RESUMO
Introduction: To study the clinical presentation, laboratory profile, echocardiographic details, management, and outcomes of children who were diagnosed to have multisystem inflammatory syndrome in children (MIS-C) in the immediate postoperative period after surgery for congenital heart defects (CHDs). Materials and Methods: This is a prospective case-control study that included children diagnosed to have MIS-C in the postoperative period based on clinical signs, rise in inflammatory markers, and echocardiographic features of ventricular dysfunction or coronary involvement. Management included intravenous immunoglobulin (IVIG), steroids, and antiplatelet medications in addition to routine postoperative care. Out of the 461 children who underwent surgery for CHD between April 1st, 2021, and November 30th, 2021, 18 children were diagnosed with MIS-C. After the initial routine postoperative course, all 18 children had sudden worsening in clinical and laboratory parameters. Other causes such as bacterial infection were ruled out. All of these children had features of MIS-C with ventricular dilatation and dysfunction, coronary artery involvement, and reactive COVID-19 immunoglobulin G antibody. There was a significant improvement in coronary artery dimensions after IVIG administration (P = 0.001). The involvement of the left main coronary artery was associated with significantly increased length of intensive care unit (ICU) and hospital stay (P = 0.019). Mean ICU and hospital stay was prolonged in the MIS-C group. There were two deaths in this group due to severe left ventricular dysfunction. Conclusions: During the pandemic, a proportion of patients undergoing elective cardiac surgery may develop unexpected worsening in clinical status due to MIS-C. A high index of suspicion and prompt treatment with IVIG and steroids may be helpful in improving outcomes.
RESUMO
BACKGROUND: Congenital Heart diseases (CHDs) account for 1/3rd of all congenital birth defects. Etiopathogenesis of CHDs remain elusive despite extensive investigations globally. Phenotypic heterogeneity witnessed in this developmental disorder reiterate gene-environment interactions with periconceptional factors as risk conferring; and genetic analysis of both sporadic and familial forms of CHD suggest its multigenic basis. Significant association of de novo and inherited variants have been observed. Approximately 1/5th of CHDs are documented in the ethnically distinct Indian population but genetic insights have been very limited. This pilot case-control based association study was undertaken to investigate the status of Caucasian SNPs in a north Indian cohort. METHOD: A total of 306 CHD cases sub-classified into n = 198 acyanotic and n = 108 cyanotic types were recruited from a dedicated tertiary paediatric cardiac centre in Palwal, Haryana. 23 SNPs primarily prioritized from Genome-wide association studies (GWAS) on Caucasians were genotyped using Agena MassARRAY Technology and test of association was performed with adequately numbered controls. RESULTS: Fifty percent of the studied SNPs were substantially associated in either allelic, genotypic or sub-phenotype categories validating their strong correlation with disease manifestation. Of note, strongest allelic association was observed for rs73118372 in CRELD1 (p < 0.0001) on Chr3, rs28711516 in MYH6 (p = 0.00083) and rs735712 in MYH7 (p = 0.0009) both on Chr 14 and were also significantly associated with acyanotic, and cyanotic categories separately. rs28711516 (p = 0.003) and rs735712 (p = 0.002) also showed genotypic association. Strongest association was observed with rs735712(p = 0.003) in VSD and maximum association was observed for ASD sub-phenotypes. CONCLUSIONS: Caucasian findings were partly replicated in the north Indian population. The findings suggest the contribution of genetic, environmental and sociodemographic factors, warranting continued investigations in this study population.
