ABSTRACT
An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.
Subject(s)
Coronary Thrombosis/drug therapy , Fibrinolytic Agents/administration & dosage , Hypoplastic Left Heart Syndrome/complications , Thrombolytic Therapy , Tissue Plasminogen Activator/administration & dosage , Coronary Thrombosis/diagnostic imaging , Coronary Thrombosis/etiology , Coronary Thrombosis/physiopathology , Extracorporeal Membrane Oxygenation , Humans , Hypoplastic Left Heart Syndrome/diagnostic imaging , Hypoplastic Left Heart Syndrome/physiopathology , Infant , Male , Recombinant Proteins/administration & dosage , Treatment OutcomeABSTRACT
BACKGROUND: Pediatric scoliosis surgery is associated with considerable blood loss and allogenic transfusions. Transfusions contribute to morbidities and cost. A perioperative pediatric blood management program was implemented at our institution. Patients received preoperative evaluation, cell salvage, topical hemostasis, antifibrinolytics, and hypotensive anesthesia. STUDY DESIGN AND METHODS: The study was a 2-year retrospective cohort review of the program's population from September 2007 through August 2009. RESULTS: A total of 110 scoliosis surgeries were performed with only 34 and 12% of the patients requiring preoperative oral iron and erythropoietin, respectively. Neuromuscular scoliosis patients had more repaired segments and a larger transfusion rate than idiopathic scoliosis patients (36% vs. 1.7%, p = 0.001). Transfused patients had more blood loss relative to their blood volume (p = 0.001) and blood loss was associated with higher Cobb angles (p = 0.04). Logistic regression revealed that blood loss (p = 0.001), number of segments fused (p = 0.004), and lower patient weight (p = 0.007) are associated with increased odds for transfusion. Twelve patients (10.9%) were identified with low von Willebrand activity with a trend toward higher blood losses (p = 0.07) with lower activity levels. CONCLUSION: Transfusion requirements in scoliosis patients are dependent on blood loss as determined by Cobb angles and number of segments fused relative to the patients' blood volume as determined by weight. Implementation of a blood management protocol resulted in a low transfusion rate and unexpectedly led to the preoperative diagnosis of a number of patients with low levels of von Willebrand activity.