S. No. Authors Year Age/Sex Diagnosis, investigations, surgical and necropsy findings Surgery Results Follow-up
1. Gerlis LM and Ho SY et al18 1989 5 years 11 months/F Case 1 (Necropsy): Died of cerebral ischemia-postop TOF. Postmortem-TOF, several large MAPCAs, RAA, retroaortic BCV joining RSVC below azygous vein above the right superior cavoatrial junction Postoperative TOF Not applicable Not applicable
Male infant Case 2 (Necropsy): Died of complex cardiac malformation. PM examination: concordant AV connection, DORV, VSD, destroposed aorta, PAD, retroaortic BCV anterior to the arterial duct between the arch and pulmonary trunk, joined RSVC between azygous vein and right atrium Not applicable Not applicable Not applicable
7 week/F Case 3 (Necropsy): Died attempted surgical correction-common arterial trunk. Concordant AV connection, subtruncal VSD, quadricuspid truncal valve, retroaortic BCV below the aortic arch, joined below the azygous vein Not applicable Not applicable Not applicable
2. Kerschner L et al7 1988 ? child, ? sex Necropsy, relation to arterial duct ?, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
3. Daser P et al39 1902 68 years/M Necropsy, anterior to arterial duct, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
4. Ghon A et al64 1908 4½ months/F Necropsy, posterior to arterial duct, right aortic arch, absent 4th aortic arch, isolated LSA Not applicable Not applicable Not applicable
5. Nutzel H et al40 1914 74 years/M Necropsy, left aortic arch, right superior pulmonary vein to RSVC Not applicable Not applicable Not applicable
6. Martin CP et al65 1931 17 years/M Necropsy, right aortic arch, anterior to arterial duct, TOF Not applicable Not applicable Not applicable
7. Walter L et al41 1931 19 years/M Necropsy, anterior to arterial duct, left aortic arch, subthyroid venous anastomosis Not applicable Not applicable Not applicable
8. Adachi B et al‑37 1933 41 years/M Necropsy, posterior to arterial duct, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
9. Adachi B et al37 1933 20 years/M Necropsy, anterior to arterial duct, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
10. Friedman SM et al42 1945 66 years/M Necropsy, anterior to arterial duct, left aortic arch, left jugular vein anomaly Not applicable Not applicable Not applicable
11. Roberts JR et al1 1951 7 years/F Angio done, BCV located subpulmonary, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
12. Sherman FE et al38 1963 ? Necropsy, anterior to arterial duct, left aortic arch, pulmonary atresia, VSD Not applicable Not applicable Not applicable
13. Sherman FE et al38 1963 ? Necropsy, anterior to arterial duct, left aortic arch, pulmonary atresia, VSD Not applicable Not applicable Not applicable
14. Yoshida Y et al63 1975 68 years/M Necropsy, anterior to arterial duct, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
15. Kitamura S et al66 1981 69 years/M Necropsy, anterior to arterial duct, left aortic arch, no associated anomalies Not applicable Not applicable Not applicable
16. Cloez JL et al8 1982 1½ year/M Echo+Angio, left aortic arch, TOF Not mentioned Not mentioned Not mentioned
17. Smallhorn JE et al19 1985 7 patients Right aortic arch (6), left aortic arch (1), RVOT anomalies, “frequent: intracardiac anomalies (6), VSDs?, central pulmonary arteries absent (1) Not mentioned Not mentioned Not mentioned
18.
Townsend MD et al13
2008
9 years/F
Echo+Cath+Angio Diagnosis: day 8 bicuspid aortic valve, AP window repaired under DHCA, no mention of retroaortic BCV, persistent systemic arterial desaturation 9 years- hospitalized ventilated, +ve pressure, with nitric oxide. TTE- suprasystemic RVSP, CT-angio- posterior pulmonary venous drainage with a confluence draining in the LA. LSVC connected to LA appendage- LA roof junction, retroaortic BCV connected to LSVC and drained to SVC-RA junction. Saline contrast echo- bubble in LA and RA
A 5 cm long window like communication between LSVC and LAA to extend to the roof of the LA; biatrial drainage of retroaortic BCV- direct suture closed of the window
Unremarkable, SaO2 normalized.
Not mentioned
19. Towbin JA et al12 1987 18 months/M Echo- TOF, high ASD, absent hepatic portion of IVC with azygous continuation, LA isomerism, RAA, retroaortic BCV (contrast echo with left hand injection), confirmed the diagnosis Not mentioned Not mentioned Not mentioned
20. Walsh R et al29 2017 5 years/M Cardiology evaluation of a murmur. Echo- double aortic arch. MRI- evaluation of vascular ring and airway compression, left dominant double aortic arch with a retroaortic BCV. No airway compression Conservative management Not applicable Not applicable
21. Morhy Borges Leal S23 2002 9 months/F Diagnosis- Echo, TOF+PS, right aortic arch, no associated anomalies, retroaortic BCV Done Survived Not mentioned
1 year 3 months/M Echo, TOF+PS, right aortic arch, no associated anomalies, retroaortic BCV Done Not mentioned Not mentioned
2 years 1 month/F Echo, TOF+PS, left aortic arch, no associated anomalies, , retroaortic BCV Surgery done Not mentioned Not mentioned
7 years 5 months/F Echo, TOF, PA atresia, right aortic arch, absent LPA, hypoplastic RPA, MAPCA’s present, retroaortic BCV Surgery done Not mentioned Not mentioned
1 year 1 month/F Echo, TOF+PS, left aortic arch, associated supravalvar PS, retroaortic BCV Surgery done Not mentioned Not mentioned
7 years/F Echo, TOF+PS, right aortic arch, retroaortic BCV Surgery done Not mentioned Not mentioned
1 month/M Echo, TOF+PS, right aortic arch, retroaortic BCV Surgery done Not mentioned Not mentioned
8 months/F Necropsy, TAPVC, AVSD, DORV, right isomerism, left aortic arch, Echo diagnosis Necropsy Not applicable Not applicable
20 years/M Echo, TOF, PS, right aortic arch, no associated anomalies Surgery done Not mentioned Not mentioned
36 years/F Echo, ASD, PS, left aortic arch, no associated anomalies Surgery done Not mentioned Not mentioned
18 years/F Echo, TOF, PS, left aortic arch Surgery done Not mentioned Not mentioned
8 months/M Echo, TOF, PS, right aortic arch, Surgery done Not mentioned Not mentioned
15 years/F Echo, Normal heart, left aortic arch Not applicable Not applicable Not applicable
1 month/F Echo+ angio, TOF pulmonary atresia, left aortic arch, no associated anomalies Not mentioned Not mentioned Not mentioned
22. Ming Z et al35 2009 4 months/M Pulmonary atresia, VSD, PAD, ASD, right aortic arch, right tracheal bronchus, CT-angio, mild compression of the esophagus by retroesophageal left BCV, Not mentioned Not mentioned Not mentioned
6 months/M Echo, VSD, CT angio- retroesophageal BCV Not mentioned Not mentioned Not mentioned
6 days/F Pulmonary atresia, VSD, PAD, left aortic arch, CT-angio,retroesophageal left BCV Not mentioned Not mentioned Not mentioned
6 months/F VSD, PAD, CT angio, retroaortic BCV divided into two branches. Anterior and thinner branch was located above the aortic arch, drained into SVC; posterior thicker branch routed posterior to esophagus and joined the azygous vein before draining to RSVC Not mentioned Not mentioned Not mentioned
23. Kim HJ et al28 1994 61 years/F Sudden retrosternal pain, CXR- prominent aorta, mild widening of superior mediastinum, CT- highly enhancing venous structure lateral to aortic arch. The retroaortic BCV joined RSVC below the azygous vein; venography- confirmed the CT findings. Diagnosis of chronic liver disease Not mentioned Not mentioned Not mentioned
46 years/M CXR- widened superior mediastinum, CT- retroaortic BCV. The vein abruptly changes its course medially at the level of APW, continued between aortic arch and lower trachea joined RSVC below azygous vein Not mentioned Not mentioned Not mentioned
24. Elami A et al57 1985 6 years/F Cyanosed TOF, CXR- classical TOF, Echo- TOF, RVOT infundibular gradient 97mmHg, absent pulmonary valve, confluent PA, RAA with mirror image branching, juxtaductal COA gradient 48 mmHg, PFO, retroaortic BCV, cath angio confirmed the diagnosis Right thoracotomy extended resection end to end eanastomosis; 2 months later, ICR-TOF Survived At 12 months asymptomatic
25. Mill MR et al2 1993 3 days/M IAA, APW, VSD anomalous RSA from the DTA, retroaortic BCV Not mentioned Not mentioned Not mentioned
50 months/F TOF, right aortic arch, PFO, retroaortic BCV Not mentioned Not mentioned Not mentioned
6 months/F TOF, right aortic arch, ASD, LSVC, retroaortic BCV Not mentioned Not mentioned Not mentioned
25 months/M TOF, right aortic arch, Cornelia de Lange syndrome, retroaortic BCV Not mentioned Not mentioned Not mentioned
19 months/F TOF, right aortic arch, Trisomy 21, retroaortic BCV Not mentioned Not mentioned Not mentioned
55 months/M TOF, right aortic arch, PFO, retroaortic BCV Not mentioned Not mentioned Not mentioned
24 months/M TOF, right aortic arch, retroaortic BCV Not mentioned Not mentioned Not mentioned
26. Amerasekera SSH, McGurk SP69 2009 30 years/M MRI, structurally normal heart, retroaortic BCV Not mentioned Not mentioned Not mentioned
27. Fujimoto K et al10 1992 54 years/F CXR- “bucking” of the aortic arch, suggestive of aortic aneurysm, MRI- anomalous left BCV Not mentioned Not mentioned Not mentioned
28. Curtil A et al77 1999 N=25 patients TOF, right aortic arch (19, 70%), ultrasonographic diagnosis (19, 70%), during surgery diagnosis established (6, 22%), associated cardiomyopathy (5), malformational syndromes (2), retroesophageal subclavian artery (2), Di-Georges syndrome (1) One stage ICR (13), systemic pulmonary shuntICR (10), not operated (2) Not mentioned Not mentioned
29. Kitamura S et al66 1981 69 years/M Necropsy study- retroaortic left BCV (diameter 13mm, length 7.9 cm), right SVC (diameter 15mm, length 6.7 cm) Not mentioned Not mentioned Not mentioned
30.
Chen SJ et al78
2005
N=30 patients (18 males)
Age: 13 days-36 years, median 1 year 7 months, left anomalous BCV (27), right anomalous BCV (1), anomalous BCV bridging between bilateral SVC (2), TOF (23), RAI (4), ASD (1), VSD (1), DORV (1), RAA (21), TOF with P atresia (10), no LSVC
Not mentioned
Not mentioned
Not mentioned
31. Semionov A, Kosiu KJ et al33 2017 48 years/F Diagnosis- carcinoma colon, no CHD, no vascular anomaly, contrast CT- normal heart and great vessels, retroaortic BCV Not mentioned Not mentioned Not mentioned
32. Kwon OH et al47 2014 72 years/M Evaluation of incidental lung mass on CXR, CT chest- evaluation of pulmonary nodule, double left BCV and PLSVC, left BCV – anterior branch, normally placed left BCV; posterior branch below the aortic arch, drained in RSVC, PLSVC- connected to A via coronary sinus Right middle lobectomy for squamous cell carcinoma Survived Not mentioned
33. Shaffer EW 1986 3 years/F Evaluation of murmur, cyanosis, hypoxic spell at 5 months age, Cath- TOF, RAA, mirror image branching, left BT shunt at 5 months age, repeat cath, Echo- diagnosis confirmed, retroaortic BCV Intracardiac repair Not mentioned Not mentioned
34. Kulkarni S et al24 2008 0.4 years/M Mitral atresia, hypoplastic LV, DORV, LAA, VSD, PS, normal pulmonary atresia, retroaortic BCV Surgery done Not mentioned Not mentioned
0.8 years/F TOF, RAA, normal pulmonary arteries, retroaortic BCV Surgery done Not mentioned Not mentioned
1.5 years/M TOF, RAA, retroaortic BCV Surgery done Not mentioned Not mentioned
16 years/F TOF, RAA, pulmonary atresia, retroaortic BCV Surgery done Not mentioned Not mentioned
1.5 years/M TOF, PA, post BT shunt, pulmonary atresia, retroaortic BCV Surgery done Not mentioned Not mentioned
1.5 years/M TOF, RAA, pulmonary artery normal, retroaortic BCV Surgery done Not mentioned Not mentioned
0.4 years/F TOF, severe PS, post BT shunt, RAA, retroaortic BCV Surgery done Not mentioned Not mentioned
0.1 years/M TOF, sevre PS, RAA, retroaortic BCV Surgery done Not mentioned Not mentioned
35. Yigit AE et al31 15 years/M Recurrent lung infection, CXR- mediastinal widening, CT angio – retroaortic BCV, divided into 2 branches at thoracic inlet. Anterior and thinner branch- above the aortic arch, anterior to CA then passed between the brachiocephalic trunk, and LCCA- ten joined SVC, posterior thicker branch- coursed posterior to trachea + esophagus joined the azygous vein at the level of T2 and finally to RSVC, no cardiovascular anomaly. Not mentioned Not mentioned Not mentioned
36. Shim MS et al46 2010 53 years/M Chest wall pain, CT- lung carcinoma, RUL, retroaortic BCV divided into two branches at the level of aortic arch. Anterior branch- above the aortic arch coursed anterior to the LCCA and BCA- joined SVC, posterior branch below the aortic arch, posterior to ascending aorta, both joined RSVC separately, no cardiovascular anomaly Refused treatment Not applicable Not applicable
37. Topcuoglu OM et al45 2014 62 years/M HIV infection with cold, contrast CT- double left BCV, anterior vein normally located, posterior vein thinner, accessory branch coursing posterior to the aortic arch, anterior vein joined RSVC, accessory vein joined superior to azygous vein, diameter LBCV 12.5mm, accessory BCV 2.2 mm, RBCV 13mm Not mentioned Not mentioned Not mentioned
38. Takada Y et al17 1992 6 patients M / 2 patients F Mean age 35.7 years (range 14-58 years), CT- retroaortic BCV (6), double BCV (2), associated CHD (2), MRI same findings Not mentioned Not mentioned Not mentioned
39. Khoury NJ et al22 2008 53 years/M Hypertension, hyperlipidemia, interscapular pain, MR angio+CT angio- high distal aortic arch, descending portion of the high arch- narrower in caliber for 6 cm, ascending portion- dilated, pseudocoarctation aorta, retroaortic BCV Not mentioned Not mentioned Not mentioned
40. Bartoli JM et al55 42 years/M Known case of AR for 30 years, CXR LVE aortography- grade IV AR, high aortic arch, CT- high aortic arch dilated at the origin of LSA, retroaortic BCV, pseudocoarctation aorta AVR bioprosthetic (CE bio 27mm) Not mentioned Not mentioned
41. Subirana MI et al43 1986 23 years/F Cyanotic CHD- evaluation, Echo, DORV, straddling tricuspid valve, 2 large VSDs, angio double left BCV above + below the aortic arch Left modified BT shunt Survived Not mentioned
42. Koutlas TC et al56 1998 2 years Diagnosis after birth- tricuspid atresia, VSD, normally related great arteries, mild deviation infundibular septum, hypoplastic RV. BAS – PA banding at 3 weeks age, Cath at 2 years- non-distorted branch PAS, mean pressure 12 mmHg, QP:QS 0.9:1, PVR 2 woods unit. Retroaortic BCV passed between LPA (anterior) and left main bronchus (posterior), 1.5 cm below the carina- compression of the left BCV between LPA and left main bronchus CPB, circulatory arrest at 18°C, left cavopulmonary anastomosis, end to side LPA. MPA divided distal to PAB. Hemi Fontan type right cavopulmonary connection, proximal MPA to aorta anastomosis like DKS, 1 year later- fenestrated lateral tunnel Fontan Survived Not mentioned
43. Agarwal R et al‑74 2006 1 year 5 months Diagnosis- tricuspid atresia, pulmonary atresia, RAA, retroaortic anomalous BCV. Day 1- LMBT, 6 months- Aorta, RPA (central shunt), 9 months- blocked central shunt, narrowed LMBT, stenting of the narrowed PAD, 1 year 5 months- narrowed LMBT, narrowed stended ductus, parallel course of the retroaortic BCV Bi-directional Glenn, PA reconstruction. Aorta was transected for unimpeded visualization Survived Asymptomatic, SaO2 87%
44. Konstantinov EI et al5 2003 New born/F Weight 3.12 kg. Echo- COA, unrestrictive VSD, posterior malalignment outlet septum, subaortic stenosis, restrictive PFO, almost closed ductus, bicuspid aortic valve, good ventricular function, aortic annulus 0.5 cm, pulmonary annulus 1.1 cm, tricuspid annulus 1.2 cm, ascending aorta proximal to LCA 0.45 cm, juxtaductal COA, retroaortic BCV, PGE1 0.05 µg/kg/min. CPB- bicaval cannulation, cooled to 25°C, cardioplegic arrest, PAD ligated, circulatory arrest for 25 min, ascending aorta transected – BCV transposed in front of ascending aorta, hypoplastic aorta ligated distal to LSA- ascending aorta continuity restored by a “semilunar anastomosis, descending aorta to ascending aorta anastomosis Survived Discharged home on 10th postoperative day
45. Nakamura Y et al32 2006 70 year/M Diagnosis- post aortic left innominate vein, distal aortic arch aneurysm Total arch replacement, antegrade cerebral perfusion at 20°C Survived Not mentioned
46. Lim ZN et al75 2018 3 month/F Echo, CHD, cyanosis, TOF, RAA, retroaortic BCV CPB, intracardiac repair Survived At 2 months, asymptomatic
47. Chu MW et al56 2007 4 weeks neonate Echo, MR angio, respiratory distress, interrupted aortic arch, aberrant left subclavian artery, VSD, retroaortic BCV CPB, VSD closure- Gortex patch, restoration of aortic continuity Survived At 3 months, asymptomatic
48. Kawara T et al48 2003 3 months/M Cath, angio, Tricuspid atresia, anomalous BCV, RVOTO, VSD, pulmonary stenosis, left aortic arch Bilateral bidirectional Glenn without CPB Survived At 2 years awaiting completion Fontan