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May 1973, Vol 125, No. 5 >
Article | May 1973

Systemic Venous Anomalies and Partial Heterotaxia With Normal Heart FREE

P. Syamasundar Rao, MB, DCH; Marian E. Molthan, MD
[+] Author Affiliations

Received for publication Feb 7, 1972; accepted Jan 25, 1973.

Reprint requests to Department of Pediatrics, Medical College of Georgia, Augusta, GA 30902 (Dr. Rao).


Am J Dis Child. 1973;125(5):749-752. doi:10.1001/archpedi.1973.04160050089019.
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References
Comments

A case of infrahepatic interruption of inferior vena cava with hemiazygos continuation into a persistent left superior vena cava and partial visceral heterotaxia in association with an otherwise normal heart is presented. The peculiarity of this rare lesion without cardiac malformation and the possible explanation for it are discussed. The clinical significance of systemic venous anomalies is discussed.

REFERENCES

1 +
Lev M, Rowlatt UF:  The pathologic anatomy of mixed levocardia . Am J Cardiol 8:216-263, 1961;.
2 +
Arcilla RA, Gasul BM:  Congenital dextrocardia: Clinical, angiocardiographic, and autopsy studies on 50 patients . J Pediatr 58:39-58, 251-262, 1961;.
3 +
Van Praagh R, et al:  Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications . Am J Cardiol 13:510-531, 1964;.
4 +
Campbell M, Deuchar DC:  The left-sided superior vena cava . Br Heart J 16:423-439, 1954;.
5 +
Anderson RC, Adams P Jr, Bruke B:  Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava)-report of 15 new cases . J Pediatr 59:370-383, 1961;.
6 +
Moller JH, et al:  Congenital cardiac disease associated with polysplenia: A developmental complex of bilateral "leftsidedness." Circulation 36:789-799, 1967;.
7 +
Derra VE, Loogen F, Satter P:  Anomalien der unteren Hohlvene . Dtsch Med Wochenschrift 90:689-695, 1965;.
8 +
Kaindl VF, et al:  Über eine seltene Anomalie der Vena Cava inferior . Radiol Austriaca 18:45-52, 1968;.
9 +
Leachman RD, Sloves AJ:  Relationship of the azygos venous system to normal atrial situs in levocardia . Circulation 29:901-904, 1964;.
10 +
Hastreiter AR, Rodriguez-Coronel A:  Discordent situs of thoracic and abdominal viscera . Am J Cardiol 22:111-118, 1968;.
11 +
Ruttenberg HD, et al:  Syndrome of congenital cardiac disease with asplenia: Distinction from other forms of congenital cyanotic cardiac disease . Am J Cardiol 13:387-406, 1964;.
12 +
Ivemark BI:  Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood: An analysis of the heart malformations in the splenic agenesis syndrome with 14 new cases . Acta Paediatr Scand 44( (suppl 104) ):1-110, 1955;.
13 +
Rodin AE, Sloane JA, Ngheim QX:  Polysplenia with severe congenital heart disease and Howell-Jolly bodies . Am J Clin Pathol 58:127-134, 1972;.
14 +
Putschar WGJ, Manion WC:  Congenital absence of the spleen and associated anomalies . Am J Clin Pathol 26:429-470, 1956;.
15 +
Diener KA:  Agenesia del bazo: Presentacion de un caso sin malformaciones congenitas del corazon . Bol Med Hosp Infant 19:711-715, 1962;.
16 +
Baillie M:  An account of a remarkable transposition of the viscera . Philosophical Trans 78:350-363, 1788;.
17 +
Tucker AS, Liebman J, Bolande RP:  Heterotaxy as related to congenital heart disease . Ann Radiol 9:178-184, 1966;.
18 +
Ruttenberg HD:  Corrected transposition of the great vessels: Splenic syndromes (asplenia, polysplenia) , in Moss AJ, Adam FH (eds): Heart Disease in Infants, Children, and Adolescents . Baltimore, Williams & Wilkins Co, 1968;, chap 26.
19 +
Freedom RM:  The heterotaxy syndrome , abstracted. Circulation 44( (suppl 2) ):115, 1971;.
20 +
Hancock EW:  Coronary sinus rhythm in sinus venous defect and persistent left superior vena cava . Am J Cardiol 14:608-615, 1964;.
21 +
Furuse A, et al:  Coronary sinus rhythm in anomalous systemic venous connection . Jap Heart J 9:200-207, 1968;.
22 +
Momma K, Linde LM:  Abnormal rhythms associated with persistent left superior vena cava . Pediatr Res 3:210-216, 1969;.
23 +
Lucus RV, Schmidt RE:  Anomalous venous connections, pulmonary and systemic , in Moss AJ, Adams FH (eds): Heart Disease in Infants, Children and Adolescents . Baltimore, Williams & Wilkins Co, 1968;, chap 32.
24 +
Fraser RS, et al:  Left superior vena cava . Am J Med 31:711-716, 1961;.
25 +
Keith JD, Rowe RD, Vlad P: Heart Disease in Infancy and Childhood . New York, Macmillan Publishers, 1967;, pp 493-498.
26 +
Haeger K, Juhlin I, Krook H:  Eisemenger's complex accompanied by double superior venae cavae, the left draining into the left atrium . Am Heart J 50:471-475, 1955;.
27 +
Peel AAF, et al:  Anomalous venous drainage with death from cardiac catheterization . Scott Med J 1:83-85, 1956;.
28 +
Cooley DA, Latson JR, Keats AS:  Surgical considerations in repair of ventricular and atrial septaldefects utilizing cardiopulmonary bypass . Surgery 43:214-225, 1958;.
29 +
Bosher LH:  Problems in extracorporeal circulation relating to venous cannulation and drainage . Ann Surg 149:652-663, 1959;.
30 +
Effler DB, Greer AE, Sifers EC:  Anomaly of the vena cava inferior: Report of a fatality after ligation . JAMA 146:1321-1322, 1951;.
31 +
Edward EA:  Clinical anatomy of lesser variations of classifying the anomalies of this vessel . Angiology 2:85-99, 1951;.
32 +
Gryska PF, Earthrowl FH Jr:  Left-sided inferior vena cava . Arch Surg 94:363-364, 1967;.
33 +
Magbitang MH, Hayford FC, Blake JM:  Dilated azygos vein simulating mediastinal tumor; Report of a case . N Engl J Med 263:598-600, 1960;.
34 +
Stern WZ, Bloomberg AE:  Idiopathic azygos phlebectasia simulating mediastinal tumor . Radiology 77:622-625, 1961;.
35 +
Petersen RW:  Infrahepatic interruption of inferior vena cava with azygos continuation (persistent right cardinal vein) . Radiology 48:304-307, 1965;.
36 +
Beddingfield CW, Young DA:  Anomalous inferior vena cava producing hilar tumor . Arch Surg 98:67-68, 1969;.

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References

1 +
Lev M, Rowlatt UF:  The pathologic anatomy of mixed levocardia . Am J Cardiol 8:216-263, 1961;.
2 +
Arcilla RA, Gasul BM:  Congenital dextrocardia: Clinical, angiocardiographic, and autopsy studies on 50 patients . J Pediatr 58:39-58, 251-262, 1961;.
3 +
Van Praagh R, et al:  Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications . Am J Cardiol 13:510-531, 1964;.
4 +
Campbell M, Deuchar DC:  The left-sided superior vena cava . Br Heart J 16:423-439, 1954;.
5 +
Anderson RC, Adams P Jr, Bruke B:  Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava)-report of 15 new cases . J Pediatr 59:370-383, 1961;.
6 +
Moller JH, et al:  Congenital cardiac disease associated with polysplenia: A developmental complex of bilateral "leftsidedness." Circulation 36:789-799, 1967;.
7 +
Derra VE, Loogen F, Satter P:  Anomalien der unteren Hohlvene . Dtsch Med Wochenschrift 90:689-695, 1965;.
8 +
Kaindl VF, et al:  Über eine seltene Anomalie der Vena Cava inferior . Radiol Austriaca 18:45-52, 1968;.
9 +
Leachman RD, Sloves AJ:  Relationship of the azygos venous system to normal atrial situs in levocardia . Circulation 29:901-904, 1964;.
10 +
Hastreiter AR, Rodriguez-Coronel A:  Discordent situs of thoracic and abdominal viscera . Am J Cardiol 22:111-118, 1968;.
11 +
Ruttenberg HD, et al:  Syndrome of congenital cardiac disease with asplenia: Distinction from other forms of congenital cyanotic cardiac disease . Am J Cardiol 13:387-406, 1964;.
12 +
Ivemark BI:  Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood: An analysis of the heart malformations in the splenic agenesis syndrome with 14 new cases . Acta Paediatr Scand 44( (suppl 104) ):1-110, 1955;.
13 +
Rodin AE, Sloane JA, Ngheim QX:  Polysplenia with severe congenital heart disease and Howell-Jolly bodies . Am J Clin Pathol 58:127-134, 1972;.
14 +
Putschar WGJ, Manion WC:  Congenital absence of the spleen and associated anomalies . Am J Clin Pathol 26:429-470, 1956;.
15 +
Diener KA:  Agenesia del bazo: Presentacion de un caso sin malformaciones congenitas del corazon . Bol Med Hosp Infant 19:711-715, 1962;.
16 +
Baillie M:  An account of a remarkable transposition of the viscera . Philosophical Trans 78:350-363, 1788;.
17 +
Tucker AS, Liebman J, Bolande RP:  Heterotaxy as related to congenital heart disease . Ann Radiol 9:178-184, 1966;.
18 +
Ruttenberg HD:  Corrected transposition of the great vessels: Splenic syndromes (asplenia, polysplenia) , in Moss AJ, Adam FH (eds): Heart Disease in Infants, Children, and Adolescents . Baltimore, Williams & Wilkins Co, 1968;, chap 26.
19 +
Freedom RM:  The heterotaxy syndrome , abstracted. Circulation 44( (suppl 2) ):115, 1971;.
20 +
Hancock EW:  Coronary sinus rhythm in sinus venous defect and persistent left superior vena cava . Am J Cardiol 14:608-615, 1964;.
21 +
Furuse A, et al:  Coronary sinus rhythm in anomalous systemic venous connection . Jap Heart J 9:200-207, 1968;.
22 +
Momma K, Linde LM:  Abnormal rhythms associated with persistent left superior vena cava . Pediatr Res 3:210-216, 1969;.
23 +
Lucus RV, Schmidt RE:  Anomalous venous connections, pulmonary and systemic , in Moss AJ, Adams FH (eds): Heart Disease in Infants, Children and Adolescents . Baltimore, Williams & Wilkins Co, 1968;, chap 32.
24 +
Fraser RS, et al:  Left superior vena cava . Am J Med 31:711-716, 1961;.
25 +
Keith JD, Rowe RD, Vlad P: Heart Disease in Infancy and Childhood . New York, Macmillan Publishers, 1967;, pp 493-498.
26 +
Haeger K, Juhlin I, Krook H:  Eisemenger's complex accompanied by double superior venae cavae, the left draining into the left atrium . Am Heart J 50:471-475, 1955;.
27 +
Peel AAF, et al:  Anomalous venous drainage with death from cardiac catheterization . Scott Med J 1:83-85, 1956;.
28 +
Cooley DA, Latson JR, Keats AS:  Surgical considerations in repair of ventricular and atrial septaldefects utilizing cardiopulmonary bypass . Surgery 43:214-225, 1958;.
29 +
Bosher LH:  Problems in extracorporeal circulation relating to venous cannulation and drainage . Ann Surg 149:652-663, 1959;.
30 +
Effler DB, Greer AE, Sifers EC:  Anomaly of the vena cava inferior: Report of a fatality after ligation . JAMA 146:1321-1322, 1951;.
31 +
Edward EA:  Clinical anatomy of lesser variations of classifying the anomalies of this vessel . Angiology 2:85-99, 1951;.
32 +
Gryska PF, Earthrowl FH Jr:  Left-sided inferior vena cava . Arch Surg 94:363-364, 1967;.
33 +
Magbitang MH, Hayford FC, Blake JM:  Dilated azygos vein simulating mediastinal tumor; Report of a case . N Engl J Med 263:598-600, 1960;.
34 +
Stern WZ, Bloomberg AE:  Idiopathic azygos phlebectasia simulating mediastinal tumor . Radiology 77:622-625, 1961;.
35 +
Petersen RW:  Infrahepatic interruption of inferior vena cava with azygos continuation (persistent right cardinal vein) . Radiology 48:304-307, 1965;.
36 +
Beddingfield CW, Young DA:  Anomalous inferior vena cava producing hilar tumor . Arch Surg 98:67-68, 1969;.

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