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Case Reports
. 2018 Jan;7(1):e12-e15.
doi: 10.1055/s-0038-1637743. Epub 2018 Mar 22.

Staged Surgical Palliation for HLHS in a Girl with Severe Factor X Deficiency

Ahmed F Elmahrouk  1   2 , Mohamed F Ismail  1   3 , Abdulbadee Bugis  1 , Nashwa Badawy  1   4 , Hesham Mohamed Aboelghar  5   6 , Tamer Hamouda  1   7 , Ahmed Jamjoom  1
Affiliations
Case Reports

Staged Surgical Palliation for HLHS in a Girl with Severe Factor X Deficiency

Ahmed F Elmahrouk et al. Thorac Cardiovasc Surg Rep. 2018 Jan.

Abstract

Background Factor X deficiency (also known as Stuart-Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication. Conclusion The need to maintain hemostasis while aiming to prevent intracardiac thrombosis requires multidisciplinary team approach including hematologist, cardiac surgeon, pediatric cardiac intensivist, and anesthesiologist along with meticulous hemostasis during surgery and careful monitoring of coagulation profile in the postoperative period.

Keywords: Norwood procedure; factor X deficiency; hypoplastic left heart syndrome; superior cavopulmonary anastomosis; total cavopulmonary connection.

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Figures

Fig. 1
Fig. 1
A graph of the timeline for international normalized ratio [INR] measurements showing derangement, with returning near normal values after treatment at the time of surgeries.
Fig. 2
Fig. 2
A graph of the timeline for prothrombin time (PT) measurements showing derangement, with returning near normal values after treatment at the time of surgeries.
See this image and copyright information in PMC

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