J Thorac Cardiovasc Surg 2007;134:1067-1069
© 2007 The American Association for Thoracic Surgery
Cor triatriatum and total anomalous pulmonary venous connection to the coronary sinus
Kazutoshi Tachibana, MD*,
Nobuyuki Takagi, MD,
Hisayoshi Osawa, MD,
Motoki Takamuro, MD,
Masato Yokozawa, MD,
Hideki Tomita, MD,
Tetsuya Higami, MD
Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan.
Received for publication December 30, 2006; accepted for publication January 8, 2007.
* Address for reprints: Kazutoshi Tachibana, MD, Department of Thoracic and Cardiovascular surgery, Sapporo Medical University and hospital, S-1 W-16 chuo-ku, Sapporo, 060-8543 Japan. (Email: aad72150{at}pop17.odn.ne.jp).
| |
Drs Takagi, Higami, and Tachibana (left to right)
|
|
Clinical Summary
Patient 1
A 22-day-old infant was referred to our hospital because of hypoxia. The neonate received ventilatory support. A two-dimensional echocardiogram revealed the presence of coronary sinus (CS) total anomalous pulmonary venous connection (TAPVC) with a large atrial septal defect (ASD); the presence of a membrane within the left atrium (LA) was suspected. The neonate underwent an emergency operation with conventional cardiopulmonary bypass. The right atrium was opened, and drainage of all 4 pulmonary veins (PVs) into the CS was confirmed. There was no communication between the PVs and the LA. The membrane in the LA was identified through a secundum ASD. The anomaly was easily repaired by means of complete excision of the membrane through the ASD. The CS was then "deroofed" and cut back into the LA. An autologous pericardial patch was sewn in place to reconstitute the atrial septum so that the CS (and thus the PVs) could drain into the LA. At follow-up 12 months after the operation, she remains well and is developing normally.
Patient 2
A 17-year-old girl was referred with the diagnosis of ASD. Echocardiography and cardiac catheterization raised the suspicion of an unroofed CS but could not confirm the diagnosis. Three-dimensional computed tomographic analysis provided really useful information and confirmed the presence of cor triatriatum with CS TAPVC (Figure 1). During cardiopulmonary bypass, the right atrium was incised longitudinally. The CS was noticeably enlarged. An ASD was not detected, and one was created in the position of the fossa ovale. There were communications between the dorsal chamber, which was receiving the PVs, and the ventral chamber, which had a left atrial appendage, and between the dorsal chamber and the CS. The obstructive membrane paralleled the posterior wall of the ventral chamber. The left- and right-sided components of the obstructive membrane were incised toward the orifice of the communication between the dorsal chamber and the CS (Figure 2, A). Then a tongue-like flap of the obstructive membrane was flipped down and anastomosed to the edge of the orifice (Figure 2, B). After completion of the intra-atrial rerouting with the obstructive membrane, a wide communication between the common PV and the LA was created, whereas the route of left-right shunting was closed, and the coronary vein was drained directly into the right atrium. After this operation, the patient was asymptomatic and returned to a normal school life.
View larger version (105K):
[in this window]
[in a new window]
|
Figure 1. Three-dimensional computed tomographic scan (patient 2). The left atrium was subdivided into 2 chambers: the true left atrium (LA) and the accessory chamber (AC). The accessory chamber also communicates with the coronary sinus (CS) and receives the pulmonary veins (PVs).
|
|
View larger version (75K):
[in this window]
[in a new window]
|
Figure 2. A, The left- and right-side obstructive membrane was incised toward the orifice of the communication between the accessory chamber (AC) and the coronary sinus (CS). B, A tongue-like flap of the obstructive membrane was flipped down and anastomosed to the edge of the orifice. CS, coronary sinus; LA, left atrium; RA, right atrium; PV, pulmonary vein.
|
|
Discussion
The embryogenesis of cor triatriatum remains controversial and is usually explained by citing the entrapment or malincorporation theory.1
The diverticulum, or common PV, grows toward the developing lungs and ultimately connects with the PVs, which have already formed. When this drainage pathway is established, the early connections between the splanchnic plexus and the umbilical-vitelline and cardinal venous system become of secondary importance and are largely lost. As the LA grows, the common PV is absorbed into its posterior wall, and the PVs enter the LA individually.
Depending on the stage at which normal embryogenesis is interrupted, a variety of connections of the AC to the atria and cardinal venous system can occur. If atresia of the common PV occurs very early, a major drainage route can be established through either the umbilicovitelline or cardinal venous system (total anomalous pulmonary venous connection). If stenosis occurs at a late stage, the result may be stenosis of the common PV (cor triatriatum). If stenosis occurs at a very late stage, after absorption of the common PV into the LA, the result will be stenosis or atresia of individual PVs. Concomitant cor triatriatum and total anomalous pulmonary venous connection, presented here, occur over the first 2 stages. In patient 2, normal embryogenesis might have been interrupted later than embryogenesis in patient 1.
Careful two-dimensional echocardiography could be the procedure of choice in identifying the subdividing membrane. Unfortunately, cineangiography is often unsuccessful in demonstrating the abnormal left atrial membrane. However, a three-dimensional computed tomographic scan provides really useful information for the evaluation of such a complicated association.
Both surgical repairs were achieved simply; notably, in patient 2 intra-atrial rerouting was completed with the obstructive membrane. Cor triatriatum has a lot of variations and can be repaired by using various methods.2,3 It is important to consider the possible use of the obstructive membrane and to repair as simply as possible.
In summary, although rare, concomitant cor triatriatum and TAPVC can be treated successfully if the diagnosis is confirmed in detail and sufficient information is available preoperatively.
References
- Van Praagh R, Corsini I. Cor triatriatum: pathological anatomy and a consideration of morphogenesis based on 13 post-mortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human embryos. Am Heart J 1969;78:379-405.[Medline]
- Vouhe PR, Baillot-Venant F, Fermont L, et al. Cor triatriatum and total anomalous pulmonary venous connection: a rare surgically correctable anomaly. J Thorac Cardiovasc Surg 1985;90:443-445.[Abstract]
- Salomone G, Tiraboschi R, Bianchi T, Ferri F, Crippa M, Parenzan L. Cor triatriatum. Clinical presentation and operative results. J Thorac Cardiovasc Surg 1991;101:1088-1092.[Abstract]
