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Technical Tips for Three Congenital Heart Operations: Modified Ross-Konno Procedure, Optimal Ventricular Septal Defect Exposure by Tricuspid Valve Incision, Coronary Unroofing and Endarterectomy for Anomalous Aortic Origin of the Coronary Artery

  • Constantine Mavroudis
    Correspondence
    Address reprint requests to Constantine Mavroudis, MD, Center for Pediatric and Congenital Heart Surgery, Cleveland Clinic Children's Hospital, Cleveland Clinic Lerner School of Medicine, Case Western Reserve University, 9500 Euclid Avenue/M41, Cleveland, OH 44195
    Affiliations
    Center for Pediatric and Congenital Heart Surgery, Cleveland Clinic Children's Hospital, Cleveland, Ohio
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  • Carl L. Backer
    Affiliations
    Division of Cardiovascular-Thoracic Surgery, Children's Memorial Hospital, Chicago, Illinois
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      The phrase in my experience, which is usually followed by a litany of seemingly profound and erudite assertions, ought to be accompanied by carefully analyzed data or be relegated to the categories of conclusions based on selective memory.
      Generally, medical students, residents, or young attending physicians/surgeons are wary of this phrase and politely cast an appropriate, silent, skeptical, and disguised look at the orator. Yet, when we attend specialized surgical meetings, we listen attentively to select colleagues who suggest various nuances of surgical techniques, oftentimes without measurements, based on memory, and sometimes without representative drawings. These sessions have helped us learn new operations and have been some of the greatest educational experiences of our professional lives.
      This article on surgical technique is based somewhat on our experiences but documents, to the best of our knowledge and abilities, the measured basis of our conclusions. The topics for illustration and discussion include the modified Ross-Konno operation, tricuspid valve incision for ventricular septal defect closure, and coronary unroofing/endarterectomy for anomalous aortic origin of the coronary arteries.

      Modified Ross-Konno Operation

      The Konno operation was developed to significantly enlarge a small aortic annulus by incising the interventricular septum resulting in an aortoseptoplasty with prosthetic valve placement.
      • Konno S.
      • Imai Y.
      • Iida Y.
      • et al.
      A new method for prosthetic valve replacement in congenital aortic stenosis associated with hypoplasia of the aortic valve ring.
      Because of subsequent atrioventricular arrhythmias, left ventricular dysfunction in some patients, and a daunting reoperation for valve re-replacement, the Ross-Konno operation was introduced.
      • Reddy V.M.
      • Rajasinghe H.A.
      • Teitel D.F.
      • et al.
      Aortoventriculoplasty with the pulmonary autograft: The “Ross-Konno” procedure.
      This operation resulted in improved hemodynamics, limited interventricular septal incision, and freedom from anticoagulation. Because of a higher incidence of complete heart block in our experience with both the Konno and the standard Ross-Konno operation, we recently introduced the modified Ross-Konno procedure.
      • Mavroudis C.
      • Backer C.L.
      • Kaushal S.
      Aortic stenosis and aortic insufficiency in children: Impact of valvuloplasty and modified Ross-Konno procedure.
      In the current commentary, we explain the drawings and describe nuances of the operation.
      Figure thumbnail gr2
      Figure 2Circumferential resection of the aortic valve and annulus demonstrates the enlarged left ventricular outflow tract. The annulus of the aortic valve, especially when small, serves as a restrictive fibrous skeleton, which maintains the diminutive size of the left ventricular outflow. When this fibrous skeleton is resected, the remaining muscle can stretch to accommodate the larger pulmonary autograft. The small incision at the left ventricular free wall (shown by the 11 blade) can further open the left ventricular outflow tract to implant the larger pulmonary autograft. LA = left atrium; LAA = left atrial appendage; LCA = left coronary artery; PA = pulmonary artery; RA = right atrium; RCA = right coronary artery; RV = right ventricle.
      Figure thumbnail gr4
      Figure 4The pulmonary autograft is implanted using the pledgeted suture technique. In addition, the left coronary artery anastomosis to the autograft and the right coronary artery anastomosis are in process. We generally mobilize the coronary arteries extensively to allow tension-free anastomoses. LA = left atrium; LCA = left coronary artery; RA = right atrium; RCA = right coronary artery; RV = right ventricle.
      Figure thumbnail gr5
      Figure 5The last step of the neoaortic reconstruction is demonstrated. The proximal neoaorta, comprising the implanted pulmonary autograft and implanted coronaries, is being connected to the ascending aorta using running suture technique. Occasionally the ascending aorta will require reduction (resection of a portion of the aortic wall) or enlargement (placement of an enlarging patch) aortoplasty to form an appropriately sized aortic root to prevent neoaortic regurgitation. Ao = aorta; PA = pulmonary artery; RA = right atrium; RV = right ventricle.
      Figure thumbnail gr6
      Figure 6The modified Ross-Konno operation is completed after a pulmonary homograft has been placed to reconstruct the right ventricular outflow tract. We have used oversized pulmonary homografts for this part of the operation, although others have used bovine internal jugular grafts (Contegra; Medtronic, Inc, Minneapolis, MN) as well as aortic homografts. Ao = aorta; PA = pulmonary artery; RA = right atrium; RV = right ventricle.
      Figure thumbnail gr1
      Figure 1The patient has been placed on aortobicaval cardiopulmonary bypass. Aortic cross-clamping, coronary artery mobilization, and autograft harvest from the right ventricular outflow tract are depicted. The dotted line, posterior to the course of the left main coronary artery, represents the lateral left ventricular free-wall incision that will be used to enlarge the left ventricular outflow tract, which will accommodate the pulmonary autograft implantation. The particulars of these maneuvers are well described elsewhere but certainly require careful dissection and attention to the details of anatomy to avoid injury to the pulmonary autograft, the coronary arteries, and the surrounding structures. We employ antegrade and retrograde cold blood cardioplegia augmented by topical iced saline administered every 20 minutes for myocardial preservation. Ao = aorta; LCA = left coronary artery; PA = pulmonary artery; RA = right atrium; RCA = right coronary artery; RV = right ventricle.
      Figure thumbnail gr3
      Figure 3The pulmonary autograft implantation technique after annular resection and the left free-wall enlarging incision is depicted. Ordinarily, we use a double running suture technique for the implantation; however, the muscular neck of the left ventricular outflow tract will not hold sutures as well as the previously resected annulus. The individual, interrupted pledgeted sutures help to provide the stability that the implantation procedure requires to sustain systemic pressure. LCA = left coronary artery; PA = pulmonary artery; RA = right atrium; RCA = right coronary artery; RV = right ventricle.

      Circumferential and Radial Tricuspid Valve Incision for Ventricular Septal Defect Closure

      Primary or concomitant ventricular septal defect closure is required for approximately 20% of all congenital heart repairs.
      • Mavroudis C.
      • Backer C.L.
      • Jacobs J.P.
      Ventricular septal defect.
      Postoperative residual ventricular septal defects, small or large, usually do not close spontaneously with time and have the capacity to negatively impact patients' lives by long-term decongestive pharmacologic therapy, risk of endocarditis, and problem of insurance coverage (persistent murmur). For these and many other reasons, it is imperative that the surgeon establishes excellent exposure of the ventricular septal defect to ensure accurate exposure and secure closure without the possible attendant complications of heart block and tricuspid regurgitation. The following drawings describe the circumferential and radial tricuspid valve incisions to optimally expose a difficult ventricular septal defect for secure patch closure.
      Figure thumbnail gr7
      Figure 7A perimembranous ventricular septal defect (circular dotted line) is represented behind the septal leaflet of the tricuspid valve and oftentimes extends toward the anterior leaflet. When complex chordal attachments are present, retraction and optimal exposure may result in chordal disruption and tricuspid insufficiency. It is for these reasons that a circular incision is performed to visualize the ventricular septal defect. Ao = aorta; CS = coronary sinus; IVC = inferior vena cava; PA = pulmonary artery; SVC = superior vena cava; TV = tricuspid valve; VSD = ventricular septal defect.
      Figure thumbnail gr8
      Figure 8The circumferential incision (circular dotted line) is performed to detach that portion of the tricuspid valve to optimally expose the ventricular septal defect, thereby avoiding the possibility of chordal rupture. Great care is taken to avoid injury to the subtended aortic valve leaflets, which are in close proximity to the projected tricuspid incision. Oftentimes, a dose of antegrade cardioplegia can help in identifying the aortic leaflets through this approach. CS = coronary sinus; VSD = ventricular septal defect.
      Figure thumbnail gr9
      Figure 9The tricuspid valve is retracted, which results in excellent exposure. Pledgeted sutures are placed around the edges of the defect with special care to avoid injury to the tricuspid valve, the aortic valve, and the conduction system. At least 2 pledgeted sutures are placed through the tricuspid annulus from the right atrial cavity to anchor the polytetrafluoroethylene patch. These sutures are then placed through the patch, which can be tied in place, thereby closing the ventricular septal defect.
      Figure thumbnail gr10
      Figure 10The closure of the ventricular septal defect is completed showing the tied pledgeted sutures anchoring the polytetrafluoroethylene patch in place.
      Figure thumbnail gr11
      Figure 11The tricuspid valve is reattached using the interrupted suture technique, which theoretically has the advantage of allowing annular growth.
      Figure thumbnail gr12
      Figure 12The completed ventricular septal defect using the circumferential tricuspid incision technique is shown.
      Figure thumbnail gr13
      Figure 13A perimembranous ventricular septal defect (circular dotted line) is represented behind the septal leaflet of the tricuspid valve. Occasionally, the tricuspid chordae are anatomically organized in such a manner that retraction will not offer appropriate exposure or, worse still, attempted retraction for exposure will result in chordal rupture and tricuspid regurgitation. Under these circumstances, a radial incision can be performed with special reference to the aortic valve and the conduction system. When the anatomy is not clear, the surgeon can elect to administer antegrade cardioplegia to demonstrate the aortic leaflet through the ventricular septal defect. An 11 blade can be used to incise the valve to the tricuspid annulus as shown in the drawing (interrupted line). This maneuver allows excellent exposure of the ventricular septal defect. CS = coronary sinus; VSD = ventricular septal defect.
      Figure thumbnail gr14
      Figure 14Interrupted pledgeted sutures are placed around the edges of the ventricular septal defect with great care to avoid injury to the tricuspid valve and its related chordae, the aortic valve, and the conduction system, which is located in the triangle of Koch (not displayed). These sutures are then placed in the polytetrafluoroethylene patch.
      Figure thumbnail gr15
      Figure 15The polytetrafluoroethylene patch is tied in place, which is facilitated by the exposure afforded by the radial incision in the septal leaflet of the tricuspid valve.
      Figure thumbnail gr16
      Figure 16The septal leaflet of the tricuspid valve is repaired by individual, interrupted fine sutures. A cold saline-filled bulb syringe can be used to float the tricuspid leaflets to test for competency of the valve.
      Figure thumbnail gr17
      Figure 17(A) A transected aortic root demonstrates a normal left main coronary artery and an abnormal intramural course of the right coronary artery, which takes its origin from the left coronary cusp, traverses intramurally (within the wall of the aorta) toward the right coronary cusp, and emerges there to perfuse the heart. Oftentimes these intramural segments are stenotic and can cause ischemic symptoms at rest or during exercise. Occasionally, the first symptom may be cardiac arrest, which can lead to death if not appropriately treated. (B) The unroofing procedure is performed along the length of the intramural course. The dissection ends where the artery emerges from the aorta to supply the heart. In the majority of cases, the incised and unroofed segment is very thick and may require a localized endarterectomy to fully unroof the coronary artery course. (C) The tacking sutures are placed at the neo-orifice and serve to reattach the intimal layers, thereby preventing dissection and thrombosis. We employ interrupted 8-0 Prolene (Polypropylene Suture; Ethicon, Inc, Somerville, NJ) for this purpose and have found the result to be satisfactory. LCA = left coronary artery; RCA = right coronary artery.
      Figure thumbnail gr18
      Figure 18(A) The same anatomy is shown as in except that the course of the intramural right coronary artery is more proximal to the aortic annulus (upstream) and traverses the intersinus distance below the commissure. This obviously contraindicates the operation that is shown in because it will involve an unwanted incision across the aortic leaflets. (B) The unroofing and endarterectomy procedures can be accomplished on both sides of the commissure without injury to the aortic leaflets. (C) The tacking sutures are placed to prevent intimal dissection. LCA = left coronary artery; RCA = right coronary artery.
      Figure thumbnail gr19
      Figure 19(A) A transected aortic root demonstrates a normal right coronary artery and an abnormal intramural course of the left main coronary artery, which takes its origin from the right coronary cusp, traverses intramurally (within the wall of the aorta) toward the left coronary cusp, and emerges there to perfuse the heart. (B) The unroofing procedure is performed along the length of the intramural course. The dissection ends where the artery emerges from the aorta to supply the heart. In the majority of cases, the incised and unroofed segment is very thick and may require a localized endarteretomy to fully unroof the coronary artery course. (C) The tacking sutures (8-0 Prolene) are placed at the neo-orifice and serve to reattach the intimal layers, thereby preventing dissection and thrombosis. LCA = left coronary artery; RCA = right coronary artery.
      Figure thumbnail gr20
      Figure 20(A) The same anatomy is shown as in except that the course of the intramural left coronary artery is more proximal to the aortic annulus (upstream) and traverses the intersinus distance below the commissure. This obviously contraindicates the operation that is shown in because it will involve an unwanted incision across the aortic leaflets. (B) The unroofing and endarteretomy procedures can be accomplished on both sides of the commissure without injury to the aortic leaflets. (C) The tacking sutures are placed to prevent intimal dissection. LCA = left coronary artery; RCA = right coronary artery.
      Figure thumbnail gr21
      Figure 21(A) The anomalous origin and pathway of the left main coronary artery is depicted. The origin is within the right coronary cusp adjacent to the takeoff of the right coronary artery. The artery is elongated and courses to the right and posterior of the aorta to emerge at the usual location and origin of the left main coronary artery near the left coronary cusp. Note that the course of the coronary artery is not intramural and the usual unroofing approach as described in the previous figures is not indicated. (B) Instead, we have chosen to perform side-to-side anastomoses for these abnormal coronary artery courses. (C) This procedure succeeds in a neo-orifice formation more closely related to the normal coronary artery anatomy. a = artery; LAD = left anterior descending coronary artery; LCA = left coronary artery; RCA = right coronary artery.

      Anomalous Aortic Origin of the Coronary Artery

      Anomalous aortic origin of the coronary artery has been associated with myocardial ischemic syndromes and sudden death. Although some authors
      • Boissier F.
      • Coolen N.
      • Nataf P.
      • et al.
      Sudden death related to an anomalous origin of the right coronary artery.
      advocate coronary artery bypass as treatment for this problem, others
      • Tavaf-Motamen H.
      • Bannister S.P.
      • Corcoran P.C.
      • et al.
      Repair of anomalous origin of right coronary artery from the left sinus of Valsalva.
      note the high incidence of coronary bypass occlusion due to competitive flow from the native coronary artery. Pulmonary translocation had been advocated by Rodefeld and associates to relieve the potential extrinsic pulmonary artery pressure on the anomalous coronary artery,
      • Rodefeld M.D.
      • Culbertson C.B.
      • Rosenfeld H.M.
      • et al.
      Pulmonary artery translocation: A surgical option for complex anomalous coronary artery anatomy.
      but Gulati and coworkers later modified this approach to include pulmonary artery translocation only for single coronary ostium without an intramural course; intramural coronary artery unroofing was applied to all patients with intramural courses.
      • Gulati R.
      • Reddy V.M.
      • Culbertson C.
      • et al.
      Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches.
      Recent reports document the high incidence of intramural courses, commissural takeoffs, and acute angulation of these anomalous coronary arteries, all of which can be associated with significant intermittent coronary obstruction.
      • Romp R.L.
      • Herlong J.R.
      • Landolfo C.K.
      • et al.
      Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery.
      • Jaggers J.
      • Lodge A.J.
      Surgical therapy for anomalous aortic origin of the coronary arteries.
      Herein we describe the different techniques that we have used to establish unobstructed pathways for some of these anomalous coronary arteries.

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