Summary
The predilection of children with congenital heart disease (CHD) to infection may be explained in part by an underlying immunodeficiency disorder. Some 13 syndromes in which immunodeficiency and CHD may coexist have been reported in the medical literature. In addition, immunoglobulin and T-cell deficiencies have been found in nonsyndromal patients with CHD. The diagnosis of immunodeficiency should be entertained in such children, as early recognition of an immunodeficiency disorder can result in improved antimicrobial and immunological management.
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Aggett PJ, Harries JT, Harvey BAM, Soothill JE (1979) An inherited defect of neutrophil mobility in Shwachman's syndrome.J Pediatr 94:391–394
Aiuti F, Fiorilli M, Quinti I (1983) Thymopoietin pentapeptide treatment of primary immunodeficiencies.Lancet 1:551–555
Ammann AJ, Wara DW, Cowan MJ, Barrett DJ, Steihm R (1982) The Di George syndrome and the fetal alcohol syndrome.Am J Dis Child 136:906–908
Barrett DJ, Ammann AJ, Wara DW, Cowan MJ, Fisher TJ, Stiehm ER (1981) Clinical and immunologic spectrum of the Di George syndrome.J Clin Lab Immunol 6:1–6
Biggar WD, Ramirez RA, Rose V (1981) Congenital asplenia: immunologic assessment and a clinical review of eight surviving patients.Pediatrics 67:548–551
Condie RM, O'Reilly RJ (1984) Prevention of cytomegalovirus infection by prophylaxis with an intravenous, hyperimmune native unmodified globulin: randomized trial in bone marrow transplant recipients.Am J Med 76(suppl 3A):134–141
Conley ME, Beckwith JB, Mancer JFK, Tenchkoff L (1979) The spectrum of Di George syndrome.J Pediatr 94:883–890
Cunningham-Rundles C, Siegal FP, Smithwick EM, Lion-Boule A, Cunningham-Rundles S, O'Malley J, Barandum S, Good RA (1984) Efficacy of intravenous immunoglobulin in primary humoral immunodeficiency disease.Ann Intern Med 101:435–439
Di George AM (1968) Congenital absence of the thymus and its immunologic consequence: concurrence with congenital hypoparathyroidism.Birth Defects 4:116–121
Fischer A, Griscelli C, Freidrich W, Kubarak B, Levinsky R, Morgan G, Vossen J, Wagemaker G, Landais P (1986) Bone-marrow transplantation for immunodeficiencies and osteoporosis: European survey 1968–1985.Lancet 2:1080–1083
Freedom RM, Rosen FA, Nadas AD (1972) Congenital cardiovascular disease and anomalies of the third and fourth pharyngeal pouch.Circulation 46:165–172
Fucillo DA, Steele RW, Hensen SA, Vincent MM, Hardy JB, Bellanti JA (1974) Impaired cellular immunity to rubella virus in congenital rubella.Infect Immun 9:81–84
Gallin JI (1981) Abnormal phagocyte chemotaxis: pathophysiology, clinical manifestations and management of patients.Rev Infect Dis 3:1196–1220
Hall CB, McBride JT, Walsh EE (1983) Aerosolized ribavirin treatment of infants with respiratory syncytial viral infection: a randomized double-blind study.N Engl J Med 308:1443–1447
Hancock MP, Huntley CC, Sever JL (1968) Congenital rubella syndrome with immunoglobulin disorder.J Pediatr 72:636–645
Hassiz A (1986) Intravenous immunoglobulin: pharmacological aspects and therapeutic use.Vox Sang 51:10–17
Hughes WT, Tufenkeji H, Bartley DL (1983) Symposium on anti-infection therapy: the immune compromised host.Pediatr Clin North Am 30:103–120
Jensen K, Petersen PH, Nielsen EL, Dahl G, Nielsen J (1976) Serum immunoglobulin M, G and A concentration levels in Turner's syndrome compared with normal women and men.Hum Genet 31:329–334
Johnson S, Knight R, Marmer DJ, Steele RW (1981) Immune deficiency in fetal alcohol syndrome.Pediatr Res 15:908–911
Johnston RB (1982) Defects of neutrophil function.N Engl J Med 307:134–436
Kaplan EL (1977) Infective endocarditis in the pediatric age group: an overview. In: Kaplan EL, Turanto AV (eds)Infective endocarditis: AHA symposium, monograph 52. AHA, Dallas
Kiel EA, Drummond WH, Barrett DJ (1984) Prevalence of T lymphocyte abnormalities in infants with congenital heart disease.Am J Dis Child 138:143–146
Kretschmer R, Say B, Brown D, Rosen FS (1968) Congenital aplasia of the thymus gland (Di George syndrome).N Engl J Med 279:1295–1301
Kretschmer RR, Lopez-Osuna M, De la Rosa L, Armendares S (1974) Leucocyte function in Down's syndrome: quantitative NBT reduction and bactericidal capacity.Clin Immunol Immunopathol 2:449–455
Lammer EJ, Chen DT, Hoar RH, Agnish ND, Benke PJ, Braun JT, Curry CJ, Fernhoff PM, Grix AW, Lott IT, Richard JM, Sun SC (1985) Retinoic acid embryopathy.N Engl J Med 313:837–841
Levin S, Schlesinger M, Handzel Z, Hahn T, Altman Y, Czernobilsky B, Boss J (1979) Thymic deficiency in Down's syndrome.Pediatrics 63:80–87
Lischner HE (1972) Di George syndrome(s).J Pediatr 81:1041–1044
MacDonald NE, Hall Ce, Suffin SC, Alexson C, Harris PJ, Manning JA (1982) Respiratory syncytial viral infection in infants with congenital heart disease.N Engl J Med 307:397–400
Ochs HD, Wedgwood RJ (1980) Disorders of the B-cell system. In: Stiehm ER, Fulginiti VA (eds)Immunologic disorders in infants and children. WB Saunders, Toronto, p 255
Olson GB, South MA, Good RA (1967) Phytohemagglutinin unresponsiveness of lymphocytes from babies with congenital rubella.Nature 214:635–696
Prince GA, Hemming VG, Chanock PM (1986) The use of purified immunoglobulin in the therapy of respiratory syncytial virus infection.Pediatr Infect Dis 5:S201–206
Radford DJ (1985) Truncus arteriosus and facial dysmorphism.Aust Paediatr J 21:131–133
Radford DJ, Lachman R, Thong YH (1986) The immunocompetence of children with congenital heart disease.Int Arch Allergy Appl Immunol 81:331–336
Radford DJ, Perkins L, Lachman R, Thong YH (1988) The spectrum of the Di George syndrome in patients with truncus arteriosus.Pediatr Cardiol 9:95–101
Ross SC, Densen P (1984) Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency.Medicine 63:243–273
Savilahti E, Rapola J (1984) Frequent myocardial lesions in Shwachman's syndrome.Acta Paediatr Scand 73:642–651
Seger R, Wildfeuer A, Buchinger G, Romen W, Catty D, Dybas L, Haferkamp O, Stroder J (1976) Defects in granulocyte function in various chromosome abnormalities (Down's, Edwards', cri-du-chat syndrome).Klin Wochenschr 54:177–183
Sharma SD (1986) The macrophages.Clin Immun Allergy 6:1–28
Shimizu T, Takao A, Ando M, Hirayama A (1984) Conotruncal anomaly face syndrome: its heterogeneity and association with thymus involution. In: Nora JJ, Takao A (eds)Congenital heart disease, causes and processes. Futura, New York, pp 29–41
Shortman K (1984) T-cell development in the thymus.Nature 309:583–584
Smith DW (1982)Recognisable patterns of human malformation, 3rd edn. WB Saunders, Philadelphia, p 460
South MA, Montgomery JR, Rawls WE (1975) Immune deficiency in congenital rubella and other viral infections.Birth Defects 11:234–238
Taussig HB (1960)Congenital malformation of the heart. Harvard University Press, Cambridge, pp 166–167
Thong YH (1978) Impaired neutrophil kinesis in the Shwachman-Diamond syndrome.Aust Paediatr J 14:34–37
Thong YH (1986) Immunomodulation by antimicrobial agents. In: Jeljazewicz J, Pulverer G (eds)Antimicrobial agents and immunity. Academic Press, London, pp 87–129
Thong YH, Robertson EF, Rischbieth HG, Pollard AC (1978) Successful restoration of immunity in the Di George syndrome with fetal thymic epithelial transplant.Arch Dis Child 53:580–584
Vogler LB, Lawton AR (1985) Ontogeny of B cells and humoral immune functions.Clin Immun Allergy 5:235–252
Waldman JD, Rosenthal A, Smith AL, Shurin S, Nadas AS (1977) Sepsis and congenital asplenia.J Pediatr 90:555–559
Waters AH, Webster ADB (eds) (1985) Intravenous immunoglobulins in immunodeficiency syndromes and idiopathic thrombocytopenic purpura.R Soc Med (Lon)
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Radford, D.J., Thong, Y.H. The association between immunodeficiency and congenital heart disease. Pediatr Cardiol 9, 103–108 (1988). https://doi.org/10.1007/BF02083708
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DOI: https://doi.org/10.1007/BF02083708