Balancing the Scales: Understanding Hemostatic Abnormalities in Children with Uncorrected Cyanotic Congenital Heart Disease

Abstract

Background: Children with uncorrected cyanotic congenital heart disease often develop hemostatic abnormalities due to chronic hypoxia, increasing bleeding and thrombotic risks.

Objective: This study aimed to evaluate hemostatic abnormalities in children with uncorrected cyanotic congenital heart disease (CCHD), focusing on thrombocytopenia, coagulation factor deficiencies, bleeding and thrombotic manifestations, and the relationship between hematocrit and coagulation derangements.

Methodology: This study was a cross-sectional study carried out between April and December of 2023 in the Department of Pediatric Cardiology and Hematology, the Children Hospital and Institute of Child Health, Multan, Pakistan. Seventy-eight children who had a confirmed CCHD but were aged 1 to 15 years were recruited through consecutive sampling. Clinical analysis and laboratory tests, such as Complete Blood Count (CBC), Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT), International Normalized Ratio (INR) and coagulation factor tests (to rule out abnormal screening) were conducted. In patients with recent stroke, Thrombophilia (Protein C, Protein S, Antithrombin III) tests were done. The analysis was made in SPSS v25; p 0.05 was regarded as significant.

Results: In 78 patients, the average age of the patients was 1–5 years (n=30, 38.5) at a male-to-female ratio of 1.4:1. The most common lesion was Tetralogy of Fallot (TOF) (n=53, 68%). Hemostatic abnormalities were thrombocytopenia (47%), increased PT (44%), increased INR (41%), and increased APTT (34%). Hematocrit (>55) was raised in 29% and was significantly related to deranged APTT (p=0.02). The level of bleeding manifestations was 7 patients (9%), and 4 (5.1) had a stroke history. Five percent of the patients were found to be deficient in coagulation factors.

Conclusion: Children with uncorrected CCHD exhibit significant hemostatic abnormalities, including thrombocytopenia and coagulation factor deficiencies, which may remain subclinical but carry risk for bleeding or thrombotic events. Awareness and early evaluation of these abnormalities are essential for optimized perioperative care and improved outcomes in resource-limited settings.