Coagulation abnormalities in children with uncorrected congenital cyanotic heart disorders

Abstract Background Congenital Cyanotic heart disease (CCHD) is a congenital defect of the heart that reduces the amount of oxygen delivered to the body. Coagulation is the physiological process of maintaining hemostasis in the body. Multiple coagulation and hematological abnormalities have been found in patients with CCHD that can lead to both bleeding tendency or/and thrombosis. Aim To assess the presence of certain hematological and coagulation abnormalities among pediatric patients with CCHD comparing them to normal control and assess for the presence of complications resulting from these abnormalities. Methods This analytical observational case control study included 76 patients (37 cases diagnosed with congenital cyanotic heart disease and 39 healthy control) with age ranged from 6 months to 13 years old who were following up in pediatric cardiology clinic in Cairo University Specialized Pediatric Hospital. Both cases and control were tested for PT, PTT, INR, CBC differential and D-dimer, the results of both groups were compared. Results Children with congenital cyanotic disease had a statistically significant higher values of PT, PTT, INR and D-dimer compared to healthy controls with P value 0.001, 0.004, < 0.001 and < 0.001 respectively. Also, there was a statistically significant increase in the Hb, HCT in cases when compared with the control group with a (p-value < 0.001). There was a statistically significant decrease in the platelet count in cases group versus control group with p = 0.001. Eight patients (22%) showed thrombocytopenia while three patients (8%) showed thrombocytosis. There was a statistically significant longer duration of cyanosis in patients who developed complications versus patients who didn’t develop complications (p value: 0.014). Conclusion Coagulation abnormalities, in the form of prolonged PT, PTT, INR, D –dimer, high hematocrit values and thrombocytopenia, were found in children with cyanotic heart disease compared to healthy individuals. These abnormalities make them more liable for thrombotic or bleeding complications. So, routine screening of coagulation abnormalities is recommended in these patients which may aid in management decisions while awaiting definitive surgeries.