Health Evidence Bulletins - Wales (logo)
Team Leader: Dr David Fone

Date of completion: 30.9.98

6: Congenital heart disease

This bulletin is a supplement to, not a substitute for, professional skills and experience. Users are advised to consult the supporting evidence for a consideration of all the implications of a recommendation.

The Statements The Evidence
6.1 Echocardiography
6.1a. Extended mid-trimester fetal echocardiography has greater sensitivity than four chamber view alone in the pre-natal diagnosis of major cardiac defects in a low risk population (78% vs. 48%)i. In a large case-series, the extended fetal heart examination identified 18/21 major abnormalities (sensitivity 86%, specificity 99.9%)i. Echocardiography for structural abnormalities correlates well with post-natal diagnosisii,iii.
(Health gain notation 1 "beneficial")
i. Achiron R, Glaser J, Gelernter I, Hegesh J, Yagel S. Extended fetal echocardiographic examination for detecting cardiac malformations in low risk pregnancies. British Medical Journal 1992;304:671-74
(Type IV evidence – comparison of extended fetal echocardiography with standard four chamber view in 5400 fetuses in low risk pregnancies between 18 and 24 weeks gestation)
ii. Allan LD, Chita SK, Sharland GK, Fagg NL, Anderson RH, Crawford DC. The accuracy of fetal echocardiography in the diagnosis of congenital heart disease. International Journal of Cardiology 1989;25:279-88
(Type IV evidence – retrospective comparison of echocardiography and post-mortem findings in 41 cases with major cardiac malformation)
iii. Oberhoffer R, Cook AC, Lang D, et al. Correlation between echocardiographic and morphological investigations of lesions of the tricuspid valve diagnosed during fetal life. British Heart Journal 1992;68:580-85.
(Type IV evidence – retrospective comparison of echocardiography and post-mortem findings in 19 cases with severe tricuspid valve malformation)
6.1b. Echocardiography is the definitive diagnostic method for the recognition and assessment of congenital and acquired heart disease in the paediatric populationi.
(Health gain notation 1 "beneficial")


i. XV Echocardiography in the Paediatric Patient. In: Cheitlin MD, Alpert JS, Armstrong WF, et al. ACC/AHA guidelines for the clinical application of echocardiography: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (committee on the clinical application of echocardiography). Journal of the American College of Cardiology 1997;29:862-79

(Type V evidence – expert opinion)


6.2 Clinical guidelines
6.2a. Evidence-based guidelines for the evaluation and management of congenital heart disease are availablei.
(Health gain notation 1 "beneficial")
i. Driscoll D, Allen HD, Atkins DL, et al. Guidelines for evaluation and management of common congenital cardiac problems in infants, children and adolescents: A statement for health care professionals from the committee on congenital cardiac defects of the council on cardiovascular disease in the young, American Heart Association. Circulation 1994; 90: 2180-88

(Type Vevidence – expert opinion)
6.2b. Evidence-based recommendations for the management of the adult with congenital heart disease are availablei.
(Health gain notation 1 "beneficial")


i. Connelly MS, Webb GD, Somerville J et al, for the Canadian Consensus Conference on Adult Congenital Heart Disease 1996. Canadian Journal of Cardiology 1998; 14: 395-452
(Type V evidence – expert opinion)

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