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  • Print publication year: 2013
  • Online publication date: September 2013

Abnormalities of the great vessels



Abnormal systemic venous connections are usually of little surgical significance, as their clinical consequences are limited. The anomalies are apt to be encountered as the surgeon pursues a more complex associated intracardiac anomaly. They are of most significance in the setting of isomeric atrial appendages, which we discuss in Chapter 10, showing how so-called visceral heterotaxy is best considered in terms of right versus left isomerism. In this chapter, we consider the features of the anomalous systemic venous connections in their own right. They may be grouped into the categories of absence or abnormal drainage of the right superior caval vein, anomalies of the inferior caval vein, persistence or abnormal drainage of the left superior caval vein, and abnormal hepatic venous connections. Abnormalities of the coronary sinus usually fall into one of these groups, although unroofing, which produces an interatrial communication through the right atrial orifice of the sinus, has been discussed in Chapter 7.

Abnormalities of the right superior caval vein

These are extremely rare. The vein may be diminished in size. Alternatively, it may be completely absent when the venous return from the head, neck, and arms passes through a persistent left superior caval vein to the right atrium by way of the coronary sinus (Figure 9.1) or, rarely, directly into the left atrium. Only this last situation requires surgical intervention. The other conditions, if encountered during an open-heart operation, would require some adjustment from the usual technique used for cannulation. Although there is no definite evidence to this effect, we would not expect these abnormalities to affect the location of the sinus node.

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Wilcox's Surgical Anatomy of the Heart
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Anderson, RC, Heilig, W, Novick, R, Jarvis, C. Anomalous inferior vena cava with azygos drainage: so-called absence of the inferior vena cava. Am Heart J 1955; 49: 318–322.
Moller, JH, Nakib, A, Anderson, RC, Edwards, JE. Congenital cardiac disease associated with polysplenia: a developmental complex of bilateral ‘left-sidedness’. Circulation 1967; 36: 789–799.
Venables, AW. Isolated drainage of the inferior vena cava to the left atrium. Br Heart J 1963; 25: 545–548.
Winter, FS. Persistent left superior vena cava: survey of world literature and report of 30 additional cases. Angiology 1954; 5: 90–132.
Bankl, H. Congenital Malformations of the Heart and Great Vessels. Baltimore, MD: Urban and Schwarzenberg, 1977; p 194.
Agnoletti, G, Annechino, F, Preda, L, Borghi, A. Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle?Cardiol Young 1999; 9: 285–290.
Edwards, JE, DuShane, JW. Thoracic venous anomalies. I. Vascular connection between the left atrium and the left innominate vein (levoatriocardinal vein) associated with mitral atresia and premature closure of the foramen ovale (case 1). II. Pulmonary veins draining wholly to the ductus arteriosus (case 2). Arch Pathol 1950; 49: 517–537.
Pinto, CAM, Ho, SY, Redington, A, Shinebourne, EA, Anderson, RH. Morphological features of the levoatriocardinal (or pulmonary-to-systemic collateral) vein. Pediatr Pathol 1993; 13: 751–761.
Lucas, R, Woolfrey, BF, Anderson, RC, Lester, RG, Edwards, JE. Atresia of the common pulmonary vein. Pediatrics 1962; 29: 729–739.
DeLisle, G, Ando, M, Calder, AL, et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976; 91: 99–122.
Gupta, SK, Gulati, GS, Juneja, R, Devagourou, V. Total anomalous pulmonary venous connection with descending vertical vein: unusual drainage to azygos vein. Ann Pediatr Cardiol 2012; 5: 188–190.
Neill, CA, Ferencz, C, Sabiston, DC, Sheldon, H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage‘scimitar syndrome’. Bull Johns Hopkins Hosp 1960; 107: 1–21.
Chauvin, M, Shah, DC, Haisseguerre, M, Marcellin, L, Brechenmacher, C. The anatomic basis of connections between the coronary sinus musculature and the left atrium in humans. Circulation 2000; 101: 647–652.
Brom, AG. Narrowing of the aortic isthmus and enlargement of the mind. J Thorac Cardiovasc Surg 1965; 50: 166–180.
Ho, SY, Anderson, RH. Coarctation, tubular hypoplasia and the ductus arteriosus: a histological study of 35 specimens. Br Heart J 1979; 41: 268–274.
Elzenga, NJ, Gittenberger-de-Groot, AC. Localised coarctation of the aorta. An age dependent spectrum. Br Heart J 1983; 49: 317–323.
Becker, AE, Becker, MJ, Edwards, JE. Anomalies associated with coarctation of the aorta. Particular reference to infancy. Circulation 1970; 41: 1067–1075.
Hamilton, WF, Abbott, ME. Coarctation of the aorta of the adult type: Part I. Complete obliteration of the descending arch at insertion of the ductus in a boy of fourteen; bicuspid aortic valve; impending rupture of the aorta; cerebral death. Part II. A statistical study and historical retrospect of 200 recorded cases, with autopsy, of stenosis or obliteration of the descending arch in subjects above the age of two years. Am Heart J 1928; 3: 381–421.
Lerberg, DB. Abbott’s artery. Ann Thorac Surg 1982; 33: 415–416.
Pierpont, M E M, Zollikofer, CL, Moller, JH, Edwards, JE. Interruption of the aortic arch with right descending aorta. Pediatr Cardiol 1982; 2: 153–159.
Ho, SY, Wilcox, BR, Anderson, RH, Lincoln, JCR. Interrupted aortic arch–anatomical features of surgical significance. Thorac Cardiovasc Surg 1983; 31: 199–205.
Barry, A. Aortic arch derivatives in the human adult. Anat Rec 1951; 111: 221–238.
Stewart, JR, Kincaid, OW, Edwards, JE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield, IL: Charles C. Thomas, 1964.
Ramos-Duran, L, Nance, JW, Schoepf, UJ, et al. Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol 2012; 198: W466–W474.
Pool, PE, Vogel, JHK, Blount, SG. Congenital unilateral absence of a pulmonary artery. The importance of flow in pulmonary hypertension. Am J Cardiol 1962; 10: 706–732.
Contro, S, Miller, RA, White, H, Potts, WJ. Bronchial obstruction due to pulmonary artery anomalies. I. Vascular sling. Circulation 1958; 17: 418–423.
Maier, HC, Gould, WJ. Agenesis of the lung with vascular compression of the tracheobronchial tree. J Pediatr 1953; 43: 38–42.
Harrison, MR, Hendren, WH. Agenesis of the lung complicated by vascular compression and bronchomalacia. J Pediatr Surg 1975; 10: 813–817.
Harrison, MR, Heldt, GP, Brasch, RC, de Lorimier, AA, Gregory, GA. Resection of distal tracheal stenosis in a baby with agenesis of the lung. J Pediatr Surg 1980; 15: 938–943.
Gross, RE. Surgical management of patent ductus arteriosus with summary of four surgically treated cases. Ann Surg 1939; 110: 321–356.
Ali Kahn, MA, Al Yousef, S, Mullins, CE, Sawyer, W. Experiences with 205 procedures of transcatheter closure of ductus arteriosus in 182 patients, with special reference to residual shunts and long term follow-up. J Thorac Cardiovasc Surg 1992; 104: 1721–1727.
Lloyd, TR, Fedderly, R, Mendelsohn, AM, Sandhu, SK, Beekman, RH. Transcatheter occlusion of patent ductus arteriosus with Gianturco coils. Circulation 1993; 88: 1414–1420.
Pontius, RG, Danielson, GK, Noonan, JA, Judson, JP. Illusions leading to surgical closure of the distal left pulmonary artery instead of the ductus arteriosus. J Thorac Cardiovasc Surg 1981; 82: 107–113.
Mendel, V, Luhmer, J, Oelert, H. Aneurysma des Ductus arteriosus bei einem Neugeborenen. Herz 1980; 5: 320–323.
Wilcox, BR, Peters, RM. The surgery of patent ductus arteriosus: a clinical report of 14 years’ experience without an operative death. Ann Thorac Surg 1967; 3: 126–131.
Faulkner, SL, Oldham, RR, Atwood, GF, Graham, TP. Aortopulmonary window, ventricular septal defect and membranous pulmonary atresia with a diagnosis of truncus arteriosus. Chest 1974; 65: 351–353.
Ogden, JA. Congenital anomalies of the coronary arteries. Am J Cardiol 1970; 25: 474–479.
Becker, AE. Variations of the main coronary arteries. Edited by Becker, AE, Losekoot, T, Marcelletti, C, Anderson, RH, Edinburgh, Churchill Livingstone. Pediatr Cardiol 1983; 3: 263–277.
Smith, A, Arnold, R, Anderson, RH, et al. Anomalous origin of the left coronary artery from the pulmonary trunk. Anatomic findings in relation to pathophysiology and surgical repair. J Thorac Cardiovasc Surg 1989; 98: 16–24.
Takeuchi, S, Imamura, H, Katsumoto, K, et al. New surgical method for repair of anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1979; 78: 7–11.
Ishikawa, T, Otsuka, T, Suzuki, T. Anomalous origin of the left main coronary artery from the non-coronary sinus of Valsalva. (Letter)Pediatr Cardiol 1990; 11: 173–174.
Kragel, AH, Roberts, WC. Anomalous origin of either right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol 1988; 62: 771–777.
Debich, DE, Williams, KE, Anderson, RH. Congenital atresia of the orifice of the left coronary artery and its main stem. Int J Cardiol 1989; 22: 398–404.
McKay, R, Anderson, RH, Cook, AC. The aorto-ventricular tunnels. Cardiol Young 2002; 12: 563–580.