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Repair of aorto-right ventricular tunnel with pulmonary stenosis and an anomalous origin of the left coronary artery

Repair of aorto-right ventricular tunnel with pulmonary stenosis and an anomalous origin of the... Abstract A 5-month-old infant with aorto-right ventricular tunnel, pulmonary stenosis and a sole supply of the left coronary artery from the tunnel underwent closure of the ventricular end of the tunnel, subsequent to balloon pulmonary valvuloplasty. Care was taken not to obstruct the aortic end of the tunnel in order to maintain the left coronary artery perfusion. Aorto-right ventricular tunnel, Left coronary artery 1 Introduction Aorto-right ventricular tunnel (ARVT) is a rare congenital communication between one of the aortic sinuses and the right ventricle. It may be accompanied by pulmonary stenosis and coronary abnormalities. We report a successful surgical correction of this complex malformation, diagnosed preoperatively with echocardiography and magnetic resonance imaging (MRI). 2 Case report The asymptomatic female full term infant was evaluated at the age of 2 weeks because of a heart murmur. Systolic and diastolic to and fro murmur grade III was noted along the left sternal border, there was no systolic click, the peripheral pulses were not bounding. Electrocardiogram (ECG) demonstrated mild right ventricular hypertrophy, chest X ray was normal. Echocardiography showed a thickened stenotic pulmonary valve with a maximum instantaneous gradient 75 mmHg, right ventricular hypertrophy and a large tunnel connecting the left coronary aortic sinus with the right ventricle. The origin of ARVT at the aortic aspect was narrow, followed by an aneurysmatic dilatation. The to and fro flow within ARVT revealed a 34 mmHg gradient from the aorta to the right ventricle and 5 mmHg gradient in opposite direction. Right coronary artery was dilated and originated normally, left coronary artery could not be visualized. Echocardiography and MRI demonstrated a very tiny vessel in the area where the left anterior descending (LAD) artery should be expected, but we could not demonstrate its origin from the left coronary sinus. A balloon pulmonary valvuloplasty has been performed at the age of 5 months, but closing the ARVT with coils was not performed due to an unsuitable anatomy of tunnel and because the tunnel origin of LAD could not be reliably excluded even by angiography. During cardiac surgery, the ARVT gave the appearance of a ‘third great artery’ arising from the heart (Fig. 1 ), connecting the anterior wall of the right ventricle with the left sinus of Valsalva. The right coronary artery was dilated. The LAD was running parallel to ARVT, with a small transverse communication in the middle of the tunnel. Because the left coronary artery originated obviously from ARVT, we decided to leave the aortic orifice of the tunnel open. The right ventricular orifice was closed with a patch and the tunnel diameter was reduced. The pulmonary valve was tricuspid and mildly stenotic. The postoperative recovery was uneventful. Ten months later, the baby is asymptomatic, with a systolic murmur grade 2/6 corresponding to echocardiographic findings of a mild valvar and some degree of supravalvar pulmonary stenosis with a maximum systolic gradient 45 mmHg. There is no residual communication between the ARVT and the right ventricle. Echocardiography demonstrated no aortic or pulmonary valve regurgitation. Both ventricles show normal systolic function and no ischemic changes are observed on ECG. Fig. 1 Open in new tabDownload slide Surgical view of the ARVT. AO, aorta; PA, pulmonary artery; ARVT, aorto-right ventricular tunnel. Fig. 1 Open in new tabDownload slide Surgical view of the ARVT. AO, aorta; PA, pulmonary artery; ARVT, aorto-right ventricular tunnel. 3 Discussion The first description of ARVT was published in 1961 [1]. The case reports on ARVT remain scarce – we found ten reports in the literature [2–10]. Our patient underwent surgical correction, even though it was our initial intention to perform the transcatheter closure of the ARVT along with a balloon valvuloplasty of the stenotic pulmonary valve. However, positioning the coils into the tunnel seemed to be unreliable and unsafe, the baby was too small for a safe using of Amplatzer duct occluder, and the origin of the hypoplastic left coronary artery from the tunnel could not be excluded even after selective contrast injection. Interestingly, in the literature five of the ten cases report on coexistent coronary anomalies [2,3,9,7,10]. This unusual form of aorto-ventricular communication in our patient highlights the importance of identifying all associated anomalies. The management could have been postponed beyond the neonatal period, because the presence of pulmonary stenosis prevented development of a heart failure. The ostium of a coronary artery may be malposed within the aortic sinus. In our patient, however, the communication with ARVT was the only supply of left coronary artery. It is therefore important not to interrupt the coronary artery flow by closing the aortic end of the tunnel. The same index of suspicion should be kept in mind, when a transcatheter treatment of this malformation is to be considered in the future. Reimplantation of the left coronary artery into the aorta with closure of the aortic tunnel entrance was considered impossible as the left coronary artery did not spring from the aorta but from the mid-part of the tunnel. For this reason it was not possible to create a left coronary artery with a cuff of aortic wall tissue that could be reimplanted in a safe fashion. Therefore, it was decided not to close the aortic opening of the tunnel in order to maintain coronary perfusion. The tunnel diameter was reduced surgically with an intention to prevent later aneurysmatic dilatation of the remaining portion of the tunnel. References [1] Houel J. , Raynaud R. , Morand P. , Pinet F. . Communication aorte-ventricule droit: à propos de 3 observations , Arch Mal Coeur Vaiss , 1961 , vol. 54 (pg. 1346 - 1356 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [2] Bharati S. , Lev M. . Aortico-right ventricular tunnel , Chest , 1973 , vol. 63 pg. 197 Google Scholar Crossref Search ADS WorldCat [3] Saylam A. , Tuncali T. , Ikizler C. , Aytac A. . Aorto-right ventricular tunnel , Ann Thorac Surg , 1974 , vol. 18 (pg. 634 - 637 ) Google Scholar Crossref Search ADS PubMed WorldCat [4] Jureidini S.B. , De Mello D. , Nouri S. , Kanter K. . Aortico-right ventricular tunnel with critical pulmonary stenosis: diagnosis by two-dimensional and Doppler echocardiography and angiography , Pediatr Cardiol , 1989 , vol. 10 (pg. 99 - 103 ) Google Scholar Crossref Search ADS PubMed WorldCat [5] Westaby S. , Archer N. . Aortico-right ventricular tunnel , Ann Thorac Surg , 1992 , vol. 53 (pg. 1107 - 1109 ) Google Scholar Crossref Search ADS PubMed WorldCat [6] Kleinkamp G. , Minami K. , Thies W.R. , Dohmann R. , Raute Kreinsen U. , Meyer H. , Korfer R. . Aorta-right ventricular tunnel with a rudimentary valve and an anomalous origin of the left coronary artery , J Thorac Cardiovasc Surg , 1992 , vol. 104 (pg. 1759 - 1760 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [7] Rosengart T.K. , Redel D.A. , Stark J.F. . Surgical repair of aorto-right ventricular tunnel in an infant , Ann Thorac Surg , 1993 , vol. 55 (pg. 520 - 522 ) Google Scholar Crossref Search ADS PubMed WorldCat [8] Vargas F.J. , Molina A. , Martinez J.C. , Ranzini M.A. , Vazquez J.C. . Aortico-right ventricular tunnel , Ann Thorac Surg , 1998 , vol. 66 (pg. 1793 - 1795 ) Google Scholar Crossref Search ADS PubMed WorldCat [9] v Son J.A.M. , Hambsch J. , Schneider P. , Mohr F.W. . Repair of aortico-right ventricular tunnel , Eur J Cardiothorac Surg , 1998 , vol. 14 (pg. 214 - 217 ) Google Scholar Crossref Search ADS PubMed WorldCat [10] Talwar S. , Choudhary U.J. , Kothary S.S. , Airan B. . Aortico-right ventricular tunnel , Int J Cardiol , 1999 , vol. 70 (pg. 201 - 205 ) Google Scholar Crossref Search ADS PubMed WorldCat © 2002 Elsevier Science B.V. All rights reserved. Elsevier Science B.V. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Cardio-Thoracic Surgery Oxford University Press

Repair of aorto-right ventricular tunnel with pulmonary stenosis and an anomalous origin of the left coronary artery

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Publisher
Oxford University Press
Copyright
© 2002 Elsevier Science B.V. All rights reserved.
Subject
Case report
ISSN
1010-7940
eISSN
1873-734X
DOI
10.1016/S1010-7940(02)00115-X
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Abstract

Abstract A 5-month-old infant with aorto-right ventricular tunnel, pulmonary stenosis and a sole supply of the left coronary artery from the tunnel underwent closure of the ventricular end of the tunnel, subsequent to balloon pulmonary valvuloplasty. Care was taken not to obstruct the aortic end of the tunnel in order to maintain the left coronary artery perfusion. Aorto-right ventricular tunnel, Left coronary artery 1 Introduction Aorto-right ventricular tunnel (ARVT) is a rare congenital communication between one of the aortic sinuses and the right ventricle. It may be accompanied by pulmonary stenosis and coronary abnormalities. We report a successful surgical correction of this complex malformation, diagnosed preoperatively with echocardiography and magnetic resonance imaging (MRI). 2 Case report The asymptomatic female full term infant was evaluated at the age of 2 weeks because of a heart murmur. Systolic and diastolic to and fro murmur grade III was noted along the left sternal border, there was no systolic click, the peripheral pulses were not bounding. Electrocardiogram (ECG) demonstrated mild right ventricular hypertrophy, chest X ray was normal. Echocardiography showed a thickened stenotic pulmonary valve with a maximum instantaneous gradient 75 mmHg, right ventricular hypertrophy and a large tunnel connecting the left coronary aortic sinus with the right ventricle. The origin of ARVT at the aortic aspect was narrow, followed by an aneurysmatic dilatation. The to and fro flow within ARVT revealed a 34 mmHg gradient from the aorta to the right ventricle and 5 mmHg gradient in opposite direction. Right coronary artery was dilated and originated normally, left coronary artery could not be visualized. Echocardiography and MRI demonstrated a very tiny vessel in the area where the left anterior descending (LAD) artery should be expected, but we could not demonstrate its origin from the left coronary sinus. A balloon pulmonary valvuloplasty has been performed at the age of 5 months, but closing the ARVT with coils was not performed due to an unsuitable anatomy of tunnel and because the tunnel origin of LAD could not be reliably excluded even by angiography. During cardiac surgery, the ARVT gave the appearance of a ‘third great artery’ arising from the heart (Fig. 1 ), connecting the anterior wall of the right ventricle with the left sinus of Valsalva. The right coronary artery was dilated. The LAD was running parallel to ARVT, with a small transverse communication in the middle of the tunnel. Because the left coronary artery originated obviously from ARVT, we decided to leave the aortic orifice of the tunnel open. The right ventricular orifice was closed with a patch and the tunnel diameter was reduced. The pulmonary valve was tricuspid and mildly stenotic. The postoperative recovery was uneventful. Ten months later, the baby is asymptomatic, with a systolic murmur grade 2/6 corresponding to echocardiographic findings of a mild valvar and some degree of supravalvar pulmonary stenosis with a maximum systolic gradient 45 mmHg. There is no residual communication between the ARVT and the right ventricle. Echocardiography demonstrated no aortic or pulmonary valve regurgitation. Both ventricles show normal systolic function and no ischemic changes are observed on ECG. Fig. 1 Open in new tabDownload slide Surgical view of the ARVT. AO, aorta; PA, pulmonary artery; ARVT, aorto-right ventricular tunnel. Fig. 1 Open in new tabDownload slide Surgical view of the ARVT. AO, aorta; PA, pulmonary artery; ARVT, aorto-right ventricular tunnel. 3 Discussion The first description of ARVT was published in 1961 [1]. The case reports on ARVT remain scarce – we found ten reports in the literature [2–10]. Our patient underwent surgical correction, even though it was our initial intention to perform the transcatheter closure of the ARVT along with a balloon valvuloplasty of the stenotic pulmonary valve. However, positioning the coils into the tunnel seemed to be unreliable and unsafe, the baby was too small for a safe using of Amplatzer duct occluder, and the origin of the hypoplastic left coronary artery from the tunnel could not be excluded even after selective contrast injection. Interestingly, in the literature five of the ten cases report on coexistent coronary anomalies [2,3,9,7,10]. This unusual form of aorto-ventricular communication in our patient highlights the importance of identifying all associated anomalies. The management could have been postponed beyond the neonatal period, because the presence of pulmonary stenosis prevented development of a heart failure. The ostium of a coronary artery may be malposed within the aortic sinus. In our patient, however, the communication with ARVT was the only supply of left coronary artery. It is therefore important not to interrupt the coronary artery flow by closing the aortic end of the tunnel. The same index of suspicion should be kept in mind, when a transcatheter treatment of this malformation is to be considered in the future. Reimplantation of the left coronary artery into the aorta with closure of the aortic tunnel entrance was considered impossible as the left coronary artery did not spring from the aorta but from the mid-part of the tunnel. For this reason it was not possible to create a left coronary artery with a cuff of aortic wall tissue that could be reimplanted in a safe fashion. Therefore, it was decided not to close the aortic opening of the tunnel in order to maintain coronary perfusion. The tunnel diameter was reduced surgically with an intention to prevent later aneurysmatic dilatation of the remaining portion of the tunnel. References [1] Houel J. , Raynaud R. , Morand P. , Pinet F. . Communication aorte-ventricule droit: à propos de 3 observations , Arch Mal Coeur Vaiss , 1961 , vol. 54 (pg. 1346 - 1356 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [2] Bharati S. , Lev M. . Aortico-right ventricular tunnel , Chest , 1973 , vol. 63 pg. 197 Google Scholar Crossref Search ADS WorldCat [3] Saylam A. , Tuncali T. , Ikizler C. , Aytac A. . Aorto-right ventricular tunnel , Ann Thorac Surg , 1974 , vol. 18 (pg. 634 - 637 ) Google Scholar Crossref Search ADS PubMed WorldCat [4] Jureidini S.B. , De Mello D. , Nouri S. , Kanter K. . Aortico-right ventricular tunnel with critical pulmonary stenosis: diagnosis by two-dimensional and Doppler echocardiography and angiography , Pediatr Cardiol , 1989 , vol. 10 (pg. 99 - 103 ) Google Scholar Crossref Search ADS PubMed WorldCat [5] Westaby S. , Archer N. . Aortico-right ventricular tunnel , Ann Thorac Surg , 1992 , vol. 53 (pg. 1107 - 1109 ) Google Scholar Crossref Search ADS PubMed WorldCat [6] Kleinkamp G. , Minami K. , Thies W.R. , Dohmann R. , Raute Kreinsen U. , Meyer H. , Korfer R. . Aorta-right ventricular tunnel with a rudimentary valve and an anomalous origin of the left coronary artery , J Thorac Cardiovasc Surg , 1992 , vol. 104 (pg. 1759 - 1760 ) Google Scholar PubMed OpenURL Placeholder Text WorldCat [7] Rosengart T.K. , Redel D.A. , Stark J.F. . Surgical repair of aorto-right ventricular tunnel in an infant , Ann Thorac Surg , 1993 , vol. 55 (pg. 520 - 522 ) Google Scholar Crossref Search ADS PubMed WorldCat [8] Vargas F.J. , Molina A. , Martinez J.C. , Ranzini M.A. , Vazquez J.C. . Aortico-right ventricular tunnel , Ann Thorac Surg , 1998 , vol. 66 (pg. 1793 - 1795 ) Google Scholar Crossref Search ADS PubMed WorldCat [9] v Son J.A.M. , Hambsch J. , Schneider P. , Mohr F.W. . Repair of aortico-right ventricular tunnel , Eur J Cardiothorac Surg , 1998 , vol. 14 (pg. 214 - 217 ) Google Scholar Crossref Search ADS PubMed WorldCat [10] Talwar S. , Choudhary U.J. , Kothary S.S. , Airan B. . Aortico-right ventricular tunnel , Int J Cardiol , 1999 , vol. 70 (pg. 201 - 205 ) Google Scholar Crossref Search ADS PubMed WorldCat © 2002 Elsevier Science B.V. All rights reserved. Elsevier Science B.V.

Journal

European Journal of Cardio-Thoracic Surgery – Oxford University Press

Published: Jun 1, 2002

Keywords: Aorto-right ventricular tunnel Left coronary artery

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