Late donor cardiectomy performed after heterotopic transplantation
MedWire News: A team of UK cardiologists have successfully removed the donor heart from a pediatric patient who 10 years previously underwent a heterotopic transplantation for advanced cardiomyopathy.
Crucially, this first-of-a -kind procedure allowed the patient to the cease immunosuppressant therapy that had contributed to refractory post-transplant lymphoproliferative disorder (PTLD).
“In cases of infant cardiomyopathy… it is possible for the patient's own heart to make a full recovery if it is given adequate support to do so,” Magdi Yacoub (Heart Science Centre, Imperial College London) and colleagues comment in The Lancet.
The incidence of pediatric cardiomyopathy is highest during the first year of life - 8 to 12 times higher than after the first year - and the prognosis is very poor, the authors explain.
In 1994, an 8-month-old girl presented to their clinic with signs of severe heart failure, secondary to dilated cardiomyopathy.
Normally orthotopic cardiac transplantation is the only effective form of treatment for such children with severe forms of disease. However, in this particular case there were several important contraindications to the procedure, including fixed elevated pulmonary vascular resistance with a transpulmonary gradient of 23 mm Hg, poor general condition, and small size of the child.
When the patients was aged 2 years in 1995, the team therefore, chose to perform a heterotopic transplantation with preservation of the hypertrophied recipient right ventricle to deal with increased pulmonary vascular resistance.
After transplantation, the child’s condition improved substantially with resolution of the signs of chronic heart failure over the following 2 weeks. The donor and recipient hearts functioned completely independently.
However, in the proceeding years the patient developed multiple, recurrent episodes of PTLD associated with Epstein-Barr virus (EBV) infection that were resistant to several therapies, including reduction of immunosuppression.
Furthermore, in 2005 an echocardiogram showed that while the native heart was functioning normally, the donor heart had become impaired due to attempts to reduce immunosuppressive drugs.
Yacoub and colleagues decided to remove the malfunctioning heterotopic donor heart in February, 2006 when the patient was aged 13 years. Following surgery, the immunosuppressant drugs were removed altogether, and the patient subsequently made a complete recovery from the EBV PTLD.
Discussing their findings, the researchers say that the techniques developed for this procedure could potentially be adapted to newly available mechanical heart devices.
MedWire (www.medwire-news.md) is an independent clinical news service provided by Current Medicine Group, a part of Springer Science+Business Media. © Current Medicine Group Ltd; 2009
By Andrew Czyzewski