Journal of the Korean Pediatric Society 1990;33(5):642-650.
Published online May 31, 1990.
A Clinical Study on Hematological Findings in Children with Cyanotic Congenital Heart Disease.
Seong Ho Kim1, Heung Jae Lee1, Hahng Lee2
1Department of Pediatrics, Sejong General Hospital, Pucheon, Korea
2Department of Pediatrics, College of Medicine, Hanyang University, Seoul, Korea
청색증형 선천성 심질환아의 혈액학적 소견에 대한 임상연구
김성호1, 이홍재1, 이항2
1부천세종병원 소아과
2한양대학교 의과대학 소아과학교실
Received: 22 November 1989   • Accepted: 15 February 1990
Children with cyanotic congenital heart disease (CCHD) and secondary polycythemia exhibit increased susceptibility to hemorrhage or thrombosis. The hemostatic disorder manifests itself by the occurrence of excessive hemorrhage after surgery, and by increased incidence of cerebrovascular accident of a thrombotic nature during life. To assess whether the severity of polycythemia in children with CCHD is correlated with coagula- tion abnormalities, hematologic studies with preoperative and postoperative blood counts, preoper- ative arterial oxygen saturation and coagulation tests were performed in 74 children with CCHD, confirmed by cardiac catheterization during the period of 2 years from January, 1986 to December, 1987 in the Department of Pediatrics, Hanyang University Hospital. The study patients were divided into two groups according to the severity of polycythemia, comparing the results-in Group I (Het^50%) with those in Group H (Het<50%), and the results in Group A (age <3 years) with those in Group B (age ^3 years). The summary of the rusults from the study and the observation were as follow: 1) Compared to the children in Group II with lesser degree of polycythemia, the mean value of platelet counts was significantly lower in children in Group I in more severe polycythemic group, but the mean values in both group were within normal range. However, the mean values for bleeding time, prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen level, and thrombo test showed no significant differences between the two groups (Table 2). 2) Frequency of thrombocytopenia, with platelet count below 100,000/mm3 was higher in more severe polycythemic group with 3 patients in Group I and none in Group II, while all 3 patients were in Group A and none in Group B (Fig. 1). The number of the patients with prolonged PT over 15 seconds (sec) was 6 in Group I with 2 in Group II,and 5 in Group A with 3 in Group B (Fig. 3). The number of low fibrinogen level below 125 mg/dl was 5 in Group I with 2 in Group II, and 6 in Group A with one in Group B (Fig. 4). These results indicated that the chance of hemostatic problems occurring with CCHD was higher in the more severely polycythemic children with Het above 50%, and in children under the age of 3 years (Table 3). 3) There was a correlation between Het and preoperative aortic oxygen saturation (SaO2), and mean value of SaO2 was significantly lower in Group I as expected (Fig. 5, 6). 4) Hemoglobin values and Het were significantly reduced and platelet counts increased postoper- atively after corrective surgery (Table 4). 5) One out of 7 children with documented iron deficiency anemia developed hemiparesis from cerebral thrombosis (Table 5). 6) In conclusion, in children with CCHD polycythemia becomes more severe as SaO2 falls, and the chance of hemostatic problems such as hemorrhage and thrombosis increases as polycythemia gets severe. Ultimately corrective surgery could improve these hemostatic defects, but proper preoperative assessment of coagulation status is imperative in order to avoid excessive operative and postoper- ative hemorrhage and to administer promptly appropriate therapies for hemorrhage encountered during or after the surgery, thereby enabling to achieve better success with corrective surgery and survival.
Key Words: Cyanotic congenital heart disease, Hematologic findings, Coagulation, Polycythemia

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