Open-heart surgery in an infant with heterozygous factor VII deficiency.
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.
Matsuhisa
Hironori
H
First Department of Surgery, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan. matsuhisa_kch@hp.pref.hyogo.jp
Yoshimura
Naoki
N
Niimi
Hideki
H
Ichida
Fukiko
F
D000015:Abnormalities, Multiple; D016063:Blood Loss, Surgical / Q000517:prevention & control; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002315:Cardiopulmonary Bypass; D003029:Coagulants / Q000008:administration & dosage; D004252:DNA Mutational Analysis; D004310:Double Outlet Right Ventricle / Q000097:blood / Q000150:complications / Q000601:surgery*; D005167:Factor VII / Q000235:genetics; D005168:Factor VII Deficiency / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D015942:Factor VIIa / Q000008:administration & dosage; D018729:Fontan Procedure; D006471:Gastrointestinal Hemorrhage / Q000209:etiology* / Q000517:prevention & control; D006345:Heart Septal Defects, Ventricular / Q000097:blood / Q000150:complications / Q000601:surgery*; D006579:Heterozygote; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care; D019106:Postoperative Hemorrhage / Q000209:etiology* / Q000517:prevention & control; D011994:Recombinant Proteins / Q000008:administration & dosage; D016896:Treatment Outcome
D003029:Coagulants; D011994:Recombinant Proteins; D005167:Factor VII; C103587:recombinant FVIIa; D015942:Factor VIIa