Introduction

With the successful repair and palliation of many forms of congenital heart disease in childhood, a growing population of people are now surviving into adulthood with even complex cardiac lesions . These people have a desire to live as ‘normal’ lives as possible, and having children is part of their expectations for the future.

Congenital heart disease is a rare cause of maternal cardiac mortality; however, a large proportion of women attending pre-pregnancy cardiac obstetric clinics have congenital heart disease . The fact that these women have known cardiac disease enables comprehensive pre-conception counselling and optimisation of care to take place . In contrast, many women with acquired heart disease often present for the first time during pregnancy. Unsurprisingly, most maternal cardiac fatalities occur within this group .

It is difficult to establish precisely the extent of maternal morbidity associated with congenital heart disease. In one of the largest studies on cardiac maternal morbidity , congenital heart disease was not labelled as a separate category. The small number of participants with congenital heart disease were listed under those with valve disease. It has been hypothesised that improved pre-conception counselling and antenatal care have led to an improvement in maternal outcomes in congenital heart disease compared with other forms of maternal cardiac disease .

A wide spectrum of congenital heart lesions exists, ranging from the benign lesion with no effect on longevity to life-shortening conditions that may be highly symptomatic. Some women may present with a cardiac lesion that has a unique anatomy and physiology, and therefore preconception advice and management plans must be tailored to the individual patient. It is understandable that the evidence base for practice is rather patchy in this heterogenous population, and many of the treatment recommendations are based on clinical consensus . It is, therefore, essential that these women are cared for by a team of experienced clinicians in a multi-disciplinary environment .

Principles of pre-conception counselling

Preconception counselling should be offered to all women with congenital heart disease who are of child-bearing age. This advice must be given by a specialist multi-disciplinary team with both cardiac and obstetric input. The advice given should be tailored to the unique anatomy and physiology of each particular women. The key components of comprehensive counselling are presented in Table 1 .

Several simple scoring systems have been developed to assist clinicians in their attempts to effectively risk stratify women contemplating pregnancy. The most commonly used of these are the Cardiac Disease in Pregnancy (CARPREG) score and the ZAHARA (Zwangerschap bij Aangeboren Hartafwijking) score ( Table 2 ) . Although far from comprehensive, these scoring systems offer additional tools to approximate risk. An alternative scale that is also informative is the modified World Health Organization pregnancy classification. This has four categories of risk from class I (little additional risk from pregnancy) to class IV (pregnancy is extremely high risk and should be considered contraindicated) .

Principles of pre-conception counselling

Preconception counselling should be offered to all women with congenital heart disease who are of child-bearing age. This advice must be given by a specialist multi-disciplinary team with both cardiac and obstetric input. The advice given should be tailored to the unique anatomy and physiology of each particular women. The key components of comprehensive counselling are presented in Table 1 .

Several simple scoring systems have been developed to assist clinicians in their attempts to effectively risk stratify women contemplating pregnancy. The most commonly used of these are the Cardiac Disease in Pregnancy (CARPREG) score and the ZAHARA (Zwangerschap bij Aangeboren Hartafwijking) score ( Table 2 ) . Although far from comprehensive, these scoring systems offer additional tools to approximate risk. An alternative scale that is also informative is the modified World Health Organization pregnancy classification. This has four categories of risk from class I (little additional risk from pregnancy) to class IV (pregnancy is extremely high risk and should be considered contraindicated) .