Changes in plasma free and sulfoconjugated dopamine in patients with congenital heart disease who underwent cardiac operation

It has been suggested that plasma sulfoconjugated dopamine (DA) may serve as a source or reservoir for free DA in plasma. Moreover, it has also been reported that the plasma levels of conjugated DA may be used as an index predicting heart failure in patients with heart disease. Therefore, in the present study, we have measured the plasma levels of free and sulfoconjugated DA in patients with congenital heart disease who underwent total corrective operations. The patients were divided into two groups with (6 patients with tetralogy of Fallot, TOF) or without (5 patients with ventricular septal defect without pulmonary hypertension, VSD) cyanosis (mean age of 2.11 years). Blood samples were collected before and after operation from the patients, and plasma free and sulfoconjugated DA levels were measured using high performance liquid chromatography. Preoperative levels of free DA in patients in both groups were higher than the level in age matched control subjects. The plasma level of conjugated DA in TOF was higher than that in the controls and was the highest in VSD before operation. DA infusion early after operation caused a rise in plasma free and conjugated DA, however, the levels of increased free DA were lower in the VSD than in the TOF group. After discontinuing DA infusion, the plasma levels of free DA remained higher, while those of conjugated DA decreased to a level lower than the preoperative values in both groups. As the plasma levels of free and sulfoconjugated DA vary with hemodynamics, it was assumed that the difference in the plasma sulfoconjugated DA level between the groups before operation was due to the influence of pulminary blood flow on catecholamine homeostasis. Since the decrease in conjugated DA has been postulated to be an index of sustained heart failure, it is conceivable that it takes a long time for patients who underwent cardiac operations in infancy to recover from heart failure.     

右腋下小切口心内直视手术224例

目的总结右腋下小切口径路手术治疗先天性心脏病的经验,并探讨其相关的问题。方法224例先天性心脏病患者采用右腋下小切口径路经第3或第4肋间进胸,在体外循环下行心内直视术,其中室间隔缺损(VSD)修补术168例,房间隔缺损(ASD)修补术48例,法洛四联症(TOF)根治术6例,右心室双出口(DORV)和埃布斯坦综合征(Ebsteinsyndrome)矫治术各1例。结果全组术后死亡1例(0.45%),死于急性肺水肿。发生并发症13例(5.8%)。右腋下小切口径路手术患者的体外循环时间、主动脉阻断时间、呼吸机辅助呼吸时间和术后住院天数与同期胸骨正中切口径路手术患者比较差别无统计学意义(P>0.05),但术中出血量和术后胸腔引流量较胸骨正中切口径路少(P<0.01)。术后随访214例,随访时间2个月~7年,除3例患者术后早期心功能稍差(射血分数<0.50)和2例VSD患者术后发生轻度残余漏外,其余患者均恢复良好。结论对具有该术式适应证的患者经右腋下小切口径路行心内直视手术,有安全可靠、术中出血少和美观等优点,但对TOF以及复杂先心病患者慎用本术式。     

Anterior chest wall deformities and congenital heart disease

Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.     

Tetralogy of fallot: a surgical perspective

Tetralogy of Fallot (TOF) is an index lesion for all paediatric and congenital heart surgeons. In designing an appropriate operation for children with TOF, the predicted postoperative physiology must be taken into account, both for the short and long term. A favourable balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of biventricular function. A unified repair strategy to limit both residual PS and PI is presented, along with supportive experimental evidence. A strategy for dealing with coronary anomalies and some comments regarding best timing of operation are also included.     

Tetralogy of Fallot Cardiac Function Evaluation and Intelligent Diagnosis Based on Dual‐Source Computed Tomography Cardiac Images

Tetralogy of Fallot (TOF) is the most common complex congenital heart disease (CHD) of the cyanotic type. Studies on ventricular functions have received an increasing amount of attention as the development of diagnosis and treatment technology for CHD continues to advance. Reasonable options for imaging examination and accurate assessment of preoperative and postoperative left ventricular functions of TOF patients are important in improving the cure rate of TOF radical operation, therapeutic evaluation, and judgment prognosis. Therefore, with the aid of dual‐source computed tomography (DSCT), cardiac images with high temporal resolution and high definition, we measured the left ventricular time–volume curve using image data and calculating the left ventricular function parameters to conduct the preliminary evaluation on TOF patients. To comprehensively evaluate the cardiac function, the segmental ventricular wall function parameters were measured, and the measurement results were mapped to a bull's eye diagram to realize the standardization of segmental ventricular wall function evaluation. Finally, we introduced a new clustering method based on auto‐regression model parameters and combined this method with Euclidean distance measurements to establish an intelligent diagnosis of TOF. The results of this experiment show that the TOF evaluation and the intelligent diagnostic methods proposed in this article are feasible.     

Surgical correction of tetralogy of Fallot in a 61-year-old patient

Tetralogy of Fallot (TOF) is a congenital heart disease that is usually diagnosed and treated during infancy. Only 3% of such patients reach the age of 40 without surgery. We describe a 61-year-old woman with uncorrected TOF that was successfully treated by radical surgery. The patient presented with exertional dyspnea, insomnia, and malaise. Echocardiography and cardiac catheterization indicated a dilated, severely hypertrophic right ventricle, ventricular septal defect, an overriding aorta, and infundibular stenosis in the right ventricular outflow tract. All symptoms disappeared after full surgical correction, which remains the preferred treatment for adult TOF because it confers long-term survival and an improved quality of life.     

Perioperative cardiovascular complications in patients with hypertrophic cardiomyopathy

BACKGROUND: The aim of this study was to examine the incidence of cardiac complications in patients with hypertrophic cardiomyopathy (HCM) during noncardiac surgery. METHODS: A retrospective study was made for surgical patients in the period of 1989-2000 at Kitasato University Hospital. RESULTS: Thirty out of 66000 patients were preoperatively diagnosed as HCM. Sixty percent of the HCM patients had one or more perioperative cardiovascular complications. There were perioperative congestive heart failure in 3 patients (10%), and myocardial ischemia in 4 patients (13%). However, there were no myocardial infarction, no life-threatening dysarrthythmia and no cardiac death. Factors which appeared to be associated with the perioperative cardiovascular complications were the type of HCM (HOCM), major surgery, general anesthesia and preoperative medication with a beta-blocker or a calcium channel blocker. CONCLUSIONS: It is suggested that patients with HCM undergoing noncardiac surgery have a high incidence of cardiac complications such as congestive heart failure and myocardial ischemia.     

体肺分流术在治疗肺血减少型先天性心脏病中的应用评价

目的分析体肺分流术在肺血减少型先天性心脏病治疗中的应用效果。方法对2005年4月至2009年2月我院20例肺血减少型心脏病患者行体肺分流手术,其中Waterston术（升主动脉吻合至右肺动脉）12例,中心分流术8例。结果围术期死亡1例,术后半年心功能衰竭1例,余病例元远期死亡。结论对肺动脉发育不良的患者,体肺分流术可减轻患者的缺氧症状,改善患者的心功能,促进肺血管的发育,并为进一步的手术创造条件。     

低浓度一氧化氮吸入筛选重度肺动脉高压根治手术适应证

目的　以一氧化氮吸入作为评估先天性心脏病 (先心病 )重度肺动脉高压病人肺血管扩张能力的方法 ,寻找筛选肺动脉高压手术适应证的最佳标准。方法　选择先心病重度肺动脉高压病人 5 3例 ,分别行右心导管检查 ,测定每例在基础状态下 ,吸入纯氧或一氧化氮各时点血流动力学参数 ,并进行统计学分析。结果　 5 3例吸入一氧化氮、纯氧与基础状态下相比较 ,肺动脉收缩压 ,舒张压及平均压两两之间均存在明显差异 (P <0 0 5 ) ;主动脉收缩压 ,舒张压及平均压两两之间均无明显差异 (P >0 0 5 ) ;肺循环阻力与体循环阻力之比在吸入一氧化氮及吸氧后均明显降低 (P <0 0 5 ) ,而吸入一氧化氮和吸氧之间无明显差异 (P >0 0 5 )。 36例采取手术治疗。结论　术前吸入一氧化氮肺循环阻力与体循环阻力之比下降与否与手术疗效密切相关。     

Anatomically Corrected Malposition of the Great Arteries with Tubular Hypoplasia of the Aortic Arch: A Case Report of Surgical Correction

Abstract Anatomically corrected malposition of the great arteries (ACMGA) is rare congenital cardiac anomaly. In a heart with ACMGA, both great arteries arise from the appropriate ventricle but they exit parallel to each other. The pulmonary trunk is located posterior and to the right of the aorta. ACMGA associated with tubular hypoplasia of the aortic arch is an extremely rare cardiac malformation and it has previously been reported in only four patients. In addition, successful surgical repair for this combination of anomalies was achieved in only one patient. We describe clinical and morphologic features in a neonate with this complex anomaly. The patient successfully underwent the staged operation . (J Card Surg 2010;25:412‐415)     

Cost-utility analysis of salvage cardiac extracorporeal membrane oxygenation in children

BACKGROUND: Indications for extracorporeal membrane oxygenation therapy have expanded to include cardiopulmonary arrest and support after congenital heart surgery. Data from a national registry have reported that cardiac patients have the poorest survival of all extracorporeal membrane oxygenation recipients. Concerns have been raised about the appropriateness of such an aggressive strategy, especially in light of the high costs and potential for long-term neurologic disability. We reviewed our experience with salvage cardiac extracorporeal membrane oxygenation to determine the cost-utility, which accounts for both costs and quality of life. METHODS: Medical records of patients with congenital heart disease receiving salvage cardiac extracorporeal membrane oxygenation between January 2000 and May 2004 were reviewed. Charges for all medical care after the institution of extracorporeal membrane oxygenation were determined and converted to costs by published standards. The quality-of-life status of survivors was determined with the Health Utilities Index Mark II. RESULTS: Salvage cardiac extracorporeal membrane oxygenation was instituted in 32 patients (18 for cardiopulmonary arrest and 14 for cardiac failure after heart surgery) at a median age of 2.0 months (range, 4 days to 5.1 years). Congenital heart disease was present in 27 (84%). The mean duration of extracorporeal membrane oxygenation support was 5.1 +/- 4.1 days. Survival to hospital discharge was 50%, including 1 patient bridged to heart transplantation. Survival to 1 year was 47%. The mean score of the Health Utilities Index for the survivors was 0.75 +/- 0.19 (range, 0.41-1.0). The median cost for hospital stay after the institution of extracorporeal membrane oxygenation was USD 156,324 per patient. The calculated cost-utility for salvage extracorporeal membrane oxygenation in this population was USD 24,386 per quality-adjusted life-year saved, which would be considered within the range of accepted cost-efficacy (< USD 50,000 per quality-adjusted life-year saved). CONCLUSIONS: Salvage cardiac extracorporeal membrane oxygenation results in reasonable survival and is justified on a cost-utility basis.     

Early diagnosis in hypertrophic cardiomyopathy--case report

Disproportionate heart muscle enlargement compared with little or no chamber enlargement are characteristic for hypertrophic cardiomyopathy (HCM). The clinical course of HCM is highly variable. Many patients are asymptomatic or mildly symptomatic and may be relatives of patients with known disease. Unfortunately, the first clinical manifestation of the disease may be sudden death, frequently occurring in children and young adults, often during or after physical exertion. We present 2 cases of HCM who emphasize the variability of clinical and ECG modifications and underline the importance of routine echocardiography in patients with atypical cardiac symptoms in order to identify the disease and prevent high risk for sudden death.     

Extracorporeal membrane oxygenation for the circulatory support of children after repair of congenital heart disease

We have treated 39 infants and children with congenital heart disease with extracorporeal membrane oxygenation during the past 5 years. Thirty-six were treated for low cardiac output or pulmonary vasoreactive crisis after repair of congenital heart defects. Twenty-two (61%) survived. Most patients were cannulated from the neck via the right internal jugular vein and the right common carotid artery. Six patients were cannulated from the chest, including three who had separate drainage of the left side of the heart with a left atrial cannula. Two of these patients survived and were the only survivors of the nine patients cannulated in the operating room because they could not be weaned from cardiopulmonary bypass after open cardiac operations. We also reviewed 312 patients (the predictor study series) having open cardiac operations before the availability of extracorporeal membrane oxygenation; 27 of these patients died. Data were collected at 1 and 8 hours postoperatively to determine if any parameters might predict early mortality. With these parameters used as criteria, patients who went on extracorporeal membrane oxygenation were as sick as those who died before extracorporeal membrane oxygenation was available. The most common complication was bleeding related to heparinization. The mean transfusion requirement in survivors was 1.50 +/- 1.13 ml/kg/hr, 5.63 +/- 7.0 ml/kg/hr in the nonsurvivors, and 7.46 +/- 8.29 ml/kg/hr in those cannulated in the operating room because they could not be weaned from bypass. Four children had intracranial hemorrhage, and two of them died. There was one late death. Nine of the 22 survivors are entirely normal. All survivors who do not have Down's syndrome are considered to have normal central nervous system function. We conclude that extracorporeal membrane oxygenation can improve survival in patients with both pulmonary artery hypertension and low cardiac output after operations for congenital heart disease.     

胸部不同切口治疗小儿常见先天性心脏病的比较

目的 比较胸部不同切口治疗小儿常见先天性心脏病的临床结果.方法 回顾分析1999年1月至2001年12月采用胸部不同切口治疗小儿常见先天性心脏病1 669例,并根据胸部不同切口分成正中胸骨切口组(Med组),胸骨下段小切口组(Pt组)和胸部右侧切口组(Rat组),其中行房间隔缺损(ASD)修补术245例, 室间隔缺损(VSD)修补术1 005例,法洛四联症(TOF)根治术419例.结果 ASD修补术中,Rat组肺部并发症的发生率高于Med组和Pt组(P<0.05);VSD修补术中,Rat组术前肺动脉高压的比率低于 Med 组和Pt组(P<0.05),而术后肺部并发症的发生率则高于Med组和Pt组(P<0.05);TOF根治术中,Med组术前血红蛋白浓度、动脉血氧饱和度分别高于及低于Rat组和Pt组(P<0.05),术中跨肺动脉瓣环补片比率却高于Rat组和Pt组(P<0.05).结论 手术切口的选择应当保证心内畸形的矫正,最大限度地减少手术并发症,保证患者的安全.     

Magnetic resonance imaging findings of persistent primitive hepatic venous plexus with complex congenital cardiac disease.

Persistence of the primitive hepatic venous plexus (PPHVP) is an uncommon anomaly that can present associated with complex congenital heart disease and inferior vena cava (IVC) development anomalies and complicate endovascular and surgical procedures. We report a case of PPHVP in a patient with complex congenital heart disease with unexplained persistent arterial desaturation after Kawashima operation and describe magnetic resonance angiography (Angio-MRI) findings leading to the diagnosis. We recommend that IVC-Angio-MRI be performed in preoperative evaluation of patients with complex congenital heart disease and left isomerism when redirection of systemic venous blood to the pulmonary arterial circulation is considered.     

Anaesthetic management of an adolescent for scoliosis surgery with a Fontan circulation

Advances in the treatment of congenital heart disease have led to a new group of adolescents or adults patients with cardiac anomalies. The anaesthetic management of these patients can be challenging especially when they are scheduled for major noncardiac surgery inducing haemodynamic instability. We report the case of a 14-year-old boy scheduled for posterior spinal fusion for idiopathic scoliosis who underwent a Fontan operation in infancy for pulmonary atresia with right ventricle hypoplasia. The preoperative investigations and the anaesthetic management are described.     

Combined spinal epidural anesthesia with epidural volume extension technique for hysterectomy in patient with unpalliated cyanotic heart disease--a case-report

Adults with unrepaired cyanotic heart disease scheduled for a non-cardiac surgery present serious concerns for the anesthesiologists. Both general and regional anesthetic techniques have been safely reported in Eisenmenger's syndrome and adults with Tetralogy of Fallot (TOF) for non-cardiac surgery. The safety of low-dose sequential combined spinal epidural (CSE) technique in high-risk cardiac cases has recently been emphasized in obstetric anesthetic practice but to our knowledge, this technique has not been reported for hysterectomy in cyanotic heart disease patients in anesthetic literature. We report the successful management of hysterectomy in a patient with ventricular septal defect and pulmonary atresia (VSD-PA) using CSE anesthesia with epidural volume extension (EVE) technique.     

双调转术治疗矫正型大动脉转位合并心内畸形

目的总结双调转手术治疗矫正型大动脉转位（cTGA）合并心内畸形的经验。方法2002年4月至2004年6月，行双调转手术治疗cTGA合并心内畸形病儿7例，其中男6例，女1例；年龄4—15岁，平均8．4岁。SLL型6例，IDD型1例。合并室间隔缺损6例，右室双出口1例，继发孔房间隔缺损2例，肺动脉狭窄6例；3例SLL型为右旋心，1例IDD型为左旋心。手术方式包括改良Senning＋Rastelli 4例，Mustard＋Rastelli＋双向Glenn手术、Senning＋Rastelli手术、改良Senning＋Switch手术各1例。结果术后早期死亡1例，为cTGA合并室间隔缺损、肺动脉高压，行改良Senning＋Switch手术者，死因为左心功能衰竭。早期主要并发症有低心排血量综合征、房室分离各1例，胸腔积液和低蛋白血症2例。随访2—24个月，恢复良好，复查多普勒超声心动图、心电图和X线胸片显示，窦性心律5例，结性心律1例，心功能均为Ⅰ级。结论双调转手术可以达到解剖矫治cTGA合并心内畸形，早期手术死亡率低，中、远期心功能效果良好。SLL型病例采用改良Senning心房内调转手术效果优于Senning和Mustard手术。     

Cardiac arrest at induction of anesthesia in a child with undiagnosed right-ventricular dependent coronary circulation: a case report

Pediatric perioperative cardiac arrest occurs in 1.4 per 10,000 anesthetics, with an overall mortality rate of 26%. The etiology of the arrest is identifiable in the majority of these patients. We report the case of a child with a complex congenital heart defect, who sustained a cardiac arrest at induction of anesthesia, secondary to right-ventricular dependent coronary circulation. We discuss the incidence, risks, anesthetic management and outcomes of pediatric cardiac arrest in the perioperative period especially in patients with complex congenital heart disease.     

Placement of a continuous-flow ventricular assist device in the failing ventricle of an adult patient with complex cyanotic congenital heart disease

For patients with end-stage heart failure and contraindications to transplantation, insertion of a continuous-flow left ventricular assist device (LVAD) is an effective treatment strategy. We present a case of LVAD insertion in a 46-year-old man with cyanotic complex congenital heart disease and an extensive surgical history who presented with failure of his systemic ventricle. The insertion of an LVAD in our patient restored cardiac output and improved cyanosis and native ventricular function. As the number of patients with congenital heart defects surviving to adulthood increases, destination LVAD therapy may be increasingly considered as an alternative.