Hausdorf G, Loebe M
Deutsches Herzzentrum Berlin, Germany.
Z Kardiol. 1994;83 Suppl 2:91-100.
A refractory low-cardiac-output syndrome is, in pediatric patients, most often due to impaired myocardial function after corrective surgery in congenital heart disease. Therapy has to focus on postoperative adaptation, which usually takes place within a few days. We report on three therapeutic strategies to "bridge" this phase of postoperative adaptation. Improving the contractile state of the myocardium using enoximone was attempted in 16 neonates with low-cardiac-output syndrome refractory to catecholamines (Dosage: loading-dose 1 mg/kg in 10 min intravenously, followed by an infusion of 10 mcg/kg/min). In 12/16 neonates cardiac index increased by more than 20% ("responder"), while in 4/16 neonates cardiac index remained unchanged ("non-responder"). All non-responders succumbed due to refractory low-cardiac-output syndrome, while only one responder died in low-cardiac-output syndrome. Hemodynamically, enoximone resulted in an increase of cardiac index and stroke volume (p < 0.003), a reduced systemic vascular resistance (p < 0.0022), and reduced right and left atrial pressures (p < 0.003). Heart rate and mean arterial pressure remained unchanged. No rhythm disturbances were observed. Another therapeutic approach to postoperative low-cardiac-output syndrome is atrial decompression by creating an atrial septal defect. Due to the possibility of later transcatheter closure of these defects, the acceptance for the intraoperative creation of an atrial communication to decompress the right or left atrium is increased. The defect size is critical and should be below 9 mm, so that the Rashkind-PDA-Occluder can be used for later transcatheter closure. We performed such a "surgical-interventional" decompression in 18 patients (age: 2 weeks to 7 years). In six patients the atrial defect was created because of an underdeveloped left ventricle (body weight 2.9-9.2 kg), in 12 patients for right atrial decompression during a total cavopulmonary shunt (body weight 15.2-54.2 kg). A spontaneous closure of the defect did not occurred in any of the patients, thus, transvenous closure of the defect was performed 2 to 10 weeks postoperatively. In the follow-up period of 4 to 22 months no complications such as thrombus formation, thromboembolic events or infections occurred. In low-cardiac-output syndrome refractory to all therapeutic measures mechanical circulatory support is the final method to keep the patient alive. In 11 children with refractory low-cardiac-output syndrome mechanical circulatory support was performed. In three of these, extracorporal membrane oxygenation (ECMO) was used, in eight children a ventricular assist device (Berlin Heart) was used.(ABSTRACT TRUNCATED AT 400 WORDS)
在儿科患者中，难治性低心排血量综合征最常见的原因是先天性心脏病矫正手术后心肌功能受损。治疗必须专注于术后适应，这通常在几天内发生。我们报告了三种治疗策略来“过渡”这一术后适应阶段。对16例对儿茶酚胺难治的低心排血量综合征新生儿尝试使用依诺昔酮改善心肌收缩状态（剂量：静脉内10分钟内负荷剂量1mg/kg，随后以10mcg/kg/min输注）。16例新生儿中有12例心脏指数增加超过20%（“反应者”），而4例新生儿心脏指数保持不变（“无反应者”）。所有无反应者均因难治性低心排血量综合征死亡，而只有1例反应者死于低心排血量综合征。血流动力学上，依诺昔酮导致心脏指数和每搏量增加（p<0.003），全身血管阻力降低（p<0.0022），左右心房压力降低（p<0.003）。心率和平均动脉压保持不变。未观察到心律失常。术后低心排血量综合征的另一种治疗方法是通过创建房间隔缺损进行心房减压。由于这些缺损后期有可能通过导管闭合，术中创建心房交通以减压右心房或左心房的接受度增加。缺损大小至关重要，应小于9mm，以便后期可使用拉什金德动脉导管未闭封堵器进行导管闭合。我们对18例患者（年龄：2周至7岁）进行了这种“外科-介入”减压。6例患者因左心室发育不全（体重2.9-9.2kg）创建房间隔缺损，12例患者在全腔静脉肺动脉分流术期间为右心房减压（体重15.2-54.2kg）。所有患者均未出现缺损自发闭合，因此术后2至10周进行了经静脉缺损闭合。在4至22个月的随访期内，未发生血栓形成、血栓栓塞事件或感染等并发症。对于所有治疗措施均难治的低心排血量综合征，机械循环支持是维持患者生命的最终方法。对11例难治性低心排血量综合征儿童进行了机械循环支持。其中3例使用了体外膜肺氧合（ECMO），8例儿童使用了心室辅助装置（柏林心脏）。（摘要截断于400字）
