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Re: Maternal outcome of pregnancy following Mustard procedure

Dear Editor

We were interested to read Dr Thorne’s article on pregnancy in heart disease and have been developing our service locally for pregnancy in congenital heart disease.[1] We have been particularly concerned with the counselling and management of women following Mustard procedure as many of this unique group are now of child bearing age and there is little data as to their pregnancy outcome.[2]

We carried out a case note review of the 18 women followed up at our institution after Mustard procedure for simple transposition of the great arteries. Seven women had a total of eight pregnancies that they intended to carry to term. The mean age at conception was 24.5 years (range 19-31 years). Five women (six pregnancies) were in NYHA class 1 and had good ventricular function before conception. All six babies were born without neonatal complication. No change in patient's exercise tolerance was reported in the year following each delivery, however two had deterioration in cardiac function on echo. Subsequently one went on to require cardiac transplant 11 years following delivery (31 years following the Mustard procedure). Two women were in NYHA 2 at conception. One was 31 years of age and had poor systemic ventricular function. She was admitted 18 weeks pregnant requiring intra-venous inotropes. She was counselled as to the risks of continuing her pregnancy and decided against termination. She died 25 weeks pregnant following a VF arrest. The second patient (28 years old) presented 38 weeks pregnant with poor cardiac function. In view of deteriorating maternal condition caesarean section was performed. Following delivery she remains in heart failure despite maximal therapy and is currently on the active cardiac transplant list.

In our series, as others have described, asymptomatic women with good echocardiographic function tolerate pregnancy well. [3] At the moment our limited data suggests that outcome of pregnancy in symptomatic patients with atrial switch procedure can be disastrous with life threatening deterioration of their ventricular function. The role of exercise testing and assessment of systemic ventricular performance under loading conditions mimicking pregnancy may have a place in this group of patients prior to preconception counselling. Given the potential for development of impaired systemic ventricular function in all patients following Mustard procedure we agree with Dr. Thorne’s suggestion that earlier pregnancy should perhaps be advised.

References

1) Thorne SA. Pregnancy in heart disease. Heart 2004;90:450-456

2) Moons P, Gewillig M, Sluysmans T, et al. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart 2004;90:307-313

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Re: Re Pregnancy in heart disease, author's response

Dear Editor

Steve Yentis raises relevant discussion points.[1] A detailed discussion of anaesthetic methods was beyond the scope of the article and Steve's comments regarding the safety of low-dose epidural are welcome.

I agree that assisted vaginal delivery is the safest mode of delivery for most women with heart disease. His comments about instrumental vaginal delivery for women with Marfan syndrome underlines the importance of specialist obstetric units for women with heart disease. However aortic disease in Marfan syndrome does carry a significant risk and elective Caesarean section may still be a safer option outside units with particular expertise in this condition.

Reference

1. S A Thorne. Pregnancy in heart disease. Heart 2004; 90: 450-456.

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Re: Re: Pregnancy in heart disease

Dear Editor

Sara Thorne give a comprehensive account of heart disease in pregnancy [1] which, as she says, represents an increasing challenge to high-risk obstetric teams. I wish to raise three points.

First, in most cases the "good analgesia" in labour mentioned by Dr Thorne should be provided (anticoagulation allowing) by epidural analgesia using modern low-dose solutions of local anaesthetic/opioid, since this both preserves cardiovascular stability and allows extension of the epidural block to anaesthesia for caesarean section if required.[2] This is in contrast to the traditional and outdated view that cardiac disease is an absolute contra-indication to all regional techniques -- though use of strong local anaesthetics by rapid injection (for example, single-shot spinal anaesthesia for caesarean section) may indeed be hazardous.

Second, although Dr Thorne recommends elective caesarean section for patients with aortic lesions, at the Chelsea and Westminster Hospital we have developed a team-based approach whereby patients with aortic lesions such as Marfan's disease and coarctation do not necessarily undergo elective caesarean section, the risks from regional or general anaesthesia (cardiovascular instability) and surgery (deep vein thrombosis, bleeding, infection and the need for uterotonic drugs with their potentially dangerous cardiovascular side effects) often being considered greater than those of epidural analgesia and elective instrumental delivery.[3]

Third, Heart's readers may not be aware that the International Journal of Obstetric Anesthesia, though not yet listed in the National Library of Medicine's database, has an extensive collection of reports of cardiac disease in pregnancy including the Obstetric Anaesthetists' Associations' UK Registry of High-risk Obstetric Anaesthesia.[4,5] The latter includes over 300 cases of cardiorespiratory disease managed in the UK since 1996.

References

1. Thorne SA. Pregnancy in heart disease. Heart 2004; 90: 450-456.

2. Suntharalingam G, Dob D, Yentis SM. Obstetric epidural analgesia in aortic stenosis: a low-dose technique for labour and instrumental delivery. International Journal of Obstetric Anesthesia 2001; 10: 129-34.

3. Yentis S, Gatzoulis MA, Steer P. Pregnancy and coarctation of the aorta. Journal of the Royal Society of Medicine 2003;96: 471.

4. Dob DP, Yentis SM. UK Registry of High-risk Obstetric Anaesthesia: report on cardiorespiratory disease. International Journal of Obstetric Anesthesia 2001; 10: 267-72.

5. Lewis N, Dob DP, Yentis SM. UK Registry of High-risk Obstetric Anaesthesia: arrhythmias, cardiomyopathy, aortic stenosis, transposition of the great arteries and Marfan’s syndrome. International Journal of Obstetric Anesthesia 2003; 12: 28-34.