Morrow W R, Naftel D, Chinnock R, Canter C, Boucek M, Zales V, McGiffin D C, Kirklin J K
University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
J Heart Lung Transplant. 1997 Dec;16(12):1255-66.
The major limiting factor to successful heart transplantation in infants is the limited supply of donors. To examine the impact of donor limitations on survival after listing, a multiinstitutional study was designed to identify risk factors for death while waiting and for longer interval to transplantation.
Between January 1 and December 31, 1993, 118 infants 6 months of age or younger (86 younger than 29 days) were listed for heart transplantation from 21 institutions. The primary diagnosis was hypoplastic left-sided heart syndrome (HLHS) in 70 (59%), other congenital defects in 32 (27%), cardiomyopathy or myocarditis in 13 (11%), and other diagnoses in 3. Among the 48 patients without HLHS, 32 (67%) required inotropic, mechanical, or prostaglandin support, whereas 16 (33%) did not.
At 6 months after listing, only 6% remained on the list awaiting transplantation, 59% underwent transplantation. 31% died while waiting, and 4% were removed from the list. The greatest mortality rate before transplantation was among patients with HLHS in whom the actuarial mortality rate if they were unable to receive a transplant was 77% at 6 months, compared with 52% in patients without HLHS and without inotropic or mechanical support (p = 0.05). By multivariable analysis, risk factors for death while waiting included inotropic support (p = 0.02), smaller size (p = 0.0007), and blood type O (p = 0.003). Surgical procedures before listing did not significantly influence pretransplantation mortality rates. The interval from listing to transplantation increased with young age (p = 0.01) in patients without HLHS and smaller size (p = 0.001) and blood group O (p = 0.0006) for patients with HLHS. The effect of blood type O on mortality rates and longer interval to transplantation was due to the distribution of type O donor hearts to non-type O recipients. Palliative operations after listing did not favorably influence survival; nine patients underwent first-stage Norwood while waiting, and six died before transplantation.
The mortality rate is unacceptably high among infants awaiting transplantation, particularly in patients with HLHS. Infants receiving intravenous inotropes or mechanical support at listing are at high risk of early death while waiting. The distribution of blood group O donors to non-blood group O recipients results in higher mortality rates among blood group O recipients. Greater emphasis should be placed on medical strategies to improve survival while waiting and on expanding existing graft resources.
婴儿心脏移植成功的主要限制因素是供体供应有限。为了研究供体限制对列入名单后生存率的影响，设计了一项多机构研究，以确定等待期间死亡和移植间隔时间延长的风险因素。
1993年1月1日至12月31日期间，来自21个机构的118名6个月及以下（86名小于29天）的婴儿被列入心脏移植名单。主要诊断为左心发育不全综合征（HLHS）70例（59%），其他先天性缺陷32例（27%），心肌病或心肌炎13例（11%），其他诊断3例。在48例非HLHS患者中，32例（67%）需要使用正性肌力药物、机械支持或前列腺素支持，而16例（33%）不需要。
列入名单6个月后，只有6%的患者仍在等待移植，59%的患者接受了移植。31%的患者在等待期间死亡，4%的患者被从名单中移除。移植前死亡率最高的是HLHS患者，如果他们无法接受移植，6个月时的精算死亡率为77%，而没有HLHS且没有正性肌力药物或机械支持的患者为52%（p = 0.05）。通过多变量分析，等待期间死亡的风险因素包括正性肌力药物支持（p = 0.02）、体型较小（p = 0.0007）和O型血（p = 0.003）。列入名单前的手术操作对移植前死亡率没有显著影响。对于非HLHS患者，从列入名单到移植的间隔时间随年龄减小而增加（p = 0.01），对于HLHS患者，随体型较小（p = 0.001）和O型血（p = 0.0006）而增加。O型血对死亡率和移植间隔时间延长的影响是由于O型供体心脏分配给非O型受体。列入名单后的姑息性手术对生存率没有有利影响；9名患者在等待期间接受了一期诺伍德手术，6名患者在移植前死亡。
等待移植的婴儿死亡率高得令人无法接受，尤其是HLHS患者。列入名单时接受静脉正性肌力药物或机械支持的婴儿在等待期间有早期死亡的高风险。O型供体向非O型受体的分配导致O型受体的死亡率更高。应更加重视改善等待期间生存率的医疗策略以及扩大现有的移植物资源。
