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of the feeding artery [5]. Meanwhile, systemic artery-topulmonary vein fistulae without lung sequestration are rare and are usually diagnosed at an early age [5]. Aberrant vessels originating from the aortic arch and descending aorta have not been reported in adult patients, to our knowledge. Systemic artery-topulmonary vein fistulae could be treated with ligation or percutaneous closure of the fistulous tract. Because the two aorta-to-pulmonary vein fistulae in our patient were narrow and tortuous enough to enable the performance of percutaneous procedures, we successfully occluded the fistulae by performing coil embolization. The diagnosis of multiple systemic artery-topulmonary vein fistulae is difficult, and developing a therapeutic strategy is important. Multimodality imaging is crucial for diagnosing multiple fistulae. CT or magnetic resonance imaging is useful in determining the entire course of the tract, the morphologic size and complexities of the fistula, and the presence of associated lung sequestration. The treatment plan for multiple aorta-topulmonary vein fistulae should be reasonable and tailored for patient-specific conditions. As a result of the mutual exchange of opinions between the cardiologist, cardiac surgeon, and radiologist, the sequential treatment of the multiple systemic artery-to-pulmonary vein fistulae in our patient was successful.

References

Anomalous Aortic Origin of the Left Coronary Artery From the Right Coronary Sinus: Diagnosis and Surgical Repair of Intramural Retrovalvular Coronary Artery Mangesh Jadhav, MD, Andreas Pflaumer, MD, Phillip S. Naimo, MD, and Igor E. Konstantinov, MD, PhD Departments of Cardiothoracic Surgery and Cardiology, Royal Children’s Hospital, Murdoch Children’s Research Institute, and University of Melbourne, Melbourne, Victoria, Australia

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coronary artery could be difficult due to its intramural course and proximity to the aortic valve. We described the surgical management of a child after sudden hemodynamic collapse and the coronary translocation technique that prevented distortion of the aortic root and allowed successful translocation of the abnormal coronary artery despite its intramural course immediately behind the aortic valve commissure. (Ann Thorac Surg 2015;100:2357–9) Ó 2015 by The Society of Thoracic Surgeons

A

nomalous aortic origin of a coronary artery (AAOCA) is a rare anomaly that accounts for approximately 0.2% of cardiac malformations [1] and may result in sudden cardiac death [2, 3]. A large proportion of AAOCA also have an interarterial or intramural course, or both [2]. Although an anomalous right CA from the left coronary sinus has been described more often, an anomalous left CA (LCA) from the right coronary sinus has a much greater risk of sudden death [3]. A healthy 10-year-old girl collapsed suddenly while running. She was resuscitated at a peripheral hospital, had ST depression in the inferolateral leads, moderately reduced left ventricular (LV) systolic function and increased lactate (6.3 mmol/L). She was urgently transferred to the intensive care unit of the Royal Children’s Hospital with stable vital signs on adrenalin (0.15 mg/kg/min) and O2 at 4 L/min. Her electrocardiogram showed frequent premature ventricular contractions, short runs of nonsustained ventricular tachycardia, and persistent ischemic changes. A transthoracic echocardiogram demonstrated minimal LV dilatation and preserved systolic function with no obvious wall motion abnormality. She had cardiac arrest and was emergently placed onto extracorporeal membrane oxygenation support. Cardiac catheterization was performed, right ventricular (RV) biopsy specimen was normal, and the initial coronary angiogram appeared normal, with good flow in all coronary arteries. Owing to elevated troponin and a high index of suspicion of a coronary anomaly, a focused transthoracic echocardiogram was performed on extracorporeal membrane oxygenation and demonstrated the anomalous origin of LCA from the right coronary sinus with an interarterial course. This was confirmed on a cardiac computed tomography angiogram (Fig 1). The intramural segment of the left main CA was too short for unroofing in a way that a neoostium could be created far enough into the left coronary sinus to allow commissural resuspension. Intraoperatively, the intramural LCA was exposed by detaching the aortic valve commissure (Fig 2A and B). The aortic wall of the neoleft coronary sinus was Accepted for publication Feb 12, 2015.

Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct translocation of the left Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

Address correspondence to Dr Konstantinov, Royal Children’s Hospital, Flemington Rd, Parkville, Victoria 3029, Australia; e-mail: igor. [email protected].

0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.107

FEATURE ARTICLES

1. Tachibana M, Mukouhara N, Hirami R, et al. Double congenital fistulae with aneurysm diagnosed by combining imaging modalities. Acta Med Okayama 2013;67:305–9. 2. Dodge-Khatami A, Mavroudis C, Backer CL. Congenital heart surgery nomenclature and database project: anomalies of the coronary arteries. Ann Thorac Surg 2000;69:S270–97. 3. Mangukia CV. Coronary artery fistula. Ann Thorac Surg 2012;93:2084–92. 4. Mavroudis C, Backer CL, Rocchini AP, Muster AJ, Gevitz M. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63:1235–42. 5. Jariwala P, Ramesh G, Sarat Chandra K. Congenital anomalous/aberrant systemic artery to pulmonary venous fistula: closure with vascular plugs & coil embolization. Ind Heart J 2014;66:95–103.

CASE REPORT JADHAV ET AL AAOC OF LCA FROM RIGHT CORONARY SINUS

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CASE REPORT JADHAV ET AL AAOC OF LCA FROM RIGHT CORONARY SINUS

Ann Thorac Surg 2015;100:2357–9

Fig 1. Computed tomography shows (A and B) left coronary artery origin from the right coronary sinus and its proximal interarterial course and (C and D) its intramural course in relation to aortic the valve. (A and B) The venous cannula is in the right atrium.

partially resected and reimplanted into the right coronary sinus after the LCA transfer, and the aortic valve was reattached so that the normal anatomy of the aortic root was preserved (Fig 2C and D). The ostium of the LCA

FEATURE ARTICLES

Fig 2. (A) Illustration shows the origin and course of the anomalous left coronary artery (LCA) from the right coronary sinus. (B) Intraoperatively, the aortic valve cusp was detached, exposing a retrovalvular intramural course of the LCA. (C) This maneuver allowed unroofing of the intramural LCA. (C and D) The LCA button was then rotated approximately 60 degrees clockwise and was transposed to the left coronary sinus. (RCA ¼ right coronary artery.)

was enlarged, and the button was rotated to prevent kinking of the LCA. An intraoperative epicardial echocardiogram confirmed good flow into the LCA after translocation. Owing

Ann Thorac Surg 2015;100:2359–62

to poor LV function, she was supported with extracorporeal membrane oxygenation, which was weaned off in 2 days after normalization of the LV function. The patient has normal aortic valve function, no aortic insufficiency, is asymptomatic, and after normal results on an exercise test and Holter electrocardiogram monitoring, is involved in competitive swimming at 1 year after the operation.

Comment

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

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the anomalous CA has been described with good early results [3, 5]. Mainwaring and colleagues [3] described 6 patients who underwent CA reimplantation with no deaths. Similarly, Erez and colleagues [5] described 9 patients (mean age, 12 years; range, 4 months to 23 years) operated on between 2003 and 2005, of whom 7 patients underwent CA reimplantation and 2 patients underwent unroofing of the anomalous CA, with no deaths after a mean follow-up of 1 year. In the child described in this report, prompt medical attention prevented sudden death, and an echocardiogram was helpful in establishing the diagnosis that was confirmed by computed tomography images. The surgical technique described here allowed successful translocation of the abnormal CA despite its intramural course immediately behind the aortic valve commissure.

References 1. Davis JA, Cecchin F, Jones TK, Portman MA. Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 2001;37:593–7. 2. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a coronary artery. Ann Thorac Surg 2009;88:844–8. 3. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic origin of a coronary artery: mid-term results after surgical repair in 50 patients. Ann Thorac Surg 2011;92:691–7. 4. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a coronary artery: a single-centre experience. Ann Thorac Surg 2014;98:941–6. 5. Erez E, Tam VKH, Doublin NA, Stakes J. Anomalous coronary artery with aortic origin and course between the great arteries: improved diagnosis, anatomic findings, and surgical treatment. Ann Thorac Surg 2006;82:973–7. 6. Frommelt PC, Berger S, Pelech AN, Bergstrom S, Williamson JG. Prospective identification of anomalous origin of left coronary artery from the right sinus of Valsalva using transthoracic echocardiography: importance of colour Doppler flow mapping. Pediatr Cardiol 2001;22:327–32. 7. Thankavel PP, Balakrishnan P, Ramaciotti C. Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method. Pediatr Cardiol 2013;34:842–6.

Neonatal Repair in a Patient With Heterotaxy, Truncus Arteriosus, Pulmonary Artery Sling, and Tracheal Stenosis Petros V. Anagnostopoulos, MD, Eugene C. Kenny, PAC, Amy L. H. Peterson, MD, Scott A. Hagen, MD, and J. Scott McMurray, MD American Family Children’s Hospital, the University of Wisconsin Hospital and Clinics; Department of Surgery, University of Wisconsin School of Medicine and Public Health; Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin

We present a newborn with heterotaxy features, multiple congenital anomalies, truncus arteriosus with long segment tracheal stenosis, and a left pulmonary artery sling. The patient had complete neonatal repair with slide 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.105

FEATURE ARTICLES

The first manifestation of the AAOCA may be sudden death, although many patients may remain asymptomatic [2–4]. Several factors have been proposed to contribute to sudden death, including the intramural course, acute angle of take-off, systolic stretching, and compression of the anomalous CA between the great arteries during exercise [3, 4]. The electrocardiogram is often normal in asymptomatic patients, whereas in only 50% of symptomatic patients it is abnormal [3, 5]. The diagnosis can be made by careful transthoracic echocardiogram, yet is not always easy. A color Doppler examination improves the evaluation by identifying flow within the anomalous CA [6]. A parasternal long-axis sweep between aorta and pulmonary artery can visualize the proximal LCA and increases the accuracy when used in conjunction with parasternal short-axis view [7]. Cardiac computed tomography confirms the diagnosis. Surgical repair is recommended in all patients with an anomalous LCA from the right coronary sinus due to the increased risk of sudden death [2, 3]. In addition, symptomatic patients with an anomalous right CA from the left coronary sinus should undergo surgical repair, unless it is a nondominant anomalous right CA in an asymptomatic patient [2, 3]. Methods of repairing AAOCA may involve unroofing of the anomalous CA with an intramural course [2–4], direct reimplantation of the anomalous CA [3, 5], CA bypass grafting [2], or pulmonary artery translocation [3]. The unroofing procedure has been reported with good early results (2–4); however, the long-term outcomes are yet to be established. Experience with surgical repair of the AAOCA in children is limited. Sharma and colleagues [4] described 75 patients (median age, 46 years; range, 13 to 70 years) operated on between 1992 and 2011, all of whom underwent surgical unroofing of an AAOCA, with no early deaths, and 1 late death after mean follow-up of 1.5 years. Similarly, Mainwaring and colleagues [3] described 50 patients (median age, 14 years; range, 5 days to 47 years) operated on between 1999 and 2010, with 35 patients undergoing surgical unroofing. They reported no deaths after mean follow-up of 5.3 years. Unfortunately, simple unroofing of the intramural CA is not always feasible due to proximity of the aortic valve, as it was in our patient. Such a patient would require reimplantation of the CA after unroofing and reattachment of the aortic valve. The reimplantation is more technically difficult and has a risk of obstruction of the reimplanted CA due to kinking [2]. Reimplantation of

CASE REPORT ANAGNOSTOPOULOS ET AL TRUNCUS REPAIR AND SLIDE TRACHEOPLASTY

Anomalous Aortic Origin of the Left Coronary Artery From the Right Coronary Sinus: Diagnosis and Surgical Repair of Intramural Retrovalvular Coronary Artery.

Anomalous aortic origin of the left coronary artery from the right coronary sinus is a rare congenital anomaly that may cause sudden death. Direct tra...
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