Polderman Florens N, Cohen Joeri, Blom Nico A, Delhaas Tammo, Helbing Wim A, Lam Jan, Sobotka-Plojhar Marta A, Temmerman Arno M, Sreeram Narayanswani
Wilhelmina Children's Hospital, UMC Utrecht, Pediatric Cardiology, Utrecht, The Netherlands.
Int J Cardiol. 2004 Jun;95(2-3):171-6. doi: 10.1016/j.ijcard.2003.03.026.
It is known that children with previously diagnosed heart defects die suddenly. The causes of death are often unknown.
The aim of the study was to identify all infants and children within the Netherlands with previously diagnosed heart disease who had a sudden unexpected death (SUD), and to identify the possible cause of death.
Retrospective, cross-sectional study.
All children (<19 years) with a previously diagnosed heart defect and SUD between January 1990 and June 2001 in seven out of eight tertiary centres in the Netherlands were identified using the hospital databases. We excluded patients receiving compassionate care. Diagnoses, clinical status and circumstances of death were sought from case notes and post mortem reports. Deaths were classified as of cardiac or non-cardiac origin.
We identified 150 cases of SUD (89 male) at a median age of 2.3 years (range 18 days-18.9 years); 49/150 patients (33%) were </=1 year. Diagnostic categories included left to right shunts (N=34, 14 </=1 year), cyanotic lesions (N=38, 13 <==1 yar), cardiomyopathy (N=11, 2 </=1 year), univentricular heart (N=24, 10 </=1 year), obstructive lesions (N=11, 3 </=1 year), arrhythmia (N=8, 0 </=1 year), and miscellaneous defects (N=18, 5 </=1 year). 108/150 patients (72%) had been operated of whom 61 (57%) had corrective surgery. Of the infants with SUD, 32/49 (65%) had undergone surgery (11 corrective); 76/101 older children had undergone previous surgery (50 corrective). 114/150 children (76%) died of a cardiac cause. Causes of death were arryhythmia (59), heart failure (25), shunt occlusion (10), pulmonary hypertensive crisis (8) and acute myocardial infarction (4). 30/49 infants (61%) died of a cardiac cause; causes of death included arrythmia (10), heart failure (8), shunt occlusion (7), acute myocardial infarction (2).
SUD in children with heart defects were predominantly of cardiac origin. Pumpfailure and arrhythmias were the terminal events in a significant number of patients in the entire population.
已知先前被诊断患有心脏缺陷的儿童会突然死亡。死亡原因通常不明。
本研究的目的是确定荷兰境内所有先前被诊断患有心脏病且意外突然死亡（SUD）的婴幼儿及儿童，并确定可能的死亡原因。
回顾性横断面研究。
利用医院数据库，确定了1990年1月至2001年6月期间荷兰八家三级医疗中心中七家中心内所有先前被诊断患有心脏缺陷且发生SUD的儿童（小于19岁）。我们排除了接受临终关怀的患者。从病历和尸检报告中查找诊断、临床状况及死亡情况。死亡被分类为心脏源性或非心脏源性。
我们确定了150例SUD病例（89例男性），中位年龄为2.3岁（范围为18天至18.9岁）；49/150例患者（33%）年龄小于等于1岁。诊断类别包括左向右分流（N = 34，14例小于等于1岁）、青紫型病变（N = 38，13例小于等于1岁）、心肌病（N = 11，2例小于等于1岁）、单心室心脏（N = 24，10例小于等于1岁）、梗阻性病变（N = 11，3例小于等于1岁）、心律失常（N = 8，0例小于等于1岁）以及其他杂类缺陷（N = 18，5例小于等于1岁）。108/150例患者（72%）接受过手术，其中61例（57%）接受了矫正手术。在患有SUD的婴儿中，32/49例（65%）接受过手术（11例为矫正手术）；76/101例大龄儿童先前接受过手术（50例为矫正手术）。114/150例儿童（76%）死于心脏原因。死亡原因包括心律失常（59例）、心力衰竭（25例）、分流阻塞（10例）、肺动脉高压危象（8例）以及急性心肌梗死（4例）。30/49例婴儿（61%）死于心脏原因；死亡原因包括心律失常（10例）、心力衰竭（8例）、分流阻塞（7例）、急性心肌梗死（2例）。
患有心脏缺陷儿童的SUD主要源于心脏。在整个人口中，相当数量的患者最终发生泵衰竭和心律失常。
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