Andersons pediatric cardiology 1744

Treatments Aimed Primarily at Reducing Sudden Death Mortality in patients with heart failure is related either to terminal end-organ failure or sudden death Although the treatment options discussed in this chapter, until now, aim to maintain the patient's stability, there has been increasing interest in the use of therapies that aim primarily to reduce the risk of sudden death among patients with failing hearts As a result, implantation of a cardioverter-defibrillator is now widely recommended in the treatment of selected adults with chronic stable heart failure.58 Early studies with these devices suggested benefit in those patients who had suffered an aborted sudden death episode (secondary prevention),112–114 although subsequent randomized trials showed clear benefit in the primary prevention of sudden death in adults with a left ventricular ejection fraction less than 35%, and mild to moderate HFrEF (Fig 65.9).115 FIG 65.9 Estimates of death from any cause in the SCD-HeFT trial (From Bardy GH, Lee KL, Mark DBR, et al Amiodarone or an implantable cardioverter-defibrillator (ICD) for congestive heart failure N Engl J Med 2005;352:225–237.) A meta-analysis of 13 studies in pediatric dilated cardiomyopathy demonstrated a 27% incidence of sudden death in children who died of this disease (Table 65.1).116 One retrospective analysis showed increased risk for sudden death in children with idiopathic dilated cardiomyopathy who were younger and had more dilated and thinner-walled left ventricles at the time of diagnosis.117 Thus appropriate application of cardioverter-defibrillator therapy to children with heart failure could clearly save lives, although there are few data on their use in children Although not proven, possible indications include children with heart failure and the following: an aborted sudden death episode, severe symptomatic cardiomyopathy awaiting heart transplantation, and after heart transplantation with severe graft vasculopathy awaiting retransplantation.118,119 Many of the technical challenges described above for resynchronization therapy in children apply to the use of an implantable cardioverter-defibrillator Table 65.1 Sudden Cardiac Failure and Total Mortality in Pediatric Series of Dilated Cardiomyopathy 1985 24 1988 32 1990 23 12 17 11 63 53 48 33 36 43 37 15 30 63 25 44 Number Number of of Deaths Sudden Due to Deaths CHF 11 10 1991 25 18 72 12 60 80 17 1991 36 1991 81 12 30 33 37 59 42 25 20 33 64 11 19 1991 63 10 16 48 11 20 1994 19 1994 63 17 37 27 39 19 21 21 36 61 10 Mean 1-Year 5-Year Mortality FollowYear Patients Deaths Mortality Mortality (%) up (%) (%) (Months) Risk Factors for Mortality Risk Factors for Arrhythmia Severe MR Age >2 y Low EF, EFE, family history CT ratio >17%, EF 25 mm Hg Persistent CHF Undefined Age >2 years; no improvement No Yes No Yes Yes No No 1995 28 1998 62 Total 456 29 172 32 47 38 49 47 46 11 38 27 22 50 73 46 27% 28 126 73% Undefined EFE, RHF No No CHF, Congestive heart failure; CT, cardiothoracic ratio on chest roentgenogram; EF, ejection fraction; EFE, endocardial fibroelastosis; LVEDP, left ventricular end diastolic pressure; MR, mitral regurgitation; RHF, right heart failure Modified from Silka MJ, Szmuszkovicz JR Arrhythmias and sudden cardiac death in pediatric heart failure In: Shaddy RE, Wernovsky G, eds Pediatric Heart Failure Boca Raton, FL: Taylor & Francis; 2005:441  ... Modified from Silka MJ, Szmuszkovicz JR Arrhythmias and sudden cardiac death in pediatric heart failure In: Shaddy RE, Wernovsky G, eds Pediatric Heart Failure Boca Raton, FL: Taylor & Francis; 2005:441 ... resynchronization therapy in children apply to the use of an implantable cardioverter-defibrillator Table 65.1 Sudden Cardiac Failure and Total Mortality in Pediatric Series of Dilated Cardiomyopathy 1985 24 1988 32 1990 23 12 17 11 63 53 48 33 36 43 37...cardioverter-defibrillator (ICD) for congestive heart failure N Engl J Med 2005;352:225–237.) A meta-analysis of 13 studies in pediatric dilated cardiomyopathy demonstrated a 27% incidence of sudden death in children who died of this