Complex congenital anomalies of superior vena cava and pulmonary veins with left-sided inferior vena cava/ Sol yerlesimli inferiyor vena kava ile superiyor vena kava ve pulmoner venlerin kompleks konjenital anomalileri.

Introduction

Development of inferior vena cava (IVC) begins at fifth week of gestation. Three paired venous channels that develop sequentially (posterior cardinal, subcardinal, supracardinal) anastomose and regress (1). Left sided IVC develops as a result of the persistence of the left supracardinal vein, and prevalence of this anomaly is estimated to be 0.2-0.5% (2). Persistent left superior vena cava (SVC) is a well-known anomaly, and found in 0.5-4% of general population. The most frequent anomaly accompanies with persistent left SVC is total anomalous pulmonary venous return (10%) (3). The incidence of pulmonary vein anomalies is about 0.5% (4).

We report a first case of complex venous system anomaly, which is a combination of three anomalies in systemic and pulmonervenous system.

Case report

A 20-year-old asymptomatic male was referred to our clinic for delineation of the cause of the enlarged cardiac silhouette on his chest X-ray obtained for routine check-up. His medical and family history and physical examination were unremarkable except 1/6-grade systolic murmur best heard at lower sternal region. Standard surface electrocardiogram and blood chemistry were in normal ranges. Echocardiographic examination revealed enlarged coronary sinus, right atrium and right ventricle. For identifying the cause of enlargement of right sided chambers, contrast echocardiographic examination with agitated saline was performed. During contrast injection via lower extremity veins primarily coronary sinus, secondarily right atrium filled with contrast (Fig. 1A). However, IVC was not visualized in substernal view (Fig. 1B). Instead of IVC, hepatic veins emptied into right atrium individually at the substernal echocardiographic view (Fig. 1B). This finding led us to suspect the absence of hepatic segment of the IVC and venous drainage of lower extremity is maintained via persistent left SVC. Further injection from the right antebrachial vein showed the right atrial filling from SVC (Fig. 1C), which also leads us to suspect persistent left SVC with intact right SVC.

Thoraco-abdominal computed tomography (CT) showed that right jugular and subclavian veins come together, form the right sided SVC, which drains to the right atrium, and left internal jugular vein with left subclavian vein forms the left SVC, which drains to the right atrium via the coronary sinus (Fig. 2). Besides these anomalies left pulmonary veins drain to left atrium, right pulmonary veins drain to the right atrium (Fig. 3). Bilateral iliac veins converge and form the left sided IVC (Fig. 4). Left-sided IVC proceeds as hemiazygos vein (Fig. 5). Venous drainage of abdomen and lower extremities maintained by left subclavian vein via hemiazygos conduit. Computed tomography images also revealed malrotation of the spleen but not polysplenia.

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Discussion

While each of left-sided IVC, double SVC and partial pulmonary venous return anomalies reported individually (5, 6), there was no knowledge about the presence of all these three anomalies at the same time except left atrial isomerism. However, our case cannot be diagnosed as left atrial isomerism because of absence of left sidedness such as two left lung and bronchi. A necropsy serial with double SVC declared that all the cases with left SVC had continuity with the coronary sinus (7). Ventricular septal defect and atrioventricular connection anomalies are the most frequent additional congenital anomalies (7). No accompanying cardiac anomaly was determined in our patient. Although, absence of IVC is associated with polysplenia in 84% of cases (8), our case had not polysplenia, but malrotation of the spleen with a hilus located inferiorly was present. Gouley et al. (9) described a case of absent hepatic portion of the IVC with hemiazygos drainage direct into the left atrium, with questionable, persistent left SVC. In other series, the hemiazygos drained into the left SVC, which mostly drains into the coronary sinus as in our case (10). Although the left sided IVC has been described previously, we have not found any report having three different anomalies all together. We suspected the drainage of subdiaphragmatic venous return to persistent SVC and absence the hepatic segment of IVC by agitated saline contrast echocardiography, and confirmed the diagnosis by multidetector CT.

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Conclusion

Our case is unique for the presence of all three congenital systemic and pulmonary venous anomalies, come together. Determining of venous anomalies is of particular importance before implantation of cardiac pacemakers and implantable cardioverter defibrillators.

References

1. Sandercoe GD, Brooke-Cowden GL. Developmental anomaly of the inferior vena cava. ANZ J Surg 2003; 73: 356-60.

2. Dahnert W. Radiology review manual. Baltimore: Williams and Wilkins;1996.

3. Demos TC, Posniak HV, Pierce KL, Olson MC, Muscato M. Venous anomalies of the thorax. AJR Am J Roentgenol 2004; 182: 1139-50.

4. Oliver JIM, Gallego P, Gonzalez A, Dominguez FJ, Aroca A, Mesa JIM. Sinus venosus syndrome: atrial septal defect or anomalous venous connection? A multiplane transoesophageal approach. Heart 2002; 88: 634-8.

5. Guray Y, Yelgec NS, Guray U, Yilmaz MB, Boyaci A, Korkmaz S. Left-sided or transposed inferior vena cava ascending as hemiazygos vein and draining into the coronary sinus via persistent left superior vena cava: case report. Int J Cardiol 2004; 93: 293-5.

6. Aydogdu S, Tumgor G, Parildar M, Arikan C, Aydin U, Yuzer Y, et al. Acute hepatic vein thrombosis after liver transplantation in a child with biliary atresia and absent inferior vena cava. Transplant Proc 2006; 38: 1459-60.

7. Munoz Castellanos L, Nivon MK, Zapata Carreon RR, Salinas Sanchez HC. Left superior vena cava and anomalies associated with it. Arch Cardiol Mex 2003; 73: 175-84.

8. Rose V, Izukawa T, Moes CA. Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases with special reference to diagnosis and prognosis. Br Heart J 1975; 37: 840-5.

9. Ginaldi S, Chuang VP, Wallace S. Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr 1980; 4:112-4.

10. Downing OF. Absence of the inferior vena cava. Pediatrics 1953;12: 675-80.

Cem Koz, Mehmet Yokusoglu, Mustafa Tasar*, Fatih Ors*, Celal Genc

From Departments of Cardiology and *Radiology, Faculty of Medicine, Gulhane Military Academy, Ankara, Turkey

Address for Correspondence/Yazisma Adresi: Dog. Dr. Mehmet Yokusoglu, Gulhane Askeri Tip Akademisi ve Askeri Tip Fakultesi, Kardiyoloji Anabilim Dali, Gn. Tevfik Saglam Cad. 06010 Etlik, Ankara, Turkey Phone: [phone] Fax: [phone] E-mail: [e-mail]