Paediatric Congenital Heart Disease


The understanding, diagnosis and treatment of congenital malformations of the heart has been revolutionized over the past few decades, reflecting not only a greater appreciation of the morphological variability to be found within the malformed hearts, but also huge advances in the design and construction of machines available for diagnosis, treatment and life support. Nowadays, despite the anatomical complexity, accurate diagnosis is to be expected in all cases, with the greater majority of patients expected to survive treatment, which is being undertaken progressively earlier in life.

Keywords: sequential segmental analysis; cross‐sectional echocardiography; catheterization; angiography; interventional catheterization; open heart surgery

Figure 1.

The three segments within the heart and their junctions.

Figure 2.

The four basic patterns of arrangement of the organs within the body. The overall arrangement of the isomeric variants is often called visceral heterotaxy. Bronchial morphology depends on the bronchial length, the morphologically right bronchus being significantly shorter than the left (arrows). The arrangement within the heart, in the first analysis, reflects the structure of the morphologically right appendage (MRA) and the morphologically left appendage (MLA).

Figure 3.

In this patient, an interatrial communication in the oval fossa has been closed by insertion, through a catheter, of an umbrella with two discs made of nitinol wire. In (a) the left half of the double umbrella (arrow) has been opened in the left atrium (LA). In (b) the arrangement is shown in which the right‐sided umbrella (arrow) is opened in the right atrium (RA), sandwiching the margins of the deficient atrial septum.



Anderson RH and Ho SY (1997) Continuing medical education, Sequential segmental analysis – description and categorization for the millenium. Cardiology in the Young 7: 98–116.

Anderson RH and Wilcox BR (1995) Understanding cardiac anatomy – the prerequisite for optimal cardiac surgery. Annals of Thoracic Surgery 59: 1366–1375.

Becker AE and Anderson RH (1983) How should we describe hearts in which the aorta is connected to the right ventricle and the pulmonary trunk to the left ventricle? A matter for reason and logic. American Journal of Cardiology 51: 911–912.

Falcao S, Daliento L, Ho SY, Rigby ML and Anderson RH (1999) Cross‐sectional echocardiographic assessment of the extent of the atrial septum relative to the atrioventricular junction in atrioventricular septal defect. Heart 81: 199–206.

Franklin RCG, Anderson RH, Daniëls O et al. (1999) Report of the coding committee of the Association for European Paediatric Cardiology. Cardiology in theYoung 9: 633–657.

Gatzoulis MA, Rigby ML and Redington AN (1995) Interventional catheterisation in paediatric cardiology. European Heart Journal 16: 1767–1772.

Van Mierop LHS (1971) Transposition of the great arteries, Clarification or further confusion? American Journal of Cardiology 28: 735–738 (editorial).

Van Praagh R (1971) Transposition of the great arteries. II. Transposition clarified. American Journal of Cardiology 28: 739–741.

Van Praagh R (1972) The segmental approach to diagnosis in congenital heart disease. In: Bergsma D (ed.) Birth Defects, Original Article Series, vol. VIII, no. 5. The National Foundation – March of Dimes, pp. 4–23. Baltimore: Williams and Wilkins

Wood P (1958) The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. British Medical Journal 2: 701–709.

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Anderson, Robert H, and Redington, Andrew N(Apr 2001) Paediatric Congenital Heart Disease. In: eLS. John Wiley & Sons Ltd, Chichester. [doi: 10.1038/npg.els.0003277]