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Repair of Anomalous Coronary Artery From the Pulmonary Artery by Aortic Implantation

  • Anthony Azakie
    Correspondence
    Address reprint requests to Anthony Azakie, MD, Pediatric Specialists of Texas, Methodist Children׳s Hospital, 4410 Medical Dr #540, San Antonio, TX 78229.
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    Pediatric Specialists of Texas, Methodist Children׳s Hospital, San Antonio, TX
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Open ArchivePublished:October 05, 2015DOI:https://doi.org/10.1053/j.optechstcvs.2015.09.001
      Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital cardiac lesion, is an important cause of myocardial ischemia and infarction in children, and carries a high mortality in the first year of life. In the current era, repair of ALCAPA is most commonly performed by aortic implantation. Coronary elongation techniques are available for cases where the ectopic coronary artery originates at point in the pulmonary artery that is distant from the aortic root. In cases where clinically significant mitral regurgitation (MR) is present mitral valve repair is usually not performed. The early outcomes for aortic implantation of ALCAPA are excellent in the current era, with survival rates exceeding 90%. A small number of children, those who present with cardiogenic shock and very poor ventricular function, may require post-repair mechanical circulatory support using ECMO or LVAD and still have high survival rates and expect good long-term recovery. Following repair of ALCAPA by aortic implantation, ventricular functional parameters such as depressed ejection fraction, ventricular dilation and MR should recover within 8 months of repair.

      Keywords

      Introduction

      Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly that usually presents in infancy.
      • Wesselhoeft H.
      • Fawcett J.S.
      • Johnso 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.
      A minority of children who escape clinical detection and survive infancy can present the same in adulthood. The lesion is associated with coronary steal, a left-to-right shunt, and myocardial ischemia that worsens as the pulmonary vascular resistance drops in the first few weeks of life. Myocardial ischemia and infarction ensue. Mitral regurgitation (MR) can develop because of papillary muscle ischemia or infarction, or because of left ventricular (LV) dilation and mitral annular enlargement. If left unrepaired, most patients with this lesion die in infancy.
      A number of surgical strategies have been used to address ALCAPA, including anomalous coronary ligation, aortic implantation, Takeuchi tunneling, coronary artery bypass grafting, or anastomosis to the subclavian artery.
      • Alexi-Meskishvili V.
      • Nasseri B.A.
      • Nordmeyer S.
      • et al.
      Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children.
      • Novick W.M.
      • Li X.F.
      • Anic D.
      • et al.
      Anomalous left coronary artery from the pulmonary artery: Intermediate results of coronary elongation.
      • Ando M.
      • Mee R.B.
      • Duncan B.W.
      • et al.
      Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method.
      • Turley K.
      • Szarnicki R.J.
      • Flachsbart K.D.
      • et al.
      Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery.
      • Sodian R.
      • Rassoullian D.
      • Beiras-Fernandez A.
      • et al.
      ALCAPA with the ectopic orifice at the non-facing sinus: successful anatomic repair by creation of an autologous extrapulmonary tunnel.
      • Ginde S.
      • Earing M.G.
      • Bartz P.J.
      • et al.
      Late complications after Takeuchi repair of anomalous left coronary artery from the pulmonary artery: case series and review of literature.
      • Azakie A.
      • Russell J.L.
      • McCrindle B.W.
      • et al.
      Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
      In the current era, immediate repair is indicated and it involves establishment of a dual coronary system. Our preferred approach is aortic implantation even in cases where the anomalous coronary artery originates leftward and laterally from the main pulmonary artery, where tunneling may be a desirable option.
      • Novick W.M.
      • Li X.F.
      • Anic D.
      • et al.
      Anomalous left coronary artery from the pulmonary artery: Intermediate results of coronary elongation.
      • Ando M.
      • Mee R.B.
      • Duncan B.W.
      • et al.
      Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method.
      • Turley K.
      • Szarnicki R.J.
      • Flachsbart K.D.
      • et al.
      Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery.
      • Sodian R.
      • Rassoullian D.
      • Beiras-Fernandez A.
      • et al.
      ALCAPA with the ectopic orifice at the non-facing sinus: successful anatomic repair by creation of an autologous extrapulmonary tunnel.
      The Takeuchi tunnel is associated with a number of complications such as tunnel leak or obstruction of the main pulmonary artery.
      • Ginde S.
      • Earing M.G.
      • Bartz P.J.
      • et al.
      Late complications after Takeuchi repair of anomalous left coronary artery from the pulmonary artery: case series and review of literature.
      A number of coronary artery elongation techniques are available to allow for successful, tension-free anastomosis to the aortic root.
      • Novick W.M.
      • Li X.F.
      • Anic D.
      • et al.
      Anomalous left coronary artery from the pulmonary artery: Intermediate results of coronary elongation.
      • Ando M.
      • Mee R.B.
      • Duncan B.W.
      • et al.
      Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method.
      • Turley K.
      • Szarnicki R.J.
      • Flachsbart K.D.
      • et al.
      Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery.
      • Sodian R.
      • Rassoullian D.
      • Beiras-Fernandez A.
      • et al.
      ALCAPA with the ectopic orifice at the non-facing sinus: successful anatomic repair by creation of an autologous extrapulmonary tunnel.
      In cases where there is clinically significant MR, the mitral valve should be left alone, especially in infants, as recovery of function is expected.
      • Azakie A.
      • Russell J.L.
      • McCrindle B.W.
      • et al.
      Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
      In the adult or adolescent with ALCAPA and moderate-to-severe MR, mitral valvuloplasty should be considered.
      Although aortic implantation is the technique of choice for repair of ALCAPA, there are very rare circumstances, such as ALCAPA from the right pulmonary artery (PA), where the anomalous vessel courses through the aortic wall and where alternate strategies may need to be developed to avoid coronary artery injury. Most cases of ALCAPA from the right PA, even with an intramural segment in the aortic wall, can be repaired by implantation.
      • Ando M.
      • Mee R.B.
      • Duncan B.W.
      • et al.
      Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method.
      In the rare case where mobilization of the vessel appears hazardous, other techniques such as creation of an in situ coronary pouch, subclavian artery anastomosis, or tunneling should be considered.
      Postoperatively, mechanical circulatory support may be required in children who fail to wean from cardiopulmonary bypass, an event that is likely in the child who presents cardiogenic shock.
      • Azakie A.
      • Russell J.L.
      • McCrindle B.W.
      • et al.
      Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
      Either extracorporeal membrane oxygenation or left ventricular assist device support can be used to allow for LV recovery with good outcomes. We prefer the use of extracorporeal membrane oxygenation to provide right ventricular support as the right ventricle may have been subjected to coronary steal, ischemia, and elevated PA pressures in the setting of MR (Figure 1, Figure 2, Figure 3, Figure 3, Figure 3, Figure 3, Figure 4, Figure 4, Figure 4, Figure 5, Figure 5).
      Figure thumbnail gr1
      Figure 1After induction of general anesthesia, central venous and radial arterial monitoring lines are placed. Reduction in pulmonary vascular resistanceas seen with hyperventilation, alkalosis, or administration of high FiO2is avoided. A median sternotomy is performed and the thymus gland is removed. The pericardium is opened and a patch is harvested for later use. The child is systemically heparinized and purse strings are placed in the distal ascending aorta, right superior vena cava, and inferior vena cava. The pulmonary arteries are dissected and encircled with heavy silk snares. The ALCAPA may be visualized at this point and fine polypropylene suture can be used to mark the recipient site on the ascending aorta. After ascending aortic and bicaval cannulation, a cardioplegia needle is inserted into the ascending aorta. High-flow CPB is instituted to support the child with mild-moderate hypothermia. CPB = cardiopulmonary bypass.
      Figure thumbnail gr2
      Figure 2Soon after the institution of CPB, the aorta is cross-clamped and cold blood cardioplegia is administered through the aortic root. Immediate clamping of the aorta is performed after initiation of CPB, so as to prevent coronary steal into the decompressed PA. The PA tourniquets are tightened to prevent runoff of cardioplegia into the PA via the ALCAPA. A 30-40 ml/kg of a 4:1 ratio of Buckberg or del Nido cardioplegia solution to blood, enriched with aspartate and glutamate, is given at the initial dose, and if necessary 5-10 ml/kg is given every 20-30 minutes thereafter. CPB = cardiopulmonary bypass. The caval snares are tightened, the right atrium is opened, and the left heart is decompressed through the atrial septum. Attention is then turned to the PA that is transected proximal to the bifurcation. The coronary orifice that in most cases originates from the posterior (facing) sinus is identified, and then a large button is excised and the proximal coronary artery is mobilized. An anterior aortotomy is made to allow for direct visualization of the aortic valve, and extension of the incision to the point where the coronary artery is translocated. CPB = cardiopulmonary bypass.
      Figure thumbnail gr3a
      Figure 3(A) An incision has been made in the ascending aorta above the sinotubular junction and extended leftward. The aortotomy allows for direct visualization, so that a medially based trapdoor incision is made in the recipient aortic root site. The coronary artery is minimally rotated, so that it is implanted without tension.
      Figure thumbnail gr3b
      Figure 3(A) An incision has been made in the ascending aorta above the sinotubular junction and extended leftward. The aortotomy allows for direct visualization, so that a medially based trapdoor incision is made in the recipient aortic root site. The coronary artery is minimally rotated, so that it is implanted without tension.
      Figure thumbnail gr3c
      Figure 3(A) An incision has been made in the ascending aorta above the sinotubular junction and extended leftward. The aortotomy allows for direct visualization, so that a medially based trapdoor incision is made in the recipient aortic root site. The coronary artery is minimally rotated, so that it is implanted without tension.
      Figure thumbnail gr3d
      Figure 3(A) An incision has been made in the ascending aorta above the sinotubular junction and extended leftward. The aortotomy allows for direct visualization, so that a medially based trapdoor incision is made in the recipient aortic root site. The coronary artery is minimally rotated, so that it is implanted without tension.
      Figure thumbnail gr3b
      Figure 3Continued (B) After “reimplantation” of the coronary artery, the aortic root is closed with polypropylene suture with the cardioplegia site used to vent the aortic root and LV. A patch may be used to minimize tension on the aortic root closure or suture line.
      Figure thumbnail gr3c
      Figure 3Continued (C and D) The trapdoor method uses minimal rotation of the coronary artery, allowing the coronary artery to remain almost in situ for the aortic implantation. The use of the trapdoor decreases the arc or the rotation used to transfer the coronary artery, and minimizes the risk of ischemia from stretching or kinking.
      Figure thumbnail gr3d
      Figure 3Continued (C and D) The trapdoor method uses minimal rotation of the coronary artery, allowing the coronary artery to remain almost in situ for the aortic implantation. The use of the trapdoor decreases the arc or the rotation used to transfer the coronary artery, and minimizes the risk of ischemia from stretching or kinking.
      Figure thumbnail gr4a
      Figure 4(A) The anomalous coronary artery can originate leftward from the lateral aspect of the posterior-facing sinus or from the anterior nonfacing pulmonary sinus, making its implantation more challenging because of its distant relationship with the aortic root. In this case, a ring of MPA tissue is harvested together with the coronary sinus button. MPA = main pulmonary artery.
      Figure thumbnail gr4b
      Figure 4(A) The anomalous coronary artery can originate leftward from the lateral aspect of the posterior-facing sinus or from the anterior nonfacing pulmonary sinus, making its implantation more challenging because of its distant relationship with the aortic root. In this case, a ring of MPA tissue is harvested together with the coronary sinus button. MPA = main pulmonary artery.
      Figure thumbnail gr4c
      Figure 4(A) The anomalous coronary artery can originate leftward from the lateral aspect of the posterior-facing sinus or from the anterior nonfacing pulmonary sinus, making its implantation more challenging because of its distant relationship with the aortic root. In this case, a ring of MPA tissue is harvested together with the coronary sinus button. MPA = main pulmonary artery.
      Figure thumbnail gr4b
      Figure 4Continued (B) The ring is then divided at a point most distal from the coronary orifice.
      Continued (C) The MPA tissue is tubularized, thus elongating the coronary artery, allowing for (D) minimal tension during aortic implantation. MPA = main pulmonary artery.
      Figure thumbnail gr4c
      Figure 4Continued (E) Alternatively, an anterior flap of PA wall is harvested with the coronary artery sinus button and augmented posteriorly with an autologous pericardial patch to elongate the coronary artery.
      The interatrial defect is closed, the heart aggressively deaired, and the cross-clamp is released. The patient is given a loading dose of milrinone during the rewarming phase of CPB, and the aortic root is vented through the cardioplegia cannula. CPB = cardiopulmonary bypass.
      Figure thumbnail gr5a
      Figure 5(A) The sinus defect in the pulmonary root is filled with a redundant autologous pericardial patch. The pulmonary arteries are extensively mobilized and the MPA anastomosis is completed. Care is taken to avoid compression of the implanted coronary artery by the posterior PA anastomosis.
      Figure thumbnail gr5b
      Figure 5(A) The sinus defect in the pulmonary root is filled with a redundant autologous pericardial patch. The pulmonary arteries are extensively mobilized and the MPA anastomosis is completed. Care is taken to avoid compression of the implanted coronary artery by the posterior PA anastomosis.
      Figure thumbnail gr5b
      Figure 5Continued (B) When an extensive amount of PA wall is used for coronary elongation, a generous autologous pericardial patch is used for the PA reconstruction, and direct tissue-to-tissue contact in the PA anastomosis is used to allow for growth.
      The patient is fully warmed and weaned from CPB on dopamine, adrenaline, and milrinone infusions. A left atrial pressure line is inserted for continuous monitoring in the postoperative setting. The chest may be left open for delayed sternal closure. Infrequently, the child may have to be placed on ECMO. In that event, VA ECMO is preferentially used via transsternal cannulation of the ascending aorta and right atrium. A cannula is placed in the left atrial appendage to optimize LV decompression and recovery. CPB = cardiopulmonary bypass; ECMO = extracorporeal membrane oxygenation; VA = venoarterial.

      Comment

      In the current era, the outcomes for aortic implantation of ALCAPA are excellent. Hospital and long-term survival exceed 95%. Excellent outcomes are expected also for the small percentage of patients who require mechanical circulatory support to allow for recovery of hibernating, stunned, or chronically ischemic myocardium. Data from the Hospital for Sick Children, Toronto, suggest that ventricular functional parameters—ejection fraction, LV dilation, and MR—usually recover within 1 year.
      • Azakie A.
      • Russell J.L.
      • McCrindle B.W.
      • et al.
      Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome.
      By repeated measures and mixed linear regression analysis, the ejection fraction and degree of LV dilation usually improve by 4 months after the operation (Fig. 6). Improvements in MR lag behind, but in most cases the degree of MR should be mild or less in most patients 7-8 months after the repair. If severe MR persists in the late postoperative period, then causes of recurrent myocardial ischemia should be investigated. We have not used routine postoperative anticoagulation, and currently include cardiac computed tomography angiography in addition to echocardiography during routine follow-up imaging.
      Figure thumbnail gr6
      Figure 6Time course (months) for ventricular recovery of (A) ejection fraction (%).
      Continued (B) Ventricular dilation (ratio of measured left ventricular end-diastolic [LVED] diameter to 95% of normal).
      Continued (C) Mitral valve regurgitation (0 = none, 0.5 = trace, 1= mild, 1.5 = mild to moderate, 2 = moderate, 2.5 = moderate to severe) as determined by repeated measures of mixed linear regression ananlysis.

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