伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗的临床探讨

目的 探讨伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗的可行性和效果.方法 回顾性分析7例伴有体肺侧支血管的发绀型先天性心脏病镶嵌治疗患者的临床资料.全组患者于手术当天在导管室行主动脉造影和选择性体肺侧支血管造影,根据情况选择合适的弹簧圈对体肺侧支血管进行栓塞,随后立即移至外科手术室行外科矫治手术.结果 7例患者共发现体肺侧支血管15支,除其中的1支使用ev3公司可控性弹簧圈2枚栓塞外,其余均使用COOK公司非可控弹簧圈共20枚达到完全栓塞,顺利完成外科矫治手术.未出现介入治疗并发症,未发生体肺侧支栓塞后严重低氧血症,无矫治术后脱离体外循环机困难,外科根治术后未出现灌注肺、低心排血量综合征等严重并发症.术后无需行补救性体肺侧支栓塞.结论 伴有体肺侧支血管的发绀型先天性心脏病患者镶嵌治疗是可行、有效的.     

发绀型先天性心脏病伴体肺动脉侧支循环的内外科联合治疗

目的 评价内、外科联合治疗对发绀型先天性心脏病伴体肺动脉侧支循环的临床效果.方法 回顾性分析27例经内、外科联合治疗的发绀型先天性心脏病伴体肺动脉侧支循环患者的临床资料,着重分析侧支血管栓堵方法 、外科手术方法 、治疗结果 及并发症.结果 24例存活,3例死亡,病死率11.1%(3/27),死因分别为心室颤动1例、低心排血量综合征1例、呼吸衰竭1例.66支侧支血管为完全阻断,11支血管为部分阻断.栓堵相关并发症:2例缺氧发作:3支侧支血管渗漏,放置弹簧圈后侧支循环均达完全阻断.结论 内、外科联合治疗伴有体肺动脉侧支循环的发绀型先天性心脏病是行之有效的方法.     

Extramural coronary arteries in adults with cyanotic congenital heart disease

Dilatation and tortuosity of extramural coronary arteries are prevalent in cyanotic congenital heart disease. Two pathogenetic variables are operative, namely endothelial vasodilator substances and medial structural abnormalities.     

Nitric oxide metabolism in adults with cyanotic congenital heart disease

Endothelial-derived nitric oxide (NO) diffuses abluminally to regulate blood flow by activating soluble guanylate cyclase in medial smooth muscle. However, a significant fraction of NO diffuses luminally, where the extremely high reaction rate with red blood cell hemoglobin (Hb) effectively reduces luminal concentration to zero. The erythrocytosis of cyanotic congenital heart disease has potentially opposing effects, namely, a reduction in medial smooth muscle NO bioavailability because of the increase in luminal consumption of the molecule and, conversely, an increase in the elaboration of NO in response to the high endothelial shear stress of the erythrocytotic perfusate. NO metabolism in cyanotic congenital heart disease is unknown. Accordingly, this study aimed to establish the metabolic fate of NO and to determine the degree to which its levels are altered. Blood samples from 25 nonfasting patients with cyanotic congenital heart disease and 25 nonfasting normal controls were collected in Vacutainer tubes containing citrate dextrose and in separate Vacutainer tubes containing a solution that specifically preserves S-nitrosated Hb. Total NO species, plasma S-nitrosated proteins, iron nitrosyl Hb, and S-nitrosated Hb were quantified using chemiluminescence. In conclusion, a significant increase in plasma concentrations of NO metabolites and a modest increase in iron nitrosyl Hb levels were found, suggesting increased luminal consumption caused by erythrocytosis and further suggesting that hypoxemia might activate nonoxidative NO metabolic pathways and enhance tissue oxygen delivery.     

Thyrotoxicosis increases right to left shunt in congenital cyanotic heart disease

We wish to report two cases of congenital cyanotic heart disease in whom coexisting thyrotoxicosis increased the right to left shunt. We review the literature on the cardiac and respiratory effects of thyroid hormone to try to explain this phenomenon. Two patients, one with Fallot's tetralogy (diagnosed at 68 yars of age), the other with a ventricular septal defect, developed thyrotoxicosis which resulted in a dramatic but reversible deterioration in their cardiac condition. We conclude that thyrotoxicosis increases right to left shunt in congenital cyanotic heart disease. This effect is profound but reversible. We suggest that when an unexplained rapid deterioration occurs in patients with congenital cyanotic heart disease, thyrotoxicosis should be excluded.     

有搏动性双向Glenn术治疗小婴儿复杂先心病的临床分析

目的 回顾性总结3岁以内复杂先心病患儿行有搏动性双向Glenn术的治疗效果,探讨双向Glenn分流术的手术时机.方法 收集2008年1月至2013年7月间3岁内接受双向Glenn术的77例患儿资料,根据年龄分为A组(小婴儿组,＜6个月,23例)和B组(婴幼儿组,6-36个月,54例),比较两组患儿围手术期及术后随访资料.结果 A、B两组患儿的手术时间[(148.20±21.21)min、(154.87±52.37)min]、呼吸机辅助时间[(30.63±23.86)h、(24.61±18.83)h]、ICU滞留时间[(6.35±4.16)d、(4.90±2.57)d]、术后住院时间[(12.11±3.68)d、(10.03±4.82)d]、血制品使用量[(401.21±276.79)ml、(435.76±226.95)ml]及术后引流量[(15.68±7.32)ml/kg、(17.78±8.43)ml/kg]比较差异均无统计学意义(P＞0.05).术后两组患儿肺动脉均较术前明显发育(P＜0.05),两组间发育程度比较差异无统计学意义(P＞0.05).18例患儿(A组6例,B组12例)后期接受了TCPC治疗.结论 对于无法行一期根治的复杂发绀型先心病患儿,即使是＜6个月的小婴儿,有搏动性双向Glenn术都是安全、有效的治疗方法.     

发绀型先天性心脏病改良体-肺动脉分流术中血流动力学变化

目的:使用压力记录分析法(PRAM)监测发绀型先天性心脏病患儿改良体-肺动脉(m BT)分流术中血流动力学的变化,分析其临床应用价值,并以此作为选择容量治疗或强心治疗的依据。方法:选择28例择期行m BT分流术的先天性心脏病患儿,主要诊断法洛四联症19例,肺动脉狭窄+室间隔缺损6例,肺动脉闭锁+室间隔缺损3例,月龄2~24个月,中位月龄10.6(6.2,18.9)个月,体质量(10.5±5.1)kg,Nakata指数平均(125±41)mm~2/m~2。在切皮前(T0)、切心包1 min(T1)、部分阻断肺动脉1 min和10 min(T2、T3)、部分阻主动脉后(T4)、人工血管开放通血1 min和10 min(T5、T6)、术毕(T7),记录循环周期效率(CCE)、心排血指数(CI)、心每搏指数(SVI)、收缩压(SBP)、重脉压与舒张压差值(Pdic-a)、压力升支最大斜率(dp/dt_(max))、体循环阻力指数(SVRI)、脉压变异度(PPV)。结果:各时间点dp/dt_(max)的平均值均>1.0 mm Hg/ms(1mm Hg=0.133k Pa)。人工血管通血前(T0-T4),T3点CCE、CI、SVI均处于最低点,其中CCE_(T3)明显低于CCE_(T0-T1)(P均<0.05),CI_(T3)明显低于CI_(T0-T2)(P均<0.01),SVI_(T3)明显低于SVI_(T0-T2)(P<0.01,P<0.01和P<0.05);Pdic-a_(T4)处于最低点,显著低于Pdic-a_(T0-1)(P<0.01和P<0.05);SVRI_(T3)处于最高点,明显高于SVRI_(T0)(P<0.01)。人工血管通血后(T5-T7),T5点CCE、CI、SVI、Pdic-a均处于最低点,其中CCE_(T5)明显低于CCE_(T7)(P<0.05),CI_(T5)明显低于CI_(T6-T7)(P均<0.05),SVI_(T5)明显低于SVI_(T6-T7)(P均<0.05),Pdic-aT5明显低于Pdic-aT6-7(P<0.01);PPVT5明显高于PPVT6-T7(P<0.01)。在T6和T7点,PPV与Pdic-a呈显著负相关(r=-0.51,r=-0.53,P均<0.01)。结论:m BT术中血流动力学维护重点,在人工血管通血前以强心为主,应用正性肌力药维护心功能,在人工血管通血后通过扩容维护有效循环血容量。通过CCE、CI、SVI、dp/dt_(max)、PPV、Pdic-a等血流动力学监测指标,精准判断液体治疗和应用正性肌力药治疗的权重,从而积极防治患儿术中低氧血症,稳定血流动力学状态。     

Use of self expanding stents in stenotic aortopulmonary shunts in adults with complex cyanotic heart disease.

OBJECTIVE: To describe the use of self expanding stents in treating long segment stenosis of aortopulmonary shunts (APS) in adults. DESIGN: Clinical records, catheterisation data, cineangiograms, and operation notes of four consecutive patients undergoing stent implantation since December 1994 were studied retrospectively. SETTING: A tertiary referral centre for cardiac disease. SUBJECTS: Four patients underwent cardiac catheterisation because of clinical deterioration. Their age ranged between 23 and 32 years. The underlying diagnosis was complex cyanotic heart disease in all. Three had a stenotic interposition graft, and one had a classic Blalock shunt. RESULTS: There was one technical failure owing to migration of the stent distal to an ostial stenosis. The ability index, resting oxygen saturation, and exercise tolerance improved in the remainder. Their medium term results have been excellent. CONCLUSIONS: This technique may further palliate adult patients with complex congenital heart disease, though the long term patency of stents is unknown.     

Dynamics of oxygen uptake during exercise in adults with cyanotic congenital heart disease

The dynamic increase in oxygen uptake (VO2) at the start of exercise reflects the circulatory adjustments to metabolic changes induced by the exercise. Because VO2 measured at the lungs is the product of pulmonary blood flow and arteriovenous oxygen difference, pathologic conditions affecting the capacity of these factors to change would be expected to alter VO2 kinetics. To determine whether measurement of VO2 kinetics can detect conditions in which the pulmonary blood flow response to exercise is abnormal, VO2 was measured, breath-by-breath, during the transition from rest to exercise in 13 adults with cyanotic congenital heart disease (central venoarterial shunting) and in nine normal subjects. The increase in VO2 above baseline during the first 20 sec of exercise (phase I), reflecting the immediate increase in pulmonary blood flow, was diminished in the patients compared with that in normal subjects (14.8 +/- 10.9 vs. 49.8 +/- 19.2 ml of oxygen) (p less than .001). The patients' phase I responses correlated with their reported physical activity tolerance (p less than .01). In addition, the second phase of the VO2 response kinetics was prolonged in patients compared with normal subjects (half-time = 63 +/- 13 vs 15 +/- 13 sec) (p less than .001). We conclude that striking disturbances in VO2 kinetics occur in patients with cyanotic congenital heart disease and that these measurements provide a useful noninvasive means of evaluating the degree to which the increase in pulmonary blood flow is constrained in response to exercise.     

Neurocognitive profiles in adolescents and young adults with congenital heart disease

Introduction and Objectives

The objectives of this study were to assess the neuropsychological performance (NP) of adolescents and young adults with congenital heart disease (CHD), comparing them with a group of healthy controls, to determine whether there are different neurocognitive phenotypes in CHD, and to identify their relation to sociodemographic, neonatal, clinical and psychological adjustment variables.