Pediatr Radiol (1991) 21:158-159
Pediatric Radiology 9 Springer-Verlag 1991
Systemic artery-pulmonary vein fistula without sequestration C. P e r n o t I , R S i m o n I , J. C. H o e f f e l 2, A . M. W o r m s I , E M a r c o n t a n d J. P r e v o t 3 1 Department of Cardiology, 2 Department of Radiology, and 3 Department of Surgery, Children Hospital, Vandoeuvre, France Received: 8 July 1990; accepted: 31 July 1990
Abstract. Fistulae between an artery arising from the aorta and a p u l m o n a r y vein associated with absence of a branch of the pulmonary artery, but without anomalies of the bronchial tree, are exceedingly rare. We report a case in a child who presented with a continuous vascular m u r m u r and developed heart failure due to the left-left shunt. Left lobectomy was performed and showed that the entire left lower lobe was occupied by a huge arteriovenous fistula between an artery stemming from the aorta and the pulmonary veins.
showed a huge arterial branch arising from the aorta which vascularized the inferior lobe of the left lung. There was rapid venous drainage towards the inferior left pulmonary vein with several opacities which looked like fistulae. Aortography with catheterization of the large abnormal artery showed the branches to the lingula and the left lower lobe (Fig. 3). Bronchography showed that the main left bronchus was displaced upward. The bronchus of the left superior lobe was normal. The lingular bronchus was displaced. The inferior lobe bronchus was displaced posteriorly. The antero-basal and latero-basal segmental bronchi of the lungs were opacified. Diagnosis of a fistula between an anomalous branch of the aorta and a pulmonaryvein was made. At surgery there was an enormous
fstula between a systemic artery and a pulmonaryvein.The left inferior pulmonaryvein was enlarged and dilated. A left lower lobectomy was performed and the anomalous artery ligated. Pathological examination demonstrated a complex pulmonary malformation with a systemic supply but without interruption of normal bronchial continuity.
Discussion This case demonstrates an aorta pulmonary venous fistula different from the c o m m o n fistula between the pulmonary artery and p u l m o n a r y vein.
Systemic artery-pulmonary vein fistula with absence of a branch of the pulmonary artery is very rare in childhood.
Case report S.C., a female, presented at the age of one month with moderate cardiac failure and a continuous murmur over the left anterior chest and back. At 12 months a chest X-Ray showed a cardiothoracic ratio of 49% with increased pulmonaryvascular markings. A vertical b and of increased density was also visible behind the left heart. The electrocardiogram demonstrated left ventricular hypertrophy. Cardiac ultrasound demonstrated left ventricular dilatation. However, the left atrial/aortic ratio was not increased. Right heart cardiac catheterization revealed normal pulmonary artery pressures. The catheter could not be passed through apatent ductus arteriosus. Oxymetry did not show any evidence of left to right shunt at pulmonary artery level. Angiography in the main pulmonary artery showed absence of opacification of the left inferior lobar pulmonary artery (Fig. 1) in the left anterior oblique view. Angiographyinthe left pulmonary artery showed a hypoplastic left pulmonary artery with an absent inferior lobar branch. Opacifcation of the left ventricle on axial view (Fig. 2) and on frontal view
Fig. 1. Injectionwithin the pulmonary artery (left anterior oblique view): no opacification of a left inferior lobar artery
C. Pernot et al.: Systemic artery-pulmonary vein fistula without sequestration
Fig. 2. Ventriculography: longitudinal axial view following passage of the catheter from the right atrium into the left ventricle. No ventricular septal defect. The descending aorta gives rise to an arterial branch that supplies the left lower lobe
159 There are also three cases [2, 3, 7] which are similar to our case but the pulm o n a r y artery and its branches are normal and the abnormality is a fistula between thoracic aorta and a normal p u l m o n a r y vein. In our case, there was no inferior pulmonary artery, there was no bronchial abnormality. The shunt is left-left, in contrast to the right-left shunt of pulm o n a r y arteriovenous fistula or the leftright arteriovenous fistula of the head or of the limbs. As it is not a sequestration the main reason for surgery is ligature of the a b n o r m a l artery, and l o b e c t o m y is necessary only if the lung has been damaged.
Acknowledgement. The authors would like to thank Dr. G. Sholler Department of Radiology Children's Hospital Camperdown (Australia) and Dr. RB. Guyer Department of Radiology Royal South Hants Hospital Southampton (Great Britain) for their help in reviewing the manuscript.
References
Fig. 3. Aortography with passage of the catheter into the abnormal artery. Very large dilated tortuous artery giving off branches to the lingula and to the left lower lobe except for the superior segment of the inferior lobe
A n abnormal artery arose from the aorta and supplied an a r e a of the lung which had lost its usual p u l m o n a r y artery supply. The p u l m o n a r y venous return from the segment was through a normally draining p u h n o n a r y vein. The p u l m o n a r y segment involved l o o k e d like a vascular sponge and displaced the normal bronchial tree. It is a form of vascular malformation which can be identified as pseudo-sequestration. Similar cases have b e e n r e p o r t e d in the literature with arteriographic and pathological proof. Merlier [5] described a boy of 6 years with an abnormal artery arising from the abdominal
aorta and normal bronchi. L o b e c t o m y was performed. Begin [1] described two patients, The first, a female of 3 years, had an a b n o r m a l artery arising from the thoracic aorta. The bronchi were normal. A left lower l o b e c t o m y was performed. The second, a female of 14 years, had also an abnormal artery arising from the abdominal aorta. The bronchus was normal. Right lower lob e c t o m y was performed. T h e authors also speak of arterial sequestration without bronchial abnormalities. Others cases include a young adult of 20 years [4] and a female of 46 years of age [6].
1. Begin R Lamarre A, Blanchette G (1985) La sequestration pulmonaire. Etude retrospective de 27 cas en centres hospitaliers pediatrique et adulte avec remise en question de l'approche therapeutique d'emblee chirurgicale. Union Med Canada 114:301 2. Currarino G, Willis K, Miller W (1975) Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. A report of three cases. J Pediatr 87:554 3. Ernst S, Bruschke A (1971) An aberrant systemic artery to the right lung with normal pulmonary tissue. Chest 60:606 4. Gamondes JR Devolfe Ch, Morin A, Tabib A, Loire R (1984) Sequestration pulmonaire intra-lobaire du type I de Pryce. A propos d'une observation anatomo-chirurgicale. Rev Pneumol Clin 40:209 5. Merlier M, Rochainzamir A, Rojas-Miranda A, Levasseur R Sulzer JD, Verley JM, Langlois J, Binet JR Le Brigand H (1970) Aspects anatomo-cliniques des sequestrations pulmonaires, Apropos de 46 observations. Ann Chit Thorac Cardiovasc 9: 511 6. Perrotin M, Novikoff N, Dauptain J, Ecoiffief J, Cabrol C, Himbert F, Maurice P (1973) Sequestration pulmonaire arterielte pure et souffle continu. Arch Mal Coeur 8:1077 7. Scott LR Perry LW (1969) Systemic arterypulmonary vein fistulas. Congenital and acquired left to left shunt. Am J Cardio123: 872
Dr. J. C. Hoeffel Department of Radiology Children Hospital Atlee du Morvan F-54511 Vandoeuvre Cedex France
Pediatric Radiology 9 Springer-Verlag 1991
Systemic artery-pulmonary vein fistula without sequestration C. P e r n o t I , R S i m o n I , J. C. H o e f f e l 2, A . M. W o r m s I , E M a r c o n t a n d J. P r e v o t 3 1 Department of Cardiology, 2 Department of Radiology, and 3 Department of Surgery, Children Hospital, Vandoeuvre, France Received: 8 July 1990; accepted: 31 July 1990
Abstract. Fistulae between an artery arising from the aorta and a p u l m o n a r y vein associated with absence of a branch of the pulmonary artery, but without anomalies of the bronchial tree, are exceedingly rare. We report a case in a child who presented with a continuous vascular m u r m u r and developed heart failure due to the left-left shunt. Left lobectomy was performed and showed that the entire left lower lobe was occupied by a huge arteriovenous fistula between an artery stemming from the aorta and the pulmonary veins.
showed a huge arterial branch arising from the aorta which vascularized the inferior lobe of the left lung. There was rapid venous drainage towards the inferior left pulmonary vein with several opacities which looked like fistulae. Aortography with catheterization of the large abnormal artery showed the branches to the lingula and the left lower lobe (Fig. 3). Bronchography showed that the main left bronchus was displaced upward. The bronchus of the left superior lobe was normal. The lingular bronchus was displaced. The inferior lobe bronchus was displaced posteriorly. The antero-basal and latero-basal segmental bronchi of the lungs were opacified. Diagnosis of a fistula between an anomalous branch of the aorta and a pulmonaryvein was made. At surgery there was an enormous
fstula between a systemic artery and a pulmonaryvein.The left inferior pulmonaryvein was enlarged and dilated. A left lower lobectomy was performed and the anomalous artery ligated. Pathological examination demonstrated a complex pulmonary malformation with a systemic supply but without interruption of normal bronchial continuity.
Discussion This case demonstrates an aorta pulmonary venous fistula different from the c o m m o n fistula between the pulmonary artery and p u l m o n a r y vein.
Systemic artery-pulmonary vein fistula with absence of a branch of the pulmonary artery is very rare in childhood.
Case report S.C., a female, presented at the age of one month with moderate cardiac failure and a continuous murmur over the left anterior chest and back. At 12 months a chest X-Ray showed a cardiothoracic ratio of 49% with increased pulmonaryvascular markings. A vertical b and of increased density was also visible behind the left heart. The electrocardiogram demonstrated left ventricular hypertrophy. Cardiac ultrasound demonstrated left ventricular dilatation. However, the left atrial/aortic ratio was not increased. Right heart cardiac catheterization revealed normal pulmonary artery pressures. The catheter could not be passed through apatent ductus arteriosus. Oxymetry did not show any evidence of left to right shunt at pulmonary artery level. Angiography in the main pulmonary artery showed absence of opacification of the left inferior lobar pulmonary artery (Fig. 1) in the left anterior oblique view. Angiographyinthe left pulmonary artery showed a hypoplastic left pulmonary artery with an absent inferior lobar branch. Opacifcation of the left ventricle on axial view (Fig. 2) and on frontal view
Fig. 1. Injectionwithin the pulmonary artery (left anterior oblique view): no opacification of a left inferior lobar artery
C. Pernot et al.: Systemic artery-pulmonary vein fistula without sequestration
Fig. 2. Ventriculography: longitudinal axial view following passage of the catheter from the right atrium into the left ventricle. No ventricular septal defect. The descending aorta gives rise to an arterial branch that supplies the left lower lobe
159 There are also three cases [2, 3, 7] which are similar to our case but the pulm o n a r y artery and its branches are normal and the abnormality is a fistula between thoracic aorta and a normal p u l m o n a r y vein. In our case, there was no inferior pulmonary artery, there was no bronchial abnormality. The shunt is left-left, in contrast to the right-left shunt of pulm o n a r y arteriovenous fistula or the leftright arteriovenous fistula of the head or of the limbs. As it is not a sequestration the main reason for surgery is ligature of the a b n o r m a l artery, and l o b e c t o m y is necessary only if the lung has been damaged.
Acknowledgement. The authors would like to thank Dr. G. Sholler Department of Radiology Children's Hospital Camperdown (Australia) and Dr. RB. Guyer Department of Radiology Royal South Hants Hospital Southampton (Great Britain) for their help in reviewing the manuscript.
References
Fig. 3. Aortography with passage of the catheter into the abnormal artery. Very large dilated tortuous artery giving off branches to the lingula and to the left lower lobe except for the superior segment of the inferior lobe
A n abnormal artery arose from the aorta and supplied an a r e a of the lung which had lost its usual p u l m o n a r y artery supply. The p u l m o n a r y venous return from the segment was through a normally draining p u h n o n a r y vein. The p u l m o n a r y segment involved l o o k e d like a vascular sponge and displaced the normal bronchial tree. It is a form of vascular malformation which can be identified as pseudo-sequestration. Similar cases have b e e n r e p o r t e d in the literature with arteriographic and pathological proof. Merlier [5] described a boy of 6 years with an abnormal artery arising from the abdominal
aorta and normal bronchi. L o b e c t o m y was performed. Begin [1] described two patients, The first, a female of 3 years, had an a b n o r m a l artery arising from the thoracic aorta. The bronchi were normal. A left lower l o b e c t o m y was performed. The second, a female of 14 years, had also an abnormal artery arising from the abdominal aorta. The bronchus was normal. Right lower lob e c t o m y was performed. T h e authors also speak of arterial sequestration without bronchial abnormalities. Others cases include a young adult of 20 years [4] and a female of 46 years of age [6].
1. Begin R Lamarre A, Blanchette G (1985) La sequestration pulmonaire. Etude retrospective de 27 cas en centres hospitaliers pediatrique et adulte avec remise en question de l'approche therapeutique d'emblee chirurgicale. Union Med Canada 114:301 2. Currarino G, Willis K, Miller W (1975) Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. A report of three cases. J Pediatr 87:554 3. Ernst S, Bruschke A (1971) An aberrant systemic artery to the right lung with normal pulmonary tissue. Chest 60:606 4. Gamondes JR Devolfe Ch, Morin A, Tabib A, Loire R (1984) Sequestration pulmonaire intra-lobaire du type I de Pryce. A propos d'une observation anatomo-chirurgicale. Rev Pneumol Clin 40:209 5. Merlier M, Rochainzamir A, Rojas-Miranda A, Levasseur R Sulzer JD, Verley JM, Langlois J, Binet JR Le Brigand H (1970) Aspects anatomo-cliniques des sequestrations pulmonaires, Apropos de 46 observations. Ann Chit Thorac Cardiovasc 9: 511 6. Perrotin M, Novikoff N, Dauptain J, Ecoiffief J, Cabrol C, Himbert F, Maurice P (1973) Sequestration pulmonaire arterielte pure et souffle continu. Arch Mal Coeur 8:1077 7. Scott LR Perry LW (1969) Systemic arterypulmonary vein fistulas. Congenital and acquired left to left shunt. Am J Cardio123: 872
Dr. J. C. Hoeffel Department of Radiology Children Hospital Atlee du Morvan F-54511 Vandoeuvre Cedex France
