Interactive CardioVascular and Thoracic Surgery 21 (2015) 272–274 doi:10.1093/icvts/ivv077 Advance Access publication 8 May 2015
CHALLENGE OF THE MONTH – CONGENITAL
Cite this article as: Careddu L, Oppido G, Ialonardi M, Gargiulo G. Aortic atresia with interrupted aortic arch: a combination incompatible with life? Interact CardioVasc Thorac Surg 2015;21:272–4.
Aortic atresia with interrupted aortic arch: a combination incompatible with life?† Lucio Careddu*, Guido Oppido, Magda Ialonardi and Gaetano Gargiulo Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy * Corresponding author. Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy. Tel: +39-051-2143218; fax: +39-051-2143157; e-mail: [email protected] (L. Careddu). Received 1 December 2014; received in revised form 24 February 2015; accepted 12 March 2015
Keywords: Aortic atresia • Interrupted aortic arch • Hybrid operation • Yasui operation
CLINICAL PROBLEM Aortic valve atresia is a common feature of hypoplastic left heart syndrome, which consists of various degrees of underdevelopment of the left ventricle aorta complex; nevertheless it can also be associated with a large ventricular septal defect (VSD) and a rather developed left ventricle. Atresia of the aortic valve prevents antegrade blood flow from the left ventricle to the ascending aorta, so perfusion of the coronary arteries, ascending aorta and aortic arch is provided by the ductus arteriosus in a retrograde fashion.
Interrupted aortic arch (IAA) is quite often associated with aortic or subaortic stenosis; however, very few cases of IAA have been reported in association with aortic valve atresia. Aortic valve atresia with IAA is an extremely rare association which would be incompatible with life, unless blood flow is provided to the ascending aorta and coronary arteries from (i) the ductus/descending aorta through ‘collaterals’ [1], or (ii) from the pulmonary trunk and pulmonary arteries through an aorto-pulmonary window [2] or bilateral ductus [3]. In the absence of a direct connection, blood flow to the coronary arteries is exclusively provided by the Circle of Willis through both carotid arteries, in a reverse
Figure 1: (A) Angiography shows Willis’ circle coronary dependent circulation. (B) Angiography shows large subclavian and vertebral arteries and diminutive carotid arteries and ascending aorta visualized by retrograde filling. † Presented at the 28th Annual Meeting of the European Association for CardioThoracic Surgery, Milan, Italy, 11–15 October 2014.
© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
L. Careddu et al. / Interactive CardioVascular and Thoracic Surgery
CASE DESCRIPTION A full-term female newborn of an uncomplicated pregnancy was transferred to our unit shortly after birth with a prenatal diagnosis of aortic valve atresia. Prostaglandin-E1 infusion was started promptly and echocardiography confirmed [S,D,S] aortic valve atresia, a large cono-ventricular VSD and a rather developed left ventricle; moreover it unexpectedly revealed type B interrupted (left) aortic arch and an aberrant right subclavian artery. Blood flow through the ascending aorta and aortic arch was clearly inverted as well as that through both carotid arteries. Angiography confirmed the diagnosis and showed blood flowing from the ductus to the descending aorta, to both subclavian (left and aberrant right subclavian artery) and vertebral arteries, and from the Circle of Willis to both carotid arteries downward to the ascending aorta and coronary arteries (Fig. 1A and B). In conclusion, coronary blood flow was assured by bilateral retrograde carotid artery flow in a sort of Circle of Willis dependent circulation, exclusively supplied by both vertebral arteries. Consequently we believe that the presence of an aberrant subclavian artery was necessary for survival. Moreover, if the presence of a large VSD led to a rather developed left ventricle, a hypoplastic ascending aorta and small
carotid arteries would have made cannulation for cardiopulmonary bypass extremely challenging and unsafe.
REFERENCES [1] Baker-Smith CM, Milazzo AS, Frush DP, Jaggers J, Kirby ML, Kanter RJ et al. Double aortic arch with aortic atresia and left-sided type B interruption. Congenit Heart Dis 2010;5:316–20. [2] Rosenquist GC, Taylor JF, Stark J. Aortopulmonary fenestration and aortic atresia. Report of an infant with ventricular septal defect, persistent ductus arteriosus, and interrupted aortic arch. Br Heart J 1974;36: 1146–8. [3] Decaluwe W, Delhaas T, Gewillig M. Aortic atresia, interrupted aortic arch type C perfused by bilateral arterial duct. Eur Heart J 2005;26: 2333. [4] Dibardino DJ, Heinle JS, Andropoulos DA, Kerr CD, Morales DL, Fraser CD Jr. Aortic atresia and type B interrupted aortic arch: diagnosis by physiologic cerebral monitoring. Ann Thorac Surg 2005;79:1758–60. [5] Yew G, Coleman D, Calder L. Aortic valvar atresia, interrupted aortic arch, and quadricuspid pulmonary valve: a rare combination. Pediatr Cardiol 2005;26:455–9.
Treatment solution by Careddu et al. Authors: Lucio Careddu, Guido Oppido, Magda Ialonardi and Gaetano Gargiulo Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy doi: 10.1093/icvts/ivv099 © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. The treatment of choice for aortic valve atresia with interrupted aortic arch was a biventricular repair at 12 months. On Day 6 of life, the patient underwent selective pulmonary arteries banding and ductus stenting with the aim to promote ascending and aortic arch growth for further repair. The hybrid procedure was preferred because it was judged to be safer
Figure 1: (A) Left carotid artery division and connection to the main pulmonary artery ( proximally), and to the right carotid artery with azygos vein segment interposition (distally). (B) Biventricular repair. Right ventricle to pulmonary artery conduit (Contegra). Dacron conduit connection of the main pulmonary artery (neo aorta) to the aortic arch.
CHALLENGE OF THE MONTH
fashion; in these situations, the presence of an aberrant subclavian artery could be crucial [4]. To the best of our knowledge only 16 cases of aortic valve atresia and IAA have been reported in the literature to date; only 3 of these cases had no direct connection to the ascending aorta and all had an aberrant right subclavian artery [5], thus proving that an aberrant subclavian artery receiving blood directly from the ductus arteriosus is indispensible for survival.
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CHALLENGE OF THE MONTH – CONGENITAL
Cite this article as: Careddu L, Oppido G, Ialonardi M, Gargiulo G. Aortic atresia with interrupted aortic arch: a combination incompatible with life? Interact CardioVasc Thorac Surg 2015;21:272–4.
Aortic atresia with interrupted aortic arch: a combination incompatible with life?† Lucio Careddu*, Guido Oppido, Magda Ialonardi and Gaetano Gargiulo Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy * Corresponding author. Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy. Tel: +39-051-2143218; fax: +39-051-2143157; e-mail: [email protected] (L. Careddu). Received 1 December 2014; received in revised form 24 February 2015; accepted 12 March 2015
Keywords: Aortic atresia • Interrupted aortic arch • Hybrid operation • Yasui operation
CLINICAL PROBLEM Aortic valve atresia is a common feature of hypoplastic left heart syndrome, which consists of various degrees of underdevelopment of the left ventricle aorta complex; nevertheless it can also be associated with a large ventricular septal defect (VSD) and a rather developed left ventricle. Atresia of the aortic valve prevents antegrade blood flow from the left ventricle to the ascending aorta, so perfusion of the coronary arteries, ascending aorta and aortic arch is provided by the ductus arteriosus in a retrograde fashion.
Interrupted aortic arch (IAA) is quite often associated with aortic or subaortic stenosis; however, very few cases of IAA have been reported in association with aortic valve atresia. Aortic valve atresia with IAA is an extremely rare association which would be incompatible with life, unless blood flow is provided to the ascending aorta and coronary arteries from (i) the ductus/descending aorta through ‘collaterals’ [1], or (ii) from the pulmonary trunk and pulmonary arteries through an aorto-pulmonary window [2] or bilateral ductus [3]. In the absence of a direct connection, blood flow to the coronary arteries is exclusively provided by the Circle of Willis through both carotid arteries, in a reverse
Figure 1: (A) Angiography shows Willis’ circle coronary dependent circulation. (B) Angiography shows large subclavian and vertebral arteries and diminutive carotid arteries and ascending aorta visualized by retrograde filling. † Presented at the 28th Annual Meeting of the European Association for CardioThoracic Surgery, Milan, Italy, 11–15 October 2014.
© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
L. Careddu et al. / Interactive CardioVascular and Thoracic Surgery
CASE DESCRIPTION A full-term female newborn of an uncomplicated pregnancy was transferred to our unit shortly after birth with a prenatal diagnosis of aortic valve atresia. Prostaglandin-E1 infusion was started promptly and echocardiography confirmed [S,D,S] aortic valve atresia, a large cono-ventricular VSD and a rather developed left ventricle; moreover it unexpectedly revealed type B interrupted (left) aortic arch and an aberrant right subclavian artery. Blood flow through the ascending aorta and aortic arch was clearly inverted as well as that through both carotid arteries. Angiography confirmed the diagnosis and showed blood flowing from the ductus to the descending aorta, to both subclavian (left and aberrant right subclavian artery) and vertebral arteries, and from the Circle of Willis to both carotid arteries downward to the ascending aorta and coronary arteries (Fig. 1A and B). In conclusion, coronary blood flow was assured by bilateral retrograde carotid artery flow in a sort of Circle of Willis dependent circulation, exclusively supplied by both vertebral arteries. Consequently we believe that the presence of an aberrant subclavian artery was necessary for survival. Moreover, if the presence of a large VSD led to a rather developed left ventricle, a hypoplastic ascending aorta and small
carotid arteries would have made cannulation for cardiopulmonary bypass extremely challenging and unsafe.
REFERENCES [1] Baker-Smith CM, Milazzo AS, Frush DP, Jaggers J, Kirby ML, Kanter RJ et al. Double aortic arch with aortic atresia and left-sided type B interruption. Congenit Heart Dis 2010;5:316–20. [2] Rosenquist GC, Taylor JF, Stark J. Aortopulmonary fenestration and aortic atresia. Report of an infant with ventricular septal defect, persistent ductus arteriosus, and interrupted aortic arch. Br Heart J 1974;36: 1146–8. [3] Decaluwe W, Delhaas T, Gewillig M. Aortic atresia, interrupted aortic arch type C perfused by bilateral arterial duct. Eur Heart J 2005;26: 2333. [4] Dibardino DJ, Heinle JS, Andropoulos DA, Kerr CD, Morales DL, Fraser CD Jr. Aortic atresia and type B interrupted aortic arch: diagnosis by physiologic cerebral monitoring. Ann Thorac Surg 2005;79:1758–60. [5] Yew G, Coleman D, Calder L. Aortic valvar atresia, interrupted aortic arch, and quadricuspid pulmonary valve: a rare combination. Pediatr Cardiol 2005;26:455–9.
Treatment solution by Careddu et al. Authors: Lucio Careddu, Guido Oppido, Magda Ialonardi and Gaetano Gargiulo Pediatric Cardiac Surgery, Adult Congenital Unit, S. Orsola-Malpighi Hospital, University of Bologna Medical School, Bologna, Italy doi: 10.1093/icvts/ivv099 © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. The treatment of choice for aortic valve atresia with interrupted aortic arch was a biventricular repair at 12 months. On Day 6 of life, the patient underwent selective pulmonary arteries banding and ductus stenting with the aim to promote ascending and aortic arch growth for further repair. The hybrid procedure was preferred because it was judged to be safer
Figure 1: (A) Left carotid artery division and connection to the main pulmonary artery ( proximally), and to the right carotid artery with azygos vein segment interposition (distally). (B) Biventricular repair. Right ventricle to pulmonary artery conduit (Contegra). Dacron conduit connection of the main pulmonary artery (neo aorta) to the aortic arch.
CHALLENGE OF THE MONTH
fashion; in these situations, the presence of an aberrant subclavian artery could be crucial [4]. To the best of our knowledge only 16 cases of aortic valve atresia and IAA have been reported in the literature to date; only 3 of these cases had no direct connection to the ascending aorta and all had an aberrant right subclavian artery [5], thus proving that an aberrant subclavian artery receiving blood directly from the ductus arteriosus is indispensible for survival.
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