Surgical technique I| Volume 9, ISSUE 1, P56-62, 2006

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Scimitar Syndrome

  • Ulf Gudjonsson
    Section of Cardiothoracic Surgery, James W. Riley Hospital for Children and Indiana University School of Medicine, Indianapolis, IN.
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  • John W. Brown
    Address reprint requests to John W. Brown, MD, Section of Cardiothoracic Surgery, Indiana University School of Medicine, 545 Barnhill Dr, EH 215, Indianapolis, IN, 46202-5123
    Section of Cardiothoracic Surgery, James W. Riley Hospital for Children and Indiana University School of Medicine, Indianapolis, IN.
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      Scimitar syndrome is a rare congenital anomaly consisting in part of right pulmonary venous return to the inferior vena cava. There is a clear bimodal presentation of this syndrome with either an infantile manifestation or a pediatric/adult form. The infantile variant is marked by a higher incidence and severity of associated defects, heart failure, pulmonary hypertension, and significant mortality. The patient with the pediatric/adult form is less severely affected and may be asymptomatic on diagnosis. In this article, we review the historical aspects, presentation, and pathophysiology of Scimitar syndrome and discuss available treatment strategies. We emphasize the safe and effective approach developed at Indiana University that obviates both the need for an intra-atrial baffle or use of cardiopulmonary bypass. The results with our alternative approach to Scimitar syndrome are summarized and they compare favorably with other published reports.


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        • Gao Y.A.
        • Burrows P.E.
        • Benson L.N.
        • et al.
        Scimitar syndrome in infancy.
        J Am Coll Cardiol. 1993; 22: 873-882
        • D Cruz I.A.
        • Aracilla R.A.
        Anomalous venous drainage of the left lung in to the inferior vena cava.
        Am Heart J. 1964; 67: 539-544
        • Brown J.W.
        • Ruzmetov M.
        • Minnich D.J.
        • et al.
        Surgical management of Scimitar syndrome.
        J Thorac Cardiovasc Surg. 2003; 125: 238-245
        • Cooper G.
        Case of malformation of the thoracic viscera.
        Lond Med Gaz. 1836; 18: 600-601
        • Chassinat R.
        Observation of remarkable anatomic abnormalities.
        Arch Gen Med. 1836; 11: 80-91
        • Dotter C.T.
        Anomalous pulmonary vein.
        Am J Med Sci. 1949; 218: 31-86
        • Drake E.H.
        • Lynch J.P.
        Bronchiectasis associated with anomaly of the right pulmonary vein and right diaphragm; Report of a case.
        J Thorac Surg. 1950; 19: 433-437
        • Kirklin J.W.
        • Ellis Jr, F.H.
        • Wood E.T.
        Treatment of anomalous pulmonary venous connections in association with interatrial communications.
        Surgery. 1956; 39: 389-398
        • Koch S.
        Repair of Scimitar syndrome.
        Radiology. 1960; 75: 592
        • Ruggieri M.
        • Abbate M.
        • Parano E.
        • et al.
        Scimitar vein anomaly with multiple cardiac malformations, craniofacial, and central nervous system abnormalities in a brother and sister.
        Am J Med Genet. 2003; 116: 170-175
        • Huddleston C.B.
        • Mendeloff E.N.
        Scimitar syndrome.
        Adv Cardiac Surg. 1999; 11: 161-178
        • Kiely B.
        • Filler J.
        • Stone S.
        • et al.
        Syndrome of anomalous venous drainage of the right lung to the inferior vena cava.
        Am J Cardiol. 1967; 20: 102-116
        • Huddleston C.B.
        • Exil B.
        • Canter C.E.
        • et al.
        Scimitar syndrome presenting in infancy.
        Ann Thorac Surg. 1999; 67: 154-160
        • Gikonyo D.K.
        • Tandon R.
        • Lucas Jr, R.V.
        • et al.
        Scimitar syndrome in neonates.
        Pediatr Cardiol. 1986; 6: 193-197
        • Schramel F.M.
        • Westermann C.J.
        • Knaepen P.J.
        • et al.
        The Scimitar syndrome.
        Eur Respir J. 1995; 8: 196-201
        • Kramer U.
        • Dornberger V.
        • Fenchel M.
        • et al.
        Scimitar syndrome.
        Eur Radiol. 2003; 13: L147-L150
        • Dickinson D.F.
        • Galloway R.W.
        • Massey R.
        • et al.
        Role of embolization of systemic arterial supply to right lung.
        Br Heart J. 1982; 47: 468-472
        • Pfammater J.-P.
        Infantile Scimitar syndrome with pulmonary hypertension-Successful treatment with coil embolization.
        Cardiol Young. 1997; 7: 454-457
        • Laros C.D.
        • Westermann C.J.
        Dilatation, compensatory growth, or both after pneumonectomy during childhood and adolescence. A thirty-year follow-up study.
        J Thorac Cardiovasc Surg. 1987; 93: 570-576
        • Zubiate P.
        Surgical correction of anomalous pulmonary venous connection.
        Ann Surg. 1962; 156: 234-250
        • Shumacker Jr, H.B.
        • Judd D.
        Partial anomalous pulmonary venous return with reference to drainage into the inferior vena cava and to an intact atrial septum.
        J Cardiovasc Surg (Torino). 1964; 45: 271-278
        • Calhoun R.F.
        • Mee R.B.
        A novel approach to Scimitar syndrome.
        Ann Thorac Surg. 2003; 76: 301-303
        • Dupuis C.
        • Charaf L.A.
        • Breviere G.M.
        • et al.
        The adult form of Scimitar syndrome.
        Am J Cardiol. 1992; 70: 502-507