(Acta Paediatr Jon 1992: 34: 573
- 5781
The Very Low Birthweight Neonate With an Interrupted Aortic Arch and Dextrocardia Yoshihiro Wakazono, M.D.,’ ltaru Yarnada, M.D.,’ Keiichi Kagajou, M.D.,’ Masaaki Miyazaki, M.D.,’ Wlasayoshi Isshiki, M.D.,’ Naomasa Takayama, M.D.,’ Shunji Shinnoka, M.D.,2 Tetsuo Tomino, M.D.,’ and Keizou Furuya, M.D.,3 Departments of Pediatrics, 2Cardiologyand ’Pathology, Ehime Prefectural Central Hospital, Matsuyama, Japan
Type 1A interruption of the aortic arch complex accompanied by mirror-image dextrocardia with situs inversus was diagnosed by counter-current aortography through the rapial artery. This technique is useful for aortography of the very low birthweight neonate. Key Words Type 1A IAA complex, Dextrocardia, Very low birthweight neonate, Aortography by radial artery injection
Introduction
Case Report
Interruption of the aortic arch (IAA) is a rare but deadly congenital cardiovascular anomaly particularly in very low birthweight babies. We encountered an IAA complex neonate accompanied by mirror-image dextrocardia with situs inversus and dextroviscera. This combination in a very low birthweight baby is rare. Diagnosis was made using countercurrent aortography through radial artery, instead of cardiac catherization. This examination was performed in an incubator for very low birthweight neonates. In addition, we report the findings of the autopsy of the present case.
Diagnosis A iemale infant was delivered spontaneously at 35 weeks gestation, weighed 1,372g (Apgar score 8), and was She fourth child of a 35 year old mother whose three previous babies were normal. There was no history of congenital heart disease in either parent’s family. On August 21, 1989, the baby was transferred to our hospital on the second day of life because of cyanosis, tachycardia and tachypnea. Physical examination on admission revealed a pale, tachypneic neosate. Her weight was 1,369 g, height 37 cm, head circumference 27 cm and chest circumference 24 cm. Her heart rate Cas 124 beatdmin and her respiratory rate was 78 beatdmin. She showed peripheral cyanosis but no cyanosis of the trunk, She had a flat and large forehead, hyperteiorism and a cleft soft palate. Through auscultation it was determined that the cardiac apex was right-sided. There was no murmur. Both femoral pulses we‘re palpable. She did not exhibit nasal alar breathing or grunting, but her chest was slightly retracted.
Received January 22, 1992 Revised May 6, 1992 Accepted May 22, 1992 Correspondence address: Dr Yoshihiro Wakazono, Department of Pediatrics, Faculty of Medicine, Kyoto University, 54 Shogoin-Kawahara-cho,Sakyo-ku, Kyoto 606, Japan.
514 (84) Y. Wakazono et al. There was no rile. The abdomen was soft and flat, and there was no liver enlargement. A chest X-ray revealed the enlargement of the heart (cardiothoracic ratio = 0.62), dextrocardia with situs inversus and a stomach bubble on the right side (Fig. 1). An electrocardiogram showed sinus rhythm and typical findings of mirror-image dextrocardia ( - 180' inversion of P, Q, R, S and T at lead I) and hypertrophy of the right ventricle (T-wave positive at leads V 1 and V2; Fig. 2). Laboratory findings on admission showed high levels of serum basal urinary sodium, creatinine, slightly low levels of serum Na, C1, Ca and transient hyperglycemia. There was no chromosomal abnormality (Table 1). Two-dimensional echocardiographic examination demonstrated the presence of ventricular septal defect (VSD), atrial septal defect (ASD) and the absence of the aortic arch. These findings suggested IAA complex and dextrocardia with situs inversus. A 24-gauge angiocatheter was inserted percutaneously through the right radial artery, and 2 ml iopamidol was administered through this prior to a chest and abdomi-
Fig. 1: Chest X-ray.
Fig. 2: Electrocardiograph.
nal X-ray. This revealed that the iopamidol was channelled from the radial artery through to the brachial artery and right subclavian artery to the ascending aorta. There was no descending aorta. We diagnosed IAA type A and dextrocardia with situs inversus (Fig. 3). Prostaglandin El was infused to maintain ductal patency and dopamine as a pre-operative course. Urine volume gradually decreased, acidosis was progressive, and her general condition gradually worsened. An emergency operation was performed at 5 days of age.
Operation The IAA was repaired by posterolateral thoracotomy. The diameters of the aortic arch and the right subclavian artery were 2.5 mm and 3.0 mm respectively. It appeared that the junction of the aortic arch and descending aorta was blocked by a membrane. However, the pulmonary artery ran through the patent ductus arteriosus (PDA) to the descending aorta. The subclavian artery was used for the repair. It was incised longitudinally to the descending .aorta to make a flap after incision of that membrane. The descending aorta was covered by a subclavian flap. The PDA (diameter 15 mm) was ligated, and banding of the pulmonary trunk was performed. In this way, blood flow was directed from the ascending aorta through the aortic arch, the subclavian flap and to the descending
Acta Paediatr Jpn
Low birthweight neonate ( 8 5 ) 515 Table 1. Laboratory findings on admission Peripheral blood
Blood chemistry
RBC 326 1 0 4 / ~ ~ 3 GOT 40 Ku Hb 12.9 g/dl GPT 5 Ku Ht 37.6% T-Bil 5.4 mg/dl PLT 17.2 104/mm3 TP 4.0 g/dl WBC 10,500/mm3 BUU 20.5 mg/dl Bands 5% CRTN 2.9 mg/dl Segments 59% Na 131 mEq/l Eosinophils 2% K 4.2 mEq/l C1 95 mEq/l Lymphocytes 33% Monocytes 1% Ca 3.8 mEq/l BGA (025Vmin) Glu 174 mg/dl PH 1.34 HPT 26% pa* 41 mmHg PO 93 mmHg CRP 1' Hd0322 mmolfl - 3 mmol/l Karyotype 46, XX BE 0,Sat 91% RBC, red blood cells; Hb, hemoglobin; Ht, hematocrit; PLT, platelet count; WBC, white blood cells; GOT, glutamate oxaloacetate transaminase; GPT, glutamic-pyruvic transaminase; T-Bil, total bilirubin; TP, total protein; BUN, blood urea nitrogen; CRTN, creatinine; HPT, hepaplastin test, CRP, C-reactive protein.
However, heart failure progressed and she died at 9 days of age.
Autopsy Autopsy revealed situs inversus totalis and no thymus. Her right lung was divided into two parts and her left lung divided into three parts. All other viscera were in the mirror-image dextra position (Fig. 4). The heart exhibited dextrocardia with situs inversus and IAA. We recognized the junction at the subclavian flap and the descending aorta, the ligated PDA and pulmonary arterial banding (Fig. 5). The opened heart revealed VSD type 1 (8 x 8 mm; Fig. 6), and ASD (5 x 5 mm). The pulmonary and aortic valves were normal.
Discussion
Fig. 3: Aortography by radial artery injection.
aorta. One day after operation, urine volume increased and her general condition improved.
Vol. 34 No. 5 October 1992
Interrupted aortic arch is a rare congenital malformation that is usually accompanied by severe intracardiac anormalies. According to Van Praagh, IAA represented 1.4% of autopsied infants with congenital heart disease [ 11, which coincided with the New England Regional Infant Cardiac Program (NERICP) incidence of 1.3% of congenital heart disease [2]. On the other hand, the rate of dextrocardia with a normal spleen was 0.037 per 1000 live births
576 (86) Y. Wakazono et a/.
Fig. 6: The opened heart revealed VSD type 1 (8 x 8 mm; arrowed) and ASD ( 5 x 5 mm). The pulmonary and aortic valves were normal.
Fig. 4: Results of the autopsy. She had situs inversus totalis and no thymus. Her right lung was divided into two parts and her left lung divided into three parts. All other viscera were in the mirror-image dextra position.
Fig. 5 : The heart was diagnosed as dextrocardia with situs inversus and IAA. We recognized the junction between subclavian flap (+-) and the descending aorta the ligated PDA and pulmonary arterial banding. (4).
according to the NERICP. Cases of IAA with mirror-image dextrocardia are rare. Ekici et al. reported a case of IAA with mirror-image dextro-
cardia [3], and a few cases of dextrocardia associated with aortic coarctation have been reported [4,5]. However, it does not seem that IAA with dextrocardia has been reported in the very low birthweight baby. IAA has always been associated with other defects, most commonly PDA and VSD. In Japan, various anomalies accompanied by IAA have been reported [6]. In the present case, PDA, VSD, ASD and dextrocardia were identified. IAA has been classified anatomically into atresia of the aortic arch and absence of the aortic arch. However, circulation is interrupted and both anomalies are clinically treated as I A A [7]. IAA has been classified by Celoria and Patton into three groups: in type A, interruption is distal to the left subclavian artery; in type B, interruption exists between the subclavian and the left common carotid arteries; and in type C, interruption is distal to the brachiocepharic (innominate) artery, or to the right common carotid artery if the right subclavian artery is aberrant [8]. According to Washio [9], this anomaly can be divided into two further groups according to blood flow: type 1 is accompanied by pulmonary-ductus-descending aorta-trunk (PDDT), and type 2 (not accompanied by PDDT). The present case was type 1A IAA. It is said that in IAA there is a preferential flow of blood into the sixth arches, and the blood flow into the fourth arches is accordingly
Acta Paediatr Jpn
Low birthweight neonate (87) 577 reduced at the time of normal disappearance of components of the embryonic aortic arch system [lo]. Conal maldevelopment resulting from the displacement of the crista supraventricularis into the left ventricle has been implicated as the main factor. Another study included mitral valve obstruction, truncus arteriosus with preferential flow to the pulmonary circulation and hypoplasia of the ventricle as important factors [ 101. In the present case, there was no evidence of mitral valve obstruction, truncus arteriosus or left ventricle hypoplasia. Ekici reported that IAA could be explained by reduced blood flow into the ascending aorta due to the large leftright shunt through the VSD [3]. However, it is doubtful whether only VSD can explain reduced blood flow into the ascending aorta, in consideration of pulmonary hypertension in utero. We recognized the usefulness of aortography by radial artery injection instead of cardiac catherization for the very low birthweight neonate. Kobayashi et al. performed aortography by radial artery injection in 43 patients and diagnosed aortic arch disorders in 28 patients [ 111. In very low birthweight babies, radial artery injection is a useful diagnostic technique. It is said that after the availability of prostaglandin (PG) therapy, the condition of most neonates improves and can be ready for operation [12]. However, the present case did not improve with PG therapy. Various surgical treatments have been reviewed. In aortic repair, methods include direct anastomosis, subclavian flap construction, aortoplasty, Blalock-Park repair and tube graft repair [13-161. Barratt-Boyes et al. reported the first successful one-stage repair of IAA [ 171. Previous studies concluded that primary repair was the best approach for IAA with VSD [ 18-20]. Recently, Scott reported that one-stage complete repair of all cardiac defects should be performed whenever feasible [21]. According to Benatar et al. primary total correction using deep hypothermia with or without circulatory arrest was the procedure of choice for cases of type B interruption, while for neonates with type A interruption and VSD, it was necessary to review the need to close the VSD at a later stage after the repair of interruption [22].
Vol. 34 No. 5 October 1992
However, Ooshima et al. reported good results for a two step procedure for IAA without LVOTO: repair of the interruption followed by closure of the VSD. Other studies have insisted that the staged repair of IAA with VSD was very reliable despite the condition of the infant or major associated cardiac anomalies, and could be recommended for infants at high risk for primary repair [23,24]. In the present case, we selected two-step repair (subclavian flap construction, PA banding and PDA ligation in the first step) as an emergency procedure. Type B interruption is frequently associated with the DiGeorge syndrome [25]. However, this could not be investigated in detail as there was no thymus. References 1. van Praagh R, Bernhard WF, Rosenthal A. Interrupted aortic arch surgical treatment. Am J Cardiol 1971;27: 200. 2. Fyler C, Buckey P, Hallenbrand E et al. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980 65: 377. 3. Ekici E, Tuncali T. Interrupted aortic arch, mirror-image dextrocardia with situs inversus and Sprengel’s anomaly. Turk J Pediatr 986; 28: 77. 4. Kamau P, Miles V, Toews W. Surgical repair of coarctation of the aorta in infants less than six months of age. J Thorac Cardiovasc Surg 1981; 81: 171. 5. Zelikovsky A, Vidne B, Levy MJ. Mirror-image dextrocardia with situs inversus and coarctation of the aorta. Chest 1974; 66: 297. 6. Ono S, Sone K, Kunimine Y. Associated anomalies in interrupted aortic arch. Heart 1982; 14: 1501. 7. Moller JH, Edwards JE. Interruption of the aortic arch. Anatomic patterns and associated cardiac malformations. Am J Roentgen01Rad Ther Nucl Med 1965; 95: 557. 8. Celoria GC,Patton RB. Congenital absence of the aortic arch. Am Heart J 1959; 58: 407. 9. Wasio M, Sakurai T, Irizawa N et al. Surgical repairofIAA. Heart 1971; 3: 632. 10. Moore GW, Hutchins GM. Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches. Am J Cardiol 1978; 42: 467. 11. Kobayashi T, Sone K, Kobayashi T et al. Diagnosis of congenital heart diseases with aortography by means of radial artery injection. Kitakanto igaku 1991; 41: 335. 12. Heymann MA, Berman W, Rudolph Am, Whitman V. Dilatation of the ductus arteriosus by prostaglandin E, in aortic arch abnormalities. Circulation 1979: 59: 169.
578 (88) Y. Wakazono et al, 13. Monro JL, Bunton RW, Sutherland GR. Correc-
19. Turley K,Yee ES, Ebert PA. The total repair of
tion of interrupted aortic arch. J Thorac Cardiovasc Surg 1989;98:42 I. Sell JE, Jonas RA, Mayer JE. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96: 864. Qureshi SA, Maruszewski B, McKay R. Determinants of survival follrwing repair of interrupted aortic arch in infancy. Int J Cardioll990;26:303. Collins-Nakai RL,Macdonald R, Parisi-Buckley L. Interrupted aortic arch in infancy. J Pediatr 1976;88: 959. Barratt-Boyes BG, Nicholls TT, Brandt PWT. Aortic arch interruption associated with patent duct arteriosus, ventricular septal defect and total anomalous pulmonary venous connection. Total correction in an 8 day old infant by means of profound hypothermia and limited cardiopulmonary bypass. J Thorac Cardiovasc Surg 1972;63:
interrupted arch complex in infants. Circulation 1984;70: 116. 20. Schumacher G,Screiber R, Meisner H, Lorenz HP, Sebening F, Buhlmeyer K. Interrupted aortic arch. Pediatr Cardiol 1986;7: 89. 2 1. Scott WA, Rocchini AP, Bove EL et al. Repair of interrupted aortic arch in infancy. J Thorac Cardiovasc Surg 1988;96:564. 22. Benatar A, Antunes MJ, Kinsley RH. Aortic arch interruption in the neonate, with emphasis on early diagnosis and management. S Afr Med J
367. 18. Norwood WI, Lang P, Castaneda AR, Hougen TJ.
2483. 25. van Mierop LH, Kutsche LM. Cardiovascular
14. 15. 16. 17.
Reparative operations for interrupted aortic arch with ventricular septal defect. J Thorac Cardiovasc Surg 1983;86:832.
1989;75:315. 23. Irwin ED, Braulin EA, Forker JE. Staged repair of interrupted aortic arch and ventricular septal defect in infancy. Ann Thorac Surg 199 1;52: 632. 24. Yanaguchi M,Ohashi H, Imai M. Surgical treatment of interrupted aortic arch in infants under three months of age. Jpn J Thorac Surg 1989;37: anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. Am JCardiol 1986;58: 133.
Acta Paediatr Jpn
- 5781
The Very Low Birthweight Neonate With an Interrupted Aortic Arch and Dextrocardia Yoshihiro Wakazono, M.D.,’ ltaru Yarnada, M.D.,’ Keiichi Kagajou, M.D.,’ Masaaki Miyazaki, M.D.,’ Wlasayoshi Isshiki, M.D.,’ Naomasa Takayama, M.D.,’ Shunji Shinnoka, M.D.,2 Tetsuo Tomino, M.D.,’ and Keizou Furuya, M.D.,3 Departments of Pediatrics, 2Cardiologyand ’Pathology, Ehime Prefectural Central Hospital, Matsuyama, Japan
Type 1A interruption of the aortic arch complex accompanied by mirror-image dextrocardia with situs inversus was diagnosed by counter-current aortography through the rapial artery. This technique is useful for aortography of the very low birthweight neonate. Key Words Type 1A IAA complex, Dextrocardia, Very low birthweight neonate, Aortography by radial artery injection
Introduction
Case Report
Interruption of the aortic arch (IAA) is a rare but deadly congenital cardiovascular anomaly particularly in very low birthweight babies. We encountered an IAA complex neonate accompanied by mirror-image dextrocardia with situs inversus and dextroviscera. This combination in a very low birthweight baby is rare. Diagnosis was made using countercurrent aortography through radial artery, instead of cardiac catherization. This examination was performed in an incubator for very low birthweight neonates. In addition, we report the findings of the autopsy of the present case.
Diagnosis A iemale infant was delivered spontaneously at 35 weeks gestation, weighed 1,372g (Apgar score 8), and was She fourth child of a 35 year old mother whose three previous babies were normal. There was no history of congenital heart disease in either parent’s family. On August 21, 1989, the baby was transferred to our hospital on the second day of life because of cyanosis, tachycardia and tachypnea. Physical examination on admission revealed a pale, tachypneic neosate. Her weight was 1,369 g, height 37 cm, head circumference 27 cm and chest circumference 24 cm. Her heart rate Cas 124 beatdmin and her respiratory rate was 78 beatdmin. She showed peripheral cyanosis but no cyanosis of the trunk, She had a flat and large forehead, hyperteiorism and a cleft soft palate. Through auscultation it was determined that the cardiac apex was right-sided. There was no murmur. Both femoral pulses we‘re palpable. She did not exhibit nasal alar breathing or grunting, but her chest was slightly retracted.
Received January 22, 1992 Revised May 6, 1992 Accepted May 22, 1992 Correspondence address: Dr Yoshihiro Wakazono, Department of Pediatrics, Faculty of Medicine, Kyoto University, 54 Shogoin-Kawahara-cho,Sakyo-ku, Kyoto 606, Japan.
514 (84) Y. Wakazono et al. There was no rile. The abdomen was soft and flat, and there was no liver enlargement. A chest X-ray revealed the enlargement of the heart (cardiothoracic ratio = 0.62), dextrocardia with situs inversus and a stomach bubble on the right side (Fig. 1). An electrocardiogram showed sinus rhythm and typical findings of mirror-image dextrocardia ( - 180' inversion of P, Q, R, S and T at lead I) and hypertrophy of the right ventricle (T-wave positive at leads V 1 and V2; Fig. 2). Laboratory findings on admission showed high levels of serum basal urinary sodium, creatinine, slightly low levels of serum Na, C1, Ca and transient hyperglycemia. There was no chromosomal abnormality (Table 1). Two-dimensional echocardiographic examination demonstrated the presence of ventricular septal defect (VSD), atrial septal defect (ASD) and the absence of the aortic arch. These findings suggested IAA complex and dextrocardia with situs inversus. A 24-gauge angiocatheter was inserted percutaneously through the right radial artery, and 2 ml iopamidol was administered through this prior to a chest and abdomi-
Fig. 1: Chest X-ray.
Fig. 2: Electrocardiograph.
nal X-ray. This revealed that the iopamidol was channelled from the radial artery through to the brachial artery and right subclavian artery to the ascending aorta. There was no descending aorta. We diagnosed IAA type A and dextrocardia with situs inversus (Fig. 3). Prostaglandin El was infused to maintain ductal patency and dopamine as a pre-operative course. Urine volume gradually decreased, acidosis was progressive, and her general condition gradually worsened. An emergency operation was performed at 5 days of age.
Operation The IAA was repaired by posterolateral thoracotomy. The diameters of the aortic arch and the right subclavian artery were 2.5 mm and 3.0 mm respectively. It appeared that the junction of the aortic arch and descending aorta was blocked by a membrane. However, the pulmonary artery ran through the patent ductus arteriosus (PDA) to the descending aorta. The subclavian artery was used for the repair. It was incised longitudinally to the descending .aorta to make a flap after incision of that membrane. The descending aorta was covered by a subclavian flap. The PDA (diameter 15 mm) was ligated, and banding of the pulmonary trunk was performed. In this way, blood flow was directed from the ascending aorta through the aortic arch, the subclavian flap and to the descending
Acta Paediatr Jpn
Low birthweight neonate ( 8 5 ) 515 Table 1. Laboratory findings on admission Peripheral blood
Blood chemistry
RBC 326 1 0 4 / ~ ~ 3 GOT 40 Ku Hb 12.9 g/dl GPT 5 Ku Ht 37.6% T-Bil 5.4 mg/dl PLT 17.2 104/mm3 TP 4.0 g/dl WBC 10,500/mm3 BUU 20.5 mg/dl Bands 5% CRTN 2.9 mg/dl Segments 59% Na 131 mEq/l Eosinophils 2% K 4.2 mEq/l C1 95 mEq/l Lymphocytes 33% Monocytes 1% Ca 3.8 mEq/l BGA (025Vmin) Glu 174 mg/dl PH 1.34 HPT 26% pa* 41 mmHg PO 93 mmHg CRP 1' Hd0322 mmolfl - 3 mmol/l Karyotype 46, XX BE 0,Sat 91% RBC, red blood cells; Hb, hemoglobin; Ht, hematocrit; PLT, platelet count; WBC, white blood cells; GOT, glutamate oxaloacetate transaminase; GPT, glutamic-pyruvic transaminase; T-Bil, total bilirubin; TP, total protein; BUN, blood urea nitrogen; CRTN, creatinine; HPT, hepaplastin test, CRP, C-reactive protein.
However, heart failure progressed and she died at 9 days of age.
Autopsy Autopsy revealed situs inversus totalis and no thymus. Her right lung was divided into two parts and her left lung divided into three parts. All other viscera were in the mirror-image dextra position (Fig. 4). The heart exhibited dextrocardia with situs inversus and IAA. We recognized the junction at the subclavian flap and the descending aorta, the ligated PDA and pulmonary arterial banding (Fig. 5). The opened heart revealed VSD type 1 (8 x 8 mm; Fig. 6), and ASD (5 x 5 mm). The pulmonary and aortic valves were normal.
Discussion
Fig. 3: Aortography by radial artery injection.
aorta. One day after operation, urine volume increased and her general condition improved.
Vol. 34 No. 5 October 1992
Interrupted aortic arch is a rare congenital malformation that is usually accompanied by severe intracardiac anormalies. According to Van Praagh, IAA represented 1.4% of autopsied infants with congenital heart disease [ 11, which coincided with the New England Regional Infant Cardiac Program (NERICP) incidence of 1.3% of congenital heart disease [2]. On the other hand, the rate of dextrocardia with a normal spleen was 0.037 per 1000 live births
576 (86) Y. Wakazono et a/.
Fig. 6: The opened heart revealed VSD type 1 (8 x 8 mm; arrowed) and ASD ( 5 x 5 mm). The pulmonary and aortic valves were normal.
Fig. 4: Results of the autopsy. She had situs inversus totalis and no thymus. Her right lung was divided into two parts and her left lung divided into three parts. All other viscera were in the mirror-image dextra position.
Fig. 5 : The heart was diagnosed as dextrocardia with situs inversus and IAA. We recognized the junction between subclavian flap (+-) and the descending aorta the ligated PDA and pulmonary arterial banding. (4).
according to the NERICP. Cases of IAA with mirror-image dextrocardia are rare. Ekici et al. reported a case of IAA with mirror-image dextro-
cardia [3], and a few cases of dextrocardia associated with aortic coarctation have been reported [4,5]. However, it does not seem that IAA with dextrocardia has been reported in the very low birthweight baby. IAA has always been associated with other defects, most commonly PDA and VSD. In Japan, various anomalies accompanied by IAA have been reported [6]. In the present case, PDA, VSD, ASD and dextrocardia were identified. IAA has been classified anatomically into atresia of the aortic arch and absence of the aortic arch. However, circulation is interrupted and both anomalies are clinically treated as I A A [7]. IAA has been classified by Celoria and Patton into three groups: in type A, interruption is distal to the left subclavian artery; in type B, interruption exists between the subclavian and the left common carotid arteries; and in type C, interruption is distal to the brachiocepharic (innominate) artery, or to the right common carotid artery if the right subclavian artery is aberrant [8]. According to Washio [9], this anomaly can be divided into two further groups according to blood flow: type 1 is accompanied by pulmonary-ductus-descending aorta-trunk (PDDT), and type 2 (not accompanied by PDDT). The present case was type 1A IAA. It is said that in IAA there is a preferential flow of blood into the sixth arches, and the blood flow into the fourth arches is accordingly
Acta Paediatr Jpn
Low birthweight neonate (87) 577 reduced at the time of normal disappearance of components of the embryonic aortic arch system [lo]. Conal maldevelopment resulting from the displacement of the crista supraventricularis into the left ventricle has been implicated as the main factor. Another study included mitral valve obstruction, truncus arteriosus with preferential flow to the pulmonary circulation and hypoplasia of the ventricle as important factors [ 101. In the present case, there was no evidence of mitral valve obstruction, truncus arteriosus or left ventricle hypoplasia. Ekici reported that IAA could be explained by reduced blood flow into the ascending aorta due to the large leftright shunt through the VSD [3]. However, it is doubtful whether only VSD can explain reduced blood flow into the ascending aorta, in consideration of pulmonary hypertension in utero. We recognized the usefulness of aortography by radial artery injection instead of cardiac catherization for the very low birthweight neonate. Kobayashi et al. performed aortography by radial artery injection in 43 patients and diagnosed aortic arch disorders in 28 patients [ 111. In very low birthweight babies, radial artery injection is a useful diagnostic technique. It is said that after the availability of prostaglandin (PG) therapy, the condition of most neonates improves and can be ready for operation [12]. However, the present case did not improve with PG therapy. Various surgical treatments have been reviewed. In aortic repair, methods include direct anastomosis, subclavian flap construction, aortoplasty, Blalock-Park repair and tube graft repair [13-161. Barratt-Boyes et al. reported the first successful one-stage repair of IAA [ 171. Previous studies concluded that primary repair was the best approach for IAA with VSD [ 18-20]. Recently, Scott reported that one-stage complete repair of all cardiac defects should be performed whenever feasible [21]. According to Benatar et al. primary total correction using deep hypothermia with or without circulatory arrest was the procedure of choice for cases of type B interruption, while for neonates with type A interruption and VSD, it was necessary to review the need to close the VSD at a later stage after the repair of interruption [22].
Vol. 34 No. 5 October 1992
However, Ooshima et al. reported good results for a two step procedure for IAA without LVOTO: repair of the interruption followed by closure of the VSD. Other studies have insisted that the staged repair of IAA with VSD was very reliable despite the condition of the infant or major associated cardiac anomalies, and could be recommended for infants at high risk for primary repair [23,24]. In the present case, we selected two-step repair (subclavian flap construction, PA banding and PDA ligation in the first step) as an emergency procedure. Type B interruption is frequently associated with the DiGeorge syndrome [25]. However, this could not be investigated in detail as there was no thymus. References 1. van Praagh R, Bernhard WF, Rosenthal A. Interrupted aortic arch surgical treatment. Am J Cardiol 1971;27: 200. 2. Fyler C, Buckey P, Hallenbrand E et al. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980 65: 377. 3. Ekici E, Tuncali T. Interrupted aortic arch, mirror-image dextrocardia with situs inversus and Sprengel’s anomaly. Turk J Pediatr 986; 28: 77. 4. Kamau P, Miles V, Toews W. Surgical repair of coarctation of the aorta in infants less than six months of age. J Thorac Cardiovasc Surg 1981; 81: 171. 5. Zelikovsky A, Vidne B, Levy MJ. Mirror-image dextrocardia with situs inversus and coarctation of the aorta. Chest 1974; 66: 297. 6. Ono S, Sone K, Kunimine Y. Associated anomalies in interrupted aortic arch. Heart 1982; 14: 1501. 7. Moller JH, Edwards JE. Interruption of the aortic arch. Anatomic patterns and associated cardiac malformations. Am J Roentgen01Rad Ther Nucl Med 1965; 95: 557. 8. Celoria GC,Patton RB. Congenital absence of the aortic arch. Am Heart J 1959; 58: 407. 9. Wasio M, Sakurai T, Irizawa N et al. Surgical repairofIAA. Heart 1971; 3: 632. 10. Moore GW, Hutchins GM. Association of interrupted aortic arch with malformations producing reduced blood flow to the fourth aortic arches. Am J Cardiol 1978; 42: 467. 11. Kobayashi T, Sone K, Kobayashi T et al. Diagnosis of congenital heart diseases with aortography by means of radial artery injection. Kitakanto igaku 1991; 41: 335. 12. Heymann MA, Berman W, Rudolph Am, Whitman V. Dilatation of the ductus arteriosus by prostaglandin E, in aortic arch abnormalities. Circulation 1979: 59: 169.
578 (88) Y. Wakazono et al, 13. Monro JL, Bunton RW, Sutherland GR. Correc-
19. Turley K,Yee ES, Ebert PA. The total repair of
tion of interrupted aortic arch. J Thorac Cardiovasc Surg 1989;98:42 I. Sell JE, Jonas RA, Mayer JE. The results of a surgical program for interrupted aortic arch. J Thorac Cardiovasc Surg 1988;96: 864. Qureshi SA, Maruszewski B, McKay R. Determinants of survival follrwing repair of interrupted aortic arch in infancy. Int J Cardioll990;26:303. Collins-Nakai RL,Macdonald R, Parisi-Buckley L. Interrupted aortic arch in infancy. J Pediatr 1976;88: 959. Barratt-Boyes BG, Nicholls TT, Brandt PWT. Aortic arch interruption associated with patent duct arteriosus, ventricular septal defect and total anomalous pulmonary venous connection. Total correction in an 8 day old infant by means of profound hypothermia and limited cardiopulmonary bypass. J Thorac Cardiovasc Surg 1972;63:
interrupted arch complex in infants. Circulation 1984;70: 116. 20. Schumacher G,Screiber R, Meisner H, Lorenz HP, Sebening F, Buhlmeyer K. Interrupted aortic arch. Pediatr Cardiol 1986;7: 89. 2 1. Scott WA, Rocchini AP, Bove EL et al. Repair of interrupted aortic arch in infancy. J Thorac Cardiovasc Surg 1988;96:564. 22. Benatar A, Antunes MJ, Kinsley RH. Aortic arch interruption in the neonate, with emphasis on early diagnosis and management. S Afr Med J
367. 18. Norwood WI, Lang P, Castaneda AR, Hougen TJ.
2483. 25. van Mierop LH, Kutsche LM. Cardiovascular
14. 15. 16. 17.
Reparative operations for interrupted aortic arch with ventricular septal defect. J Thorac Cardiovasc Surg 1983;86:832.
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Acta Paediatr Jpn
