Br Heart J 1990;64:385-7 385 Br Heart J: first published as 10.1136/hrt.64.6.385 on 1 December 1990. Downloaded from Anomalous subaortic position of the brachiocephalic vein (innominate vein): an echocardiographic study

Jung Yun Choi, Mi Jin Jung, Young Hwi Kim, Chung II Noh, Yong Soo Yun

Abstract department of paediatrics, Seoul National In 24 (0-98%) of 2457 patients with con- University Children's Hospital to find out the genital heart disease the brachiocephalic total number of new patients with congenital vein was in an anomalous position below heart disease, the types of cardiac defect, and the aortic arch. This is a much higher the patients who had an anomalous brachio- proportion of such cases than reported cephalic vein running below the aortic arch. If so far. This high frequency may arise necessary, the diagnosis of the cardiac defects from differences in the study population was updated after cardiac catheterisation or and the method of diagnosis. This venous cardiac surgery. anomaly was more common in patients The diagnosis of a subaortic brachiocephalic with tetralogy of Fallot or ventricular vein was mainly based on the cross sectional septal defect with pulmonary atresia. echocardiographic and Doppler findings. In Patients with the venous anomaly were the first few cases cardiac catheterisation con- more likely to have a right aortic arch. firmed the anomalous position of the brachio- The anomalous course of the brachio- cephalic vein. Some cases had surgical confir- cephalic vein from the neck to the junc- mation too. tion of the superior vena cava was shown We used the x2 test or Fisher's exact test for by cross sectional echocardiography. In statistical analysis and a p value < 0-05 was doubtful cases, Doppler study usually regarded as significant. clarified the anatomical arrangement. Results

Twenty four (098%) patients with an copyright. The left innominate vein extends from the anomalous subaortic brachiocephalic vein were junction of the left internal jugular vein and found among 2457 patients with congenital the left subclavian vein to the superior vena heart disease. Twenty three patients had a cava. It runs above and in front of the aortic normally arranged atria and the left brachio- arch and in front of the left subclavian, left cephalic veins were below the aortic arch. One common carotid, and innominate arteries. In patient had atrial inversion, a left sided atrial situs inversus the arrangement of the superior vena cava, and a right brachiocephalic systemic veins is usually the mirror image of vein associated with a complex intracardiac http://heart.bmj.com/ this. defect. In this patient the right brachiocephalic Rarely the left brachiocephalic vein takes an vein was below the left aortic arch and anomalous course below the aortic arch. This intracardiac defects consisted of discordant was first described by Kershner a hundred atrioventricular connection with single atrio- years ago.' Smallhorn et al described echo- ventricular valve, single aorta from right ven- cardiographic findings of this anomaly' and tricle, and pulmonary atresia. Table 1 shows Gerlis and Ho recently reviewed a total of 25 the frequency with which the venous anomaly patients including three of their own.3 was found in several cardiac malformations. It

Though this anomaly is not clinically was very common in cases of tetralogy of Fallot on September 29, 2021 by guest. Protected important, it may be confused with the pul- and ventricular septal defect with pulmonary monary artery in cross sectional echocar- atresia. Table 2 summarises the clinical charac- diography especially in the patients with very teristics and the frequency of right aortic arch hypoplastic or atretic central pulmonary in the patients who had either tetralogy of Department of arteries. Fallot or ventricular septal defect with Paediatrics, Seoul National University Gerlis and Ho thought that this venous Children's Hospital, anomaly was very rare-about one in 500 Table I Frequency of anomalous brachiocephalic vein in Seoul, Korea congenitally malformed hearts. But we found some cardiac malformations J Y Choi that this lesion is not rare in certain cardiac M J Jung Cardiac No with No with Frequency Y H Kim defects. We found 24 cases among 2457 lesion cardiac defect anomalous vein (%) C I Noh echocardiograms of congenital heart disease in Y S Yun VSD 1042 1 0-01 21 months and we report the incidence and TOF 177 10 5-7 Correspondence to associated congenital heart diseases in this VSD + PA 57 10 17-5 Dr Jung Yun Choi, Others 1181 3* Deparanent of Paediatrics, malformation. Seoul National University Total 2457 24 0-98 Children's Hospital, 28 Yeunkun-Dong, *Each patient had truncus arteriosus, complete transposition Chongro-Ku, Seoul 110-744, Patients and methods of the great arteries, and a complex anomaly with inverted Korea. atrial situs. Accepted for publication We reviewed echocardiograms obtained be- PA, pulmonary atresia; TOF, tetralogy of Fallot; VSD, 3 July 1990 tween January 1988 and September 1989 in the ventricular septal defect. 386 Choi, Jung, Kim, Noh, Yun Br Heart J: first published as 10.1136/hrt.64.6.385 on 1 December 1990. Downloaded from Table 2 Clinical characteristics and laterality ofaortic arch in patients with subaortic associated pulmonary atresia and the brachio- brachiocephalic vein cephalic vein in an anomalous position. The Proportion of table also shows how often a right aortic arch Cardiac Age Sex Proportion right AA in was diagnosed in patients with the same cardiac defect (mean) ratio of right those without (n) (range) (M:F) AA (%) venous anomaly (%) defects but without an anomalous brachio- cephalic vein during the same period. A right TOF 1 yr 9 irmth 2 3:1 80 20-4 aortic arch was significantly more common in (10) (1 mnth-10 yr) VSD + PA 1 yr9mnth 2-3:1 50 21-3 patients with an anomalous vein than in those (10) (3 mnth-10 yr) without (p < 0.05 in tetralogy of Fallot and p < 0.05 in ventricular septal defect with AA, aortic arch. See footnote to table 1 for other abbreviations. pulmonary atresia). Five patients had a persistent ductus Figure 1 Frontal view from suprasternal notch arteriosus. Three of them had a ventricular showing the subaortic left septal defect, pulmonary atresia, a right aortic brachiocephalic vein arch, and a left ductus arteriosus arising from (arrows) running in front of the left subclavian the left innominate artery. Another patient had artery and below the left tetralogy of Fallot, left aortic arch, and a left innominate artery and ductus arteriosus and the remaining patient aortic arch. Ao, aorta; had atrial situs inversus, a left aortic arch, right IA, innominate artery. ductus arteriosus from the right innominate artery, and the intracardiac defects described earlier. The ductus arteriosus always passed in front of the anomalous brachiocephalic vein. Ten of the 19 patients without a persistent ductus arteriosus had an operation. But in the surgical descriptions of these operations there was no comment of the presence of a ligamen- tum arteriosum or its relation to the anomalous Figure 2 Suprasternal vein. para-sagittal view shows Cross sectional echocardiography in the two circular structures below the aortic arch. The suprasternal frontal plane showed the upper circle (arrows) is anomalous left brachiocephalic vein between the left brachiocephalic the right pulmonary artery (below) and the

vein and the lower one is copyright. the right pulmonary artery aorta (above). This anomalous vein could (RPA). usually be traced to the left side of the neck when the transducer is tilted leftwards and anteriorly. In patients with a right aortic arch and left innominate artery, the anomalous vein descended from the left side of the neck and crossed the left subclavian artery anteriorly, ran under the left innominate artery, and then joined the superior vena cava (fig 1). When the http://heart.bmj.com/ ductus arteriosus arose from the left in- nominate artery, slight tilting of the transducer Figure 3 Doppler showed the relation between the vein and the spectrum (B) recorded ductus. But in patients with a left aortic arch from the left this vein ran in front of the left common carotid brachiocephalic vein (A) and showing low velocity and left subclavian arteries so that it was very venousflow toward the difficult to get a simultaneous demonstration of heart. this vein and head and neck arteries.

The parasagittal view showed two circles on September 29, 2021 by guest. Protected below the aortic arch. The upper one is the anomalous vein and the lower one is the right pulmonary artery (fig 2). Doppler examination showed the typical venous flow signal toward the heart (fig 3).

Discussion The anomalous subaortic course of the left brachiocephalic vein was first described about a hundred years ago.' Gerlis and Ho reviewed 25 cases, including three of their own patients.3 They reported that all these patients had an anomalous left brachiocephalic vein. In our study we found one patient who had a right brachiocephalic vein below the left aortic arch with an inverted atrial arrangement. Gerlis and Ho estimated the incidence of this malforma- tion to be about 0 2% in their necropsy series. Anomalous subaortic position of the brachiocephalic vein (innominate vein): an echocardiographic study 387 Br Heart J: first published as 10.1136/hrt.64.6.385 on 1 December 1990. Downloaded from In our clinical study we found the anomaly in with other vessels, and in doubtful cases Dop- 0-98% of patients. This difference may be due pler study usually clarifies the situation. to the difference in the study population and The most frequently associated cardiac the method of diagnosis. The two echocar- defects were tetralogy of Fallot and ventricular diographers in our laboratory are well aware of septal defect with pulmonary atresia, both of this anomalous position of the brachiocephalic which were present in 20 of the 24 cases with vein and the echocardiograTphic findings when the venous anomaly. Another two patients had this lesion is present are so characteristic that conotruncal anomalies-truncus arteriosus or they are unlikely to miss or overdiagnose this transposition of the great arteries. There were lesion. The lower incidence found in the 22 conotruncal anomalies in total. This associa- necropsy series may have been lower because tion of anomalous subaortic brachiocephalic tetralogy of Fallot and ventricular septal defect vein with the conotruncal anomalies seems to with pulmonary atresia, both conditions in be highly significant. In addition, a right aortic which the venous anomaly is common, do not arch was more common in patients with the have a high mortality rate. Therefore these two venous anomaly than in those without. The cardiac malformations were less common in the embryological origins of the anomalous vein necropsy series. Furthermore this lesion might have been reviewed' but the relation between be missed during the routine examinations. the abnormal position of the brachiocephalic Because we do not know of another clinical vein, conotruncal cardiac anomalies, and the study ofthe incidence ofthis venous anomaly it right aortic arch has yet to be established. is impossible to tell whether there is a racial variation. This study was partly supported by the Seoul National Univer- Smallhorn et al described the echocardio- sity Children's Hospital Research Fund. graphic findings associated with anomalous brachiocephalic vein and cautioned that this lesion might be confused with the central 1 Kershner L. Zur Morphologie der Vena Cava Inferior. Anat Anz 1888;3:808-23. (Cited in reference 3 by Gerlis and pulmonary artery.2 The echocardiographic Ho.) findings in our cases are almost the same as 2 Smallhorn JF, Zielinsky P, Freedom RM, Rowe RD. et in Abnormal position of the brachiocephalic vein. Am J those described by Smallhorn al, and Cardiol 1985;S5:234-6. addition, we found that this vein was usually 3 Gerlis LM, Ho SY. Anomalous subaortic position of the brachiocephalic (innominate) vein: a review of published traceable to the left side ofthe neck. Ifthis vein reports and report of three new cases. Br Heart J is properly traced, it is not likely to be confused 1989;61:540-5. copyright. http://heart.bmj.com/ on September 29, 2021 by guest. Protected