The Surgical Anatomy of Tetralogy of Fallot with Pulmonary Atresia Rather than Pulmonary Stenosis

We examined the pertinent surgical features of the anatomy of 56 hearts having tetralogy of Fallot with pulmonary atresia instead of stenosis, or malformations with pulmonary atresia closely related to tetralogy. We took particular cognizance of the pulmonary arterial supply in 15 hearts in which this was derived through systemic-to-pulmonary collateral arteries, dissecting, as far as possible, the bronchopulmonary segmental distribution of the collateral arteries compared to the intrapericardial pulmonary arteries in 11 of these hearts. Two of the hearts had absence of intrapericardial pulmonary arteries, so that a solitary arterial trunk left the base of the heart. Evidence of an atretic subpulmonary infundibulum was found in 40 of the hearts, while such an infundibulum was lacking in the remainder. The pulmonary atresia was muscular in 43 hearts, valvar in 11, while the pulmonary trunk was absent in the other two hearts. In the hearts with collateral arteries, on average 2.6 collaterals were found in each case, varying from two to five per case. Only one of these arose from a brachiocephalic artery, the others all arising from the descending aorta. The distribution of collateral arteries in two cases was remarkably reminiscent of the arrangement of bronchial arteries. As far as could be judged, 16.5 bronchopulmonary segments on average were supplied in each heart, 5.1 exclusively by collateral arteries, 11.8 by intrapericardial pulmonary arteries and an average of 0.64 segments per case having a shared supply.     

Tetralogy of Fallot with subarterial ventricular septal defect

BACKGROUND: Tetralogy of Fallot with subarterial ventricular septal defect is frequently seen among Asians. Compared with infracristal ventricular septal defect, postoperative right ventricular outflow obstruction is more likely because of subpulmonary extension of the defect. Moreover the incidence of aortic regurgitation is a concern because of the absence of a supporting infundibulum. METHODS: Four hundred cases of classic tetralogy were reviewed, 61 of which had subarterial ventricular septal defect. RESULTS: Aortic regurgitation (of more than mild degree) was identified in 7 cases with subarterial and 7 with infracristal ventricular septal defects. The mechanism of infracristal defect was predominantly an annular dilation before surgery. In contrast 5 cases with subarterial defect had progression of aortic regurgitation after operation yielding an actuarial incidence of 29.7% at 20 years. In 2 patients the cause seemed to be fixation or plication of the aortic valve annulus by the ventricular septal patch. Compared with infracristal defect, subarterial defect was associated with increased incidence of reoperation (12.0% versus 1.9% at 10 years, p = 0.01), frequent use of transannular patch (70.5% versus 45.7%, p = 0.0004), and worse New York Heart Association (NYHA) functional class (p = 0.007). Right ventricular outflow obstruction was the reason for reoperation in 3 patients with subarterial defect and was associated with worse NYHA classification in the long-term, on multivariate analysis (p = 0.0002). CONCLUSIONS: Tetralogy with subarterial ventricular septal defect was associated with worse functional outcome. To prevent adverse outcomes, precise suturing of the distal ventricular septal patch, extensive infundibulectomy, lower threshold for transannular incision, and smaller-sized ventricular septal patch placement are warranted.     

Tetralogy of Fallot with Coronary Artery to Pulmonary Artery Fistula and Unusual Coronary Pattern: Missed Diagnosis

Abstract  Coronary arteries to pulmonary artery fistulas are rare in patients with Tetralogy of Fallot. Even rarer is the occurrence of these fistulas from a solitary coronary system. We report one such patient in whom the preoperative diagnosis was missed. The importance of a high index of suspicion for accurate preoperative diagnosis is discussed.     

Coronary artery obstruction due to membranous ridge of the right sinus valsalva associated with Tetralogy of Fallot: syncope mimics anoxic spell

We report an infant with Tetralogy of Fallot who had suffered from repetitive attacks of syncope without obvious cyanosis. Careful observation by means of echocardiography and angiography revealed that the attacks resulted from acute coronary artery obstruction due to membranous ridge covering the sinus Valsalva. Surgical resection of the abnormal ridge and repair of Tetralogy of Fallot successfully improved the patient's symptoms. Syncope in children should be extensively investigated to exclude obstruction of the coronary arteries.     

Lung perfusion with protective solution relieves lung injury in corrections of Tetralogy of Fallot

BACKGROUND: The aim of this study was to evaluate the protective effect of pulmonary perfusion with hypothermic protective solution on lung function after cardiopulmonary bypass in corrections of Tetralogy of Fallot. METHODS: Sixty-four consecutive children with Tetralogy of Fallot were randomly divided into a control group (n = 30) and a protective group (n = 34). Hypothermic protective solution was infused to the main pulmonary artery in the protective group. Hemodynamics and lung functions were monitored. Concentrations of malondialdehyde, tumor necrosis factor-alpha, von Willebrand factor, and endothelin in plasma were measured. The interleukin-6 and interleukin-8 levels in bronchoalveolar lavage fluid were also determined. Lung biopsy specimens were obtained after weaning from cardiopulmonary bypass. RESULTS: Oxygenation values (oxygen index and alveolar-arterial O(2) gradient) were better preserved in the protective group than in the control group. The time of mechanical ventilation and length of intensive care unit stay were shorter in the protective group compared with the control group. The tumor necrosis factor-alpha and malondialdehyde levels in plasma increased in both groups after operations, and the rising extents were lower in the protective group than in the control group. The von Willebrand factor and endothelin levels in plasma increased more significantly in the control group than in the protective group. The concentrations of interleukin-6 and interleukin-8 in bronchoalveolar lavage fluid were lower in the protective group than in the control group. The examination of histopathology demonstrated capillary hyperemia and hemorrhage, intra-alveolar edema, leukocytes accumulation, mitochondria swelling and vacuolation, and gas-blood barrier broadening in the control group, whereas there were no significant changes in the protective group. The intercellular adhesion molecule-1 expression on lung vascular endothelial cells was stronger in the control group. CONCLUSIONS: Lung perfusion with hypothermic protective solution during cardiopulmonary bypass relieved lung injury in corrections of Tetralogy of Fallot. The inhibition of lung vascular endothelial cell injury may be the major mechanism of relieving cardiopulmonary bypass-induced lung injury.     

法乐四联症伴肺动脉闭锁的外科治疗(附14例报告)

报告外科治疗法乐四联症伴肺动脉闭锁１４例。肺动脉瓣水平膜状闭锁１３例，主肺动脉干闭锁１例。肺动脉发育好，肺血由一粗大的动脉导管供给者９例；肺动脉发育不良、无动脉导管，肺血由体肺侧支供给者１例；肺动脉发育不良，由细的动脉导管和体肺侧支共同供给者４例。姑息性手术５例，一期根治９例，二期根治２例；死亡３例。作者对法乐四联症伴肺动脉闭锁的手术适应证、姑息性手术方法的应用及体肺侧支的外科处理原则进行了探讨。     

法洛四联症合并冠状动脉畸形的外科治疗

目的总结法洛四联症(TOF)合并冠状动脉畸形(ACA)手术治疗经验,探讨ACA的诊断,右心室流出道(RVOT)疏通手术方式的选择及其效果。方法回顾性分析2004年1月至2010年1月武汉亚洲心脏病医院手术矫治TOF合并ACA 29例,其中男18例,女11例;年龄7岁(5个月～33岁),体重18(5～51)kg。术前动脉血氧饱和度65%～91%。所有患者均行完全矫治手术。1例经右心房疏通RVOT,修补室间隔缺损;3例经右心房疏通RVOT,修补室间隔缺损,用自体心包片加宽主肺动脉;3例行肺动脉移位术(REV),5例患者采取"双通道"手术;11例采取冠状动脉下方、上方或者两边切口疏通,补片加宽的方法("单片"或"双片"法);6例行冠状动脉移植术后行跨瓣环补片重建RVOT。结果体外循环时间78(65～102)min,主动脉阻断时间50(40～82)min,手术时间150(126～178)min。手术未出现死亡以及严重冠状动脉损伤。术后2例因出血行二次开胸止血;2例出现低心排血量,强心药物支持后治愈。随访51(21～83)个月,随访过程中无死亡。所有患者术后心功能(NYHA)分级均为Ⅰ级,心脏射血分数均正常,心电图未提示心肌缺血,动脉血氧饱和度恢复至96%～99%。术后早期RVOT压差(△P)为19(8～38)mm Hg,术后随访患者无△P增加。结论对于TOF合并ACA患者,术前通过64排螺旋CT(64-MSCT)检查可以明确ACA诊断,术中根据冠状动脉情况选择合适的流出道疏通方法,手术效果良好。     

法洛四联症合并异常冠状动脉的外科治疗

目的 总结法洛四联症(TOF)合并异常冠状动脉完全矫治手术的经验.方法 自1993年3月至2006年4月对22例TOF合并异常冠状动脉的患者施行完全矫治手术,其中经右室前壁小切口径路切除右心室流出道(RVOT)内肥厚的隔束、壁束以及纤维狭窄环,并修补室间隔缺损(VSD)5例;经右心房径路疏通RVOT、修补VSD17例,其中跨肺动脉瓣环补片加宽RVOT 7例,用自体心包外管道重建RVOT 3例,同种带瓣血管重建RVOT 1例,肺动脉前壁血管片反折缝合于RVOT上缘,外用带单瓣的牛心包补片缝合,将RVOT重建为外通道2例;其余患者均经右心房-肺动脉径路解除RVOT狭窄.结果 术后早期因冠状动脉损伤死亡1例.1例误将发自右冠状动脉(RCA)的附前降支当作增粗的圆锥支予以切断,导致停体外循环困难,将左乳内动脉与该血管吻合后停机成功,其余患者均顺利恢复.术后早期右心室-肺动脉压差(△P)平均为23.4mmHg,△P＞20mmHg者9例.随访18例,平均随访13.2个月,失访3例.△P＞20mmHg者7例,其余患者△P均＜20mmHg.结论 经右心房径路修补VSD并疏通RVOT使手术更加安全,RVOT的重建方式应根据异常冠状动脉的类型等具体解剖情况来决定.