Bleeding and Thrombotic Challenges in Children with uncorrected Cyanotic Congenital Heart Diseases (CCHD)- Experience from a tertiary care pediatric hospital.

Objective: The study aimed to assess the hemostatic complications in children with uncorrected CCHD, focusing on bleeding and thrombotic manifestations, thrombocytopenia, abnormal coagulation profiles, and deficiencies in clotting factors. Methodology: This cross-sectional study was conducted from January to November 2022 at the largest tertiary care pediatric hospital in Lahore, Pakistan. Seventy-five children aged 1-14 years with diagnosed CCHD were enrolled. Detailed clinical evaluation and laboratory investigations, including Complete Blood Count, Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT), International Normalized Ratio (INR) and clotting factors' assays were performed. Thrombophilia testing was done in patients with history of recent stroke. Data was analyzed using SPSS version 24. Results: Among the 75 patients, the majority aged between 1-5 years (n=28, 37.3%) with male to female ratio of 1.5:1. The predominant diagnosis (n= 51,68%) was Tetralogy of Fallot (TOF). Hemostatic abnormalities included thrombocytopenia (48%), prolonged PT (56%), INR (43%), and APTT (36%). Bleeding manifestations were observed in 8% of the patients, while 5.3% had a history of stroke. Raised hematocrit was found to be significantly associated with deranged APTT. Coagulation factor deficiencies including factor V, combined factor VII and X and fibrinogen, were noted in 4% of the patients. Conclusion: The study highlights significant coagulation challenges in children with uncorrected CCHD, like thrombocytopenia and coagulation factors' deficiencies. Enhanced understanding of these hemostatic abnormalities is crucial for better patient care in resource-constrained settings.