CC-BY-NC-ND 4.0 · THORAC CARDIOV SURG Reports 2018; 07(01): e12-e15
DOI: 10.1055/s-0038-1637743
Case Report: Cardiac
Georg Thieme Verlag KG Stuttgart · New York

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

Ahmed F. Elmahrouk
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
Department of Cardiothoracic Surgery, Tanta University Faculty of Medicine, Tanta, Egypt
,
Mohamed F. Ismail
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
Mansoura University Faculty of Medicine, Mansoura, Egypt
,
Abdulbadee Bugis
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
,
Nashwa Badawy
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
Cairo University Kasr Alainy Faculty of Medicine, Cairo, Egypt
,
Hesham Mohamed Aboelghar
Department of Pediatrics, Menoufia University Faculty of Medicine, Shebin El-Kom, Saudi Arabia
Department of Hematology and Oncology BMT, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
,
Tamer Hamouda
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
Department of Cardiothoracic Surgery, Faculty of Medicine, Benha University, Benha, Egypt
,
Ahmed Jamjoom
Department of Cardiothoracic Surgery, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia
› Author Affiliations
Further Information

Publication History

12 November 2017

25 January 2018

Publication Date:
22 March 2018 (online)

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.