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Research Article| Volume 13, ISSUE 1, P35-39, March 2008

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Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

  • Tom R. Karl
    Correspondence
    Address reprint requests to Tom R. Karl, MS, MD, Professor of Surgery, UCSF School of Medicine, Room S-549, 513 Parnassus Avenue, San Francisco, CA 84143.
    Affiliations
    Division of Pediatric Cardiothoracic Surgery, University of California San Francisco School of Medicine, San Francisco, California.
    Search for articles by this author
      The most commonly reported coronary artery malformation leading to sudden death in children and young athletes is an anomalous coronary origin from the wrong aortic sinus, with or without a proximal course of the coronary artery between the aorta and the pulmonary artery. Although the incidence is difficult to assess, there is a 0.17% incidence in autopsy series (all variants included) and a 0.1 to 0.3% in catheter-echo diagnostic series. There may be a regional variation. In a prospective study of 1950 consecutive patients undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in 0.92% of cases, whereas the incidence of left coronary artery arising from the right aortic sinus was 0.15%.
      • Angelini P.
      Coronary artery anomalies—current clinical issues: definitions, classification, incidence, clinical relevance, and treatment guidelines.
      In a study of 2388 patients who had transthoracic echocardiographic examination of their proximal coronary anatomy as part of innocent murmur investigation and evaluation of performance status, four patients (0.2%) were found to have an anomalous origin of the coronary artery from the wrong sinus.
      • Davis J.A.
      • Cecchin F.
      • Jones T.K.
      • et al.
      Major coronary artery anomalies in a pediatric population: incidence and clinical importance.
      Whatever the true prevalence, this lesion predisposes to fatal episodes of myocardial ischemia that may occur during or shortly after strenuous exercise.
      • Basso C.
      • Corrado D.
      • Thiene G.
      Congenital coronary artery anomalies as an important cause of sudden death in the young.
      • Basso C.
      • Maron B.J.
      • Corrado D.
      • et al.
      Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
      • Liberthson R.R.
      Sudden death from cardiac causes in children and young adults.
      Timely diagnosis requires a high index of suspicion but is frequently impossible, as the majority of patients with the anomaly are asymptomatic and thus do not seek medical attention. Therefore, sudden death is frequently the first manifestation, especially in young athletes, with only 30% of patients reporting prodromal symptoms.
      • Liberthson R.R.
      Sudden death from cardiac causes in children and young adults.
      • Maron B.J.
      • Shirani J.
      • Poliac L.C.
      • et al.
      Sudden death in young competitive athletes: clinical, demographic, and pathological profiles.
      Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and false-negative results; echocardiogram, echocardiography, and stress testing all lack predictive value.
      • Basso C.
      • Maron B.J.
      • Corrado D.
      • et al.
      Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes.
      • Liberthson R.R.
      Sudden death from cardiac causes in children and young adults.
      Finally, although the major association of sudden death is with young athletes, cases have also been reported in children under 1 year of age.

      Operative Technique

      Figure thumbnail gr1
      Figure 1Sudden death from myocardial ischemia has been observed most commonly with the left coronary artery (LCA) from the right sinus but is also seen in the right coronary artery (RCA) from the left sinus, and LCA from the noncoronary sinus.
      • Alphonso N.
      • Anagnostopoulos P.V.
      • Nölke L.
      • et al.
      Anomalous coronary artery from the wrong sinus of Valsalva: a physiologic repair strategy.
      Several mechanisms have been proposed to explain the pathophysiology of acute myocardial ischemia with anomalous coronary artery origin from the wrong sinus, including the following: (1) flap closure of the slit-like opening of the coronary orifice; (2) acute (nonorthogonal) branching angle and kinking of the coronary artery as it exits the aorta; (3) intramural segment of the proximal coronary artery; (4) compression of the intramural segment by the aortic commissure; (5) compression of the coronary artery as it courses between the aorta and the pulmonary artery, accentuated by exercise-related expansion of the pulmonary artery; (6) spasm of the coronary artery as the result of endothelial injury.
      • Angelini P.
      Coronary artery anomalies—current clinical issues: definitions, classification, incidence, clinical relevance, and treatment guidelines.
      • Davis J.A.
      • Cecchin F.
      • Jones T.K.
      • et al.
      Major coronary artery anomalies in a pediatric population: incidence and clinical importance.
      The only effective treatment for this problem is surgery, and various techniques have been described, each having a role in selected cases.
      • Ono M.
      • Brown D.A.
      • Wolf R.K.
      Two cases of anomalous origin of LAD from right coronary artery requiring coronary artery bypass.
      • Garcia-Rinaldi R.
      Right coronary arteries that course between aorta and pulmonary artery.
      • Rodefeld M.D.
      • Culbertson C.B.
      • Rosenfeld H.M.
      • et al.
      Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy.
      We present herein a universal surgical strategy that could be applied for all variants of this disease, irrespective of coronary ostial configuration, proximity of aortic valve, or other features. There is no risk to the aortic commissures or valve leaflets, and all of the pathophysiological mechanisms, as we understand them, may be addressed. Ao = aorta; L = left; MPA = main pulmonary artery; N = non; R = right.
      Figure thumbnail gr2
      Figure 2Repair is performed through a median sternotomy. An autologous pericardial patch is procured during opening and fixed in 0.2% glutaraldehyde for 5 minutes, followed by a 10-minute saline rinse. The patient is placed on cardiopulmonary bypass using aortic and bicaval cannulation and a left ventricular vent and cooled to 32°C. The aorta is clamped and the heart is arrested with aortic root cold blood cardioplegia, or with a combination of antegrade and retrograde cardioplegia in cases known to have ostial stenosis. The aorta is transected. Beginning from the cut edge of the aorta, an incision is made into the ostium of the anomalous coronary and extended into the coronary itself for about 1 cm, either stopping short of the bifurcation or continuing into the larger branch. A triangular patch of the glutaraldehyde-treated autologous pericardium is sutured into this incision to enlarge the diameter of the proximal coronary artery trunk, creating an ostium of about 5 mm.
      • Patel K.
      • Davidson A.
      • Karl T.R.
      Anomalous left coronary artery arising from the right coronary cusp.
      The aortic anastomosis is completed, incorporating the base of the triangular pericardial patch into the aortic anastomotic suture line. The heart is then deaired, and the cross-clamp is removed. L = left; LCA = left coronary artery; R = right; RCA = right coronary artery.
      Figure thumbnail gr3
      Figure 3In patients in whom the anomalous coronary courses between the aorta and the pulmonary artery, the main pulmonary artery is transected just proximally to its bifurcation. The incision is carried into the left pulmonary artery branch. The main pulmonary artery is anastomosed to the left pulmonary artery. The defects at the bifurcation and in the origin of the right pulmonary artery are closed with a second pericardial patch to avoid stenosis. The goal of this translocation is to move the pulmonary artery away from the aorta, thereby reducing the chance for compression of the anomalous coronary artery as it courses between the aorta and pulmonary artery.
      • Rodefeld M.D.
      • Culbertson C.B.
      • Rosenfeld H.M.
      • et al.
      Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy.
      The patient is warmed fully and weaned from cardiopulmonary bypass, usually with good contractility of both ventricles, unless ischemic injury has occurred before operation. Ao = aorta; LCA = left coronary artery; MPA = main pulmonary artery.

      Comment

      Our technique enlarges the slit-like ostium and augments the diameter of the proximal coronary, while improving the acute angulation at takeoff. The technique can be used even when there is a common origin of the two coronary arteries. Finally, the pulmonary artery translocation increases the anatomic space between the arterial trunks and eliminates the risk of coronary artery compression. We have used this technique in seven patients (age 2 years to 52 years). One patient, who presented in cardiogenic shock, required temporary left ventricular assist device support postoperatively. All patients are symptom free at a mean follow-up interval of 29 months (range, 4 to 85 months). Laminar flow through the new ostium was seen with color Doppler or two-dimensional echo in all cases examined (6/7) and all patients in this study group have had normal echocardiogram stress echo, with no wall motion defects. There have been no reoperations nor late deaths, with all patients enjoying unrestricted activities.
      • Alphonso N.
      • Anagnostopoulos P.V.
      • Nölke L.
      • et al.
      Anomalous coronary artery from the wrong sinus of Valsalva: a physiologic repair strategy.

      Conclusions

      In conclusion, in cases of anomalous coronary artery from the wrong aortic sinus, with a proximal course between the aorta and the pulmonary artery, the combination of coronary artery angioplasty and translocation of the pulmonary artery represents a physiologic repair strategy that effectively addresses all the mechanisms that can generate ischemia. The technique is simple and effective and has shown good midterm results.

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        Major coronary artery anomalies in a pediatric population: incidence and clinical importance.
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