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Thrombolytics for late superior caval vein thrombus in a patient with tricuspid atresia and single-lung Glenn anastomosis

Published online by Cambridge University Press:  21 July 2015

Alexander R. Bonnel ,
Vijayapraveena Paruchuri  and
Wayne J. Franklin
Alexander R. Bonnel
Affiliation:
Department of Medicine, Baylor College of Medicine, Houston, Texas, United States of America
Vijayapraveena Paruchuri
Affiliation:
Adult Congenital Heart Center, Winthrop University Hospital, Mineola, New York, United States of America
Wayne J. Franklin*
Affiliation:
Texas Adult Congenital Heart Program, Texas Children’s Hospital, Houston, Texas, United States of America
*
Correspondence to: W. J. Franklin, MD, Baylor College of Medicine, Texas Adult Congenital Heart Program, Texas Children’s Hospital, 6621 Fannin Street, W19345C, Houston, TX 77054, United States of America. Tel: +832 826 1949; Fax: +832 826 9052; E-mail: wjf@bcm.edu
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Abstract

Background

Those with cyanotic heart disease have an elevated bleeding risk but also are hypercoaguable. Treating haemodynamically significant thrombi in this unique cohort poses a monumental challenge.

Case

A 29-year-old women with tricuspid atresia and left pulmonary artery atresia presented with superior caval vein syndrome. She had a right modified Blalock–Taussig shunt as a neonate. A left modified Blalock–Taussig shunt performed later failed to establish flow to her left lung. At age 5, she had a Fontan procedure to the right lung but could not tolerate the physiology and had a low cardiac output syndrome. The Fontan was taken down and she was left with a Glenn anastamosis to the right pulmonary artery. She did well for years until she had dyspnea, upper extremity oedema and “facial fullness”. On examination she was tachycardic, hypotensive, and more desaturated than baseline. She also had facial plethora.

Decision-making

Echocardiogram showed a large 9×3 mm nearly occlusive thrombus in the superior caval vein at the bifurcation of the left and right innominate veins. An emergent venogram confirmed the location and size of the thrombus. Given the thrombus burden and potential for distal embolisation through the Glenn to the single functional lung, we chose to treat the patient with thrombolytics. She had uncomplicated ICU course and was sent home on warfarin. Follow-up echocardiogram showed complete resolution of clot.

Conclusion

This case shows the importance of history and physical exam in caring for this complex cohort of adult patients with CHD.

Keywords

Adult congenitalFontancyanotic heart diseasethrombolytics
Type
Brief Reports
Information
Cardiology in the Young , Volume 26 , Issue 1 , January 2016 , pp. 209 - 213
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Copyright
© Cambridge University Press 2015 

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