Summary

左冠状动脉直接再注入与从肺动脉左冠状动脉起源异常成人主动脉(ALCAPA)

Published: April 24, 2017
doi:

Summary

ALCAPA的手术矫正强烈建议,无论年龄或intercoronary抵押的程度。该协议提出了成年型ALCAPA的直接再植入到主动脉重新建立双冠状动脉灌注的技术。只要可行,直接再注入优选其它外科手术校正技术。

Abstract

从肺动脉(ALCAPA)左冠状动脉起源异常是一种罕见的先天性异常是小儿心肌缺血和梗死的主要原因之一。如果不及时治疗,它会导致死亡率在生命的第一年90%。在谁存活到成年患者,冠状动脉偷窃现象,逆行左侧冠状动脉流量为慢性心内膜下缺血,这可能会导致左心功能不全,缺血性二尖瓣关闭不全,恶性室性心律失常和心源性猝死的基材。危及生命的表现,平均年龄为33岁,心脏性猝死31岁。因此,手术矫正高度一旦作出诊断建议,不论年龄。在成人型ALCAPA从肺动脉的右向窦起源的ALCAPA到主动脉的直接再注入是多种生理声音修复技术吨Ø重新建立双冠脉灌注系统和建议。这个协议描述成人型ALCAPA的直接再注入技术进入主动脉。

Introduction

从肺动脉(ALCAPA)左冠状动脉异常起源是一种罕见的先天性异常通常被视为分离的病变1。 ALCAPA的发生率估计为1 300000活产,0.24%和先天性心脏病2,3的0.46%之间,包括这是小儿心肌缺血和梗死的最常见的原因之一,如果不及时治疗,导致死亡率生活4的第一年90%。只有10 – 15%的婴儿存活由于大主导右冠状动脉(RCA)提供广泛的intercoronary络4的迅猛发展到成年。在新生儿期,高肺血管阻力和所得肺动脉(PA)的压力下确保顺行流从PA保持到异常左冠状动脉。由于pulmonarÿ血管阻力逐渐减小,顺行流向左冠状动脉降低。这最终导致流动的逆转,和左到右分流到PA,从而导致“冠状动脉偷5”。因此,左心室(LV)心肌灌注取决于intercoronary络从RCA 5,6。

冠状偷现象和逆行左侧冠状动脉血流提供慢性心内膜下缺血,这可能导致左心功能不全,缺血性二尖瓣反流,和由急性心肌缺血7沉淀恶性室性心律失常的基板。在成人患者的子集,在介绍的平均年龄为41岁,性别分布朝向女性患者的移位(女到男比:2:1)8。在此患者群体中,14%是无症状; 66%用本心绞痛,呼吸困难,心悸,或疲劳的症状;和现在有危及生命的症状,包括室性心律失常,晕厥和心源性猝死8 17%。危及生命的表现,平均年龄为33岁,心脏性猝死31岁8。因此,手术矫正高度只要作出诊断建议,不论年龄或intercoronary抵押1,9的程度。

根据异常左冠状动脉起源的ALCAPA进入主动脉的直接再植入是多种生理声音修复技术重建双冠状动脉灌注系统。最常见地,从ALCAPA右侧肺窦(PA的窦1),其面向主动脉窦其中主左冠状动脉通常起源(主动脉窦2)10 <起飞/ SUP>。这冠状动脉解剖是最适合于直接再注入技术。本报告的目的是描述,详细,对于成人患者在ALCAPA左冠状动脉的直接再植入技术。后面直接再注入的理由是优势-双冠状动脉的生理重建灌注它提供了一个在异常左冠状动脉与冠状动脉旁路移植11,12,13结合的结扎。

Protocol

该协议遵循苏黎世大学的人类研究伦理委员会的机构准则。 1.制备用于外科干净,以典型的方式准备了外科手术室。为了便于外科医生和灌注师之间的通信,心脏心肺机放置到患者的左侧,相对于该外科医生。 麻醉诱导前60分钟 – 由5毫克咪达唑仑,30的口服给药前用药患者。 让患者根据标准的指导方针,与直接动脉和麻醉师中心静脉压访问进行监控。 1….

Representative Results

介绍患者是一名48岁的女子与近期心绞痛加拿大心血管学会(CCS)毕业生III和偶尔的心悸发作呈现。她报告了三名波澜不惊怀孕。中度吸烟是主要的心血管危险因素。经胸超声心动图显示一个中度受损(45%)左室射血分数和无二尖瓣关闭不全。然后进行一个冠状动脉造影。它表明没有从主动脉引起的左冠状动脉主干的。右?…

Discussion

这个协议描述了ALCAPA的直接再植入到主动脉与主左冠状动脉从根据道奇-哈塔米分类10中的肺动脉的右向窦起源成年患者的详细技术。心肌保护策略和肺动脉重建都清楚地证明。这个技术的主要关键步骤是通过左冠状动脉的慷慨动员表示实现无张力的吻合术。

长时间去饱和冠状动脉灌注容忍到成年期在少数个人。谁生存本到成年的临床表现的光谱,从没有症?…

Offenlegungen

The authors have nothing to disclose.

Acknowledgements

这项工作是由瑞士心血管基金会RT的资助。

Materials

Heart surgery infrastructure:
Heart Lung Machine Stockert SIII
EOPA 24Fr. arterial cannula Medtronic 77624
Quickdraw 25Fr. femoral venous cannula Edwards QD25
LV vent catheter 17Fr. Edwards E061
Antegrade 9Fr. cardioplegia cannula Edwards AR012V
Retrograde 14Fr. cardioplegia cannula  Edwards NPC014 
Electrocautery Covidien Force FX
Name Company Catalog number
Sutures:
Polypropylene 4/0 Ethicon 8871H
Polypropylene 5/0 Ethicon 8870H
Polypropylene 6/0 Ethicon EH7400H
Braided polyesther 2/0 ligature with polybutylate coating  Ethicon X305H
Intergard dacron graft 8 mm Maquet IGW0008-30
Micro knife Sharpoint  TYCO Healthcare PTY  78-6900
Name Company Catalog number
Drugs:
Midazolam Roche Pharma N05CD08
Rocuronium MSD Merck Sharp & Dohme  M03AC09
Propofol Fresenius Kabi N01AX10
Fentanil Actavis N01AH01
Name Company Catalog number
Instruments:
Cooley Derra anastomosis clamp Delacroix-Chevalier DC40810-16
Cooley vascular clamp Delacroix-Chevalier DC40810-16
Dissection forceps Carpentier Delacroix-Chevalier DC13110-28 
Scissors Metzenbaum Delacroix-Chevalier B351751
Needle holder Ryder Delacroix-Chevalier DC51130-20 
Dissection forceps DeBakey Delacroix-Chevalier DC12000-21 
Micro needle holder Jacobson Delacroix-Chevalier DC50002-21 
Micro scisors Jacobson Delacroix-Chevalier DC20057-21 
Lung retractor Delacroix-Chevalier B803990
Allis clamp Delacroix-Chevalier DC45907-25 
O’Shaugnessy Dissector Delacroix-Chevalier B60650
Vessel loop Medline VLMINY
18 blade knife Delacroix-Chevalier B130180
Leriche haemostatic clamp Delacroix-Chevalier B86555

Referenzen

  1. Dodge-Khatami, A., Mavroudis, C., Backer, C. L. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann. Thorac. Surg. 74 (3), 946-955 (2002).
  2. Keith, J. D. The anomalous origin of the left coronary artery from the pulmonary artery. Br. Heart J. 21 (2), 149-161 (1959).
  3. Hauser, M. Congenital anomalies of the coronary arteries. Heart. 91 (9), 1240-1245 (2005).
  4. Wesselhoeft, H., Fawcett, J. S., Johnson, A. L. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation. 38 (2), 403-425 (1968).
  5. Edwards, J. E. The direction of blood flow in coronary arteries arising from the pulmonary trunk. Circulation. 29 (2), 163-166 (1964).
  6. Agustsson, M. H., Gasul, B. M., Fell, H., Graettinger, J. S., Bicoff, J. P., Waterman, D. F. Anomalous origin of the left coronary artery from the pulmonary artery diagnosis and treatment of infantile and adult types. JAMA. 180 (1), 15-21 (1962).
  7. Roberts, S. M., Banbury, T., Mehta, A. A rare case of anomalous left coronary artery from the pulmonary artery (Bland-White-Garland Syndrome) in a 68-year-old woman. Semin. Cardiothorac. Vasc. Anesth. , (2016).
  8. Yau, J. M., Singh, R., Halpern, E. J., Fischman, D. Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman. Clin. Cardiol. 34 (4), 204-210 (2011).
  9. Michielon, G., et al. Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery. Ann. Thorac. Surg. 76 (2), 581-588 (2003).
  10. Dodge-Khatami, A., Mavroudis, C., Backer, C. L. Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries. Ann. Thorac. Surg. 69 (4 Suppl), S270-S297 (2000).
  11. Talwar, S., Jha, A. J., Choudhary, S. K., Gupta, S. K., Airan, B. Repair of anomalous left coronary artery from pulmonary artery (ALCAPA) beyond infancy. Heart Surg. Forum. 16 (4), E210-E215 (2013).
  12. Toumpourleka, M., Belitsis, G., Alonso, R., Rubens, M., Moat, N., Gatzoulis, M. Late presentation and surgical repair of ALCAPA. Int. J. Cardiol. 186, 207-209 (2015).
  13. Quah, J. X., et al. The management of the older adult patient with anomalous left coronary artery from the pulmonary artery syndrome: a presentation of two cases and review of the literature. Congenit. Heart Dis. 9 (6), E185-E194 (2014).
  14. Kottayil, B. P., et al. Anomalous origin of left coronary artery from pulmonary artery in older children and adults: direct aortic implantation. Ann. Thorac. Surg. 91 (2), 549-553 (2011).
  15. Rajbanshi, B. G., Burkhart, H. M., Schaff, H. V., Daly, R. C., Phillips, S. D., Dearani, J. A. Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults. J. Thorac. Cardiovasc. Surg. 148 (1), 220-224 (2014).
  16. Neumann, A., et al. Long-term results after repair of anomalous origin of left coronary artery from the pulmonary artery: Takeuchi repair versus coronary transfer. Eur. J. Cardio-Thorac. Surg. , (2016).
  17. Peña, E., Nguyen, E. T., Merchant, N., Dennie, C. ALCAPA syndrome: not just a pediatric disease. Radiographics. 29 (2), 553-565 (2009).

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Tavakoli, R., Jamshidi, P., Yamani, N., Gassmann, M. Direct Re-implantation of Left Coronary Artery into the Aorta in Adults with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery (ALCAPA). J. Vis. Exp. (122), e55590, doi:10.3791/55590 (2017).

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