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Ann Thorac Surg 2000;70:128-130
© 2000 The Society of Thoracic Surgeons

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Original articles: Cardiovascular

Urgency of operation in infracardiac total anomalous pulmonary venous connection

^a Herzzentrum, University of Leipzig, Leipzig, Germany

Address reprint requests to Dr van Son, Herzzentrum, University of Leipzig, Russenstrasse 19, D-04289 Leipzig, Germany

	Abstract

Top Abstract Introduction Patients and methods Comment References

 
Background. Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection.

Methods. The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed.

Results. Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome.

Conclusions. In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction.

	Introduction

Top Abstract Introduction Patients and methods Comment References

 
The tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection (TAPVC) may be partially dependent on the type of connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary veins [1–7]. We reviewed our surgical experience with various subtypes of infracardiac TAPVC to study the potential relationship between pulmonary venous obstruction and the type of connection of the descending vein.

	Patients and methods

Top Abstract Introduction Patients and methods Comment References

 
Patient 1
A 2-day-old male neonate (3,200 g) was referred with the presumed diagnosis of meconium aspiration. The patient was tachypneic and cyanotic. The arterial oxygen saturation was 75% and the arterial pH was 7.31. The patient required intubation, volume substitution, and inotropic support because of respiratory and hemodynamic instability. Transthoracic echocardiography demonstrated infracardiac TAPVC with connection of the descending vein to the portal vein (Fig 1). Blood flow in the descending vein was sluggish. Closure of the ductus venosus had resulted in dilation of the portal venous system. There was a large atrial septal defect with trivial right-to-left atrial shunting.

View larger version (67K): [in this window] [in a new window]   Fig 1. (Patient 1) Echocardiogram of infracardiac total anomalous pulmonary venous connection in which the descending vein connects to the portal vein. Secondary to closure of the ductus venosus and the high resistance of the hepatic parenchyma, the portal vein is prominently dilated. Flow in the descending vein is sluggish.

Patient 2
A 5-day-old female neonate was admitted with symptoms of tachypnea and cyanosis. The arterial oxygen saturation was 78% and the arterial pH was 7.32. Echocardiography confirmed the presumed diagnosis of infracardiac TAPVC and demonstrated sluggish flow in the descending pulmonary vein that connected to the portal vein. There was a patent foramen ovale with trivial right-to-left shunting. The patient was operated on without delay. The operative technique was the same as in patient 1. The intraoperative left atrial pressure was 12 mm Hg. The patient was extubated on the 7th postoperative day. The further postoperative course was uneventful.

Patient 3
A 7-day-old female neonate had been clinically well until several hours before admission. Then she had suddenly become critically ill with pallor and tachypnea. On admission the arterial oxygen saturation was 65% and the arterial pH was 7.28. Echocardiography demonstrated infracardiac TAPVC with obstruction of the descending vein. The patient died before operation could be undertaken. Autopsy demonstrated infracardiac TAPVC with the descending vein connected to the ductus venosus, which had physiologically closed (Fig 2). Histologic examination of the lungs demonstrated severe pulmonary edema with dilated lymphatic vessels, medial hypertrophy of the arterioles, and thickening of the alveolar walls (Fig 3).

View larger version (137K): [in this window] [in a new window]   Fig 2. (Patient 3) Autopsy specimen of infracardiac type of total anomalous pulmonary venous connection. Note the connection of the descending vein (DV) to the ductus venosus (arrow).

View larger version (133K): [in this window] [in a new window]   Fig 3. (Patient 3) Lung specimen. Note the marked thickening of the alveolar walls secondary to edema of the interlobar septa and lymphatic dilation. (Elastic van Gieson, x50.)

	Comment

Top Abstract Introduction Patients and methods Comment References

 
In TAPVC the pulmonary veins drain in an infracardiac fashion in approximately 25% of cases [1–3]. The most common sites of connection in infracardiac TAPVC are the portal vein and the ductus venosus [1–3]. Much less common are the inferior vena cava, the left hepatic vein, and the gastric vein. Uncommonly, the entire pulmonary venous drainage may be through two connections [8].

Obstruction in the infracardiac type of TAPVC may be the result of increased resistance to flow through a long narrow venous channel. Also, the descending vein may be compressed at its passage through the esophageal hiatus of the diaphragm or may be obstructed at the junction with its connecting vein [2]. The most important mechanism of obstruction, however, consists of increased resistance in the connecting venous structure. Thus, in the absence of a patent ductus venosus, pulmonary venous obstruction is almost invariably present in total anomalous connection to the portal vein because of the high resistance of the hepatic parenchymal circulation. During fetal life, the ductus venosus enables blood from the umbilical vein and portal vein to enter the right atrium, thus bypassing the hepatic parenchyma [9]. In postnatal life, the ductus venosus and umbilical vein obliterate and are represented in the ligamentum venosum and ligamentum teres, respectively. Pulmonary venous connection to the ductus venosus, as present in 1 patient in this small series, may not be obstructive during the first few days of life. As the ductus venosus closes postnatally, pulmonary venous obstruction develops. In such patients, as demonstrated in patient 3 in this series, histologic examination of the lungs shows medial hypertrophy of veins and arterioles, dilated lymphatic vessels, and alveolar capillary congestion. In contrast, a connection to a hepatic vein or the inferior vena cava is usually nonobstructive because the venous return does not pass through the hepatic parenchyma or closing ductus venosus.

Echocardiography is an extremely useful technique for the diagnosis of all forms of TAPVC. In the majority of patients, the venous morphology may be defined accurately so that cardiac catheterization and angiography, which often worsen the pulmonary edema, may be bypassed. Important echocardiographic features of nonobstructed TAPVC are dilation of the right atrium, right ventricle, and pulmonary artery secondary to volume overload of the right heart. Consequently, the left ventricle is usually smaller than normal because of leftward bulging of the ventricular septum. A large atrial communication is present in the majority of cases. In contrast, when pulmonary venous obstruction is present, pulmonary blood flow is limited (with the right heart structures also dilated) and right-to-left atrial shunting is trivial or absent. Color flow Doppler echocardiography is able to trace the descending vein into its connection with the portal or systemic venous system and to identify obstruction at the level of the diaphragm, within the liver, and in the ductus venosus.

In patients with infracardiac TAPVC and pulmonary venous hypertension secondary to obstruction, operation should generally not be delayed. The operation may have to be carried out on an emergency basis in case the descending vein connects to the portal vein or to the ductus venosus that is in the process of closing. In this situation, we generally do not agree with preoperative preparation of the neonate by the infusion of prostaglandin E₁ in combination with low-dose dopamine [6]. Such treatment may deteriorate the cyanosis and the clinical state by peripheral vasodilation and shunting blood away from the lungs. Instead we favor the strategy of immediate operation, regardless of the operative risk. In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, and the patient is clinically well, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the ductus venosus, operation should generally not be delayed based on the above-mentioned considerations.

	References

Top Abstract Introduction Patients and methods Comment References

 

1. Carter R.E.B., Capriles M., Noe Y. Total anomalous pulmonary venous drainage. Br Heart J 1969;31:45-51.[Free Full Text]
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3. Duff D.F., Nihill M.R., McNamara D.G. Infradiaphragmatic total anomalous pulmonary venous return. Br Heart J 1977;39:619-626.[Abstract/Free Full Text]
4. Turley K., Tucker W.Y., Ullyot D.J., et al. Total anomalous pulmonary venous connection in infancy. Am J Cardiol 1980;45:92-97.[Medline]
5. Sano S., Brawn W.J., Mee R.B.B. Total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1989;97:886-892.[Abstract]
6. Serraf A., Bruniaux J., Lacour-Gayet F., et al. Obstructed total anomalous pulmonary venous return. Towards neutralization of a major risk factor. J Thorac Cardiovasc Surg 1991;101:601-606.[Abstract]
7. Van de Wal H.J.C.M., Hamilton D.I., Godman M.J., Harinck E., Lacquet L.K., van Oort A. Pulmonary venous obstruction following correction for total anomalous pulmonary venous drainage. Eur J Cardiothorac Surg 1992;6:545-549.[Abstract]
8. Arcieprete P., McKay R., Watson G.H., Hamilton D.I., Wilkinson J.L., Arnold R.M. Double connections in total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1986;92:146-152.[Abstract]
9. Van Son J.A.M., Falk V., Mohr F.W. Staged surgical closure of patent ductus venosus. Asian Cardiovasc Thorac Ann 1997;5:121-123.

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