Right coronary artery arising within an aorta-to-left ventricle tunnel: surgical repair in a neonate

Right coronary artery arising within an aorta-to-left ventricle tunnel: surgical repair in a neonate Abstract We herein describe the successful surgical repair of a very rare combination of an aorta-to-left ventricle tunnel with the right coronary artery arising from it. The neonate presented with signs of heart failure due to significant regurgitation of blood via the tunnel. The closure of the tunnel was feasible during neonatal period without patches. Aorta-to-left ventricle tunnel, Aorta, Surgical technique CLINICAL SUMMARY A 3-week-old boy was referred for an assessment of a cardiac murmur fortuitously diagnosed. He was born at term with a birth weight of 2990 g. The pregnancy was complicated due to gestational diabetes requiring insulin. The patient did not have family history of congenital heart disease and genetic disorders. Cardiac examination revealed a loud systolic and diastolic precordial murmur. The liver edge was palpable 1.5 cm below the costal margin. Electrocardiogram showed sinus rhythm, left ventricular hypertrophy with strain pattern and inferolateral T-wave inversion. Transthoracic echocardiogram demonstrated a large tunnel arising from the right coronary sinus anteriorly and entering the left ventricle just below the aortic valve annulus. Severe flow reversal in the descending aorta was observed. The LV was severely dilated (LV end-diastolic diameter 3 cm, Z-score +4.2; LV end-systolic diameter 2.3 cm, Z-score +5.3) with moderately reduced systolic function. The ascending aorta was also severely dilated (diameter 1.4 cm, Z-score +3.4). Aortic valve regurgitation was not observed. The left coronary artery appeared normal. The right coronary artery could not be demonstrated. Intraoperative epicardial echocardiography confirmed these findings demonstrating an aorta-to-LV tunnel (Fig. 1). Surgical closure of the fistula was performed via median sternotomy with cardiopulmonary bypass. Aortic cross-clamp was applied, and antegrade cardioplegia was administered into the aortic root. The tunnel was externally occluded to allow good filling of the coronary arteries and prompt cardiac arrest. The tunnel was transversally opened as was the aorta. The right coronary artery ostium was laid within the aortic opening of the tunnel (Fig. 2A). The right coronary artery diameter was approximately 2 mm. The tunnel wall was used to close both aortic and ventricular orifices (Fig. 2B). Aortic cross-clamp time was 27 min. Cardiopulmonary bypass time was 45 min. Postoperative course was uneventful. The postoperative echocardiogram demonstrated good flow in the right coronary artery, a competent aortic valve and good biventricular function. Figure 1: View largeDownload slide Preoperative echocardiogram showing the Ao-to-left ventricular outflow tract tunnel from the right coronary sinus in (A) the long-axis view and (B) short-axis view with good flow in the LCA. *Surgical glove filled with water for better visualization of the tunnel. Ao: aorta; LCA: left coronary artery. Figure 1: View largeDownload slide Preoperative echocardiogram showing the Ao-to-left ventricular outflow tract tunnel from the right coronary sinus in (A) the long-axis view and (B) short-axis view with good flow in the LCA. *Surgical glove filled with water for better visualization of the tunnel. Ao: aorta; LCA: left coronary artery. Figure 2: View largeDownload slide A schematic representation of (A) the location of the fistula and (B) the technique of surgical repair. Ao: aorta; LCA: left coronary artery; LVOT: left ventricular outflow tract; RCA: right coronary artery; RVOT: right ventricular outflow tract. Figure 2: View largeDownload slide A schematic representation of (A) the location of the fistula and (B) the technique of surgical repair. Ao: aorta; LCA: left coronary artery; LVOT: left ventricular outflow tract; RCA: right coronary artery; RVOT: right ventricular outflow tract. DISCUSSION Aorta-to-LV tunnels are very rare, constituting 0.1% of congenitally malformed hearts [1]. Origin of the right coronary artery from the aorta-to-LV tunnel is extremely rare. Extensive debates on the differential diagnosis with coronary arterial fistulas have occured. The key to differentiation is the fact that aorta-to-LV tunnels bypass the ventriculoarterial junction without penetrating the septal musculature [2]. Interestingly, in a recent review on children with coronary arterial fistulas operated in our institution, none had a fistula opening to the left ventricle [3]. The majority of the children with aorta-to-LV tunnel have sufficiently stable haemodynamics. Thus, surgical repair is usually performed beyond the neonatal period [1–3]. Rarely, the regurgitation volume via the tunnel is such that surgery is required in the neonatal period. Although rarely required, the successful surgical repair of an aorta-to-LV tunnel has been reported in neonates immediately after birth [4]. Development of aortic regurgitation requiring aortic valve repair or even replacement would be of a major concern in neonatal repairs. Apart from closing the connection between the aorta and left ventricle, surgical repair must provide support to the aortic leaflet, avoiding its distortion. Similarly, preservation of the coronary circulation in a neonate is of paramount importance. Successful surgical repair with the insertion of a patch at the aortic opening has been reported in a neonate [4]. However, such patch closure of the aortic opening would leave a blind-ending communication with the LV outflow tract that may result in right ventricular outflow tract obstruction. To avoid late compression of the right ventricular outflow by residual high pressure in the LV blind-ending pouch [5], insertion of a second patch at the ventricular opening has been advocated. Recurrence of the tunnel has been reported after direct closure of only the aortic opening of the tunnel [1]. Thus, we believe that the complete division of the tunnel with direct closure of both aortic and ventricular openings, as described herein, not only restores normal anatomy but also eliminates the risk of recurrence, endocarditis or right ventricular outflow tract obstruction. This technique is simple and anatomical, obviating the need for patching. Conflict of interest: none declared. REFERENCES 1 Okoroma EO, Perry LW, Scott LP, McClenathan JE. Aortico-left ventricular tunnel. Clinical profile, diagnostic features, and surgical consideration. J Thorac Cardiovasc Surg  1976; 71: 238– 44. Google Scholar PubMed  2 McKay R, Anderson RH, Cook AC. The aorto-ventricular tunnels. Cardiol Young  2002; 12: 563– 80. Google Scholar CrossRef Search ADS PubMed  3 Yim D, Yong MS, d'Udekem Y, Brizard CP, Konstantinov IE. Early surgical repair of the coronary artery fistulae in children: 30 years of experience. Ann Thorac Surg  2015; 100: 188– 94. Google Scholar CrossRef Search ADS PubMed  4 Nakamura Y, Miyaji K, Yoshii T, Ootomo Y, Kimura S. Aorto-left ventricular tunnel successfully repaired immediately after birth. Ann Thorac Surg  2016; 101: 1988– 90. Google Scholar CrossRef Search ADS PubMed  5 Knott-Craig CJ, van der Merwe PL, Kalis NN, Hunter J. Repair of aortico-left ventricular tunnel associated with subpulmonary obstruction. Ann Thorac Surg  1992; 54: 557– 9. Google Scholar CrossRef Search ADS PubMed  © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Interactive CardioVascular and Thoracic Surgery Oxford University Press

Right coronary artery arising within an aorta-to-left ventricle tunnel: surgical repair in a neonate

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Oxford University Press
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© The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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1569-9293
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1569-9285
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10.1093/icvts/ivy104
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Abstract

Abstract We herein describe the successful surgical repair of a very rare combination of an aorta-to-left ventricle tunnel with the right coronary artery arising from it. The neonate presented with signs of heart failure due to significant regurgitation of blood via the tunnel. The closure of the tunnel was feasible during neonatal period without patches. Aorta-to-left ventricle tunnel, Aorta, Surgical technique CLINICAL SUMMARY A 3-week-old boy was referred for an assessment of a cardiac murmur fortuitously diagnosed. He was born at term with a birth weight of 2990 g. The pregnancy was complicated due to gestational diabetes requiring insulin. The patient did not have family history of congenital heart disease and genetic disorders. Cardiac examination revealed a loud systolic and diastolic precordial murmur. The liver edge was palpable 1.5 cm below the costal margin. Electrocardiogram showed sinus rhythm, left ventricular hypertrophy with strain pattern and inferolateral T-wave inversion. Transthoracic echocardiogram demonstrated a large tunnel arising from the right coronary sinus anteriorly and entering the left ventricle just below the aortic valve annulus. Severe flow reversal in the descending aorta was observed. The LV was severely dilated (LV end-diastolic diameter 3 cm, Z-score +4.2; LV end-systolic diameter 2.3 cm, Z-score +5.3) with moderately reduced systolic function. The ascending aorta was also severely dilated (diameter 1.4 cm, Z-score +3.4). Aortic valve regurgitation was not observed. The left coronary artery appeared normal. The right coronary artery could not be demonstrated. Intraoperative epicardial echocardiography confirmed these findings demonstrating an aorta-to-LV tunnel (Fig. 1). Surgical closure of the fistula was performed via median sternotomy with cardiopulmonary bypass. Aortic cross-clamp was applied, and antegrade cardioplegia was administered into the aortic root. The tunnel was externally occluded to allow good filling of the coronary arteries and prompt cardiac arrest. The tunnel was transversally opened as was the aorta. The right coronary artery ostium was laid within the aortic opening of the tunnel (Fig. 2A). The right coronary artery diameter was approximately 2 mm. The tunnel wall was used to close both aortic and ventricular orifices (Fig. 2B). Aortic cross-clamp time was 27 min. Cardiopulmonary bypass time was 45 min. Postoperative course was uneventful. The postoperative echocardiogram demonstrated good flow in the right coronary artery, a competent aortic valve and good biventricular function. Figure 1: View largeDownload slide Preoperative echocardiogram showing the Ao-to-left ventricular outflow tract tunnel from the right coronary sinus in (A) the long-axis view and (B) short-axis view with good flow in the LCA. *Surgical glove filled with water for better visualization of the tunnel. Ao: aorta; LCA: left coronary artery. Figure 1: View largeDownload slide Preoperative echocardiogram showing the Ao-to-left ventricular outflow tract tunnel from the right coronary sinus in (A) the long-axis view and (B) short-axis view with good flow in the LCA. *Surgical glove filled with water for better visualization of the tunnel. Ao: aorta; LCA: left coronary artery. Figure 2: View largeDownload slide A schematic representation of (A) the location of the fistula and (B) the technique of surgical repair. Ao: aorta; LCA: left coronary artery; LVOT: left ventricular outflow tract; RCA: right coronary artery; RVOT: right ventricular outflow tract. Figure 2: View largeDownload slide A schematic representation of (A) the location of the fistula and (B) the technique of surgical repair. Ao: aorta; LCA: left coronary artery; LVOT: left ventricular outflow tract; RCA: right coronary artery; RVOT: right ventricular outflow tract. DISCUSSION Aorta-to-LV tunnels are very rare, constituting 0.1% of congenitally malformed hearts [1]. Origin of the right coronary artery from the aorta-to-LV tunnel is extremely rare. Extensive debates on the differential diagnosis with coronary arterial fistulas have occured. The key to differentiation is the fact that aorta-to-LV tunnels bypass the ventriculoarterial junction without penetrating the septal musculature [2]. Interestingly, in a recent review on children with coronary arterial fistulas operated in our institution, none had a fistula opening to the left ventricle [3]. The majority of the children with aorta-to-LV tunnel have sufficiently stable haemodynamics. Thus, surgical repair is usually performed beyond the neonatal period [1–3]. Rarely, the regurgitation volume via the tunnel is such that surgery is required in the neonatal period. Although rarely required, the successful surgical repair of an aorta-to-LV tunnel has been reported in neonates immediately after birth [4]. Development of aortic regurgitation requiring aortic valve repair or even replacement would be of a major concern in neonatal repairs. Apart from closing the connection between the aorta and left ventricle, surgical repair must provide support to the aortic leaflet, avoiding its distortion. Similarly, preservation of the coronary circulation in a neonate is of paramount importance. Successful surgical repair with the insertion of a patch at the aortic opening has been reported in a neonate [4]. However, such patch closure of the aortic opening would leave a blind-ending communication with the LV outflow tract that may result in right ventricular outflow tract obstruction. To avoid late compression of the right ventricular outflow by residual high pressure in the LV blind-ending pouch [5], insertion of a second patch at the ventricular opening has been advocated. Recurrence of the tunnel has been reported after direct closure of only the aortic opening of the tunnel [1]. Thus, we believe that the complete division of the tunnel with direct closure of both aortic and ventricular openings, as described herein, not only restores normal anatomy but also eliminates the risk of recurrence, endocarditis or right ventricular outflow tract obstruction. This technique is simple and anatomical, obviating the need for patching. Conflict of interest: none declared. REFERENCES 1 Okoroma EO, Perry LW, Scott LP, McClenathan JE. Aortico-left ventricular tunnel. Clinical profile, diagnostic features, and surgical consideration. J Thorac Cardiovasc Surg  1976; 71: 238– 44. Google Scholar PubMed  2 McKay R, Anderson RH, Cook AC. The aorto-ventricular tunnels. Cardiol Young  2002; 12: 563– 80. Google Scholar CrossRef Search ADS PubMed  3 Yim D, Yong MS, d'Udekem Y, Brizard CP, Konstantinov IE. Early surgical repair of the coronary artery fistulae in children: 30 years of experience. Ann Thorac Surg  2015; 100: 188– 94. Google Scholar CrossRef Search ADS PubMed  4 Nakamura Y, Miyaji K, Yoshii T, Ootomo Y, Kimura S. Aorto-left ventricular tunnel successfully repaired immediately after birth. Ann Thorac Surg  2016; 101: 1988– 90. Google Scholar CrossRef Search ADS PubMed  5 Knott-Craig CJ, van der Merwe PL, Kalis NN, Hunter J. Repair of aortico-left ventricular tunnel associated with subpulmonary obstruction. Ann Thorac Surg  1992; 54: 557– 9. Google Scholar CrossRef Search ADS PubMed  © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

Interactive CardioVascular and Thoracic SurgeryOxford University Press

Published: Mar 26, 2018

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