Arrhythmia and prognosis in infants, children and adolescents with hypertrophic cardiomyopathy

Abstract

In adults with hypertrophic cardiomyopathy, the annual mortality rate from sudden death is 2 to 3%, and the finding of nonsustained ventricular tachycardia during electrocardiographic (ECG) monitoring provides a marker of the patient who is at increased risk. In the young, the annual mortality rate from sudden death is even higher, approximately 6%, but the prognostic significance of arrhythmia is unknown. To determine the prevalence of arrhythmia and its relation to prognosis, 2 days of ECG monitoring was performed in 6 infants, 14 children and 33 adolescents with hypertrophic cardiomyopathy receiving no cardioactive medications. An additional 1 to 9 days (median 2) of monitoring was performed in 29 patients. All patients had sinus rhythm; 4 adolescents had episodes of paroxysmal supraventricular tachycardia, a child with the Wolff-Parkinson-White syndrome had symptomatic reentrant atrioventricular tachycardia and 5 adolescents had asymptomatic nonsustained ventricular tachycardia. During follow-up of 1 week to 7 years (median 3 years), five patients died suddenly and two had successful resuscitation from out-of-hospital ventricular fibrillation; none of these seven patients had ventricular arrhythmias during 2 to 7 days (median 3) of ECG monitoring. The two patients with ventricular fibrillation, the five with ventricular tachycardia, the one with Wolff-Parkinson-White syndrome and the seven with recurrent syncope or adverse family history, or both, received low dose amiodarone. None of these "high risk" patients died during 6 months to 6 years (median 3 years) of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Familial screening and genetic counselling in hypertrophic cardiomyopathy: the Rotterdam experience

“…Empiric use of amiodarone has been reported to be associated with improved survival in one observational study with historical controls. 40 Supported by other data we believe that implantation of a cardioverter-defibrillator is the preferred therapy for primary prevention in highrisk patients and for secondary prevention in patients who survived life-threatening arrhythmias. 41,42 Conclusions The identification of autosomal dominantly inherited genetic defects in an HCM gene provides new opportunities for accurate identification of at-risk relatives and effective screening of the relatives of HCM patients.…”

Complete Heart Block in Hypertrophic Cardiomyopathy: A Rare Association

“…1 A history of recurrent syncope episodes is one of the predictable risk factors for sudden cardiac death in younger patients. 3,4 It is important to know the mechanism causing loss of consciousness in patients with HCM because of the different management strategy, which can be explained by two underlying mechanisms: hemodynamic mechanism and arrhythmic complications. Among the arrhythmic causes for syncope, sustained ventricular tachycardia can be the cause of syncope and even sudden cardiac death in HCM.…”

National Russian Guidelines on Application of the Methods of Holter Monitoring in Clinical Practice

“…Снижение SDNN, триангулярного индекса и отношения средней дневной к средней ночной ЧСС (циркадный индекс) показали себя как предикторы плохого прогноза (в том числе ВСС) у детей с ДКМП [47,501,502], АВ блокадой 3 степени [503]. Риск ВСС при этих заболеваниях и каналопа-тиях значительно выше у больных детей, чем у взрос-лых, при этом внезапная смерть как первый симп том встречается у 9-15% пациентов [504,505].…”