EuropeanJournal of
Eur. J. Pediatr. 131, 125--131 (1979)
Pediatrics 9 by Springer-Verlag 1979
Anomalous Systemic Arterial Supply to the Basal Segments of the Lung Presenting with a Murmur Yu Ishihara 1, R y u k o F u k u d a 1, Y u t a k a A w a y a 1, Teikichi W a t a n a b e 1, A k i r a Y a m a z a k i 2, Koji H a s h i z u m e 3, Shigenori Sawaguchi 3, and Koichi Shimizu 4 Departments of Pediatrics ~ and Surgery2, Tokyo Teishin Hospital, and Departments of Surgery3 and Pathology4, National Children's Hospital, Tokyo
Abstract. A m u r m u r was discovered on routine preschool physical examination of a 5 year and 7 m o n t h - o l d boy. A lower lobe lesion was detected by chest X-ray. A o r t o g r a p h y demonstrated that a large systemic artery f r o m the descending thoracic aorta supplied the basilar segments of the left lower lobe, which had no n o r m a l p u l m o n a r y arterial supply. A clinical diagnosis of intralobar sequestration of the lung was made, and left lower lobectomy was performed.
Key words: A b n o r m a l p u l m o n a r y artery - Systemic-pulmonary shunt.
Introduction P u l m o n a r y suquestrations receive their arterial blood supply by a n o m a l o u s systemic arteries, and m a y be associated with a m u r m u r [8, 10]. Such a n o m a l o u s arteries may supply an otherwise n o r m a l lung and may come to light following the incidental finding of a m u r m u r [ 1 - - 3 , 7 , 9 ] . In this case, there was a large systemic arterial supply to the normal basilar segments of the left lower lobe, which had no n o r m a l p u l m o n a r y arterial supply.
Case Report T. N., a 5 year and 7 month-old asymptomatic boy, was referred to the Tokyo Teishin Hospital in October 1976 because of a cardiac murmur detected on routine preschool examination. He had membrana pupillaris persistens and astigmatism. There was no past history of a murmur or pneumonia. On physical examination, he was 116.1 cm in height and 19.5 kg in weight. There was no dyspnea, cyanosis, or clubbing of the fingers. The temperature and pulse were normal. A grade 2/6 systolic ejection murmur was heard best at the cardiac apex and radiated to the posterior chest. Address for offprint requests: Yu Ishihara, M.D., Department of Pediatrics, Tokyo Teishin Hospital, 2-14-23 Fujimi, Chiyodaku, Tokyo, 102, Japan
0340-6199/79/0131/0125/$01.40
Fig. I. Chest roentgenogram, There is a slight prominence of the vascular markings in the left lower lobe
Fig. 2a and b. Left pulmonary angiogram. (a) A-P view. (b) Lateral view. There is a superior segment of the lower lobe, but no pulmonary arteries to the basilar segments
Systemic Arterial Supply to Left Lower Lobe
127
Fig. 2 b
An E C G and echocardiogram were normal. Chest roentgenograms showed slight cardiac enlargement and a left lower lobe infiltrate (Fig. 1). Bronchoscopy showed all major bronchi of the left lung to be in their normal location, but bronchograms were unsatisfactory. The left pulmonary biplane arteriograms revealed a normal pulmonary arterial supply to the upper lobe and the superior segment of the lower lobe (Fig. 2). There were no pulmonary arteries to the basilar segments of the left lower lobe. The aortograms revealed a large artery arising from the descending thoracic aorta and supplying the left lower lobe. The venous drainage was noted to return to the left atrium (Fig. 3). The data from the cardiac catheterization and pulmonary function test are shown in the Table 1. The clinical diagnosis of intralobar sequestration of the lung was made. A left lower lobectomy was performed at the National Children's Hospital on May 9th 1977. At the left thoracotomy, the visceral pleura of the left lower lobe was covered with many telangiectases. A solitary artery was identified arising from the descending thoracic aorta about 2.5 cm above the diaphragm. A thrill was palpable over the anomalous artery. At its origin, the vessel was approximately 11 m m in diameter. The pulmonary artery to the lower lobe appeared
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Fig.3. Thoracic aortogram. Large systemic artery supplies the left lower lobe
Table 1. Data from cardiac catheterization, pulmonary function test and blood count
(1) Cardiac catheterization data Location
02 Sat. (%)
Main pulmonary artery Right ventricle (Systemic artery)
87.2 N.D. 96.7
Values in parentheses are mean values Sat. = Saturation N.D. = N o t done (2) Pulmonary function test VC %VC F.E.V.1.0%
1250 ml 90.6% 84.0%
(3) Blood count RBC
465 x 104/mm 3
Hb Ht
12.9 g/dl 39.0%
Pressure (mm Hg) 20/7 (12) 18/0 (8) 104/60
Systemic Arterial Supply to Left Lower Lobe
129
Fig. 4. Roentgenogram of the resected specimen. The anomalous artery injected with barium sulfate shows the extensive distribution of the vessel in the basal segments of the left lung
somewhat smaller than usual. The anomalous systemic artery was divided and a left lower lobectomy accomplished. The postoperative course was uneventful. Pathologically, roentgenograms of the anomalous artery injected with barium sulfate showed the branches of anomalous artery passing into the basilar segments of the resected lower lobe (Fig. 4). There was no direct communication between the systemic artery and a pulmonary vein. The pulmonary artery supplied the normal superior segment of the lower lobe only. Sectioning of the anomalous artery revealed it to be thick-walled and elastic. The major bronchi, minor bronchi and bronchioles of the resected lower lobe were normal. There was no evidence of inflammation, sequestration, or dysplastic change within the pulmonary parenchyma. On pathological grounds, a diagnosis of anomalous systemic arterization of the basilar segments of the left lower lobe was made.
Discussion F o u r similar cases could be f o u n d in the literature [ 4 - - 6 ] (Table 2). O f five cases w i t h this p a r t i c u l a r a n o m a l y , f o u r cases a p p e a r e d to be healthy and the p h y s i c i a n ' s a t t e n t i o n was a t t r a c t e d to the lesion by a m u r m u r . A c o n t i n u o u s m u r m u r m i g h t be expected in a lesion that p r o d u c e s a diastolic r u n - o f f b u t was
130
Y. Ishihara et al.
Table 2. Anomalous systemic origin of a artery to the normal basal segments of the lung Our case (1977)
Kirks et al. (1976)
Hessel et al. (1970)
Litwin et al. (1970)
Sex, age
M., 5 years
M., 6 years
F., 17 months
M., 6 years
M., newborn
Symptoms
(--)
(--)
(--)
Slight fatigue
Congestive heart failure
Murmur Intensity Quality Location
2/6 Systolic Apex
4/6 Continuous Left clavicle Apex, axilla, and posterior
5/6 Continuous Upper precordium Interscapular and left lung base posteriorly
3/6 Systolic Left lower sternal border
Posterior
2/6 Systolic Left lower sternal border Posterior lung base
Radiation
Left
Left
Left
Left
Right
Operation
Lobectomy
Lobectomy
Lobectomy
Anastomosis
Lobectomy
Blood pressure
96/60
Side
115/45 bounding pulse
heard in only two cases, one being initially diagnosed having a patent ductus arteriosus. The other three cases had systolic murmurs. E C G showed left ventricular hypertrophy in two cases. Eventually, congestive heart failure secondary to the left-to-left shunt may develop, and a newborn infant described by Litwin et al. [6] had cardiac failure with widening of the pulse pressures. An E C G showed right ventricular hypertrophy secondary to the leftto-right shunt at atrial level. The basilar segments of the left lower lobe are most commonly involved [5]. The chest roentgenograms show cardiac enlargement and slight prominence of the vascular markings in the involved area. There is neither an associated mass nor cystic change in the pulmonary parenchyma. Aortography and pulmonary angiography show an abnormal systemic artery to the involved segments of the lung, without a normal pulmonary artery. Kirks et al. [5] described a spectrum of congenital pulmonary abnormalities with systemic arterial supply to the lung. These include: accessory arterial supply to the lung by a systemic vessel (i.e., there is also a normal pulmonary arterial supply to the involved segments); only a systemic vessel supplying the lung, as in our case; and sequestration. This differentiation is important in treatment. F o u r cases underwent the lobectomy of the involved lung, but in a case of Hessel et al.{4] the origin of the anomalous vessel was transfered to the pulmonary artery. If there is an associated normal pulmonary arterial supply to the involved segments of the lung, the accessory systemic vessel may simply be ligated [1--3, 7, 9]. A sequestered lobe should be completely removed to prevent recurrent infection [8, 10]. Embryologically, it is thought that persistence of the vascular connection between the aorta and pulmonary parenchyma results in the anomalous arterial supply to the lung [5].
Systemic Arterial Supply to Left Lower Lobe
131
References 1. Campbell, D. C., Murney, J. A., Dominy, D. E.: Systemic arterial blood supply to a normal lung. JAMA 182, 497--499 (1962) 2. Currarino, G., Willis, K., Miller, W.: Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. J. Pediat. 87, 554--557 (1975) 3. Ernst, S. M. P. G., Bruschke, A. V. G.: An aberrant systemic artery to the right lung with normal pulmonary tissue. Chest. 60, 606--608 (1971) 4. Hessel, E. A. II., Boyden, E. A., Stamm, S. J., Sauvage, L. R.: High systemic origin of the sole artery to the basal segments of the left lung: Findings, surgical treatment, and embryologic interpretation. Surgery 67,624--632 (1970) 5. Kirks, D. R., Kane, P. E., Free, E. A., Taybi, H.: Systemic arterial supply to normal basilar segments of the left lower lobe. Am. J. Roentgenol. 126, 817--821 (1976) 6. Litwin, S. B., Plauth, E. H., Nadas, A. S.: Anomalous systemic arterial supply to the lung causing pulmonary-artery hypertension. New Eng. J. Meal. 283, 1098--1099 (1970) 7. Scott, L. P. III., Perry, L. W.: Systemic artery-pulmonary vein fistulas: Congenital and acquired left to left shunt. Am. J. Cardiol. 23, 872--876 (1969) 8. Sperling, D. R., Finck, E. J.: Intralobarbronchopulmonarysequestration: Association with a murmur over the back in a child. Amer. J. Dis. Child. 115, 362--367 (1968) 9. Varma,K.K., Clarke, C.P.: Congenital systemic-to-pulmonaryarteriovenous fistula: Report of a case. Austr. N. Zeal. J. Surg. 40, 360--362 (1971) 10. White, J. J., Donahoo, J. S., Ostrow, P. T., Murphy, J., Haller, J. A.: Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann. Thoracic Surg. 18, 286--294 (1974)
Received October 17, 1978
Eur. J. Pediatr. 131, 125--131 (1979)
Pediatrics 9 by Springer-Verlag 1979
Anomalous Systemic Arterial Supply to the Basal Segments of the Lung Presenting with a Murmur Yu Ishihara 1, R y u k o F u k u d a 1, Y u t a k a A w a y a 1, Teikichi W a t a n a b e 1, A k i r a Y a m a z a k i 2, Koji H a s h i z u m e 3, Shigenori Sawaguchi 3, and Koichi Shimizu 4 Departments of Pediatrics ~ and Surgery2, Tokyo Teishin Hospital, and Departments of Surgery3 and Pathology4, National Children's Hospital, Tokyo
Abstract. A m u r m u r was discovered on routine preschool physical examination of a 5 year and 7 m o n t h - o l d boy. A lower lobe lesion was detected by chest X-ray. A o r t o g r a p h y demonstrated that a large systemic artery f r o m the descending thoracic aorta supplied the basilar segments of the left lower lobe, which had no n o r m a l p u l m o n a r y arterial supply. A clinical diagnosis of intralobar sequestration of the lung was made, and left lower lobectomy was performed.
Key words: A b n o r m a l p u l m o n a r y artery - Systemic-pulmonary shunt.
Introduction P u l m o n a r y suquestrations receive their arterial blood supply by a n o m a l o u s systemic arteries, and m a y be associated with a m u r m u r [8, 10]. Such a n o m a l o u s arteries may supply an otherwise n o r m a l lung and may come to light following the incidental finding of a m u r m u r [ 1 - - 3 , 7 , 9 ] . In this case, there was a large systemic arterial supply to the normal basilar segments of the left lower lobe, which had no n o r m a l p u l m o n a r y arterial supply.
Case Report T. N., a 5 year and 7 month-old asymptomatic boy, was referred to the Tokyo Teishin Hospital in October 1976 because of a cardiac murmur detected on routine preschool examination. He had membrana pupillaris persistens and astigmatism. There was no past history of a murmur or pneumonia. On physical examination, he was 116.1 cm in height and 19.5 kg in weight. There was no dyspnea, cyanosis, or clubbing of the fingers. The temperature and pulse were normal. A grade 2/6 systolic ejection murmur was heard best at the cardiac apex and radiated to the posterior chest. Address for offprint requests: Yu Ishihara, M.D., Department of Pediatrics, Tokyo Teishin Hospital, 2-14-23 Fujimi, Chiyodaku, Tokyo, 102, Japan
0340-6199/79/0131/0125/$01.40
Fig. I. Chest roentgenogram, There is a slight prominence of the vascular markings in the left lower lobe
Fig. 2a and b. Left pulmonary angiogram. (a) A-P view. (b) Lateral view. There is a superior segment of the lower lobe, but no pulmonary arteries to the basilar segments
Systemic Arterial Supply to Left Lower Lobe
127
Fig. 2 b
An E C G and echocardiogram were normal. Chest roentgenograms showed slight cardiac enlargement and a left lower lobe infiltrate (Fig. 1). Bronchoscopy showed all major bronchi of the left lung to be in their normal location, but bronchograms were unsatisfactory. The left pulmonary biplane arteriograms revealed a normal pulmonary arterial supply to the upper lobe and the superior segment of the lower lobe (Fig. 2). There were no pulmonary arteries to the basilar segments of the left lower lobe. The aortograms revealed a large artery arising from the descending thoracic aorta and supplying the left lower lobe. The venous drainage was noted to return to the left atrium (Fig. 3). The data from the cardiac catheterization and pulmonary function test are shown in the Table 1. The clinical diagnosis of intralobar sequestration of the lung was made. A left lower lobectomy was performed at the National Children's Hospital on May 9th 1977. At the left thoracotomy, the visceral pleura of the left lower lobe was covered with many telangiectases. A solitary artery was identified arising from the descending thoracic aorta about 2.5 cm above the diaphragm. A thrill was palpable over the anomalous artery. At its origin, the vessel was approximately 11 m m in diameter. The pulmonary artery to the lower lobe appeared
128
Y. Ishihara et al.
Fig.3. Thoracic aortogram. Large systemic artery supplies the left lower lobe
Table 1. Data from cardiac catheterization, pulmonary function test and blood count
(1) Cardiac catheterization data Location
02 Sat. (%)
Main pulmonary artery Right ventricle (Systemic artery)
87.2 N.D. 96.7
Values in parentheses are mean values Sat. = Saturation N.D. = N o t done (2) Pulmonary function test VC %VC F.E.V.1.0%
1250 ml 90.6% 84.0%
(3) Blood count RBC
465 x 104/mm 3
Hb Ht
12.9 g/dl 39.0%
Pressure (mm Hg) 20/7 (12) 18/0 (8) 104/60
Systemic Arterial Supply to Left Lower Lobe
129
Fig. 4. Roentgenogram of the resected specimen. The anomalous artery injected with barium sulfate shows the extensive distribution of the vessel in the basal segments of the left lung
somewhat smaller than usual. The anomalous systemic artery was divided and a left lower lobectomy accomplished. The postoperative course was uneventful. Pathologically, roentgenograms of the anomalous artery injected with barium sulfate showed the branches of anomalous artery passing into the basilar segments of the resected lower lobe (Fig. 4). There was no direct communication between the systemic artery and a pulmonary vein. The pulmonary artery supplied the normal superior segment of the lower lobe only. Sectioning of the anomalous artery revealed it to be thick-walled and elastic. The major bronchi, minor bronchi and bronchioles of the resected lower lobe were normal. There was no evidence of inflammation, sequestration, or dysplastic change within the pulmonary parenchyma. On pathological grounds, a diagnosis of anomalous systemic arterization of the basilar segments of the left lower lobe was made.
Discussion F o u r similar cases could be f o u n d in the literature [ 4 - - 6 ] (Table 2). O f five cases w i t h this p a r t i c u l a r a n o m a l y , f o u r cases a p p e a r e d to be healthy and the p h y s i c i a n ' s a t t e n t i o n was a t t r a c t e d to the lesion by a m u r m u r . A c o n t i n u o u s m u r m u r m i g h t be expected in a lesion that p r o d u c e s a diastolic r u n - o f f b u t was
130
Y. Ishihara et al.
Table 2. Anomalous systemic origin of a artery to the normal basal segments of the lung Our case (1977)
Kirks et al. (1976)
Hessel et al. (1970)
Litwin et al. (1970)
Sex, age
M., 5 years
M., 6 years
F., 17 months
M., 6 years
M., newborn
Symptoms
(--)
(--)
(--)
Slight fatigue
Congestive heart failure
Murmur Intensity Quality Location
2/6 Systolic Apex
4/6 Continuous Left clavicle Apex, axilla, and posterior
5/6 Continuous Upper precordium Interscapular and left lung base posteriorly
3/6 Systolic Left lower sternal border
Posterior
2/6 Systolic Left lower sternal border Posterior lung base
Radiation
Left
Left
Left
Left
Right
Operation
Lobectomy
Lobectomy
Lobectomy
Anastomosis
Lobectomy
Blood pressure
96/60
Side
115/45 bounding pulse
heard in only two cases, one being initially diagnosed having a patent ductus arteriosus. The other three cases had systolic murmurs. E C G showed left ventricular hypertrophy in two cases. Eventually, congestive heart failure secondary to the left-to-left shunt may develop, and a newborn infant described by Litwin et al. [6] had cardiac failure with widening of the pulse pressures. An E C G showed right ventricular hypertrophy secondary to the leftto-right shunt at atrial level. The basilar segments of the left lower lobe are most commonly involved [5]. The chest roentgenograms show cardiac enlargement and slight prominence of the vascular markings in the involved area. There is neither an associated mass nor cystic change in the pulmonary parenchyma. Aortography and pulmonary angiography show an abnormal systemic artery to the involved segments of the lung, without a normal pulmonary artery. Kirks et al. [5] described a spectrum of congenital pulmonary abnormalities with systemic arterial supply to the lung. These include: accessory arterial supply to the lung by a systemic vessel (i.e., there is also a normal pulmonary arterial supply to the involved segments); only a systemic vessel supplying the lung, as in our case; and sequestration. This differentiation is important in treatment. F o u r cases underwent the lobectomy of the involved lung, but in a case of Hessel et al.{4] the origin of the anomalous vessel was transfered to the pulmonary artery. If there is an associated normal pulmonary arterial supply to the involved segments of the lung, the accessory systemic vessel may simply be ligated [1--3, 7, 9]. A sequestered lobe should be completely removed to prevent recurrent infection [8, 10]. Embryologically, it is thought that persistence of the vascular connection between the aorta and pulmonary parenchyma results in the anomalous arterial supply to the lung [5].
Systemic Arterial Supply to Left Lower Lobe
131
References 1. Campbell, D. C., Murney, J. A., Dominy, D. E.: Systemic arterial blood supply to a normal lung. JAMA 182, 497--499 (1962) 2. Currarino, G., Willis, K., Miller, W.: Congenital fistula between an aberrant systemic artery and a pulmonary vein without sequestration. J. Pediat. 87, 554--557 (1975) 3. Ernst, S. M. P. G., Bruschke, A. V. G.: An aberrant systemic artery to the right lung with normal pulmonary tissue. Chest. 60, 606--608 (1971) 4. Hessel, E. A. II., Boyden, E. A., Stamm, S. J., Sauvage, L. R.: High systemic origin of the sole artery to the basal segments of the left lung: Findings, surgical treatment, and embryologic interpretation. Surgery 67,624--632 (1970) 5. Kirks, D. R., Kane, P. E., Free, E. A., Taybi, H.: Systemic arterial supply to normal basilar segments of the left lower lobe. Am. J. Roentgenol. 126, 817--821 (1976) 6. Litwin, S. B., Plauth, E. H., Nadas, A. S.: Anomalous systemic arterial supply to the lung causing pulmonary-artery hypertension. New Eng. J. Meal. 283, 1098--1099 (1970) 7. Scott, L. P. III., Perry, L. W.: Systemic artery-pulmonary vein fistulas: Congenital and acquired left to left shunt. Am. J. Cardiol. 23, 872--876 (1969) 8. Sperling, D. R., Finck, E. J.: Intralobarbronchopulmonarysequestration: Association with a murmur over the back in a child. Amer. J. Dis. Child. 115, 362--367 (1968) 9. Varma,K.K., Clarke, C.P.: Congenital systemic-to-pulmonaryarteriovenous fistula: Report of a case. Austr. N. Zeal. J. Surg. 40, 360--362 (1971) 10. White, J. J., Donahoo, J. S., Ostrow, P. T., Murphy, J., Haller, J. A.: Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann. Thoracic Surg. 18, 286--294 (1974)
Received October 17, 1978
