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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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References

1. Imanaka, K, Takamoto, S, Murakami, A, Kaneko, Y. Right ventricular thrombosis early after bidirectional Glenn shunt. Ann Thorac Surg 1999; 68: 563–565.CrossRefGoogle ScholarPubMed
2. Germanakis, I, Sfyridaki, C, Papadopoulou, E, et al. Stroke following Glenn anastomosis in a child with inherited thrombophilia. Int J Cardiol 2006; 111: 464–467.CrossRefGoogle Scholar
3. Idorn, L, Jensen, AS, Juul, K, et al. Thromboembolic complications in Fontan patients: population-based prevalence and exploration of the etiology. Pediatr Cardiol 2013; 34: 262–272.CrossRefGoogle ScholarPubMed
4. Monagle, P, Chan, AK, Goldenberg, NA, et al. Antithrombotic therapy in neonates and children: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012; 141 (Suppl 2): e737S–e801S.CrossRefGoogle ScholarPubMed
5. Khairy, P, Fernandes, SM, Mayer, JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008; 117: 85–92.CrossRefGoogle ScholarPubMed
6. Balling, G, Vogt, M, Kaemmerer, H, Eicken, A, Meisner, H, Hess, J. Intracardiac thrombus formation after the Fontan operation. J Thorac Cardiovasc Surg 2000; 119 (4 Pt 1): 745–752.CrossRefGoogle ScholarPubMed
7. Johnson, JT, Lindsay, I, Day, RW, et al. Living at altitude adversely affects survival among patients with a Fontan procedure. J Am Coll Cardiol 2013; 61: 1283–1289.CrossRefGoogle ScholarPubMed
8. Jahangiri, M, Kreutzer, J, Zurakowski, D, Bacha, E, Jonas, RA. Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation. J Thorac Cardiovasc Surg 2000; 120: 778–782.CrossRefGoogle ScholarPubMed
9. Cromme-Dijkhuis, AH, Henkens, CM, Bijleveld, CM, Hillege, HL, Bom, VJ, van der Meer, J. Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations. Lancet 1990; 336: 1087–1090.CrossRefGoogle ScholarPubMed
10. Ravn, HB, Hjortdal, VE, Stenbog, EV, et al. Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection. Heart 2001; 85: 61–65.CrossRefGoogle ScholarPubMed
11. Odegard, KC, McGowan, FX Jr, DiNardo, JA, et al. Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure. J Thorac Cardiovasc Surg 2002; 123: 459–465.CrossRefGoogle ScholarPubMed
12. Alsoufi, B, Manlhiot, C, Awan, A, et al. Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation. Eur J Cardiothorac Surg 2012; 42: 42–48; discussion 48–49.CrossRefGoogle ScholarPubMed
13. Forbes, TJ, Rosenthal, GL, Reul, GR Jr, Ott, DA, Feltes, TF. Risk factors for life-threatening cavopulmonary thrombosis in patients undergoing bidirectional superior cavopulmonary shunt: an exploratory study. Am Heart J 1997; 134 (5 Pt 1): 865–871.CrossRefGoogle ScholarPubMed
14. Kasper, W, Konstantinides, S, Geibel, A, et al. Management strategies and determinants of outcome in acute major pulmonary embolism: results of a multicenter registry. J Am Coll Cardiol 1997; 30: 1165–1171.CrossRefGoogle ScholarPubMed
15. Gossett, JG, Rocchini, AP, Armstrong, AK. Superior vena cava thrombectomy with the X-SIZER catheter system in a child with Fontan palliation. Catheter Cardiovasc Interv 2007; 69: 28–32.CrossRefGoogle Scholar