Case Report
Scimitar Syndrome Associated With Abnormal Hepatic Venous Drainage
World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(3) 474-476 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114559291 pch.sagepub.com
Paweł Nawrocki1, Anna Wesnerowicz1, Christoph Schmidt, MD, PhD1, Edward Malec, MD, PhD1, and Katarzyna Januszewska, MD, PhD1
Abstract Scimitar syndrome is a rare congenital cardiopulmonary anomaly. We describe a 1.5-year-old boy with this uncommon malformation and an unusual hepatic venous drainage, which allows atypical surgical repair. Keywords anatomy, cardiac anatomy/pathologic anatomy, lung pathology, congenital heart surgery Submitted July 31, 2014; Accepted October 14, 2014.
Introduction Scimitar syndrome is a rare congenital cardiopulmonary anomaly characterized by anomalous drainage of pulmonary vein(s) into the inferior vena cava (IVC). It usually involves anomalous drainage of the lower and middle lobe of the right lung (79%) or the whole right lung (21%). The syndrome is frequently associated with other anomalies like sinus venosus type atrial septal defect (ASD), hypoplasia of the right pulmonary artery, hypoplasia of the right lung, dextroposition of the heart, occasionally with abnormal bronchial anatomy, or systemic arterial supply to the lower lobe of the right lung (pulmonary sequestration).1-3 We describe a 1.5-year-old boy with scimitar syndrome and unusual hepatic vein drainage system, which allows atypical surgical repair.
Case Report A 1.5-year-old boy was referred to our hospital for a surgical correction of a partial anomalous pulmonary venous connection. The boy was born in the 32nd week of pregnancy, with 2.3 kg of weight and 49 cm length. Before admission he had a history of recurrent infections of the respiratory system. On admission, the child was cachectic and debilitated with a weight of 8.1 kg and length 80 cm, with no abnormalities in physical examination. Echocardiography showed an enlarged and hypertrophic right ventricle, which in addition to the high pulmonary pressure and flow was an indication for a surgical treatment. Cardiac catheterization revealed the mean pulmonary pressure of 39 mm Hg and pulmonary to systemic flow ratio (Qp/Qs) of 2.0. Angiography showed an abnormal drainage of the middle and lower lobes of the right lung to the relatively
small IVC (Figure 1) and additional atypical hepatic venous drainage. The left and middle hepatic veins formed a truncus that drained directly into the right atrium (Figure 2), and three right hepatic veins created one vessel connected to the IVC (Figure 3). Other anomalies were sinus venosus inferior type ASD, persistent left superior vena cava, dextroposition of the heart, hypoplasia of right lung and right pulmonary artery, and systemic arterial collateral from the descending aorta to the right lung (which has been occluded during catheterization). During the surgical repair, cardiopulmonary bypass (CPB) was instituted by cannulation of the ascending aorta and separate venous cannulation of the three vessels, namely, two superior caval veins and the common trunk of the left and middle hepatic veins. The IVC was snared during the CPB. A baffle of autologous pericardium fixed in glutaraldehyde was placed into the right atrium around the whole lumen of the IVC to channel the anomalous pulmonary vein flow through the ASD into the left atrium. During the CPB, no signs of blood congestion were noticed in the lower part of the body. The IVC was ligated under the baffle and above the hepatic vein. The postoperative course was uneventful. In the follow-up after two months and six months, the patient was in a good general condition without
1 Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, University Hospital Muenster, Muenster, Germany
Corresponding Author: Katarzyna Januszewska, Division of Pediatric Cardiac Surgery, Department of Cardiothoracic Surgery, University Hospital Muenster, Albert-SchweitzerCampus 1, Geba¨ude: A1, 48149 Muenster, Germany. Email:
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Abbreviations and Acronyms ASD CPB IVC Qp/Qs
atrial septal defect cardiopulmonary bypass inferior vena cava pulmonary to systemic flow ratio
Figure 3. Drainage of the right hepatic vein into the inferior vena cava.
congestion in the lower body. The echocardiographic study showed laminar flow through the baffled pathway. There was no congestion or enlargement of the liver or the hepatic veins, and no sign of pulmonary hypertension.
Figure 1. Drainage of the scimitar vein into the inferior vena cava.
Discussion
Figure 2. Drainage of the left and middle hepatic veins into the right atrium.
Several surgical approaches have been described for a scimitar syndrome: (1) an intra-atrial patch to baffle the flow from the anomalous pulmonary venous system into the left atrium through the right atrium and ASD, (2) direct implantation of the anomalous vein to the left atrium (also with right lateral thoracotomy), (3) division and implantation of the anomalous vein into the posterolateral wall of the right atrium with patch baffling into the left atrium, and (4) resection of the lung drained by the scimitar vein.4,5 In our case, we used a baffle of autologous pericardium and placed it into the whole lumen of IVC to channel the anomalous pulmonary vein flow to the right atrium, then through the ASD into the left atrium. During the procedure, the IVC was snared, which enabled a verification of an influence of the closed inflow from the IVC to the right atrium on the hemodynamic status of the child. At the end of the operation, the IVC was safely ligated beneath the baffle and above the right hepatic vein. The blood drainage from the lower part of the body was maintained through the azygos and hemiazygos veins. Most of the hepatic veins formed a truncus, with the drainage directly to the right atrium. The right hepatic veins were connected to the IVC. The intrahepatic connections between the right and left hepatic veins present the second possible option for the venous blood flow from the lower body to the right atrium. In our case, the IVC was small and the pulmonary venous blood (scimitar vein) drained relatively distal from the
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connection between IVC and the right atrium. On the other side, the azygos vein was very well developed, and the majority of the hepatic veins drained directly to the right atrium. We took advantage of this unusual anatomy and made the described correction, because in our opinion the risk of obstruction of both the scimitar and inferior caval vein after standard baffle technique was greater than the risk of congestion in the lower part of the body. If we would have noticed any adverse sequella of the obstructed IVC drainage, the direct implantation of the IVC would be probably our choice. Although some cases of scimitar syndrome associated with persistent hepatic venous plexus and interruption of the IVC were described,6-9 to our knowledge this kind of abnormal hepatic venous drainage, which enables a modification of surgical techniques, was not reported yet.
partial anomalous pulmonary venous connection. Ann Thorac Surg. 2007;84(6): 2020-2026. Vida VL, Padalino MA, Boccuzzo G, et al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122(12): 1159-1166. C¸ic¸ek S, Arslan AH, Ugurlucan M, Yildiz Y, Ay S. Scimitar syndrome: the curved Turkish Sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2014;17(1): 56-61. Gudjonsson U, Brown JW. Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;9: 56-62. Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years experience and results of repair. J Thorac Cardiovasc Surg. 1996;112(5): 1161-1169. Agnoletti G, Bonnet D, De Blic J. Scimitar syndrome associated with absence of the right pulmonary artery and a persistent primitive hepatic venous plexus. Cardiol Young. 2005; 15(2): 216-218. Blasco PB, Garcia GG, Noguer FR, Comas JG: Persistent primitive hepatic venous plexus in a child with scimitar syndrome. Int J Cardiol. 2011;151(3): e88-e89. Madan N, Moore JW. Unusual angiographic appearance of scimitar syndrome associated with primitive hepatic venous plexus. Circulation. 2002;105(13): e78. MacDonald C, Mikhailian H, Yoo SJ, Freedom RM, Adatia I. Angiographic findings of persistent primitive hepatic venous plexus with underdevelopment of the infrahepatic vena cava in pediatric patients. Am J Roentgenol. 2000;175(5): 1397-1401.
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Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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Funding
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The author(s) received no financial support for the research, authorship, and/or publication of this article. 9.
References 1. Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG. Outcomes after surgical treatment of children with
Downloaded from pch.sagepub.com at UNIV NEBRASKA LIBRARIES on November 26, 2015