Ann Thorac Surg 2000;70:1400-1402
© 2000 The Society of Thoracic Surgeons
Case report
Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism
LeNardo D. Thompson, MDa,
Doff B. McElhinney, MDa,
V. Mohan Reddy, MDa,
Kenneth L. Jue, MDa,
Frank L. Hanley, MDa
a Divisions of Cardiothoracic Surgery and Cardiology, University of California, San Francisco, and Valley Childrens Hospital, Fresno, California, USA
Address reprint requests to Dr Thompson, University of California, San Francisco, Medical Center, 505 Parnassus Ave, San Francisco, CA 94143-0118
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Abstract
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We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.
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Introduction
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Totally anomalous pulmonary venous return (TAPVR) is a common finding in patients with right atrial isomerism and asplenia, occurring with a frequency of up to 90% [1–5]. The most commonly observed patterns of anomalous pulmonary venous drainage are supracardiac, infracardiac, and cardiac [1–3]. In TAPVR both with and without associated atrial isomerism, mixed drainage of the pulmonary veins is rare. TAPVR in patients with atrial isomerism, who generally require staged palliation for functionally univentricular congenital heart disease, is one of the factors that contributes to their poor outcome [3–6]. Drainage of the pulmonary veins below the diaphragm, with obstruction to flow, may be associated with the worst prognosis [3–5]. Recently, we have managed a patient with asplenia syndrome and functionally univentricular heart disease who had an extremely unusual pattern of TAPVR.
The patient was a full-term male infant who presented with respiratory distress at 1 month of age. A chest radiograph suggested symmetric mainstem bronchi and showed prominent pulmonary vascular markings. His electrocardiogram was abnormal, with evidence of right atrial enlargement and ventricular preexcitation. Echocardiography showed a right-sided inferior caval vein, bilateral superior caval veins, right isomerism of the atrial appendages, and a common atrium draining via a single atrioventricular valve into a large, morphologically right ventricle that gave rise to both the aorta and pulmonary trunk. The pulmonary veins were seen to drain to the portal venous system by way of a descending vertical vein. Sonography failed to identify a spleen.
After stabilization, the patient underwent banding of the pulmonary trunk to reduce pulmonary blood flow, and correction of TAPVR with anastomosis of the pulmonary venous confluence to the posterior aspect of the common atrium and ligation of the descending vertical vein. Postoperatively, the patient continued to have symptoms and signs of pulmonary overcirculation in spite of marginal systemic arterial oxygen saturation, so he underwent tightening of the band and placement of a 3 mm systemic to pulmonary arterial shunt in order to improve mixing.
One month later, the infant again had respiratory distress, severe cyanosis, ventricular dysfunction, and acidosis requiring endotracheal intubation and mechanical ventilatory support. Echocardiography and cardiac catheterization demonstrated obstruction of the pulmonary venous pathway at the site of the surgical anastomosis, with a pressure gradient of 6 mm Hg. Levophase angiography of the left pulmonary artery showed drainage both to the atrium and to the portal venous system through a descending vertical vein. He was taken to the operating room, where the obstructed venoatrial anastomosis was enlarged and the separate descending vein was anastomosed to the atrium. Intraoperative inspection clarified the pulmonary venous anatomy: The left upper and right pulmonary veins joined posterior to the atrium and then drained through a descending vein that ran intrapericardially, anterior and to the right of the inferior caval vein. The left middle and lower pulmonary veins joined and descended below the diaphragm through a separate vertical vein that was extrapericardial, located posteriorly and to the left of the inferior caval vein. Hence, this extrapericardial vertical vein was not seen at the initial repair. On retrospective review of imaging studies, it appeared as though the two separate vertical veins connected with one another below the diaphragm before draining to the portal venous system (Figs 1 and 2).
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Fig 1. Echocardiogram from a subcostal oblique parasagittal view shows the two vertical veins descending through the diaphragm and joining together, posterior to the liver.
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Fig 2. On this levophase image of a right ventricular angiogram in the left anterior oblique projection, the two vertical veins can also be appreciated faintly, as can their confluence below the diaphragm.
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Postoperatively, the patient’s respiratory status improved substantially. He has since been hospitalized once for sepsis, and underwent bilateral bidirectional cavopulmonary anastomosis without any complications. Follow-up imaging studies have shown no evidence of obstruction to pulmonary venous drainage or ventricular dysfunction.
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Comment
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Between 65% and 90% of patients with asplenia syndrome and right atrial isomerism have TAPVR as a feature of their complex congenital heart disease [1–4]. This is consistent with the pattern of "bilateral right-sidedness" that is characteristic of this form of visceroatrial heterotaxy. During normal embryogenesis, the primitive pulmonary veins fuse with an outgrowth of the left atrium to form the pulmonary venoatrial confluence [7]. Presumably, the atrial outgrowth is a patterned process that is intrinsic to a segment of the atrial tissue that has differentiated as left atrial primordium. In the context of right atrial isomerism, which is thought to result from defective lateralization (that is, abnormal development of left-right asymmetry) in the early embryo, the left atrium does not differentiate normally. Accordingly, the normal process of pulmonary venoatrial confluence does not take place, with TAPVR a frequent result.
In patients with TAPVR, there is significant variability in the pattern of anomalous drainage. The various patterns are often classified according to the site of drainage with respect to the heart: supracardiac (type 1), cardiac (type 2), infracardiac (type 3), and a combination of multiple sites (mixed, type 4) [7]. Aside from the high frequency of obstruction in infradiaphragmatic TAPVR and the relative difficulty managing mixed TAPVR, there does not appear to be any difference in management or outcome associated with this scheme of classification [8, 9]. Moreover, little is known about the developmental differences among the various patterns of drainage. If the pulmonary veins do not successfully join with the pulmonary venous atrium, or if the connection forms but subsequently becomes atretic, one or more of the connections between the veins draining the lung buds (which are derived from the primitive foregut) and the splanchnic venous plexus typically remain patent. Factors that influence which splanchnic venous connections persist in the development of TAPVR, or which systemic venous channel the aberrant pulmonary veins join, are not known. In the vast majority of cases of TAPVR, all of the pulmonary veins drain to a single site after forming a central confluence. Thus, cases of mixed drainage or of separately draining veins to the same systemic venous level probably result from an embryogenetic process that differs in certain respects, insofar as the central confluence of pulmonary veins does not develop or becomes atretic after forming.
Before the present case, there has been only one reported case of which we are aware of two separate vertical veins draining below the diaphragm [10]. In the previous case, in which the heart and central vessels were otherwise structurally normal, the entire left lung drained through one of the vertical veins while the entire right lung drained through the other, with both connecting to the portal/hepatic venous system separately. Our case differed from the previous one in that the vertical vein drained the entire right lung plus the left upper lobe while the other drained the middle and left lobes (in asplenia, both lungs are typically trilobed). In their report, Kanjuh and coworkers [10] reviewed the literature on mixed TAPVR, with a focus on cases with multiple sites of drainage to the same level. They found only eight cases with two trunks draining to the same level, all of which terminated in the supracardiac systemic veins. They found four other cases in which all of the pulmonary veins came together in a single confluence but drained with multiple subdivisions to infradiaphragmatic veins.
The unusual form of TAPVR in our patient complicated his management and may have implications for his eventual outcome. This highlights the importance of identifying all pulmonary veins and characterizing their drainage in patients with TAPVR and in patients with atrial isomerism. In most cases of mixed TAPVR, the lungs drain separately, which facilitates recognition of the multiple pathways of drainage. In our patient, however, the left upper pulmonary vein joined the right pulmonary veins. This confluence gave the impression of a single site of drainage on preoperative echocardiography.
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References
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Accepted for publication December 30, 1999.
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Abstract
Introduction
