Biomaterial Database

Open-heart surgery in an infant with heterozygous factor VII deficiency. 2010

Hironori Matsuhisa, and Naoki Yoshimura, and Hideki Niimi, and Fukiko Ichida

First Department of Surgery, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan. matsuhisa_kch@hp.pref.hyogo.jp

A four-month-old male with Taussig-Bing anomaly and multiple ventricular septal defects underwent an open-heart palliative procedure. He suffered from massive postoperative gastrointestinal bleeding. Heterozygous Arg402Stop-related factor VII deficiency was detected by genomic examinations. When he was 14 months old, a subsequent open-heart surgery with replacement therapy of recombinant factor VIIa was performed without any bleeding or thromboembolic complications. Although heterozygous factor VII deficiency is generally recognized as clinically asymptomatic, this latent bleeding disorder can appear perioperatively or postoperatively in patients who undergo cardiopulmonary bypass procedures. Consequently, the prophylactic replacement therapy with recombinant factor VII is recommended during cardiac operations.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D010166	Palliative Care	Care alleviating symptoms without curing the underlying disease. (Stedman, 25th ed)	Palliative Treatment,Palliative Supportive Care,Palliative Surgery,Palliative Therapy,Surgery, Palliative,Therapy, Palliative,Care, Palliative,Palliative Treatments,Supportive Care, Palliative,Treatment, Palliative,Treatments, Palliative
D011994	Recombinant Proteins	Proteins prepared by recombinant DNA technology.	Biosynthetic Protein,Biosynthetic Proteins,DNA Recombinant Proteins,Recombinant Protein,Proteins, Biosynthetic,Proteins, Recombinant DNA,DNA Proteins, Recombinant,Protein, Biosynthetic,Protein, Recombinant,Proteins, DNA Recombinant,Proteins, Recombinant,Recombinant DNA Proteins,Recombinant Proteins, DNA
D002315	Cardiopulmonary Bypass	Diversion of the flow of blood from the entrance of the right atrium directly to the aorta (or femoral artery) via an oxygenator thus bypassing both the heart and lungs.	Heart-Lung Bypass,Bypass, Cardiopulmonary,Bypass, Heart-Lung,Bypasses, Cardiopulmonary,Bypasses, Heart-Lung,Cardiopulmonary Bypasses,Heart Lung Bypass,Heart-Lung Bypasses
D003029	Coagulants	Agents that cause clotting.	Coagulant
D004252	DNA Mutational Analysis	Biochemical identification of mutational changes in a nucleotide sequence.	Mutational Analysis, DNA,Analysis, DNA Mutational,Analyses, DNA Mutational,DNA Mutational Analyses,Mutational Analyses, DNA
D004310	Double Outlet Right Ventricle	Incomplete transposition of the great vessels in which both the AORTA and the PULMONARY ARTERY arise from the RIGHT VENTRICLE. The only outlet of the LEFT VENTRICLE is a large ventricular septal defect (VENTRICULAR SEPTAL DEFECTS or VSD). The various subtypes are classified by the location of the septal defect, such as subaortic, subpulmonary, or noncommitted.	Taussig-Bing Anomaly,Double Outlet Right Ventricle, Noncommitted VSD,Double Outlet Right Ventricle, Subaortic VSD,Double Outlet Right Ventricle, Subpulmonary VSD,Double-Outlet Right Ventricle,Anomaly, Taussig-Bing,Double-Outlet Right Ventricles,Right Ventricle, Double-Outlet,Taussig Bing Anomaly,Ventricle, Double-Outlet Right
D005167	Factor VII	Heat- and storage-stable plasma protein that is activated by tissue thromboplastin to form factor VIIa in the extrinsic pathway of blood coagulation. The activated form then catalyzes the activation of factor X to factor Xa.	Coagulation Factor VII,Proconvertin,Stable Factor,Blood Coagulation Factor VII,Factor 7,Factor Seven,Factor VII, Coagulation
D005168	Factor VII Deficiency	An autosomal recessive characteristic or a coagulation disorder acquired in association with VITAMIN K DEFICIENCY. FACTOR VII is a Vitamin K dependent glycoprotein essential to the extrinsic pathway of coagulation.	Hypoproconvertinemia,Deficiency, Factor 7,Deficiency, Factor Seven,Deficiency, Factor VII,Factor 7 Deficiency,Deficiencies, Factor 7,Deficiencies, Factor Seven,Deficiencies, Factor VII,Factor 7 Deficiencies,Factor Seven Deficiencies,Factor Seven Deficiency,Factor VII Deficiencies,Hypoproconvertinemias