Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection

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1 The International Journal of Cardiac Imaging 16: 405±409, Ó 2000 Kluwer Academic Publishers. Printed in the Netherlands. 405 Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection H.-Y. Chen 1, S.-J. Chen 2, Y.-W. Li 2, M.-H. Wu 3, J.-K. Wang 3, Y.-F. Tsai 1, C.-C. Wu 1 & L.-K. Chen 1 1 Department of Diagnostic Radiology, Shin Kong Wu Ho-Su Memorial Hospital, Taiwan; 2 Department of Medical Imaging, Medical College and Hospital, National Taiwan University, Taiwan; 3 Department of Pediatrics, Medical College and Hospital, National Taiwan University, Taiwan, Republic of China Received 21 December 1999; accepted in revised form 12 July 2000 Key words: asplenia syndrome, computed tomography, congenital heart disease, esophageal varices, total anomalous pulmonary venous connection Abstract Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented. Introduction Anomalous pulmonary venous connection is an uncommon and severe congenital heart anomaly that is often associated with asplenia syndrome. Among the types of total anomalous pulmonary venous connection (TAPVC), the infracardiac type is the least common. It usually drains below the diaphragm into the inferior vena cava (IVC), portal vein or a erent vessel. As the portal system is the drainage route, portal hypertension may develop, and then esophageal varices have occurred in severe cases. The association of esophageal varices in asplenia syndrome with infradiaphragmatic TAPVC is unique. Case presentation Case 1 The patient was an 8-day-old male infant who had general cyanosis since birth. Echocardiography showed TAPVC with an enlarged vertical vein, which was seen to descend and join the portal venous system. The color Doppler echocardiogram demonstrated an abnormal cluster of vessels that owed upward around the descending vertical vein (Figure 1A). However, it was not possible to trace the whole course. Thereafter, the nature of these vessels could not be determined. The associated anomalies included right atrial isomerism with common atrium, common atrioventricular valve, single indeterminate ventricle, double outlet and inlet of the indeterminate ventricle, tubular hypoplasia of the aorta and large patent ductus arteriosus (PDA). Cardiac angiogram showed the

2 406 Figure 1. Case one. (A) Color-Doppler echocardiogram revealed several vessels in cross section at the hepatic hilum with one ow downward (blue; descending vein) and the others ow upward (red; varices). (B) Levophase of main pulmonary artery angiogram shows the vagus opaci ed pulmonary venous drainage at the hepatic hilum, paraspinal and lower neck regions (arrows). (C) Computed tomographic scan delineates the descending vein (arrow) located anteriorly to the esophagus that is circumvented by the varices presented as four vascular spots (v). Ao ˆ aorta; CA ˆ common atrium; LV ˆ left ventricle; RLPV ˆ right lower pulmonary vein; RV ˆ right ventricle; S ˆ spine. same ndings as demonstrated using the echocardiography, except di culty in viewing the details about the abnormal pulmonary venous drainage beyond the main portal vein, because of dilution of the contrast medium (Figure 1B). Computed tomography (CT) scans of the chest including the lower neck and the upper abdomen were obtained using an Ultrafast CT scanner (C-150L Imatron; South San Francisco, CA, USA) with EKG gating. Non-ionic iodinated contrast medium containing 37 g of iodine for 100 ml (Ultravist 370; Schering, Berlin, Germany) was administered at 2 ml/kg body weight. The slice thickness was 3 mm. The images showed bilateral superior and inferior pulmonary veins joined into a common pulmonary con uence and then a descending vertical vein, which was connected to the portal vein. Development of collat-

3 407 erals was noted around the hepatic hilum and the gastroesophageal junction. Prominent and markedly engorged esophageal venous plexus was presented as four vascular dots at the esophageal wall circumferentially (Figure 1C). They were seen in a long segment from the gastroesophageal junction to the lower neck, where multiple small channels were connected to the bilateral internal jugular veins. Thereafter, it was connected to the bilateral brachiocephalic veins, then to the superior vena cava (SVC) and into the common atrium. The common hepatic vein drained directly into the oor of the common atrium. A juxtaposed left IVC and abdominal aorta were found. Tubular hypoplasia of the aorta with a large PDA and engorged pulmonary artery were also clearly demonstrated on CT images. The ndings of cardiac anomalies were the same as described above for echocardiography. Horizontal liver without spleen and right-sided stomach were demonstrated on CT scans as on echocardiography. Case 2 This patient was a 2-day-old female infant who was presented with marked cyanosis immediately after birth. Echocardiography suggested mixed type TAPVC. Part of the pulmonary venous system drained into the right SVC. Some pulmonary veins were con uent and had an uncertain relationship with the systemic venous circulation. There were some unusual vessels behind the common atrium and in front of the descending thoracic aorta (Figure 2A). The other anomalies included right atrial isomerism with common atrium, common atrioventricular valve, and double outlet of the right ventricle, infundibular pulmonary stenosis and large PDA. The main pulmonary angiogram demonstrated that the pulmonary venous drainage failed due to severe infundibular pulmonary stenosis. Aortogram, which consisted of contrast medium passed to pulmonary artery through PDA, hardly demonstrated the details of the pulmonary venous course with only a faint and vagus opaci ed vessels superimposed at the paraspinal regions (Figure 2B). Ultrafast CT scanning was performed using the same protocol as in case 1. There was an intrapulmonary connecting vein between the right upper and right lower pulmonary vein. The right upper pulmonary vein drained into the right SVC. The other pulmonary veins were con uent as a common pulmonary vein and then to a descending vertical vein, which was connected to the esophageal venous plexus at the level of the gastrohepatic ligament. Similar to case 1, the prominent and markedly engorged esophageal varices were presented as four vascular dots circumventing the esophageal wall (Figure 2C). In the lower neck, multiple jugular plexus collaterals developed with a common connecting channel draining to the left SVC and then to the left-sided right atrium. Juxtaposed left IVC and abdominal aorta were found. The ndings of cardiac anomalies were the same as described above using echocardiography. Horizontal liver without spleen and right-sided stomach were demonstrated both on CT scans and echocardiography. All the ndings of these anomalous venous connections within the thorax and upper abdomen in case 1 and case 2 were con rmed at the operation. Discussion Anomalous pulmonary venous connection is a common manifestation associated with asplenia syndrome. However, the association of asplenia syndrome, infradiaphragmatic TAPVC and esophageal varices is quite unique [1]. According to the nature of the connection between the pulmonary veins to the systemic venous return, TAPVC is classi ed into four subtypes (supracardiac, cardiac, infracardiac and mixed types). The infracardiac type is rather uncommon [2]. In patients with infracardiac type TAPVC, the veins usually drain below the diaphragm into IVC, portal vein or an a erent vessel. As the portal system is the drainage route, portal hypertension and esophageal varices may develop in severe cases. Most of the infracardiac TAPVC have obstructive pulmonary venous drainage at the diaphragmatic level [3±5]. In this condition, portal venous hypertension hardly develops. However, in our two cases, CT images clearly demonstrated full

4 408 Figure 2. Case two. (A) Subcostal echocardiogram in oblique coronal plane revealed a strange vessel (arrowheads) between the dorsal wall of the common atrium and the descending aorta (Ao). It had continuous upward ow. (B) Levophase of aortogram showed the vagus opaci ed vessels superimposed at paraspinal regions (arrows). (C) Computed tomographic scan delineated the descending vertical vein (black arrow) located on the left side of the esophagus. There were four vascular spots (v) indicating varices surrounding the esophageal wall. Ao ˆ descending aorta; CA ˆ common atrium; LLPV ˆ left lower pulmonary vein; LV ˆ left ventricle; RV ˆ right ventricle. patency of the descending vertical vein through the diaphragm. This resulted in an over ow of the portal venous system and formation of the markedly esophageal varices. Two-dimensional echocardiogram with color Doppler is now performed routinely in infants presented with dyspnea or cyanosis. It is safe, noninvasive, and inexpensive and can be performed at the bedside. Preoperative diagnosis of usual TAPVC course is reliable [6±8]. Cardiac catheterization, an invasive type of examination, is both expensive and time-consuming. However, it is widely used to provide the angiographic pictures and the hemodynamics data. Nevertheless, in patients with infracardiac TAPVC, the drainage course beyond the vertical vein is sometime hard to gure out using either echocardiogram or cardiac catheterization. With echocardiogram, it is

5 409 di cult to trace the whole course of aberrant vessels. Frequent angiography is only faint opaci- cation of the pulmonary veins by the dilution using non-contrast blood. Ultrafast CT has the advantages of high spatial resolution, non-invasiveness, operator-independence and short scanning time. The clinical accuracy of this imaging method for detecting congenital heart disease has been shown to be high compared with angiography and echocardiography [9, 10]. It provided a detailed portrait of the pulmonary venous drainage. Magnetic resonance imaging is another noninvasive alternative [11] but required longer examination time and had less spatial resolution, moreover it was not suitable for critical patients with many vital-supporting devices. Esophageal varices are rare in newborns and are mostly caused by the extra-hepatic obstruction due to portal vein thrombosis or the intrahepatic obstruction due to biliary atresia, cystic brosis and other liver diseases. Infradiaphragmatic TAPVC is an unusual cause of esophageal varices in newborns. In our patients, esophageal varices were clearly demonstrated on CT images. They were presented as four vascular spots that circumvented the esophageal wall. The long segment venous plexus was traced from the gastroesophageal junction, upward through esophageal venous plexus and then connected into the bilateral jugular vein. Awareness of the entity in association with infradiaphragmatic TAPVC is important in clinical management and preoperative planning. Massive hematemesis may develop due to traumatic nasogastric tube insertion. Echocardiography and cardiac catheterization can de nitely help physicians make the preoperative diagnosis of TAPVC, but sometimes they may be showing the de nite course of the venous connection, especially in patients with the infracardiac type. Using EKG-gating, ultrafast CT scanning can clearly demonstrate all the cardiovascular anatomy and the pulmonary venous drainage at the same time. We suggest ultrafast CT as an imaging modality for patients with TAPVC, especially for those with the infracardiac type that has uncertain relationship with the systemic circulation on the echocardiogram and with cardiac catheterization. References 1. Rabinowitz JG, Liu L, Lindner A. Asplenia associated with infradiaphragmatic total anomalous pulmonary venous return and esophageal varices. Radiology 1969; 93: 350± Darling RC, Rothney WB, Craig JM. Total pulmonary venous drainage into the right side of the heart: report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest 1957; 6: 44± Kanjuh VI, Katkov H, Singh A, et al. A typical total anomalous pulmonary venous connection: two channels leading to infracardiac termination. Pediatr Cardiol 1989; 10: 115± Chen YC, Hsieh KS, Chi CS. Total anomalous pulmonary venous return: A clinical observation of 36 cases. Chin Med J (Taipei) 1994; 54: 44± Wang JK, Lue HC, Wu MH, et al. Obstructed total anomalous pulmonary connection. Pediatr Cardiol 1993; 14: 28± Mortera C, Tynan M, Goodwin AW, Hunter S. Infradiaphragmatic total anomalous pulmonary venous connection to portal vein ± Diagnostic implications of echocardiography. Br Heart J 1977; 39: 685± Goswami KC, Shrivastava S, Saxena A, Dev V. Echocardiographic diagnosis of total anomalous pulmonary venous connection. Am Heart J 1993; 126: 433± Brown VE, De Lange M, Dyar DA, Impastato LW, Shirali GS. Echocardiographic spectrum of supracardiac total anomalous pulmonary venous connection. J Am Soc Echocardiogr 1998; 11: 289± Chen SJ, Li YW, Wang JK, et al. Usefulness of electron beam computed tomography in children with heterotaxy syndrome. Am J Cardiol 1998; 81: 188± Chen SJ, Li YW, Wang JK, et al. Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT. J Comput Assist Tomogr 1998; 22: 560± Livolsi A, Bruno K, Marcellin L, et al. MR diagnosis of subdiaphragmatic anomalous pulmonary venous drainage in a newborn. J Comput Assist Tomogr 1991; 15(6): 1051± Address for correspondence: Shyh-Jye Chen, MD, Department of Medical Imaging, Medical College and Hospital, National Taiwan University, 7 Chung-Shan South Road, Taipei, 100 Taiwan, Republic of China Phone: ext 5586; Fax: ; james@ha.mc.ntu.edu.tw