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Ann Thorac Surg 2002;73:273-274
© 2002 The Society of Thoracic Surgeons

Case report

Pulmonary venous obstruction after total cavopulmonary connection in heterotaxy

Tetsuro Uchida, MDa, Hideki Uemura, MD*a, Toshikatsu Yagihara, MDa, Shinya Tsukano, MDb, Soichiro Kitamura, MDa

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Department of Pediatric Cardiology, National Cardiovascular Center, Osaka, Japan

Accepted for publication March 2, 2001.

* Address reprint requests to Dr Uemura, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565-8565, Japan
e-mail: huemura{at}hsp.ncvc.go.jp


   Abstract
Top
 Abstract
Introduction
 Case reports
 Comment
 References
 
Pulmonary venous obstruction is a rare complication after total cavopulmonary connection by intraatrial grafting. We experienced this complication in two patients. In one of them, a large prosthesis produced inflow obstruction of the dominant ventricle and was successfully replaced with a smaller tube. In the other patient, the intraatrial tube had become severely adherent to the common pulmonary venous orifice and was removed entirely. Conversion to an extracardiac Fontan circulation was later successfully established.


   Introduction
Top
 Abstract
 Introduction
Case reports
 Comment
 References
 
Obstruction across the pulmonary venous channel is a rare condition after the Fontan-type procedure [1, 2]. In patients with visceral heterotaxy, nonetheless, abnormal venoatrial connections are known to be almost the rule. An unusual postoperative obstruction within the pathway from the pulmonary veins to the ventricles may occur in this particular setting. We describe herein such circumstances caused by an intraatrial tube graft placed to establish the Fontan circulation.


   Case reports
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 Abstract
 Introduction
 Case reports
Comment
 References
 
Patient 1
A boy with isomeric left atrial appendages had undergone the bidirectional Glenn procedure at the age of 13 months. His cardiac malformation consisted of a severely stenotic right-sided atrioventricular valve with a right-sided hypoplastic morphologically left ventricle (l-loop) and pulmonary atresia, with the aorta arising from the dominant right ventricle. The left-sided atrioventricular valve was regurgitant. The right and left pulmonary veins were connected to the right-sided and the left-sided atria, respectively. Pulmonary resistance was calculated as 1.9 wood units · m2, with pulmonary arterial pressure and pulmonary capillary wedge pressure of 14 mm Hg and 11 mm Hg, respectively. Systemic oxygen saturation was 86%.

Plasty of the left-sided atrioventricular valve was carried out concomitantly with construction of an intraatrial channel by interposition of an 18-mm Gore-Tex tube graft between the pulmonary arteries and the orifice of the inferior vena cava (IVC). The IVC had been connected to the left-sided morphologically left atrium, and the prosthetic tube exclusively occupied the left atrium. No fenestration was placed initially. The atrial septum was resected as far as possible. The patient’s postoperative course was uneventful for a couple of days. The circulation, however, soon began to deteriorate in spite of intensive medical treatment. Echocardiography showed that the intraatrial prosthesis occupying exclusively the left-sided atrial cavity caused significant obstruction of the left-sided atrioventricular valve as the systemic ventricular inflow. On the fifth postoperative day, the intraatrial graft was replaced using a 14-mm Gore-Tex tube. A 4-mm–diameter fenestration was considered necessary eventually, because the systemic condition of the patient became significantly impaired. After the reoperation for down-sizing, no further obstruction was seen.

Eighteen months after the procedure, mean pulmonary arterial pressure was 15 mm Hg; cardiac index was calculated as 3.8 L · min-1 · m2. With occlusion of the constructed fenestration, systemic oxygen saturation became 94%. The patient is doing well 44 months after the procedure.

Patient 2
A 3-year-old boy with a common inlet left ventricle and pulmonary stenosis in the setting of right isomerism was evaluated for total cavopulmonary connection (TCPC). The procedure, carried out at another hospital, was performed concomitantly with plasty of the common atrioventricular valve. Bidirectional cavopulmonary anastomosis was employed for the superior vena cava, and a 16-mm Gore-Tex tube was placed intraatrially for draining the IVC and an independent hepatic vein.

The patient did well for 18 months, when he began to experience exertional fatigue. Systemic venous congestion became obvious 2 months later, and oliguria developed. Pleural effusion, ascites, and impaired hepatic function developed and became intractable, and the patient was eventually referred to us for further treatment.

Progressive pulmonary venous obstruction was highly suspected as a major cause of deterioration of the Fontan circulation. Echocardiography showed a pulmonary venous flow velocity of 2 m/s. Regurgitation across the common atrioventricular valve was trivial. Computerized tomography scanning unequivocally demonstrated the orientation of the intracardiac structures, as well as obstructed drainage from the common pulmonary venous chamber to the atrial cavity (Fig 1). Pulmonary arterial pressure was 22 mm Hg, and end-diastolic pressure of the systemic ventricle was 3 mm Hg. The Fontan circulation was taken down to the bidirectional Glenn physiology. Upon direct visualization pulmonary venous connections were obviously abnormal, forming a so-called common pulmonary venous chamber. The intraatrial graft previously placed had become firmly adherent to the pulmonary venous orifices.



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  		Fig 1. Demonstration of the obstructed pathway from the common pulmonary venous chamber to the atrial cavity by electron beam computed tomography. An accelerated jet was found (arrowhead), through the pulmonary venous orifice adjacent to the intraatrially placed tube graft (IAG = intraatrial graft; PV = pulmonary vein).

 
Surgical removal of the prosthesis and all fibrous tissues opened the pulmonary venous orifices to allow proper function. Postoperative pulmonary arterial pressure was 13 mm Hg, and atrial pressure was 10 mm Hg. The postoperative course was smooth with mild cyanosis. At 6 months after this procedure, the Fontan circulation was reestablished by construction of an extracardiac channel using an 18-mm Gore-Tex tube with no fenestration. The patient has been doing well for 21 months with the alternative Fontan circulation.


   Comment
Top
 Abstract
 Introduction
 Case reports
 Comment
References
 
Pulmonary venous obstruction can occur subsequent to TCPC using an intraatrial graft [1, 2]. In both cases reported here, clinical symptoms of pulmonary venous obstruction in the Fontan circulation were not pulmonary as would be seen after biventricular repair, but systemic. Previous reports have stated that obstruction of the pulmonary venous orifice was caused by a fibrous remnant of the atrial septum, by the intraatrially placed graft itself, and by the inappropriate placement of the suture line for the atrial baffle. In our patients with heterotaxy, the abnormal venoatrial connections and characteristic intracardiac structures produced obstruction from the pulmonary veins to the ventricles. Although intraatrial grafting involves relatively simple intracardiac maneuvers for establishing the Fontan circulation in such circumstances [3], it should be noted that problems may remain because of the highly complex nature of the malformations.

Pulmonary venous obstruction related to the intraatrial presence of a prosthesis might not have been produced had the extracardiac Fontan technique [4] been employed. Even when constructing an extracardiac channel, however, the surgeon must avoid external compression of the pulmonary veins by placement of a prosthetic tube. However, a 14-mm–diameter artificial tube could be too small to provide an unobstructed channel for IVC drainage when the patient reaches adolescence.

It is essential for the surgeon to recognize the need for precise orientation of the venoatrial connections so as to avoid obstruction, as well as turbulence, through the extracardiac channel to be constructed. The extracardiac channel can be long and tortuous, particularly in patients with visceral heterotaxy, a condition often characterized by unusual location of the orifices of the IVC and the independent hepatic veins, if present, as well as by abnormal patterns of pulmonary venous connection [5].


   References
Top
 Abstract
 Introduction
 Case reports
 Comment
 References
 

  1. Fogel M.A., Chin A.J. Imaging of pulmonary venous pathway obstruction in patients after the modified Fontan procedure. J Am Coll Cardiol 1992;20:181-190.[Abstract]
  2. Berman W., Fripp R.R., Yabek S.M. Late-onset pulmonary venous pathway obstruction after Fontan operation: Presentation masquerading as intra-atrial baffle leakage. Pediatr Cardiol 1997;18:49-51.[Medline]
  3. Humes R.A., Feldt R.H., Porter C.J., Julsrud P.R., Puga F.J., Danielson G.K. The modified Fontan operation for asplenia and polysplenia syndrome. J Thorac Cardiovasc Surg 1988;96:212-218.[Abstract]
  4. Marcelletti C., Corno A., Giannico S., Marino B. Inferior vena cava–pulmonary artery extracardiac conduit. J Thorac Cardiovasc Surg 1990;100:228-232.[Abstract]
  5. Uemura H., Ho S.Y., Devine W.A., Kilpatrick L.L., Anderson R.H. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995;60:561-569.[Abstract/Free Full Text]



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This Article
Right arrow Abstract Freely available
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Toshikatsu Yagihara
Soichiro Kitamura
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Related Collections
Right arrow Congenital - cyanotic

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