More families now have a choice

Editor—We support the assertion by O'Kelly and Bove in their editorial on hypoplastic left heart syndrome.1 Over the past four years our unit has performed over 100 Norwood procedures for this condition, of which 37 were in 1996. The referral pattern suggests that more families are being given the choice in the United Kingdom. We share O'Kelly and Bove's positive encouragement stance for these patients, backed by a dedicated team rather than offering terminal care. Transplantation is not an alternative practical option at present.2

Though most patients present soon after birth, an increasing number are diagnosed antenatally. Diagnosis in fetal life gives the opportunity for choice, counselling as well as planned delivery with service providers alerted. For some patients the course after the first stage can be slow and complicated with immense pressure on the parents and the rest of the family. Family support from the institutions carrying out this procedure is essential, but other parents can also provide valuable advice and help. To achieve this a national parent group has been set up, known as “Left Heart Matters,” in order to support fellow parents with individual requests but also to organise an annual get together; an information booklet for parents has also recently been published by this group. Information on Left Heart Matters and the booklet can be obtained from: Suzie Hutchinson, Cardiac Liaison Sister, Birmingham Children's Hospital, Ladywood Middleway, Birmingham B16 8ET.

Willingness to initiate treatment is crucial

Editor—Sean O'Kelly and Edward Bove state that in the United Kingdom most children born with the hypoplastic left heart syndrome will be managed with supportive terminal care. In addition, they suggest that in general there has been “little interest” in surgical palliation of the syndrome in Britain.1 We challenge these assertions.

At Guy's Hospital, London, after discussion between all relevant subspecialties, a programme of staged surgical palliation for hypoplastic left heart syndrome was begun in 1995. Eighty seven cases of the syndrome were diagnosed prenatally by fetal echocardiography during 1995 and 1996. All parents were counselled about the treatment options, and 38 sets of parents (44%) elected to continue with the pregnancy. There were five intrauterine deaths, and four pregnancies were ongoing. Of the 29 babies who were delivered, surgery was declined by the parents in six cases, and two babies died preoperatively. Twenty one babies underwent Norwood palliative surgery. In addition, five babies who were diagnosed postnatally also had surgery. Of this total group of 26 babies, there were 14 survivors (54%), of whom six subsequently survived stage 2 of the Norwood protocol with no deaths during the second stage. Of the first 13 babies, four survived, but 10 of the last 13 survived initial palliation, suggesting that with experience results have improved.

Most of our cases were diagnosed prenatally, and the number of parents who opted for termination or declined intervention postnatally suggests that many are not prepared to put their child through multiple operations with an uncertain long term outlook. We entirely agree with O'Kelly and Bove that the willingness of a unit to initiate a treatment strategy is crucial, particularly in the early stages, and think that our recent results justify our efforts. The results of postnatal treatment seem to be improving, and there is reason for cautious optimism that this trend will continue.

Quality of life is also important

Editor—Sean O'Kelly and Edward Bove suggest that we should adopt more active management of babies with hypoplastic left heart syndrome in Britain.1 Bill Brawn's results in Birmingham over the past four years are commendable but he expresses caution about the long term outcome for these babies.2

Surgery and the postoperative period are extremely difficult. Survival is important but so is quality of life. Very little has been published on neurodevelopmental outcome. One recent small follow up study from New York, however, reported that of 11 survivors, four had moderate and three severe to profound mental retardation when assessed at 11-67 months of age.3 Delayed motor development was seen in five children and cerebral palsy in two.

Palliative surgery is increasingly available for hypoplastic left heart syndrome in Britain, though surgery is still less common than in the United States. The alternative active management option of transplantation will remain constrained by a severe shortage of donor hearts.

I urge that children who survive the three stage Norwood palliation of hypoplastic left heart syndrome should have detailed neurodevelopmental follow up to ensure that they receive the necessary input for any developmental problems which become apparent and that units undertaking such surgery should carefully audit and publish quality of life outcome rather than just survival. If the neurodevelopmental outcomes are as poor in Britain as they are in the United States my view would be that parents should be very carefully counselled about treatment options, which should include offering terminal care only.