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.

Efficacy of High Dose Tranexamic Acid in Decreasing Bleeding after Cardiac Surgery for Cyanotic Congenital Heart Disease in Children Less than Ten Kilo Body Weight

“…Congenital heart defects are inherently associated with a deranged coagulation system with a pre-existing altered platelet function and altered fibrinolysis [10] [11] [12]. They are more prone for bleeding as these effects are worsened by CPB [6].…”

Impact of Thrombelastography in Paediatric Intensive Care

“…We were unable to identify any studies similar to ours. VET use in PICU following cardiac surgery has been reported for guiding transfusions and predicting bleeding 18,[25][26][27][28][29][30][31][32] . VETs have also been used in ECMO and mechanical circulatory support in PICU to guide product transfusion, measure platelet dysfunction, investigate thrombi, monitor and manage haemostasis in the presence of heparin and to detect residual heparinisation and hypercoagulability [33][34][35][36][37][38][39][40][41][42][43] , and could have a role in ECMO when it is desirable to minimise anticoagulation 44 .…”

The Safety and Efficacy of Antifibrinolytic Therapy in Neonatal Cardiac Surgery

“…Congenital heart disease (CHD) has long been associated with coagulation abnormalities [ 26 ], including platelet abnormalities [ 27 ] and fibrinolysis [ 28 ]. To overcome these adverse effects, attempts have been made to modify these effects with both lysine analogue, antifibrinolytics, and aprotinin.…”