An article published Online First and in a future edition of The Lancet reports the extraordinary story of Hannah Clark who had a donor heart grafted onto her own after suffering heart failure as a baby. She underwent surgery ten and a half years after the transplant to remove the donor heart. This was possible because her own heart had recovered satisfactorily to work on its own. Today, three and a half years after this second operation, Hannah, now aged 16, has made a complete recovery. The article is the work of leading heart surgeons Professor Sir Magdi Yacoub, Imperial College London, Heart Science Centre, Harefield Hospital, Middlesex, UK, and consultant Victor Tsang, Great Ormond Street Hospital, London, UK and collaborators.
Heart transplantation is a life-saving procedure in infants and small children with heart failure due to cardiomyopathy, which is a problem with the muscle of the heart. This condition occurs in 1.2 to 1.4 children in every 100,000. It is eight to twelve times more frequent in the first year of life than in the following years. The prognosis is very poor. Still, if the patients live long enough, it is theoretically possible for their own heart to recover. Mechanical hearts suitable for use in children are other options currently being developed.
In 1994, Hannah, aged 8 months, was brought to Harefield Hospital, UK (now a division of the Royal Brompton & Harefield NHS Foundation Trust). She had symptoms of severe heart failure secondary to cardiomyopathy. In 1995, at age 11 months, Hannah underwent a heart transplant. The donor heart was grafted onto her own heart. It took over much of the function of the heart. The new heart was inserted in parallel to the Hannah’s heart. In the long term, this allowed almost complete unloading of the left side of Hannah’s own heart. This meant it could gradually recover. But, as it is standard with all transplants of donor organs, the recipient’s immune system must be suppressed using drugs to prevent rejection of the donated organ.
Both the donor heart and Hannah’s own heart were functioning normally for the four and a half years after the transplant. At this point, it was judged necessary to remove the donor heart, which is a major operation. Hannah continued to have episodes of EBV PTLD, which by age 8 years had begun to spread and become extremely serious. To get this EBV PTLD into complete remission, chemotherapy and multiple courses of drugs were used. In January 2003, she developed symptoms suggesting that PTLD had recurred, and she went through similar drug cycles to treat it. This carried on for two more years. In 2005, an echocardiogram showed that her own heart remained functioning normally but that the function of the donor heart had become impaired. This was a reaction to the reduced levels of immunosuppressant drugs to help Hannah fight PTLD. This in turn, led to symptoms of rejection of the donor heart. Since her PTLD was apparently incurable even though it was partly immunosuppressed, the doctors decided to remove the donor heart so that immunosuppression could be stopped altogether. It was the first time this was ever done.
In February 2006, just about ten years after the original operation, surgery to remove the donor heart took place at Great Ormond Street Hospital, London, UK. Professor Sir Magdi Yacoub was the lead surgeon who performed the operation to insert the donor heart in 1995. The surgery team was led by consultant Victor Tsang and Professor Sir Magdi Yacoub. Following surgery, the immunosuppressant drugs were removed altogether, and since then Hannah has made a complete recovery from the EBV PTLD.
The authors comment: “Her post-operative course was uncomplicated and the outcome was excellent. Three-and-a-half years after surgery, the patient remains well, in complete remission from her PTLD, and has normal cardiac function.”
Professor Yacoub explains: “Apart from the overriding human element in this report, Hannah’s case has provided many lessons relevant to biology, transplantation, heart recovery and malignant disease. We all hope that this will stimulate further research and progress in this area.”
Mr Tsang remarks: “”Hannah’s case highlights that, in cases of infant cardiomyopathy such as hers, it is possible for the patient’s own heart to make a full recovery if it is given adequate support to do so. This is an important piece of knowledge as we are now gaining more experience with mechanical support for the failing heart in children.”
Hannah Clark says: “Thanks to this operation, I’ve now got a normal life just like all of my friends. I’ve just done my GCSEs (school examinations that UK children take at age 15 to 16 years), and I’ve now got a Saturday job looking after animals, which I couldn’t have done before. I’m really glad that I don’t have to rely on life-saving drugs anymore.”
“Late donor cardiectomy after paediatric heterotopic cardiac transplantation”
V Tsang, M Yacoub, S Sridharan, M Burch, R Radley-Smith, A Khaghani, B Savoldo, P J Amrolia
DOI: 10.1016/S0140-6736(09)61201-0
thelancet
Written by Stephanie Brunner (B.A.)