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J Thorac Cardiovasc Surg 1994;108:194-195
© 1994 Mosby, Inc.


LETTERS TO THE EDITOR

Thrombosis after the Fontan procedure: Transesophageal echocardiography may replace angiocardiography

Jan Lam, MD, Rodolfo Neirotti, MD, Anton E. Becker, MD

Department of Pediatric Cardiology
Department of Pediatric Cardiac Surgery
Department of Cardiovascular Pathology
Academic Medical Center
Amsterdam, The Netherlands

Claude Planché, MD

CCML
Paris, France

To the Editor:

Thrombosis of the right side of the heart after a Fontan procedure remains a serious complication with a high mortality and, at the same time, a diagnostic and therapeutic challenge. The article by Hedrick and colleagues Go 1 has highlighted these aspects. They provide an excellent overview of cases thus far reported and emphasize the difficulties in diagnosing the condition. They also promote routine echocardiographic surveillance after the Fontan operation and angiocardiographic studies once thrombosis is suspected. We would like to amend this approach by suggesting that transesophageal echocardiography presently is the diagnostic tool par excellence and, in fact, eventually may replace angiocardiography.

A 3-year-old boy with univentricular heart, mitral atresia, and pulmonary stenosis, who previously had atrial balloon septostomy (at the age of 2 months) and atrial blade septostomy (at the age of 2 years) as palliative procedures, underwent a fenestrated Fontan operation. The pulmonary artery was transected, the right atrial appendage connected to the right pulmonary artery at the site of the bifurcation, and a tunnel created to direct the blood from the caval veins to the pulmonary artery. An intraoperative transesophageal echocardiogram showed unobstructed flow into the pulmonary arteries (Fig. 1, A). The patient was not given anticoagulants. On the second postoperative day right-sided pleural effusion was diagnosed, and on day 3 the patient had massive diarrhea associated with thrombopenia. Within a few hours after this event, a sharp rise in the central venous pressure occurred accompanied by extreme distention of the jugular veins and swelling of the liver. Thrombotic occlusion was suspected, but the precordial echocardiograms were inconclusive. The decision was made to evaluate the condition with the use of transesophageal echocardiography.



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Fig. 1. , Intraoperative transesophageal echocardiogram, performed with a 7mm, 5 mHz transverse plane probe, at the end of the Fontan operation. Flow is present in both pulmonary arteries. The scanning sector is only partial in the area of the right pulmonary artery. B, Transesophageal echocardiogram in the intesive care unit at the time of the acute clinical deterioration. A 4mm, 5mHz prob is inserted through the nose. No flow is seen in the left pulmonary artery. C, At the time of reoperation the 4 mm probe is replaced with the 7 mm probe. In addition to the absence of flow, a solid mass (arrow) is seen in the left pulmonary artery. L, Left pulmonary artery; R, right pulmonary artery; S, systemic venous atrium.

 
With the child awake, a prototypical Aloka 4 mm catheter (Aloka Incorporated, Tokyo, Japan) was introduced into the esophagus after the nose was sprayed with a local anesthetic. The echographic pictures obtained nicely portrayed the site of the bifurcation of the pulmonary arteries, but, despite clear visualization of nonobstructed flow via the anastomosis between the right atrial appendage and the right pulmonary artery, no flow was seen in the left pulmonary artery (Fig. 1, B). At the time this was considered to be an artifact resulting from the projection of the left pulmonary artery. Nevertheless, the patient underwent immediate reoperation without further heart catheterization. The fenestration was enlarged and thrombi were removed from the systemic venous atrium. After the child was weaned from cardiopulmonary bypass, transesophageal echocardiography once again failed to show flow in the left pulmonary artery, but a thrombus was recognized (Fig. 1, C). Bypass was reinstituted, the left pulmonary artery was opened, and a massive thrombus occluding the left main pulmonary artery was found and removed. The patient was then weaned from cardiopulmonary bypass without difficulties. Transesophageal echocardiography in the immediate postoperative period demonstrated flow in both pulmonary arteries and no residual obstructions.

Although our present experience was obtained retrospectively, it endorses previous studies indicating the superiority of transesophageal echocardiography over conventional echocardiographic approaches for the detection of thrombus formation after the Fontan operation. Go Go 2,3 Technical achievements in miniaturizing the probes have made the transesophageal approach accessible for infants and children. In fact, a 4 mm diameter probe can now be inserted through the nose under local anesthesia, as documented in this case. Hence it appears that transesophageal echocardiography in patients with evidence of thrombotic complications of the right side of the heart after a Fontan procedure will replace angiocardiography.

References

  1. Hedrick M, Elkins RC, Knott-Craig CJ, Razook JD. Successful thrombectomy for thrombosis of the right side of the heart after the Fontan operation: report of two cases and review of the literature. J THORAC CARDIOVASC SURG 1993;105:297-301.[Abstract]
  2. Fyfe JA, Kline CH, Sade RM, Gilette PC. Transesophageal echocardiography detects thrombus formation not identified by transthoracic echocardiography after the Fontan operation. J Am Coll Cardiol 1991;18:1733-7.[Abstract]
  3. Stümper OFW, Sutherland GR, Geuskens R, Roelandt JRTC, Bos E, Hess J. Transesophageal echocardiography in evaluation and management after a Fontan procedure. J Am Coll Cardiol 1991;17:1152-60.[Abstract]



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