ObjectiveTo summarize perioperative management experience of 16 patients undergoing surgical correction of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA). MethodsWe performed a retrospective analysis of 16 patients who received surgical correction of ALCAPA between January 2005 and December 2013 in Cardiovascular Center of Children's Hospital of Fudan University. There were 6 males and 10 females with their age ranging from 2 months to 13 years (mean age of 23.25±0.63 months) and body weight ranging from 5 to 30 kg (mean body weight of 19.77±0.75 kg). All the patients underwent direct implantation of the anomalous coronary artery into the ascending aorta, and received comprehensive management regarding left ventricular ejection fraction (LVEF), heart rate, blood pressure, arterial blood gas, urinary volume, blood lactic acid and central venous pressure. ResultsPostoperative complications were low cardiac output syndrome (LCOS) in 9 patients (including intractable LCOS in 2 patients) and arrhythmia in 1 patient. One patient with intractable LCOS and arrhythmia died postoperatively with the mortality of 6.25%. Cardiopulmonary bypass time was 85-260 (135.61±35.01) minutes, aortic cross-clamping time was 40-97 (57.32±16.02) minutes, mechanical ventilation time was 34-187 (106.34±41.62) hours, length of CICU stay was 2-21 (8.13±5.02) days, and hospital stay was 12-51 (22.14±5.00) days. Postoperative LVEF and left ventricular fractional shortening were significantly higher than preoperative values (P < 0.05). ConclusionKey points for successful surgical correction of ALCAPA include meticulous perioperative management, preoperative heart function improvement, prevention of postoperative complications and use of new techniques.
【摘要】 目的 探讨脑干海绵状血管瘤患者临床表现及影响预后的相关因素。 方法 回顾分析2008年9月-2010年9月27例脑干海绵状血管瘤患者临床资料。 结果 20例（74.1%）患者CT显示出血及血肿。急性或突然神经功能废损22例，渐进性功能障碍15例，病情平稳4例。显微手术12例，放射治疗6例，保守观察9例。长期随访25例，2例再次出血，无新发病灶，无患者死亡。手术组患者年龄（Plt;0.05）、术前病情严重程度（Plt;0.05）与术后KPS评分相关。非手术组不良预后评分与患者高龄及再出血有关。 结论 展神经麻痹和面瘫患者功能恢复较差。年龄、术前病情严重程度及手术时机影响患者预后，术中全切病灶对复发和并发症发生起主要作用，如何处理合并发生的静脉畸形有待讨论。立体定向放射治疗明显降低再出血风险，但其适应证尚有争议。【Abstract】 Objective To discuss the clinical manifestations and prognostic factors of brain stem cavernous hemangioma. Methods Based on the literature, the clinical data of 27 patients having brain stem cavernous hemangioma from September 2008 to September 2010 were reviewed and analyzed. Results Twenty patients (74.1%) presented with hemorrhage and hematoma in CT scan. Acute or sudden neurological deterioration occurred to 22 patients, progressive in 15 and stable in 4. Twelve patients underwent surgical removal of the lesion; 6 underwent radiosurgery; and 9 continued with conservative management. Twenty-five patients were followed up. Two patients had rehaemorrhagia. There were no de novo lesions or death. The risk factors indicative of a possible poor postoperative KPS score in the operative group included age (Plt;0.05) and the initial clinical condition (Plt;0.05). In the non-operative group, old age and rehaemorrhagia were obviously related to the poor outcome. Conclusions Patients with abducens and facial palsy have poor functional recovery. Age, the initial clinical condition and timing of operation are the major factors correlated to surgical outcome. The factor that affects recurrence and the occurrence of complications is complete resection during the operation. How to deal with the concomitant venous malformation should be further studied. The indications for stereotactic radiosurgery are still controversial, although it has confered a reduction in the risk of rehaemorrhagia.
Objective To analyze pathologic features and surgical procedures for patients with unroofed coronary sinus syndrome (UCSS) associated with endocardial cushion defect (ECD). Methods The clinical data of 44 patients with UCSS and ECD from May 1998 to July 2016 were retrospectively reviewed. There were 18 males and 26 females with a mean age of 10.4±12.1 years (range: 5.0 months to 44.0 years) and mean weight of 25.2±20.9 kg (range 5.2-80.0 kg). According to the Kirklin and Barratt-Boyes classification, 28 patients were categorized into type Ⅰ, 5 typeⅡ , 4 type Ⅲ and 7 type Ⅳ. Among them 25 patients suffered partial ECD, 10 complete ECD, 9 transitional ECD, and 27 were associated with single atrium, 34 involved persistent left superior vena cava (PLSVC), and in 27 of the 34 patients PLSVC directly drained into the left atrium (LA). Among the 44 patients, 1 patient associated with complex anomalies underwent palliative operation, and other cardiac malformations were corrected simultaneously by surgical correction. PLSVC was ligated in 2 patients, and the intracardiac tunnels or baffles to drain PLSVC to right atrium (RA) were reconstructed in 25 patients. The associated cardiac lesions were corrected concomitantly. Results In-hospital death occurred in 2 patients, among whom 1 died of low cardiac output syndrome on postoperative day 8 and the other pulmonary infection on postoperative day 21. Thirty-one were followed up from 1 month to 10 years, and there was no death or severe complications. Conclusion When ECD is associated with PLSVC and a single atrium, UCSS may develop. Repair according to the type of UCSS is effective.
ObjectiveTo summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome. MethodWe retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013. There were 49 male and 32 female patients at age of 3.79 (range 2.07-13.02) years with preoperative room air saturation of 81% (range 63%-97%) and weight of 14.8 (10.0-36.0) kg. ResultsThere were 70 patients in the right atrial isomerism group and left in 11 patients. Dextrocardia was seen in 16 patients, and single atrium in 50 patients. Single ventricle was found in 44 patients, conotruncal defects in 40 patients, pulmonary stenosis in 72 patients, pulmonray atresia in 8 patients, common atrioventricular valve in 61 patients, and bilateral superior vena cava in 54 patients. The staged Fontan procedure was applied in 57 patients and one stage in 24 patients. Operation strategies were included intra/extracardiac conduit (n=17), extracardiac conduit (n=48), lateral tunnel (n=14) and direct cavopulmonray connection (n=2).There were 15 early deaths. Postoperative complications included low cardiac output in 15 patients, hepatic insufficiency in 35 patients, renal insufficiency in 55 patients required peritoneal dialysis and arrhythmia in 28 patients. The room air saturation was 89% (range 78%-98%) before discharge and time of follow-up was from 2 months to 6 years in 64 patients. Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation. ConclusionCompared with reported literatures in western countries, heteraotaxia patients are mostly with right atrial isomerism. Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.
Objective To study the cl inical features of infantile hemangioma and vascular malformation, to find out a proper strategy of deal ing with them. Methods From March 2000 to August 2007, 2 957 cases of infantile hemangioma and vascular malformation were treated, including 860 operative cases and 2 097 non-operative cases. There were 441 male and419 female patients in operation group, aging 6 months to 18 years (median 5 years). In 1 950 hemangioma patients of nonoperation group, there were 575 male and 1 375 female patients, aging 1 month to 14 years (median 6 months); in 147 vascular malformation patients of non-operation group, there 67 male and 80 female patients, aging 2 years to 17 years (median 7 years). In non-operative group, 147 vascular malformation patients and 1 525 infantile hemangioma patients were followed up without any medical intervention, while other 425 hemangioma patients recceived triamcinolone plus dexamethasone intralesional injection treatment. All the treatments and outcomes were recorded. Results Vascular malformation cases and infantile hemangioma cases presented totally different cl inical features. To the deadl ine of this study, 522 (34%) of 1 525 un-intervented hemangioma cases turned into involuted phase and 383 (90%) of 425 cases receiving triamcinolone plus dexamethasone intralesional injection treatment turned into involuted phase after injection treatment; no regression was noted in 147 cases of vascular malformation. The constituent ratio of infantile hemangioma in 860 operative cases was decreased gradually and the constituent ratio of vascular malformation was increased gradually as the age increasing. Conclusion Infantile hemangioma has a distinct l ife pattern. Except several specific cases need medical intervention for their special location or large ambit and unacceptable growth, most infantile hemangioma need no medical intervention. Most vascular malformations can not regress spontaneously, proper intervention is in need.