy at Elevated Heart Rate

et al. 2003

View full text Add to dashboard Cite

Coronary vasodilator reserve (CVR) is reduced in patients with left ventricular hypertrophy (LVH). However,it is not clear whether there is any difference between the coronary blood flow increase in LVH caused by hypertension (HTH) and that caused by hypertrophic cardiomyopathy (HCM) when the heart rate increases.In this study, 16 subjects with HTH, 10 subjects with HCM, and 10 subjects with normotension (NT) were investigated. Average peak velocities at rest, at pacing, and at dilatation were measured using a Doppler catheter placed at the left descending coronary artery to calculate coronary blood flow (CBF) and CVR. CVR at rest was identical in the HTH and HCM groups, and in both cases was lower than the resting CVR in NT subjects. There were significant differences in the CVR values at a pacing rate of 120 beats/min among the groups. These values were lowest in HCM, highest in NT, and intermediary in HTH subjects. And the percent increase in CBF in HCM at that pacing rate was higher than that in HTH ( p 0.05) or NT ( p 0.05). There was no difference in the percent increase in CBF at this pacing rate between the HTH and NT groups. The effects of elevated heart rate on the percent increase in CBF were different between the HTH and HCM groups. We

show abstract

A cohort study of childhood hypertrophic cardiomyopathy

Abstract: High-dose beta-blocker therapy improves survival in childhood HCM.

Cited by 151 publications

References 27 publications

The cardiofaciocutaneous syndrome

The cardiofaciocutaneous syndrome

Management of Hypertrophic Cardiomyopathy in Children

Different Coronary Blood Flow Increase in Left Ventricular Hypertrophy Due to Hypertension Compared to Hypertrophic Cardiomyopathy at Elevated Heart Rate

Contact Info

Product

Resources

About