胎龄<28周早产儿动脉导管未闭的治疗进展

动脉导管未闭(PDA)是早产儿常见疾病,目前早产儿PDA的自然发展过程仍未完全明确,PDA发生的有些高危因素仍存在争议,对PDA是否进行药物、手术干预,以及何时进行药物、手术干预仍存在争议。尽管已经有相当多的证据证实动脉导管持续开放可能有害,但目前尚缺乏关闭导管治疗方案的远期益处或害处的相关证据。大多数临床试验旨在评估短期导管开放对患儿的影响。目前尚无评估动脉导管持续开放对早产儿死亡率及并发症影响的临床试验。近年来PDA治疗上最大的变化是减少对PDA的治疗。该文重点总结胎龄28周早产儿PDA的治疗策略。     

Anticipatory perioperative management for patent ductus arteriosus surgery: Understanding postligation cardiac syndrome

Ligation of a hemodynamically significant ductus arteriosus results in significant changes in loading conditions which have predictable consequences. Postligation cardiac syndrome, defined as hypotension requiring inotropic support and failure of oxygenation and ventilation, may occur 6‐12 hours following ligation due to left ventricular systolic and diastolic failure, respectively. Afterload is the primary driver of this decompensation. In this review, we describe the pathophysiological changes in loading conditions associated with postligation cardiac syndrome and other contributors to cardiovascular dysfunction following ductal ligation. We present strategies for perioperative optimization and a physiology‐based algorithm for postoperative management guided by targeted neonatal echocardiography. The use of these strategies to reduce the frequency of postligation deterioration may be an avenue to improve outcomes for neonates in this vulnerable patient population.     

超声心动图测定未闭动脉导管内径及临床意义

目的　探讨超声心动图测定未闭动脉导管内径的可靠性及其在导管法封堵动脉导管未闭症 (PDA)中的临床意义。　方法　 4 4例 PDA患者 ,男 18例 ,女 2 6例 ,平均 10 .8± 10 .5岁。应用二维超声心动图 (2 DE)及彩色多普勒血流显像 (CDFI)测定 PDA近端及最小内径 ,并与 X线造影测值及最终所选择的封堵器直径比较。　结果　 2 DE及 CDFI显示 PDA解剖全程及测量 PDA内径的成功率分别为 5 7%和 10 0 % ;2 DE对 PDA直径测量的均值显著低于 X线测值及封堵器实际尺寸 ,其回归方程斜率更偏离 1,截距较大 ,估计标准误高 ;CDFI对 PDA直径测量的平均值与 X线测值差异无显著性 ,但平均小于封堵器尺寸 2 .9mm,其回归方程斜率更接近 1,截距小及估计标准误小。　结论　与 2 DE相比 ,CDFI能更准确地测量 PDA直径 ;在导管封堵术中选择封堵器时 ,CDFI的 PDA直径测值可与 X线测量互相补充甚至可替代 X线造影 ,避免穿刺股动脉的技术困难及大量注射造影剂的副作用     

先天性心脏病动脉导管未闭介入治疗及血流动力学变化

目的观察动脉导管未闭经皮心导管封堵术前后右心血流动力学的变化,评价其临床意义。方法对50例动脉导管未闭儿童患者,在进行介入性经皮心导管封堵术治疗前应用X线降主动脉造影测量前,术后经导管检测右心血流动力学指标变化。结果未闭动脉导管最窄处直径(5．6±1．3)mm;术前肺动脉平均压(19．1±2．3)mmHg,左向右分流量平均为(0．41±0．13)l／min,血流分流量占肺循环血流量的比例平均为(0．17±0．03);术后肺动脉平均压(14．4±1．7)mmHg,左向右分流量平均为(0．03±0．01)l／min,血流分流量占肺循环血流量的比例平均为(0．02±0．01),相应数据比较,差异有统计学意义(P＜0．05)。结论动脉导管未闭经皮心导管封堵术前后血流动力学会发生改变,肺动脉平均压,左向右分流量以及分流量占肺循环血流量的比例均会不同程度下降,这种改变可以作为评价介入治疗效果的重要指标。     

Pulmonary effects of closure of patent ductus arteriosus in premature infants with severe respiratory distress syndrome

The pulmonary effects of closure of a patent ductus arteriosus (PDA) were investigated in 11 premature infants with severe respiratory distress syndrome. Successful closure of a PDA did not improve the short-term severity of pulmonary disease (24 and 48 h after treatment) as judged by the arterial/alveolar oxygen tension ratio or the amount of ventilatory support. Also, pulmonary mechanics measured 24 h before, 24 and 48 h after treatment, were not statistically different.Conclusion Infants with severe respiratory disease requiring high ventilation pressure and high oxygen concentration, where structural changes in the lungs are already established, will rarely show short-term improvement in pulmonary disease as a result of closure of a PDA. The overall clinical condition may, however, improve as a result of reduced cardiovascular strain. Earlier treatment of a PDA could reduce the ventilation period and the possible risk of developing chronic lung disease.     

Congenital esophageal atresia successfully treated by early ligation of a tracheoesophageal fistula and delayed repair of the esophagus in a premature infant: Report of a case

We report herein the case of a premature infant with esophageal atresia (EA) and a tracheoesophageal fistula (TEF) associated with cardiac anomalies who was successfully treated by an early ligation of the TEF following gastrostomy, and delayed repair of the esophagus. A 1212-g male was born prematurely at 31 gestational weeks, at which time he was diagnosed as having EA with TEF and patent ductus arteriosus (PDA), ventricular septal defect (VSD), and atrial septal defect (ASD). A gastrostomy was initially performed but following extubation he gradually became tachypneic. A chest roentogenogram revealed atelectasis and ground-glass appearance, and reintubation was required. Ligation of the TEF was performed 53h after his birth. Following the improvement of his respiratory condition through ventilatory support and the intratracheal administration of pulmonary surfactant, he underwent repair of the esophagus on the 6th day of life. Postoperatively, he suffered from heart failure, but was treated with peritoneal dialysis and pharmacological closure of the PDA. Weaning the infant from the ventilator proved difficult, but it was finally achieved when he had reached a weight of 2268g at 3 months of age by enteral feeding. Our experience of this case demonstrates that early ligation of TEF should be performed for a premature infant with EA and TEF before respiratory distress syndrome (RDS) has developed. If a gastrostomy is required to prevent gastric distention, it should be followed by simultaneous or immediate ligation of the TEF.     

Aneurysm of the ductus diverticulum in adults The diagnostic value of three-dimensional computed tomographic scanning

Ductus arteriosus aneurysm is rare in adults and preoperative diagnosis has not been usually done. We report 2 cases of adult type ductus arteriosus aneurysm. In both cases, 3D computed tomographic scanning showed a saccular aneurysm originating from the distal aortic arch toward the left pulmonary artery, which had a notching in the orifice of the aortic side. They were successfully treated surgically though one was a ruptured aneurysm to the left pulmonary artery. In these cases, 3D-CT scan was of great value in the preoperative diagnosis of the ductus arteriosus aneurysm.     

Morphometric analysis of the great vessels in early fetal life

Pathological examination of the heart and great vessels wasperformed in 61 specimens obtained after surgical terminationof pregnancy for psychosocial indications at 9–18 weeksof gestation. The aorta and pulmonary trunk were identifiedand external diameters were measured at the level of, and distalto the aortic valve and pulmonary valve, the level of the aorticisthmus and thoracic aorta, and the proximal and distal ductusarteriosus. All eight vessel diameters increased linearly withgestational age and the ratio of the diameter of the aorticisthmus to that of the aortic valve or the distal ductus arteriosusalso increased with gestation. Early pregnancy is characterizedby rapid growth of the fetal head and this may well be the consequenceof a preferential distribution of left ventricular output infavour of the head due to relative narrowing of the aortic isthmusat this gestation.     

Anaesthetic experience with paediatric interventional cardiology

Anaesthetic and sedation techniques, complications and outcomes were reviewed in 176 children undergoing 184 interventional cardiologic procedures. Techniques included sedation only, and ketamine, inhalational or narcotic anaesthesia. Ketamine infusion was the technique most frequently used. Ketamine was associated with a higher incidence of respiratory complications (P < 0.05) than the other techniques. The higher incidence of hypercarbia (15.6 per cent), which did not affect outcome, may be attributable to the use of supplemental sedatives. The incidence of upper airway obstruction (7.8 per cent) was similar to that of previous studies. Vascular compromise resulted from the procedure in 33 patients, necessitating surgical correction in 16. Cardiac perforation occurred in four cases, causing one death. Pulmonary valve stenosis was most amenable to balloon dilatation and aortic valve stenosis least amenable. Ketamine was the anaesthetic agent preferred by cardiologists for use in the catheterisation suite when general anaesthesia was required. Vigilant monitoring by anaesthetic staff is necessary during the procedure, and avoidance of concomitant narcotics is recommended if a ketamine technique with spontaneous ventilation is used. Les techniques anesthésiques et de sédation ainsi que les complications et les issues ont été revues chez 176 enfants subissant 184 procedures cardiaques. Les techniques ont inctu soil la sédation seulement, soit l’anesthésie à la kétamine, aux agents d’inhalation ou aux narcotiques. La perfusion de kétamine était la technique la plus fréquemment utilisée. La ketamine était associée à une plus grande incidence de complication respiratoire (P < 0.05) comparativement aux autres techniques. La plus grande incidence d’hypercarbie (15.6 pour cent), n’ayant pas affecté l’issue, pourrait être attribuée à l’utilisation additionnelle de sédatifs. L’incidence d’obstruction des voies aériennes supérieures (7.8 pour cent) était similaire aux études préalables. Un problème vasculaire suite à la procédure fut observé chez 33 patients dont 16 ont requis une correction chirurgicale. Une perforation cardiaque est survenue dans quatre cas provoquant le décès d’un seul patient. La sténose de la valve pulmonaire était la procédure la plus susceptible d’être dilatée et la sténose de la valve aortique la moins susceptible. La kétamine était l’agent anesthésique préféré par les cardiologistes lors des cathétérisations quand une anesthésie générate était requise. Une surveillance vigilante par une équipe anesthésique fut nécessaire durant la procedure. Il faut aussi éviter l’administration de narcotiques si la kétamine est administrée en respiration spontanée.

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Presented in part at the Canadian Anaesthetists’ Society annual meeting in Halifax, June 1988.     

Glibenclamide treatment in a Cantú syndrome patient with a pathogenic ABCC9 gain‐of‐function variant: Initial experience

Cantú syndrome (CS), characterized by hypertrichosis, distinctive facial features, and complex cardiovascular abnormalities, is caused by pathogenic variants in ABCC9 and KCNJ8 genes. These genes encode gain‐of‐function mutations in the regulatory (SUR2) and pore‐forming (Kir6.1) subunits of K_ATP channels, respectively, suggesting that channel‐blocking sulfonylureas could be a viable therapy. Here we report a neonate with CS, carrying a heterozygous ABCC9 variant (c.3347G>A, p.Arg1116His), born prematurely at 32 weeks gestation. Initial echocardiogram revealed a large patent ductus arteriosus (PDA), and high pulmonary pressures with enlarged right ventricle. He initially received surfactant and continuous positive airway pressure ventilation and was invasively ventilated for 4 weeks, until PDA ligation. After surgery, he still had ongoing bilevel positive airway pressure (BiPAP) requirement, but was subsequently weaned to nocturnal BiPAP. He was treated for pulmonary hypertension with Sildenafil, but failed to make further clinical improvement. A therapeutic glibenclamide trial was commenced in week 11 (initial dose of 0.05 mg^–1 kg^–1 day^–1 in two divided doses). After 1 week of treatment, he began to tolerate time off BiPAP when awake, and edema improved. Glibenclamide was well tolerated, and the dose was slowly increased to 0.15 mg^?1 kg^?1day^?1 over the next 12 weeks. Mild transient hypoglycemia was observed, but there was no cardiovascular dysfunction. Confirmation of therapeutic benefit will require studies of more CS patients but, based on this limited experience, consideration should be given to glibenclamide as CS therapy, although problems associated with prematurity, and complications of hypoglycemia, might limit outcome in critically ill neonates with CS.