Aortic vasa vasorum in cyanotic congenital heart disease

Three cases of longstanding untreated cyanotic congenital heart disease are presented. In addition to collateral circulation from bronchial, intercostal, and inferior phrenic arteries, thoracic aortic vasa vasorum were visualized. We believe this may represent an additional collateral pathway to the lungs. The angiography of these cases is presented.     

Acute myocardial infarction secondary to polycythaemia in a case of cyanotic congenital heart disease

A case of coronary thrombosis resulting from secondary polycythaemia due to severe cyanotic congenital heart disease in a young girl of 19 years of age is described. This resulted in a fatal myocardial infarction. There was no evidence of coronary atheroma.     

艾司洛尔对紫绀型先天性心脏病患儿CPB过程中心肌损伤及能量代谢的影响

目的 探讨艾司洛尔对紫绀型先天性心脏病（CCHD）患儿体外循环（CPB）过程中心肌损伤的影响及机制.方法 将16例行CPB下心内直视术CCHD患儿随机分为观察组和对照组各8例,两组手术方法及麻醉方法均相同,但自麻醉诱导至术毕观察组和对照组分别静脉泵注艾司洛尔3 mg/（kg·h）及等量复方氯化钠溶液.观察两组手术一般情况,并分别于转流前（T1）、主动脉阻断后30 min（T2）、主动脉开放后30 min（T3）、术后24 h（T4）采集中心静脉血,测定肌酸激酶同工酶（CK-MB）、肌钙蛋白I（cTnI）水平,并于T1、T3取右心耳组织测定心肌组织腺甘酸含量及线粒体肿胀度（MSD）.结果 观察组复跳后心律失常发生率、多巴胺用量、ICU滞留时间及术后住院时间均显著小于对照组（P均〈0.05）;与T1时比较,两组T3和T4时血清CK-MB和cTnI水平明显升高、T3时心肌腺苷酸含量显著减少,MSD显著升高（P均〈0.01）,但观察组变化幅度均显著小于对照组（P〈0.05）.结论 艾司洛尔可通过改善心肌能量代谢及线粒体功能等途径减轻CCHD患儿CPB过程中缺血再灌注损伤.     

The haematological management of patients with cyanotic congenital heart disease: A time for consensus?

AIMS: Recurrent venesection of patients with cyanotic congenital heartdisease may be detrimental, with an increased risk of cerebrovascularevents and symptomatic iron-deficiency. The aim of this studywas to determine the venesection policies as practised in hospitalswithin a U.K. region and to determine if these policies followedcurrent recommendations. METHODS AND RESULTS: Fifty-eight consultants (56% response rate) in cardiac specialtiescompleted self-assessment questionnaires regarding the indicationsfor and practice of venesection. Sixty-one percent of thoseresponding were involved directly in the care of patients withcyanotic congenital heart disease and of these clinicians 97%used venesection. Indications for venesection varied, with 51%of those responding using an elevated haemoglobin per se (6·5–21·0g.dl^–1); 78% an elevated haematocrit (0·55–0·75)and 83% symptoms. Desired maintenance haemoglobin and haematocritlevels also varied greatly. Fifty percent of the consultantsresponding routinely screened their patients for iron deficiencyand 23% felt there was no indication for investigating a lowmean corpuscular volume. Only 18% of the policies describedfollowed any evidence based principles. CONCLUSIONS: The practice of venesecting patients with congenital cyanoticheart disease varies greatly. Policies in many hospitals donot reflect the minimal benefits and considerable risks associatedwith recurrent venesection.     

Selective coronary angiography via antegrade venous route in congenital cyanotic heart disease

An antegrade venous technique was utilised to perform selective coronary angiography in cyanotic infants and children. The procedure was successful in 88% (37/42) cases and excellent quality angiograms were recorded. The importance of proper catheter selection and details of the technique are discussed. © 1993 Wiley-Liss, Inc.     

Cyanotic congenital heart disease the coronary arterial circulation

Background: The coronary circulation in cyanotic congenital heart disease (CCHD) includes the extramural coronary arteries, basal coronary blood flow, flow reserve, the coronary microcirculation, and coronary atherogenesis. Methods: Coronary arteriograms were analyzed in 59 adults with CCHD. Dilated extramural coronaries were examined histologically in six patients. Basal coronary blood flow was determined with N-13 positron emission tomography in 14 patients and in 10 controls. Hyperemic flow was induced by intravenous dipyridamole pharmacologic stress. Immunostaining against SM alpha-actin permitted microcirculatory morphometric analysis. Non-fasting total cholesterols were retrieved in 279 patients divided into four groups: Group A---143 cyanotic unoperated, Group B---47 rendered acyanotic by reparative surgery, Group C---41 acyanotic unoperated, Group D---48 acyanotic before and after operation. Results: Extramural coronary arteries were mildly or moderately dilated to ectatic in 49/59 angiograms. Histologic examination disclosed loss of medial smooth muscle, increased medial collagen, and duplication of internal elastic lamina. Basal coronary flow was appreciably increased. Hyperemic flow was comparable to controls. Remodeling of the microcirculation was based upon coronary arteriolar length, volume and surface densities. Coronary atherosclerosis was absent in both the arteriograms and the necropsy specimens. Conclusions: Extramural coronary arteries in CCHD dilate in response to endothelial vasodilator substances supplemented by mural attenuation caused by medial abnormalities. Basal coronary flow was appreciably increased, but hyperemic flow was normal. Remodeling of the microcirculation was responsible for preservation of flow reserve. The coronaries were atheroma-free because of the salutory effects of hypocholesterolemia, hypoxemia, upregulated nitric oxide, low platelet counts, and hyperbilirubinrmia.     

A large coronary-pulmonary artery fistula in a cyanotic patient leading to severe biventricular dysfunction and heart failure

In cases of pulmonary atresia (PA) with ventricular septal defect (VSD), the commonest source of pulmonary blood flow is via major aortoplumonary collaterals. Collaterals may also arise from the coronary arteries and the reported prevalence of such coronary-pulmonary artery fistulas (CPAF) in PA with VSD is 10%. However gross congestive heart failure (CHF) and ventricular dysfunction is extremely rare in such cases. We report a 17-year-old male with PA with VSD and a large CPAF from the anterior right aortic sinus connecting to the left pulmonary artery, who presented with severe CHF. The left anterior descending and the right coronary artery both arose from the proximal part of the CPAF, possibly leading to extensive coronary steal and biventricular dysfunction.     

Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease

Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO2), oxygen pulse (O2 pulse), ventilatory equivalent for carbon dioxide (VE/CO2 at an‐ aerobic threshold), constant work‐rate endurance time (CWRET) at 75% of peak VO2, and 6‐minute walk distance (6MWD) were compared between baseline and after training by paired t test.
Result: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respec‐ tively). The mean difference of 6MWD change in the home‐program group was sig‐ nificantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training.
Conclusion: Once‐a‐week outpatient hospital‐based exercise program followed by supervised home‐based exercise program showed a significant benefit in improve‐ ment of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes.     

Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease

Background

Patients with cyanotic congenital heart disease (CCHD) have haemostatic abnormities associated with bleeding and thrombo-embolic events. The haemostatic abnormalities are not fully understood, but recent studies indicate that elevated haematocrit and fibrinogen function may be of importance.The aim of this study was to characterise the haemostatic profile and examine the potential role of haematocrit on clot formation and strength in CCHD patients. Furthermore to examine whether CCHD patients with history of haemoptysis have diminished fibrinogen function compared to those without haemoptysis.

Methods

In a prospective study 75 adult CCHD patients had haematocrit, platelet count, and plasma fibrinogen concentration examined. Furthermore thrombelastography(TEG) as well as TEG Functional Fibrinogen(TEG FF) assay evaluating fibrinogen function(FLEV) was performed. Data were compared with historical data regarding previous haemoptysis in CCHD patients.

Results

Haematocrit was 57 ± 8% and platelet counts in the lower normal range. TEG revealed a hypocoagulable condition with impaired clot formation. TEG values were correlated to haematocrit, indicating that elevated haematocrit causes impaired clot formation and strength. Despite high levels of plasma fibrinogen, TEG FF demonstrated that FLEV was diminished and negatively correlated to haematocrit. Furthermore CCHD patients with previous history of haemoptysis had significantly lower FLEV compared to CCHD patients without haemoptysis.

Conclusion

Patients with CCHD are hypocoagulable mainly due to impaired fibrinogen function. Despite a low platelet count, platelet function does not seem to be severely affected in CCHD patients. Haemostasis, and especially fibrinogen function, is negatively affected by elevated haematocrit, and fibrinogen function is diminished in CCHD patients with haemoptysis.     

The whole adult congenital heart disease patient

Adults with congenital heart disease (CHD) have unique medical and psychosocial needs. They require lifelong cardiac surveillance from medical providers with training and expertise in the care of adults with CHD. Patients with CHD must recognize the importance of ongoing surveillance and must not be lost to care in childhood, adolescence, or adulthood. This can be accomplished with the implementation of a comprehensive transition program with the collaboration of patients, parents, and both pediatric and adult health care providers. Finally, consideration of the “whole” patient demands recognition of the unique medical and psychosocial challenges of adults with CHD.     

亚临床甲状腺功能减退合并冠心病患者血脂及一氧化氮、C-反应蛋白水平分析

目的研究亚临床甲状腺功能减退（亚临床甲减）合并冠心病患者与单纯冠心病患者血脂、一氧化氮（NO）、C-反应蛋白（CRP）水平之间的差异。方法冠心病合并亚临床甲减（A组）及单纯冠心病（B组）患者各50例,均空腹抽血行血三酰甘油（TG）、总胆固醇（TC）、NO、超敏CRP检测,并比较2组各指标的差异。结果与B组患者相比,A组TG、TC、CRP均升高,NO降低,差异有统计学意义（P〈0.05或P〈0.01）。结论亚临床甲减不但影响冠心病患者脂质代谢,而且会增加冠心病患者血管内皮损伤及炎症反应,从而可能增加冠心病患者心血管病风险。     

临床综合干预措施对紫绀型先天性心脏病合并大体肺侧枝循环肺出血的预防效果观察

【摘要】 目的 探讨预防发绀型先天性心脏病合并大体肺侧支循环肺出血的有效干预措施。方法 选取2010年12月至2014年10月我院收治的发绀型先天性心脏病合并大体肺侧支循环患者50例为研究对象,根据临床实施的干预措施情况分为试验组（30例）和对照组（20例）。分别采用综合干预措施和常规干预措施。对两组患者实施干预措施后的肺出血率进行比较。结果 试验组患者的肺出血率3.33%显著低于对照组20%,组间比较差异具有统计学意义（χ2=7.0875,P=0.0078）。结论 综合干预措施可显著降低发绀型先天性心脏病合并大体肺侧支循环患者的肺出血发生率,是临床预防肺出血的理想方法之一。     

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.     

The effects of iron treatment on viscosity in children with cyanotic congenital heart disease

Objective: This study was planned to determine the effects of iron treatment in children with cyanotic congenital heart disease.

Method and Materials: A total of 39 patients with cyanotic congenital heart disease including 20 (51%) females, 19 (49%) males and whose mean age was 9.9?±?6.2 years, average weight was 33?±?18.4?kg were evaluated. Patients were categorized into two groups as having iron deficiency and no iron deficiency with respect to their ferritin levels. 4?mg/kg/day iron treatment with two valences was applied to the groups with iron deficiency for 3 months. Clinical and laboratory findings of both groups were assessed at the outset and 3 months later and viscosity measurements were carried out.

Results: Iron deficiency was identified in 21 (53.8%) out of 39 patients. Average Hb and Hct values following 3-month iron treatment increased from 14.8?±?2.4?g/dl to 16.0?±?2.0 (P?=?0.003) and from %45.8?±?7.5 to %47.6?±?7.2 (P?=?0.052), respectively. Average viscosity value, however, was 5.6?±?1.0?cP, it reduced to 5.5?±?1.0?cP value by demonstrating very little reduction (P?=?0.741). Nevertheless, O₂ sat value increased from 71.7 to 75% and complaints such as headache, visual blurriness, having frequent sinusitis decreased.

Conclusions: It was observed that iron treatment increased Hb and Hct levels in patients with cyanotic congenital heart disease without raising viscosity and it ensured improvement in clinical symptoms.     

复杂紫绀型先天性心脏病并发体肺侧支的内外科镶嵌治疗

目的:总结并发体肺侧支的复杂紫绀型先天性心脏病内外科镶嵌治疗的临床经验,探讨其治疗意义、操作技术及安全性。方法: 回顾性分析41例并发体肺侧支的复杂紫绀型先心病患者,年龄1～28（15±10）岁,体质量（18±8）kg。均行螺旋CT检查确诊,38例术前封堵侧支血管,3例术前漏诊术后封堵侧支血管。全组均行外科一期矫治手术。结果: 全组治愈37例。死亡4例:1例死于肺部感染,3例死于顽固性心力衰竭。侧支血管直径2.5～9.4（5.3±2.1） mm。每位患者放置弹簧圈3～21（10±6）枚。3例术后因侧支血管再通而二次封堵。术后4例并发肺部感染,3例肺水肿,2例灌注肺。结论: 并发体肺侧支的复杂紫绀型先心病,在围手术期应当内外科联合处理侧支血管的问题。可采用经皮介入封堵法,有效、简单、安全,可行多支、多次封堵,有效减少并发症的发生,降低手术死亡率。     

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.     

Bioelectrical impedance analysis in the management of heart failure in adult patients with congenital heart disease

Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and HF-related admission prevalence.
Results: Patients with New York Heart Association (NYHA) functional classes III-IV had a higher EI than those with NYHA classes I-II (mean ± SD, 0.398 ± 0.011 vs 0.384 ± 0.017, P < .001). EI was significantly correlated with brain natriuretic peptide level (r = 0.51, P < .001). During the mean follow-up period of 7.1 months, KaplanMeier analysis showed that a discharge EI > 0.386, the median value in the present study, was significantly associated with a future increased risk of HF-related admission (HR = 4.15, 95% CI = 1.70-11.58, P < .001). A body weight reduction during hospitalization was also related to EI reduction.
Conclusions: EI determined using BIA could be a useful marker for HF severity that could predict future HF-related admissions in adult patients with CHD.