title: Pre-operative coagulopathy management of a neonate with complex congenital heart disease: a case study.
abstract: Severe coagulation defects often develop in neonates undergoing cardiac surgery, both as a result of the surgical intervention, and as pre-existing defects in the hemostatic mechanisms. The following case report describes a newborn patient with complex congenital heart disease and respiratory failure whose pre-operative coagulopathy was aggressively managed prior to surgical correction. A 5-day-old, 2.5 kg child presented with interrupted aortic arch, ventricular septal defect, atrial septal defect, and patent ductus arteriosus. On admission, he was in respiratory arrest suffering from profound acidemia. In addition, the child was hypothermic (30.1 degrees C), septic (Streptococcus viridans), and coagulopathic (disseminated intravascular coagulation-DIC). The patient was immediately intubated and initial coagulation assessment revealed the following: prothrombin time (PT) 48.9 s (international normalized ratio (INR) 15.7), activated partial thromboplastin time (aPTT) >106 s, platelet count 30,000 mm(3), fibrinogen 15 mg dL(-1) and antithrombin III (AT-III) 10%. Before cardiac surgery could be performed, the patient's DIC was corrected with the administration of cryoprecipitate (15 ml), fresh frozen plasma (300 ml), and platelets (195 ml). In spite of the large transfusion of fresh frozen plasma, the AT-III activity, measured as a percentage, remained depressed at 33. Initial thromboelastographic (TEG) determination revealed an index of +2.02, and following 100 IU administration of an AT-III concentrate, declined to -2.32. Sequential TEG profiles were performed over several days, with the results used to guide both transfusion and medical therapy. The congenital heart defect correction was subsequently performed with satisfactory initial results, but the patient developed a fungal infection and expired on the 16th post-operative day. The present case describes techniques of coagulation management for a newborn with both a severe hemostatic defect and congenital heart disease.
authors.affiliation: Division of Clinical Perfusion Education, University of Nebraska Medical Center, Omaha 68198-5155, USA.
authors.forename: A H
authors.initials: AH
authors.lastname: Stammers
authors.forename: E D
authors.initials: ED
authors.lastname: Rauch
authors.forename: L D
authors.initials: LD
authors.lastname: Willett
authors.forename: J W
authors.initials: JW
authors.lastname: Newberry
authors.forename: K F
authors.initials: KF
authors.lastname: Duncan
mesh_terms: D000142:Acidosis, Respiratory / Q000150:complications / Q000188:drug therapy; D000900:Anti-Bacterial Agents; D000990:Antithrombin III / Q000627:therapeutic use*; D020152:Antithrombin III Deficiency / Q000150:complications*; D001780:Blood Coagulation Tests; D003102:Colloids / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004211:Disseminated Intravascular Coagulation / Q000150:complications / Q000628:therapy*; D004280:Dobutamine / Q000627:therapeutic use; D004298:Dopamine / Q000627:therapeutic use; D004359:Drug Therapy, Combination / Q000627:therapeutic use; D004697:Endocarditis, Bacterial / Q000209:etiology; D017809:Fatal Outcome; D016469:Fungemia / Q000150:complications; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery*; D006331:Heart Diseases / Q000209:etiology; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use; D010949:Plasma; D017713:Platelet Transfusion; D011183:Postoperative Complications; D011300:Preoperative Care; D017693:Sodium Bicarbonate / Q000627:therapeutic use; D013290:Streptococcal Infections / Q000150:complications / Q000188:drug therapy; D013927:Thrombosis / Q000209:etiology
chemical_list: D000900:Anti-Bacterial Agents; D003102:Colloids; D009569:Nitric Oxide; D004280:Dobutamine; D017693:Sodium Bicarbonate; D000990:Antithrombin III; D004298:Dopamine