Journal Reading - Hematological Indices in Pediatric Patients

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1. Hematological Indices in Pediatric Patients with Acyanotic Congenital Heart Disease: A Cross-Sectional Study of 248 Patients Supervisor: Dr. Yetty Movieta N, Sp.A (K) Cardiology Resident : dr. Dian Wulandari Journal Reading

2. Research Article Identity Tittle : Hematological Indices in Pediatric Patients with Acyanotic Congenital Heart Disease: A Cross-Sectional Study of 248 Patients Authors : Hanieh Mohammadi, Behzad Mohammadpour Ahranjani , Ehsan Aghaei Moghadam, Farzad Kompani , Mona Mirbeyk and Nima Rezaei Journal : Egyptian Journal of Medical Human Genetics Year : 2022 DOI : https:// doi.org /10.1186/s43042-022-00262-4

3. INTRODUCTION 01

4. Congenital cardiac defects - Most common developmental anomaly, with an incidence of 4 to 10 per 1000 live births. - Most common non-infectious causes of mortality in pediatrics .  - Increases in infants born with Down’s Syndrome as long as newborns of mothers with diabetes mellitus or mothers with congenital heart defects. - The pathogenesis of most of them has remained unknown - Congenital heart defects are comprised of two main groups based on the presence or absence of cyanosis, including acyanotic congenital heart disease and cyanotic congenital heart disease (CCHD) - Ventricular septal defect (VSD) comprises the most significant percentage of cardiac malformations, up to 40% of all congenital heart anomalies.

5. There are studies done to screen children with congenital heart diseases for hematologic abnormalities. Laboratory abnormalities of hematologic tests are common in CCHD patients. Erythrocytosis, thrombocytopenia, platelet function defects, coagulation factors deficiencies are the major hematologic disorders in patients with CCHD. Furthermore, other than what was mentioned, patients with CHD are more predisposed to cardiac infections and complications of other infections such as Intensive Care Unit hospitalization. This study aimed to assess the prevalence of hematologic disorders in pediatric patients with CHD, compare different hematology parameters between non-hypoxemic CHD patients, and evaluate neutropenia frequency, thrombocytopenia, anemia , leukopenia.

6. METHODS 02

7. + Study Design & Setting Type: C ross-sectional study Period: January 2014 – December 2019 Locations: Pediatric Cardiology Clinic, Tehran University Target: Children & Adolescents with CHD <18 years old Participant Inclusion: children and adolescents with CHD <18 years old who attended the Pediatric Cardiology Clinic, Tehran University during pre-operation visits. Exclusion: sepsis, septic shock, high fever, localized infection, radical operation due to hypoplastic pulmonary arteries, or increased pulmonary vascular resistance due to Eisenmenger syndrome, CCHD and dysmorphic features/DiGeorge syndrome, consume anti platelet or anti-inflammatory agent, Noonan or Down Syndrome, abnormal liver function test or hepatosplenomegaly, history of packed red cells or platelet transfusion. Ethics: Approved by Ethical Committee, guardian/parental written consent obtained.

8. + Study Design & Definitions Patients divided into 2 groups (according to the neutrophil count: CHD with neutropenia (neutrophil count <1500 × 109/L) 1. mild neutropenia (neutrophil count < 1500 × 109/L) 2. moderate neutropenia (neutrophil count < 1000 × 109/L) 3. Severe neutropenia (neutrophil count < 500 × 109/L) - ACHD with normal neutrophil count (neutrophil count ≥ 1500 × 109/L) - Thrombocytopenia was defined as platelet count < 150 × 109/L - Anemia as HB less than 2SD

9. + Sample collection & Measurements * Investigations from the patients’ hospital records carried out their sex, age at the pre-operation time, echocardiographic data that approved the diagnosis of CHD, and their registered hematological profile one day before cardiac surgery. * Peripheral blood samples were gathered on (K2-EDTA) (1.2 mg/mL) for complete blood count (CBC). For chemical analysis, clotted samples were obtained, and serum was sepa - rated by centrifugation for 15 min at 1000×g. CBC was performed using SysmexXt 2000i. A qualitative examination of C-reactive protein with latex agglutination test had been done to see whether the patients had an acute infection or not. * All patients underwent an echocardiographic study using a Philips iE33 machine *Statistical Analysis Statistical Program for Social Science version 26. Shapiro–Wilk  normality of data distribution. Student T-Test  p arametric quantitative variables The Mann–Whitney  compare nonparametric quantitative measures between two groups. P value < 0.05  significant

10. RESULTS 03

11. DISCUSSION 04

12. Burden of CHD CHD prevalence rising → ~12 million people worldwide Major health, social, and economic burden 589,479 years lived with disability in 2017 Mortality Trends Mortality decreasing due to improved care Shift toward non-cardiac causes (infection, malignancy) Taiwan & Australia: >50% adult ACHD deaths non-cardiac Infection = leading non-cardiac cause of death Infection Risks in CHD CHD ↑ risk of pneumonia-related death & complications Viral infections (influenza, COVID-19) → higher mortality, longer hospital stay, ↑ cost CHD patients = more vulnerable due to immune dysfunction

13. Immune Dysfunction Evidence Gene mutations (Notch, Ras/MAPK) link immunity & CHD Scarce data on immune system development in CHD Studies show ↓ WBC, ↓ T & B lymphocytes in CHD This study: 9.2% neutropenia (vs. 3% in general population) Neutropenia more common in VSD patients Hematologic Anomalies Anemia , neutropenia, coagulopathy frequently coexist Cyanotic CHD → thrombocytopenia (25% cases) This study: thrombocytopenia low (1.3%), thrombocytosis 6.4% Acyanotic CHD = less thrombocytopenia vs. cyanotic CHD Anemia in CHD Anemia common even without deficiencies 1/3 CCHD patients = iron deficiency anemia Preoperative anemia ↑ risk of complications & mortality Many cases remain undiagnosed (asymptomatic, nonspecific symptoms)

14. CONCLUSION 05

15. ACHD, especially VSD patients, at higher risk of neutropenia Hematologic issues ( anemia , neutropenia) need more focus than thrombocytopenia Screening & management of anemia essential preoperatively Further studies needed on immune dysfunction & prognosis

16. CRITICAL APPRAISAL 06

17. Population 248 children and adolescents (<18 years) with acyanotic congenital heart disease (ACHD) (VSD, ASD, PDA) Setting: Pediatric Cardiology Clinic, Tehran University of Medical Sciences (2014–2019). Exclusions: Cyanotic CHD, sepsis, genetic syndromes (Down, DiGeorge, etc.), liver dysfunction, recent transfusion. Intervention/Exposure Hematological indices measured: neutropenia, anemia , thrombocytopenia, leukopenia, thrombocytosis. Comparison - ACHD patients with neutropenia vs. ACHD patients without neutropenia. - Comparison across different defect types (VSD vs. ASD vs. PDA). - Comparison of hematologic indices between groups . Outcome Primary outcomes: Prevalence of hematological abnormalities: Anemia : 19.3%; Neutropenia: 9.2%; Thrombocytopenia: 1.2%; Thrombocytosis: 6.4% Secondary findings: Neutropenia significantly correlated with VSD (22/23 neutropenic patients had VSD, p = 0.000). Neutropenia associated with lower Hb and WBC count (p < 0.05). Anemia more frequent in neutropenic group (52% vs. 16%). No significant association between platelet count and neutropenia.

18. II Validity Study design: cross-sectional observational study. Good for prevalence, but cannot establish causality. Population: 248 children with ACHD (VSD, ASD, PDA) pre-open heart surgery. Strengths: clear inclusion/exclusion (excluded cyanotic CHD, syndromes, sepsis) Limitation: no control group (healthy children), no long-term outcomes studied only lab values Sampling method: consecutive patients at one tertiary centre (Tehran). Possible selection bias (not representative of general population) Measurement : CBC performed with standardized machine, neutropenia defined with internationl cutoffs. Outcome definitions were valid. Confounding : excluded major confounders (e.g. infections, genetic syndromes but didn’t adjust for nutritional status, socioeconomic background, or iron deficiency. Importance Highlights previously under-explored hematological abnormalities in children with ACHD, particularly neutropenia associated with VSD, which may affect immune defense and infection risk. Results highlight the need for pre-op hematology screening in ACHD kids. Applicability Population relevance: Pediatric ACHD patients—directly relevant to pediatric cardiology practice. Study done in Iran, might differ from Indonesian setting (nutrition, infection prevalence.

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