Ann Thorac Surg 2008;85:1089-1092. doi:10.1016/j.athoracsur.2007.08.045
© 2008 The Society of Thoracic Surgeons
Case Reports
Total Anomalous Pulmonary Venous Connection to the Supradiaphragmatic Inferior Vena Cava
Anna N. Seale, MBBChir,
Hideki Uemura, MD*,
Babulal Sethia, FRCS,
Alan G. Magee, MBBS,
Siew Yen Ho, PhD,
Piers E.F. Daubeney, MA, MRCP
Department of Pediatric Cardiology and Cardiothoracic Surgery, Royal Brompton Hospital, London, United Kingdom
Accepted for publication August 21, 2007.
* Address correspondence to Dr Uemura, Royal Brompton Hospital, Sydney St, London, SW3 6NP, United Kingdom (Email: h.uemura{at}rbht.nhs.uk).
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Abstract
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Total anomalous pulmonary venous connection to the inferior vena cava is a rare form of total anomalous pulmonary venous connection infrequently described in the literature. We report two cases where the pulmonary venous connection was to the supradiaphragmatic portion of the inferior vena cava. In both patients, preoperative echocardiography findings were misleading, which suggested a cardiac type of total anomalous pulmonary venous connection.
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Introduction
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Total anomalous pulmonary venous connection (TAPVC) is a form of congenital heart disease in which all the pulmonary veins from both lungs fail to connect to the left atrium and instead connect to a systemic vein or veins above the heart (supracardiac), below the heart (infracardiac), or to the right atrium or coronary sinus (cardiac). Infracardiac TAPVC accounts for approximately 20% of all TAPVC with the anomalous connection usually being below the diaphragm. Anomalous infracardiac connection to the supradiaphragmatic portion of the inferior vena cava (Fig 1) is extremely unusual; here we describe two such cases.
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Fig 1. Line drawing of total anomalous pulmonary venous connection (TAPVC) to the inferior vena cava (IVC). (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; SVC = superior vena cava.)
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Both patients presented beyond the neonatal period with similar clinical findings shown in Table 1. Echocardiography of the second patient clearly identified a separate coronary sinus of normal size. This was not clear in the first patient where echocardiographic findings were compatible with the pulmonary veins draining into a dilated coronary sinus.
At surgery, cardiopulmonary bypass was established in a standard fashion using bi-caval cannulation. The inferior vena cava (IVC) was snared without difficulty. When the right atrium was opened under cardiac arrest, the surgeons were unable to find the orifice of the pulmonary venous channel. The coronary sinus was of normal size in both cases. Eventually the ventricular mass was lifted and a pulmonary venous confluence was found behind the left atrial wall. With further dissection, a draining vein was identified from the pulmonary venous confluence coursing down obliquely toward the IVC. The connection of the draining vein was found to be at the posterior aspect of the IVC just above the diaphragmatic level and below the right atrial–IVC junction where the IVC was snared.
In both patients, repair was performed by direct anastomosis between the pulmonary venous confluence and the back of the left atrium, as is usually performed in extracardiac types of TAPVC. In the first patient, the heart was lifted to the right and in light of such unusual anatomy a decision was made to leave the draining vein open to prevent any risk of ligating the left lower pulmonary vein. An autologous pericardial patch was used to close the atrial septal defect, fenestrated due to poor ventricular function and concern over potential postoperative pulmonary hypertensive episodes. On the first postoperative day, flow through the patent descending vein was clinically significant and the channel was surgically ligated at the junction to the IVC clearly distant to the left lower pulmonary vein.
In patient 2, the approach was made through the right heart border. The atrial septal defect was directly closed and the descending vein from the pulmonary venous confluence was ligated. Both patients had uncomplicated postoperative courses and are doing well at 11 and 26 months after surgery, respectively.
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Comment
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Total anomalous pulmonary venous connection to IVC has been described as one of the rarer types of infracardiac TAPVC [1, 2]. Connection to the IVC can be above or below the diaphragm. In both of these cases the connection was found to be at the proximal portion of the IVC above the diaphragm. This infracardiac TAPVC is extremely unusual with only occasional reports in the literature [3].
In both cases, preoperative diagnosis was erroneously considered to represent the more common forms of cardiac TAPVC to the coronary sinus or directly to the RA. This is of great clinical importance to the surgical approach. In extracardiac TAPVC, the surgeon makes an anastomosis between the pulmonary venous confluence and the left atrium. The anomalous connecting vein is then ligated, unless there is a concern of residual pulmonary venous obstruction. Surgery for repair of TAPVC to the coronary sinus differs in that the enlarged coronary sinus is "de-roofed" enabling pulmonary venous return to drain into the left atrium. The atrial septal defect is enlarged and then patched to commit the opening of the coronary sinus into the left atrium. In TAPVC to the RA, the atrial septal defect is enlarged and patched, closing the defect and committing the pulmonary veins to the left atrium. Differentiation between the different types of TAPVC preoperatively is important in planning the appropriate surgical intervention.
The presence of a dilated coronary sinus, in the setting of TAPVC, may lead the unwary echocardiographer to the erroneous conclusion that this structure is the site of the pulmonary venous drainage. However, in TAPVC to the supradiaphragmatic IVC, the pulmonary venous confluence and communicating vein adopt a position and orientation inferior and posterior to the left atrium, which may be confused with a dilated coronary sinus. To prevent such misinterpretation, the finding of a dilated coronary sinus needs reconfirmation. First, is the structure indeed a coronary sinus? A separate and clearly distinct coronary sinus should be sought and, if present, it should trigger suspicion of TAPVC to IVC. Second, is the dilated coronary sinus connecting to the pulmonary veins and RA (Figs 2, 3)?
If this is not documented, or connection to the RA is only seen in subcostal views, then again there should be suspicion. Color flow Doppler may be helpful in showing additional vessels draining into the IVC, as it enters the RA; however, if diagnosis is uncertain, further imaging with angiography, magnetic resonance imaging or computed tomographic angiography should be undertaken.
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Fig 2. Echocardiographic images of (a, b) total anomalous pulmonary venous connection (TAPVC) to the inferior vena cava (IVC) and for comparison, (c, d) TAPVC to coronary sinus. (a, c) Parasternal long-axis view showing the inferior and posterior position of the pulmonary venous confluence (<) in both cases. In TAPVC to IVC, a separate coronary sinus (CS) can be seen. (b, d) Subcostal view also illustrates how these diagnoses may be confused, suggesting that the pulmonary veins (*) drain into a dilated coronary sinus. In fact, (b) in TAPVC to IVC, the veins form a confluence draining into the proximal portion of the IVC.
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Fig 3. Echocardiogram showing total anomalous pulmonary venous connection (TAPVC) to coronary sinus: parasternal long-axis with inferior tilt illustrating direct connection between the pulmonary veins (*), coronary sinus (CS), and right atrium (RA) in TAPVC to the CS.
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Both cases presented late with breathlessness and failure to thrive with the atrial communication becoming restrictive. This differs from most forms of infracardiac TAPVC in which patients present early and often unwell with cyanosis and obstruction to the pulmonary venous pathway. In TAPVC to the supradiaphragmatic IVC, the venous pathway does not pass through the diaphragm, hepatic parenchyma, or closing ductus venosus, all frequent positions of obstruction. Our experience is similar to other reports [1, 4, 5], including one of a 12-year-old presenting for the first time with TAPVC to the IVC [1].
In summary, TAPVC to the supradiaphragmatic IVC is a rare diagnosis and can masquerade as cardiac TAPVC. It is of diagnostic importance to identify the pulmonary venous confluence, coronary sinus, right atrium, and direct connections between these structures to prevent misdiagnosis. If there is any further diagnostic uncertainty, three-dimensional imaging is recommended as surgical repair of TAPVC to IVC differs with that for cardiac TAPVC.
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Acknowledgments
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We would like to thank Joanne Wolfenden and Manjit Jonsen for assistance with imaging and Emily McIntosh for artwork. Dr Anna Seale is a research fellow at the Royal Brompton Hospital supported by grants from the Harrison Heart Foundation and the Joe Gandon Memorial Trust.
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References
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- Wang ZP, Zhong F, Huang LB, Zhao W, Huang WM. Total anomalous pulmonary venous draining to inferior vena cava Thorac Cardiov Surg 2003;51:226-228.
- Duff DF, Nihill MR, McNamara DG. Infracardiac total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J 1977;39:619-626.[Abstract/Free Full Text]
- Freedom RM, Mawson JB, Yoo SJ, Benson LN. Abnormalities of pulmonary venous connections including subdivided left atrium. Congenital heart disease: textbook of angiography. Armonk, NY: Futura Publishing; 1997.
- VanSon JAM, Hambsch J, Kinzel P, Haas GS, Mohr FW. Urgency of operation in infracardiac total anomalous pulmonary venous connection Ann Thorac Surg 2000;70:128-130.[Abstract/Free Full Text]
- Duff DF, Nihill MR, Vargo TA, Cooley DA. Infradiaphragmatic total anomalous pulmonary venous return: diagnosis and surgical repair in a 10-year-old child Br Heart J 1975;37:1093-1096.[Abstract/Free Full Text]
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Abstract
Introduction
